"TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS WITH PULMONARY HYPERTENSION"
DISSERTATION SUBMITTED TO
THE TAMIL NADU Dr. M.G.R MEDICAL UNIVERSITY, CHENNAI IN PARTIAL FULFILLMENT OF THE REGULATIONS FOR THE
AWARD OF DEGREE OF M.D IN RESPIRATORY MEDICINE
BY
Dr.NAJMA MUHAMMED.K GUIDE : DR.K.ANUPAMA MURTHY DEPARTMENT OF RESPIRATORY MEDICINE
PSG INSTITUTE OF MEDICAL SCIENCES AND RESEARCH PEELAMEDU, COIMBATORE – 641004
TAMILNADU, INDIA
"TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS WITH PULMONARY HYPERTENSION"
DISSERTATION SUBMITTED TO
THE TAMIL NADU Dr. M.G.R MEDICAL UNIVERSITY, CHENNAI IN PARTIAL FULFILLMENT OF THE REGULATIONS FOR THE
AWARD OF DEGREE OF M.D IN RESPIRATORY MEDICINE
BY
Dr.NAJMA MUHAMMED.K GUIDE : DR.K.ANUPAMA MURTHY DEPARTMENT OF RESPIRATORY MEDICINE
PSG INSTITUTE OF MEDICAL SCIENCES AND RESEARCH PEELAMEDU, COIMBATORE – 641004
TAMILNADU, INDIA
"TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS WITH PULMONARY HYPERTENSION"
DISSERTATION SUBMITTED TO
THE TAMIL NADU Dr. M.G.R MEDICAL UNIVERSITY, CHENNAI IN PARTIAL FULFILLMENT OF THE REGULATIONS FOR THE
AWARD OF DEGREE OF M.D IN RESPIRATORY MEDICINE.
BY
Dr.NAJMA MUHAMMED.K GUIDE : DR.K.ANUPAMA MURTHY DEPARTMENT OF RESPIRATORY MEDICINE
PSG INSTITIUTE OF MEDICAL SCIENCES AND RESEASRCH PEELAMEDU, COIMBATORE – 641004
TAMILNADU, INDIA
CERTIFICATE
This is to certify that the thesis entitled “TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS
WITH PULMONARY HYPERTENSION” is a bonafide work of DR.NAJMA MUHAMMED.K done under the guidance and supervision of
DR.ANUPAMA MURTHY MD(CHEST) in the Department of PSG Institute of Medical Sciences and Research, Coimbatore in fulfilment of the regulations of Dr.MGR Medical University for award of M.D .Degree in Tuberculosis and Respiratory Diseases.
DR.K.ANUPAMA MURTHY Professor & Head
Department of Respiratory Medicine
DR.RAMALINGAM DEAN
DECLARATION
I hereby declare that this study dissertation entitled “TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS WITH PULMONARY HYPERTENSION” was prepared by me under the direct guidance and supervision of Professor of Respiratory Medicine, DR.K.ANUPAMA MURTHY, MD (CHEST),PSG Institute of Medical Sciences & Research, Coimbatore.
This dissertation is submitted to the Tamil Nadu Dr.MGR Medical University in fulfilment of the University regulations for the award of M.D.
Degree in Tuberculosis and Respiratory Diseases. This dissertation has not been submitted for the award of any other Degree or Diploma.
DR.NAJMA MUHAMMED.K
CERTIFICATE BY GUIDE
This to certify that the thesis entitled “TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OF BRONCHIECTASIS
WITH PULMONARY HYPERTENSION” is a bonafide work of DR.NAJMA MUHAMMED done under my direct guidance and supervision in
the Department of Respiratory medicine, PSG Institute of Medical Sciences and Research, Coimbatore in fulfilment of the regulations of DR.MGR Medical University for the award of M.D .Degree in Tuberculosis and Chest Diseases.
DR.K.ANUPAMA MURTHY Professor & Head
Department of Respiratory Medicine
ACKNOWLEDGEMENT
I shall forever remain indebted to my beloved teachers, Dr.K.Anupama Murthy, Dr.Karthikeyan Ramaraju for their constant valuable inputs guidance, patients support without which thesis would have been impossible
A special mention to the Radiology and Cardiology departments for their unyielding support and providing a good number of cases for this study. I take this opportunity to thank all the above mentioned departments
Finally I thank all my patients, who cooperated at every step and provided me the opportunity to conduct the study.
Thank you
Dr.Najma Muhammed.K
PLAGIARISM CERTIFICATE
This is to certify that this dissertation work titled “
TO CORRELATE CLINICORADIOLOGICAL CHARACTERISTICS OFBRONCHIECTASIS WITH PULMONARY HYPERTENSION
” of the candidate Dr. Najma Muhammed. K with registration number 201527151 for the award of MD in the branch of
RESPIRATORY MEDICINE. I personally verified the urkund.com website for the purpose of Plagiarism check. I found that the uploaded thesis file contains introduction to conclusion pages and result shows 7% of plagiarism in the dissertation.
Signature of the guide
CONTENTS
SL.NO CONTENTS PAGE NO
1 INTRODUCTION 1-12
2 AIMS AND OBJECTIVES 13
3 MATERIALS AND METHODS 14-16
4 REVIEW OF LITERATURE 17-51
5 RESULTS 52-62
6 DISCUSSION 63-74
7 SUMMARY 75
8 LIMITATION 76
9 CONCLUSION 77
10 BIBLIOGRAPHY 11 ANNEXURE
I) ABBREVIATIONS II) CONSENT FORM
III) PATIENTS INFORMATION FORM IV) MASTER CHART
1
INTRODUCTION
Bronchiectasis is one of the most prevalent morbidity in any community leading to recurrent lower respiratory tract infections. Bronchiectasis means abnormal and permanent dilation of medium sized subsegmental bronchi from fourth to ninth generations. First described by Laennec in 1819,Later by Sir William Osler,late by 1800 and futher defined by Reid in 1950.Bronchiectasis usually associated with mucosal thickening, mucus plugging and avariable degree of hyperinflation of lung. Bronchiectasis is associated with common and rare disease, which impact of muciliary clearance and immunity. Mucus clearance and local defence mechanisms aganist microorganisms are important in preventing recurrent pulmonary infection.Repeated infection causes damage and impedes the clearance of mucus.The airway dilatation and impairement of mucociliary clearance increases suspectibility for repeated infection in lungs,resulting in chronic infection.The abnormal airway anatomy ,chronic infection and mucus retension result in decline in respiratory function.
Bronchiectasis may be congenital or acquired. Pathogenesis has not been well defined. The combination of a microbial insult and defect in host defence may result in persistent bronchial infection and inflammation which leads to further structures to prolonged infection,result in lung damage. Other pathogenesis includes obstructive either intraluminal or extraluminal, inflammatory conditions, immunological changes.Autoimmune disease also have some association in pathogenesis of bronchiectasis. The prevalence of
2
bronchiectasis is 5-7 times greater in persons over 55years of age. In Indian adult it is third commonest non –tubercular respiratory disease with the incidence of 71per lakh.
PULMONARY HYPERTENSION
Pulmonary Hypertension is a common complication in chronic lung disease. Most revised classification of pulmonary hypertension,chronic lung disease or conditions with alveolar hypoxia are included in WHO Group III of PH –related disease. In this category of chronic lung disease of a mixed obstructive and restrictive pattern,which includes chronic bronchiectasis, cystic fibrosis and syndrome characterized by combination of pulmonary fibrosis and emphysema ,in which the prevalence of PH is almost 50%. Prevelance in India of bronchiectasis with pulmonary hypertension 1% in occupational airway disease,15% in Chronic obstructive pulmonary disease,2% in Obliterative bronchiolitis.Global prevalence in bronchiectasis of pulmonary hypertension is 7-12%.
Alveolar hypoxia is major factor for pulmonary vasoconstriction.
Endothelial level is one of the most important leading pathway for development of PH in chronic lung disease. Alveolar hypoventilation increases acute pulmonary vasoconstriction and vasodilation causes physiological shunt.
Hypoxia causes pulmonary vasoconstriction leads to increase pulmonary vascular resistance. Vasoconstriction is achieved through activation of
3
vasoconstrictor pathway or inactivation of a vasodilator pathway, or through the effects of hypoxia on vascular smooth muscle.
Figure : 1 Chest radiograph showing markedly dilated pulmonary arteries (arrows).
4
Figure 2 : Contrast-enhanced computed tomography of the chest of a patient with pulmonary arterial hypertension associated with congenital heart disease (large atrial septal defect). Massive dilatation of the pulmonary arterial trunk and branches (#). The
ratio of the diameter of aorta (Ao) to the diameter of main pulmonary artery (PA) is >1.5.
5
Figure 3: Measurements of main pulmonary artery and ascending aorta at the level of bifurcation. The main pulmonary artery (PA) size is typically taken at the level of the bifurcation of the main pulmonary artery perpendicular to the vessel wall. The aortic dimension of the ascending aorta is taken at the same level to calculate the PA to the aortic diameter (PA/Ao) ratio. The diameter is determined using the internal diameter in the contrast-enhanced image.
6
Chest radiography is not sensitive for the diagnosis of airway disease.In most severe cases of patients with bronchiectasis,as dilation of large airways which can be visualized . High –resolution computed tomography is more sensitive test than chest radiography and is useful in diagnosis of airway disease. The test is considered as gold standard for diagnosing bronchiectasis.
Greater variance in section thickness compared to HRCT sections obtained only at 10mm intervals,leads to improve the detection of bronchiectasis. HRCT technique compromises scanning for apex to base with 1-2mm cuts at 10mm intervals.
Common finding in bronchiectasis include bronchial wall thickening,centrilobular nodules mosaic attenuation by air trapping. Following tomographic criteria are used for diagnosis of CT bronchiectasis
1) Bronchial internal diameter larger than 1.5times the diameter of adjacent pulmonary artery
2) Bronchial visualization in the periphery 1-2cm from the paritel pleura
7
Figure 4: shows normal lung (right), bronchiectasis lung (left)
Figure 5 : Tracheobronchial Segments
8
TRACHEOBRONCHIAL TREE ANATOMY
Trachea divides into right- and left- primary main bronchi.
Each further divides into lobar bronchi which in turn give rise to segmental bronchi - supply air to bronchopulmonary segments.
Segmental bronchi divide dichotomously, eventually giving rise to terminal bronchioles which further terminates into respiratory bronchioles.
Originating from each respiratory bronchioles are 2-11 alveolar ducts leading to the alveolar sacs which are extended as a group of alveoli.
Airway becomes progressively narrower, shorter and more numerous, and Cross sectional area, enlarges.
Areas of tracheobronchial tree furthest from the trachea are collectively called the "distal respiratory tree"
RIGHT MAIN BRONCHUS :
The right main bronchus is 2 cm long on average and has an internal diameter of 10-16 mm. This is slightly larger than the diameter of the left main bronchus.
The bronchus intermedius of the right bronchial tree is actually quite short ,
extending for 1.0-2.5 cm until its anterior wall extends into and becomes the middle lobe bronchus. Its posterior wall extends into and becomes the right lower lobe bronchus.
9
Volume loss caused by pleural effusion, atelectasis, elevated right hemidiaphragm, as well as traction or torsion from a fibrotic or scarred upper lobe often cause shortening of this bronchus.
The RIGHT UPPER LOBE BRONCHUS
Right upper lobe bronchus divides into a. The apical bronchus
b. The anterior bronchus c. The posterior bronchus
Distally just beyond the bronchus intermedius, another division occurs into : The middle lobe bronchus with its anterior direction, dividing into
a) medial and b) lateral segmental bronchus
RIGHT LOWER LOBE BRONCHUS
The right lower lobe bronchus divides immediately into
a) Superior segmental bronchus (just across from the right middle lobebronchus), and
b) Medial basal segmental bronchus a bit more distally and along its medial wall.
10
Finally dividing into three lower lobe bronchi (Three musketeers):
c) Antero-basal d) Latero-basal e).Postero-basal
LEFT MAIN BRONCHUS
The left main bronchus is usually 4-5cm long (Right. 2cm long). Its lumen is
narrower and relatively horizontal. The usual length of the left
lower lobe bronchus beyond the origin of the superior segmental bronchus is 1cm.
Divides into upper and lower lobe bronchus.
Left upper lobe bronchus divides into apicoposterior and anterior.
Lingular – supr division, inferior division.
Left Lower lobe bronchus divides into apical,anterior basal, posterior basal, lateral basal segments.
11
Figure 6 : Bronchopulmonary segments
According to CT findings bronchiectasis is classified as Cylindrical (bronchial dilation),Varicose (with focal constrictions along airways)or Cystic (saccular dilations at the end of bronchus).
Echocardiography is commonly used to estimate systolic pulmonary artery pressure and to diagnose pulmonary hypertension in patients with advanced lung disease.It used as conventional clinical echocardiographic equipment,with 2.5 or 3.5mHz transducers.Indicative of PH on echocardiogram include increased systolic pulmonary artery hypertension or tricuspid regurgitant jet,right atrial and ventricular hypertrophy,small left ventricular dimension and dilated pulmonary artery. Echocardiography has been found to non-invasive it is preferred mortality to diagnose pulmonary hypertension.
Invasive hemodynamic assessment by RHC is pivotal in the evaluation of any
12
patient with suspected PAH. Right –Heart catherization is typically performed after the non invasive testing for PH.
Physiological parameters such as pulmonary function test that are characteristic of an obstructive disorder, confirming initial involvement of small airways. With progression of disease, a mixed functional disorder or a restrictive disorder can be found due to progressive destruction of pulmonary parenchyma that is characterised by recurrent infectious exacerbations and massive destruction of the small airways. 6-minute walk test enabling the evaluation of the degree of respiratory discomfort, in terms of determination of subjective rates according to the perfection of individual. This a vertical scale to quatify from 0-10 in which 0 represents no symptoms, and 10 represents maximum symptoms and providing an providing individual measurement of the density of the exercise.
In conclusion high –resolution CT scan can be used for diagnosis extend of involvement of segments of bronchiectasis. So predictors of clinical, physiological and radiological correlation in patients with bronchiectasis will help to determine predictors of pulmonary hypertension.
13
AIMS & OBJECTIVES
To correlate clinicoradiological characteristics of bronchiectasis with pulmonary hypertension
OBJECTIVE
Primary Objective
To correlate clinicoradiological profile of bronchiectasis Secondary Objective
To correlate clinicoradiological profile of bronchiectasis with severity of pulmonary hypertension
To correlate clinicoradiological profile with physiological charecteristics of bronchiectasis
14
MATERIALS AND METHODS
The study aim to correlate the clinicoradiological parameters of bronchiectasis,and to assess the severity of exercise capacity and Pulmonary Artery Hypertension in these patients.Clinical and radiological predictors will facilitate futher understanding of pulmonary atery hypertension and thereby affect treatment outcomes.
Study type
Prospective cross-sectional study Study Duration
12months Study Locale
PSG Institute of Medical Sciences and Research Study Method
Convenience Sampling Sample Size
40subjects
15
Inclusion Criteria
Age 18 to 65 years
Cases of Bronchiectasis diagnosed based on clinico radiological profile
Willing to participate in the study adhering to its protocol Exclusion Criteria
Patients not having acute exacerbation or active infection
Patients with Primary PulmonaryHypertension(PPH)
Patients with pulmonary hypertension attributable to primary causes other than bronchiectasis
Mental status not competent enough to consent for the study in the study adhering to its protocol
16
STUDY METHODOLOGY STEP 1:
Inclusion Criteria
STEP 2:
Informed Consent
STEP 3:
Clinical profilebronchiectasis (History, MMRC, BORG, SGRQ)
STEP 4:
Radiological profile of bronchiectasis
STEP 5:
FEV1 (measured with PFT),6MWT
STEP 6:
ECHO foe evaluation of pulmonary hypertension
STEP 7:
Analyze and Correlate
Data Analysis
Spss Software Version 24
17
REVIEW OF LITERATURE
Bronchiectasis is an important cause of respiratory morbidity but it has generally a low profile. As it is a heterogeneous condition with a large number of predisposing factors, long clinical history, the pathogenesis has not been well defined. Bronchiectasis is defined as permanent and abnormal dilatation of bronchi(1).In 1953,1.3 cases of bronchiectasis were reported in Bedford, a similar prevalence of 1.5 cases per 1000 was found from the chest radiographs obtained from routine miniature chest radiographs obtained from England and Wales in 1956 during tuberculosis eradication campaign. It is described as common in developing nations, but there is a lack of data on the actual prevalence. Generally ,bronchiectasis appears to be more common in females, with approximately two -thirds of patients in studies.
Most studies of bronchiectasis pathology were performed between 1920 and 1960,when there was readily available access to surgical and post mortem tissue. Bronchiectasis was characteristed by dilatation of subsegmental airways, which were tortuous, inflamed and obstructed by secretions. There was generally damage to the bronchioles, often with associated fibrosis and parenchymal destruction. The vascular supply to affected areas was derived mainly from hypertrophy of bronchial arteries and their anastomoses with pulmonary arteries.
18
Reid classified bronchiectasis(2) into three different types, based on macroscopic appearance of pathology specimens:
1) Tubular (or cylindrical), in which there was smooth dilatation of bronchus
2) Varicose, in which there were focal narrowings along a dilated bronchus and
3) Cystic, in which there was progressive dialation of a bronchus, which terminated in cysts or saccules. The main form currently seen on HRCT is the tubular or cylindrical form.
Figure 7
19
Figure : 8 A–D, Normal bronchus (arrow) (A), cylindric bronchiectasis with lack of bronchial tapering (arrow) (B), varicose bronchiectasis with string-of- pearls appearance (arrow) (C), and cystic bronchiectasis (arrow) (D).
20
Most definitive pathology study was performed by Whitwell(3) in a study “pathology and pathogenesis” in 1952 also described . He studied 200 operative lung samples from patients with bronchiectasis. He described the bronchial wall to be infiltrated with inflammatory cells. Ciliated epithelium was often replaced with squamous or columnar epithelium. The elastin layer was deficient or absent and, in more severe cases, there was destruction of muscle and cartilage, theses changes were responsible for bronchodilation.
He described three main forms of bronchiectasis:
1) Follicular 2) Saccular 3) Atelectactic.
1) Follicular bronchiectasis is acquired, it forms in presence of numerous lymphoid follicles situated in thickened,usually cylindrically dialated bronchial walls.
2) Saccular bronchiectasis characterised by presence of macroscopically visible thin walled, saccular bronchial dilations.
3) Atelectatic bronchiectasis , it was predominantly right-sided with follicular and saccular bronchiectasis, which involved the left lung more commonly.
.
21
Figure : 9 Cystic fibrosis. A and B, Transverse (A) and coronal (B) images show upper lobe predominance of cystic bronchiectasis(arrows) and volume loss, enlarged lung volumes, and diffuse heterogeneous attenuation.
22
Pathophysiology of Bronchiectasis
The dominant feature of bronchiectasis is airway inflammation in association with bacterial infection. It has generally been thought that the inflammation is secondary to unclearing infection. The “vicious cycle hypothesis” was proposed by Cole at al(4) explain the development of bronchiectasis. It compromised mucociliary clearance, allowing infection and colonisation of the respiratory tract. Bacteria caused inflammation, which damaged the respiratory tract, leading to more bacterial proliferation and more inflammation or damage. Thus ongoing cycle developed which caused progressive destruction of the lung. The current view is that a combination of microbial insult and defect in host defence allow establishment of persistent bronchial infection.
23
Figure : 9 Cycle of Infection and Inflammation of bronchiectasis
Mucocillary system:
Mucocillary system is apart of innate defence mechanism that clears unwanted materials from the airways.It consists of cilia,periciliary fluid layer and the mucus layer.Mucus is normally cleared by successful interaction of the cilia in the mucus and this depends on the beat frequency of the cilia,the mucus load and its physical properties ,and depeth of the periciliary fluid layer.Abnormalities in any component of the mucociliary system can result in abnormal mucociliary clearance.
24
Figure : 10 Components of the Mucociliary System
In bronchectasis, chronic inflammation in the airways results in an increase in the size and number of mucus secretory cells, that is in hypertrophy of the submucus glands and in hyperplasia and metaplasia of the globet cells extending to the bronchioles that are normally free of mucus secretory cells.
Therefore in bronchiectasis there is excessive mucus secretion throughout the conducting airways ,including the bronchioles where mucus does not exist in the healthy state. The cilia cannot transport excessive load of mucus .
In a study done by Tsang et al in 2005 showed that cilia have been found to beats slowly in bronchiectasis that is not related to genetic ciliary defects then in healthy subjects, a feature that could relate to inflammatory
25
products such as neutrophil elastase or to bacterial products especially in those colonized with pseudomonas.
Mucus in bronchiectasis can be highly viscoelastic and adhesive. These physical properties are greatly influenced by the hydration at the airway surface. Then the secreted mucus volume is excessive as bronchiectasis there is an imbalance between mucins and available water. Daviskas et al and Boucher et al studies showed that dehydration of airway mucus in bronchiectasis is evident when the percentage of solids in sputum is greater than the normal 2-3% .The increase in the percentage of solids in the mucus of bronchiectasis patients in addition to causing impairement to its transport can potentially inhibit the motility of neutrophilis within the mucus and thus their ability to kill bacteria. When the solids where increased from normal 2.5% to 6.5%,neutrophilis failed to kill bacteria.
The increased production of mucus in bronchiectasis together with the impairement of the mucociliary system leads to accumulation of mucus, chronic cough, mucus plug formation, airway obstruction, bacterial colonization and infections that fails to resolve completely. In bronchiectasis ,the failure of mucociliary system to transport mucus most likely relates to the abnormal load of mucus and the abnormal physical properties of the mucus rather that to the cilia or their movement being abnormal, except in patients with genetic ciliary defects. When the mucociliary system fails cough becomes a every important mechanism for clearing mucus. Cough become compromised
26
in patients with bronchiectasis due to highly viscoelastic and sticky mucus.
Due to severe airflow limitation in bronchiectasis cough becomes ineffective.
The impairement of the mucociliary system and the abnormal properties of mucus in bronchiectasis have been documented in studies by Currrie et al, Daviskas et al, Isawa et al using a radioaerosol technique and imaging with gamma camera.
Effects of bacteria and pathogens
Bacteria have a number of effects on the respiratory tract of which the best described is inhibition of mucociliary clearance.Hemophilius influenza,
Streptococcus pneumonia and Pseudomonas aeruginosa produce mediators that inhibit ciliary function damage ciliary epithelieum and inhibit mucus transport.Bacteria may destroy the epithelieum,release chetatic factors to attract large number of neutrophilis and produce biofilms.Biofilms are best described ibn pseudomonas infection,in which an impenetrable matrix is found around the bacteria with severe damage to the underlying lung.
Neutrophilis are found in large numbers in bronchiectasis. Readet al and Eller et al found that following injection with radiolabelled white cells 50% of the circulating neutrophilis pass into the bronchi. These neutrophilis are most prevalent in the airway lumen and release mediators such as protease and elastase which damages the bronchial wall elastin and epithelium. The bronchial cell wall is infiltrated predominantly by a macrophages, which appear to be the cells predominantly causing small airway obstruction.
27
Aetiology of Bronchiectasis
There are large number of causes that have been proposed to causes bronchiectasis. Bronchiectasis is a heterogenous condition with a long clinical history before the diagnosis is made, the exact role of causative factors is often not clear. Certain causative factors are Post –infectious bronchiectasis, most common causes described in the literature is childhood infection, particularly with pneumonia, whooping cough and measles. This appears to be particularly important in populations with poor health and recurrent infections. Infection causes structural damage to the airways, allowing persistent infection results in bronchiectasis. Seroprevalence in measles in unvaccinated populations morethan 90% and whooping cough > 50%.Finally patients with immune deficiency are more likely to have significant lung infections.
In a study by Whitwell et al(3), in 1952 a study of pathology and pathogenesis of bronchiectasis described as mycobacterial infections, both tuberculous and non – tuberculous have an association with bronchiectasis, mechanism is probably due lymph node obstruction and atelectatic.
Bronchiectasis can develop abnormal mucociliary clearance caused primarily by genetic ciliary defect. Approximately 50% of patients with primary ciliary dyskinesia have Kartagener’s syndrome ,characterised by bronchiectasis, sinusitis, and situs inversus. Another rare condition is Young’s syndrome, characterised by azoospermia and bronchiectasis that is due to highly tenacious secretions that are poorly cleared. Causes of obstruction include an inhaled
28
foreign body, slow growing tumour and twisting of an airway after lobar resection.
In a study in described by Currie et al (6) in 1987 “Impaired tracheobronchial clearance in bronchiectasis in studied retained that sputum can contribute to obstruction which leads to an important role in bronchiectasis.
Immune dysfunction associated with bronchiectasis including HIV, hypogammaglobulinaemia, type I major histocompatibility complex. Therefore ,malnutrition and socioeconomic factors may play a role in precipitating immune dysfunction.
In a study Silva et al(41) in 1989,highlighted that othercauses strongly associated with bronchiectasis such as Rheumatoid arthritis, Sjogrens syndrome, Churg –Strauss syndrome and inflammatory bowel disease.
In a cohort study by Parr et al(38) in 2000,74 subjects with α1-Antitrypsin deficiency that 70 subjects had evidence of radiological bronchiectasis and 20 had the syndrome of clinical bronchiectasis.
Microbiology of bronchiectasis
The microbiology of bronchiectasis is complex , with multiple potential pathogens. The pattern of isolates varies between different institutions. Previous studies have shown that the two major pathogens found are H.Influenza,and P.aeruginosa .Other important pathogens include Moxarella catarrhalis, S.pneumoniae,non –tuberculous mycobacteria and Aspergillus species. A consistent finding is that, despite the presence of purulent sputum,30-40% of specimens will fail to grow any pathogens.
29
As bronchiectasis progresses there appears to be a change in bacterial flora.
Subjects with mild disease usually have no pathogens isolated, while subjects with moderate disease most commonly have H.Influenzae.In most severe disease ,P.aeruginiosa is the dominant pathogen.
Assessment of bronchiectasis based on
Clinical features:
Severity of clinicopathologic events varies widely in patients with bronchiectasis. Patients have exacerbations, with chronic sputum production other symptoms include hemoptysis, especially during exacerbations and breathlessness, characterized by mild to moderate airflow obstruction, lethargy and reduced health status.
Clinical signs
Finger digital clubbing recognized as a sign of chronic suppurative lung disease.Studies reports that prevalence of clubbing 1- 2%.Overall, suggests that finger clubbing is now seen only in minority of patients with non –CF bronchiectasis.Other clinical finding in bronchiectasis is coarse crackles,reported upto 73% of patients in more recent studies.
30
Assessment of Functional status with physiological parameters
Bronchiectasis is chronic respiratory condition,characterised by cough and sputum production together with physical fatigue and reduced exercise tolerance.It causes diminished exercise capacity include dyspnea secondary to dynamic hyperinflation,altered respiratory mechanics and decreased skeletal muscle bulk.However ,there have been limited evaluation of physical function in patients with bronchiectasis and influence on respiratory disease severity and extent of disease on exercise capacity is notknown.
6-Minute Walk test
It is widely used to measure of functional status, as well as a predictor of prognosis(32).With the clinical application suggested that simple measurement reflected on the pulmonary and functional status of patients with bronchiectasis,evaluation of the 6MWT in population has been limited.
A study done by Lee at al(33) in 2008,clinical determinants of the 6-Minute Walk test in bronchiectasis provides a valuable information of the functional status in a group of patients with mild to moderate bronchiectasis and also indicates dynamic hyperinflation and increased work of breathing are responsible of disease.In this study evidence of airflow obstruction measured by using FEV1 and FVC associated 6minute walk test.
31
Pulmonary Functional Test
Patients with bronchiectasis generally have mild to moderate airflow obstruction.Both adults and children have been shown to develop progressively worsening airflow obstruction. Hogg et al have studied small airways obstruction found the factor most associated with obstruction was the presence of lymphoid follicles, which were similar.Airway reversibility may also be common in bronchiectasis.Murpghy et al and Pang et al found significant airway reversibility in 40% of subjects and two other studies found a 30% and 69% of reversibility in bronchiectasis.
Functional impairement in bronchiectasis is related to the extent of lung damage as determined by the number of bronchopulmonary segments involved.
Measurements of ventilatory capacity and lung volumes with bronchographically proven bronchiectasis showed that airways obstruction is constantly ventilatory defect.
Another smaller series shown that 71% of 34 patients with bronchiectasis had airflow limitations in FEV1/forced vital capacity ratio is less than 65%.
The findings showed that the presence of infection/inflammation was associated with increased airways obstruction. In a study done by Taylor et al,in 2010 about 204 patients showed that degree of airflow limitation has been correlated positively with both the duration of symptoms and extent of disease on CT examination. Reversibility of airflow limitation may present in bronchiectasis.Lung function tests have diagnostic value in bronchiectasis but
32
are useful in measuring the extent of impairement and demonstrating occasional response to bronchodilators or other treatments.The simple and highly reproducible tests, such as FEV1 and FVC are also clinically useful and reliable
MODIFIED BORG Dyspnea scale
This scale is an aid for the 6-minute walk test enabling the evaluation of the degree of respiratory discomfort,in terms of determination of subjective rates according to the perfection of individual.This a vertical scale to quatify from 0- 10 in which 0 represents no symptoms,and 10 represents maximum symptoms and providing an providing individual measurement of the density of the exercise.
Martinez gracia et al in 2000 in their study suggested an independent impact of BORG score in assessing the lung extent in bronchiectasis.
33
Imaging in Bronchiectasis
Chest radiography is often the first imaging modality used to investigate patients with suspected bronchiectasis(9)
Plain chest radiography
Radiographs are insensitive as a diagnostic tool for bronchiectasis, and suggest the diagnosis in lessthan 50% of cases in studies. Plain chest radiographic features have been described by Simon et al(1) in 1978,which include :
Evidence of dilated bronchi
Ring Shadows: produced by dilated bronchi seen ‘end-on’.It may be small, numerous widespread, sometimes referred as honeycomb lung. Larger ring shadows
(0.5-2cm diameter) which contain fluid levels, formed by retained mucopus.
They may be localized or widespread, giving the radiographic appearance of cystic lung.
Parrallel Lines: are produced by dilated bronchi viewed ‘side-on’. It is fine parallel, air-contaning , hairline opacities. Tramline: there walls been thickened rather than hairline, more numerous or crowded together, indicating lobar shrinkage.
Solid tubular opacities: bronchi contain mucopus which may seen as solid tubular, looking like a scaled –down column of tooth –paste. Gloved
34
finger shadow’s tangentially produce glove tending to have a rounded end, representing a large dilated bronchus containing mucopus.
Bronchography
It was for many years for the investigation of choice, for confirming bronchiectasis. Technique involves instillation of liquid contrast medium into tracheobronchial tree, under general or local anaesthesia. Aqueous or oily propyliodone was commonly used. Affected parts of lungs showed areas of bronchial dilatation, described as cylindrical, fusiform, saccular. Changes seen are found commonly in left lung and are bilateral in about one-third of cases.
Nowadays, bronchography is regarded as obsolete in most centres, HRCT having been to be more satisfactory.
Computed tomography scanning with high resolution
It should be used to diagnose bronchiectasis as technique demonstrates the airways in higher detail than standard computed tomography scanning. In a study done by Naidich et al(9) and Mcguinness et al(10) ,in 1982 and 2002 have established the use of standard criteria for diagnosis of bronchiectasis.
Most specific features are :
a) Internal diameter of a bronchus is wider than its adjacent pulmonary artery b) Failure of bronchi to taper, and
c) Visualisation of the bronchi in outer 1-2cm of lung fields.
Less specific features include mucosal wall thickening, crowding of bronchi and mucus impaction. Lobes most commonly involved in bronchiectasis are lowerlobes.
35
High resolution computed tomography
It has become the investigation of choice in bronchiectasis. Earlier studies CT was used for non- invasive and safe, this technique is used as alternative to bronchography in the diagnosis of bronchiectasis. These showed that conventional CT was less sensitive for diagnosing bronchiectasis than bronchography ,the sensitivities for 10-mm slices at 20 mm intervals .Cooke and colleagues found that conventional CT diagnosed bronchiectasis in 71% of 45 bronchiectatic lobes, sensitivity 71%,indicates that this form of CT cannot be used to ruleout bronchiectasis. Development of thin section HRCT changed this so that sensitivities increased to 82-97%. Recommended HRCT technique comprises scanning from apex to base with 1-2mm cuts at 10mm intervals.
HRCT criteria for diagnosing bronchiectasis are bronchial dilatation, and bronchial wall thickening. A bronchus is taken to be dilated if its internal diameter is greater than that of pulmonary artery branch, measurements being conventionally made in short axis of both the bronchus and the vessel.
Bronchial wall thickening is present if the thickness of the wall is at least equal to the diameter of adjacent pulmonary artery branch. The occurence of bronchial wall thickening prior to the development of bronchial dilatation is described in patients with hypogammglobulinaemia. Air trapping on expiration has been demonstrated as areas of increased transradiancy in patients with severe bronchiectasis. Scanning during inspiration and expiration may distinguish between an area of bronchiectasis and emphysematous blebs,or bullae.
36
Most commonly involved in bronchiectasis are lowerlobes usually multiple lobes are involved. HRCT has been found to have greater sensitivity than spiral CT when applied to patients in whom bronchiectasis is suspected.
Figure : 11 shows signet ring sign seen in bronchiectasis
37
Treatment of Bronchiectasis
Antimicrobial therapy is required once a acute exacerbation is identified .Patients who relapse quickly or frequently (>3times in 1year)with mucoid or mucopurulent sputum are usually managed with short course of appropriate antibiotics ,may also need longer term preventive therapy. Patients with chronic purulent sputum usually require higher antibiotics and also need of long term nebulised antibiotic therapy.
Prophylactic Therapy
Macrolides are the most common antimicrobials used in clinical practise.
Azithromycin twice weekly for 6months,has shown positive results, with reduced 24-hour sputum volume and reduced number of exacerbations.
Postural Drainage
Physiotherapy ,including postural drainage of chest secretions,has been regarded for many years is the mainstay in treatment of bronchiectasis.Postural drainage has been shown in the short term to increase rates of mucociliary clearance and to increase the expectoration of sputum,in patients whose secretions are copious.It has also been found to improve airway function .
Vaccination
In routine practises patients with bronchiectasis are vaccinated aganist influenza. Evidence is minimal regarding the pneumococcal vaccine, 23 valent pneumococcal vaccine as routine management in adults with bronchiectasis.
38
Surgical Treatment
Surgical treatment plays only a small role in management of bronchiectasis.
Surgery is indicated in the patients whose significantly disabled by persistent sputum production, recurrent infective exacerbations or haemoptysis despite all reasonable medical measures having been taken over a period of at least 12months.The disease should be confirmed radiologically, should be sufficiently localized to one lung or to part of one lung to enable curative resection and residual lung should be suffienctly healthy in terms of measured lung function.
39
PULMONARY ARTERY HYPERTENSION
Pulmonary hypertension(28) is a rare, pathologically complex disease characterized by a progressive increase in pulmonary arterial pressure associated with variable degrees of pulmonary vascular remodelling, vasoconstriction, and thrombosis. These changes leads to increased pulmonary vascular resistance, and with right-sided heart failure and death.PH is defined as an increase in mean pulmonary arterial pressure > 25mmHg at rest assessed by right heart catherisation. Pulmonary hypertension is a common complication in chronic lung disease which included in alveolar hypoxia in WHO group III of PH-related disease.PH is a haemodynamic pathophysiological state that can be found in multiple conditions.
The clinical classification of Pulmonary Hypertension has gone through a series of changes the first version was proposed in 1973 by WHO. Evian - Venice classification proposed in 1998-2003 on PAH. This is classified into five groups according to pathological, patho physiological and therapeutic characteristics. During Fourth World Symposium on PH held in 2008 in Dana Point, new classification was derived.
40
Classification:
Updated Clinical Classification of Pulmonary Hypertension(28) : 1. Pulmonary arterial hypertension
1.1 Idiopathic PAH 1.2 Heritable :
1.2.1 Bone morphogenetic protein receptor type II(BMPR2) 1.2.2 ALK-1, endoglin
1.2.3 Unknown
1.3 Drug or toxin –induced 1.4 Associated with:
1.4.1 Connective Tissue Diseases
1.4.2 Human Immunodeficiency virus (HIV) infection 1.4.3 Portal hypertension
1.4.4 Congenital heart diseases 1.4.5 Schitosomiasis
1.4.6 Chronic haemolytic anemia
1.5 Persistent pulmonary hypertension of newborn
41
1. Pulmonary venocclussive disease and/pulmonary capillary hemangiomatosis
2 . Pulmonary hypertension due to left- sided heart disease
3. Pulmonary hypertension due to lung disease and / hypoxia
4. Chronic thromboembolic pulmonary hypertension (CTEPH) 5. Pulmonary hypertension with multifactorial mechanisms
Normal pulmonary arteries have thin medial layer of circular muscle , with thickness less than 5% of diameter of vessel. Under physiological condition pulmonary circulation is characterised by variable intimal hyperplasia, medial hypertrophy, adventitial proliferation, and fibrosis that occur in close proximity to plexiform lesions. These lesions results from intimal proliferation and progresses from a cellular to a fibrotic lesion with advancing disease. As the vascular pathology progresses, pulmonary vascular resistance increases and pulmonary artery rises, in order to maintain cardiac output. So as right ventricle is able to compensate for resistance, the pressure continues to increases as the pulmonary vascular resistance increases. When contractile reserve of the right ventricle is exhausted, right ventricular systolic failure ensures. Various degrees on right ventricular diastolic dysfunction also present in PH. A combination of reduced right ventricular output and diastolic dysfunction resulting in impairing left ventricular filling and leads in
42
hemodynamic deterioration. Inflammation associated with underlying lung disease is responsible for development of PH in hypoxic states.
Group 3: Pulmonary hypertension due to Lung Disease and /or Hypoxia
Pulmonary hypertension associated with disorders of the respiratory system or hypoxemia is a category of PH that is caused mainly by inadequate oxygenation of pulmonary arterial blood result of either parenchymal lung disease, impaired control of breathing or residence at high altitude. Pulmonary arterial pressure is modest and survival depends on the severity of the pulmonary disease, rather than on the severity of the associated hypertension.
Pathophysiology
Pulmonary hypertension in the setting of chronic respiratory disease leads to increased pulmonary vascular resistance when pulmonary artery wedge pressure is normal. Increased pulmonary vascular resistance is usually secondary to effects of hypoxia and destruction of the vascular bed in lung parenchyma. While acute hypoxia leads to pulmonary vasoconstriction in the small pre-capillary arteries, chronic hypoxemia results in pulmonary vascular remodelling including medial hypertrophy and intimial proliferation of distal pulmonary arteries. Additional it includes mechanical stress secondary to hyperinflation, loss of capillaries, inflammation and endothelin –derived vasoconstriction – vasodilator imbalance.
Moreover, significant number of lung disease and severe PH, other co- morbidities may contribute pathogenesis of PH, such as left ventricular
43
diastolic dysfunction or chronic thromboembolic pulmonary hypertension.
Genetic factors may also play an important role for developing PH.
Mechanism of Pulmonary hypertension in Bronchiectasis
Alveolar hypoxia is a stimulus for pulmonary vasoconstriction. Most important pathways leading to pulmonary hypertension is endothelial level in chronic lung diseases. Alveolar hypoventilation precipitates acute pulmonary vasoconstriction in some parts of lungs and vasodilation which causes physiological shunt. Studies by Sylvester et al 2009,showed that vasoconstriction is achieved through vasoconstrictor pathway or inactivation of a vasodilator pathway, through the effects of hypoxia on vascular smooth muscle.Studies by Jing et al ,2009in rats,exposed to hypoxia suggest that hypoxia exposed arterioles develop smooth muscles in the walls of non muscular pre capillary blood vessels which persist after the removal of the stimulus and contributes to on going pulmonary hypertension.
Development of pulmonary hypertension may result in both intermittent and chronic. The effector pathway suggested L-type calcium channels, non –specific cation channels and voltage –dependent potassium channels, whereas mitochondria and nicotinamide adenine dinucleotide phosphate oxidase described as oxygen sensors. Reactive oxygen species, redox couples and adenosine monophoshate – activated kinases are the mediators of hypoxic pulmonary vasoconstriction. Endothelin -1 pathway, nitric oxide pathway, and reactive oxygen species helps for development of
44
pulmonary hypertension. Studies by Bosc et al,in 2010 demonstrated that Endothelin -1 is an important mediator of systemic hypertension in intermittent hypoxic states.ROS are highly reactive and unstable free radicals.Intermittent hypoxia stimulates the synthesis and release of ROS through the tyrosine hydroxylase system leading to the development of systemic hypertension. Studies Somer et al ,in 2008 showed that ROS was also been implicated in the induction of Endothelin -1 and in angiostensinogen synthesis with all theses agents believed to contribute to the development of pulmonary hypertension induced by intermittent hypoxia.
Figure : 12
45
Pulmonary vascular remodelling
Studies by Orr et al in 2012, of the vasculature in hypoxic pulmonary hypertension demonstrated changes including intimal thickening, medial hypertrophy and muscularization of small arterioles. When the balance between apoptosis and proliferation of endothelial cells in the pre – capillary pulmonary blood vessles, altered the proliferation, and overall resistance pattern is increased.Studies by Pak et al 2007,in neonatal calves and rodent models showed that chronic hypoxia triggers endothelial cell proliferation .Acute hypoxia triggers adventitial fibroblast proliferation within hours of exposure while medial hypertrophy and hyperplasia takes longer to develop. Fibroblasts stimulated by chronic hypoxia can transform into smooth muscle cells.Chronic hypoxia in rat models results in doubling of muscular arteries with proliferation into non muscularized vessels.
Role of systemic inflammation
Inflammation associated with underlying lung disease may be partly responsible for the development of pulmonary hypertension in hypoxic states.
Inflammatory cells have been detected in local vascular structures, evidence of systemic inflammation with raised inflammatory makers, such as CRP, and TNF-α .Studies by Chao et al in 2009,in rats exposed to hypoxia ,alveolar macrophages play a critical role in the inflammatory process ,with inflammation occurring in the presence of reduced alveolar PaO2.
46
Diagnosis of Pulmonary Hypertension
The evaluation process with suspects PH requires a series of investigations confirm the diagnosis, clarify the clinical group of PH to evaluate functional and hemodynamic impairment.
Clinical presentation
Symptoms includes breathlessness, fatigue, weakness, angina and syncope.
Physical signs of PAH include left parasternal lift second heart sound, pansystolic murmur of tricuspid regurgitation. Lung sounds are usually normal.
Right –Heart catheterization
Invasive hemodynamic assessment by RHC is pivotal in the evaluation of any patient with suspected PAH. Right –Heart catherization is typically performed after the non invasive testing for PH. In RHC essential measurements include:
o Oxygen saturation o Right atrial pressure o Pulmonary artery pressure o Left sided filling pressure o Cardiac index/Cardiac output o Systolic blood pressure o Heart rate
o Response to acute vasodilators
47
In recent study of 94 patients with bronchiectasis,31 patients (32.9%)had PH,defined as systolic Pulmonary artery pressure of ≥40mmHg on Doppler echocardiography100.Significant correlation was obseverd between right ventricular dimensions and systolic pulmonary artery pressure(r=0.74)while RV dimension were inversely related to PaO2 values(r=0.37).
Chest radiograph
In 90% of patients with PAH the chest radiograph is abnormal at time of diagnosis. Findings include loss of peripheral blood vessels. Right atrium and ventricle enlargement more seen in advanced cases. Chest radiograph allows associated moderate –to – severe lung disease, or pulmonary venous hypertension due to left heart disease. The degree of PH in any given patient doesnot correlate with extent of radiographic abnormalities.
Pulmonary Function Tests
This testing is a necessary part of initial evalutation of all patients with pulmonary hypertension, to exclude or characterised the underlying airway or parenchymal lung disease. Patients with PAH usually have decreased lung diffusion capacity for carbon monoxide and mild to moderate reduction of lung volumes. A decrease in lung volume together with a decrease in diffusion capacity for carbon dioxide tension, may indicate pulmonary hypertension.
48
Echocardiography
Doppler echocardiography should performed as a noninvasive screening test to detect elevated pulmonary artery pressure. It provides an estimation of right ventricular function and pulmonary artery pressure and can reveal other underlying cardiac abnormalities. Signs indicative of pulmonary hypertension on echocardiogram include increased pulmonary artery hypertension or tricuspid regurgitant jet, right atrial and ventricular hypertrophy, flattening of intraventricular septum, small left ventricular dimension and dilated pulmonary artery. Echocardiography is useful in disease progression, removing the need for repeated pulmonary artery catheterizations.
Patients with suspected pulmonary hypertension, right-sided heart catheterization is required to confirm the presence of PH, to establish the specific diagnosis, and to determine the severity and prognosis of pulmonary hypertension. It also can be used to evaluate for vasoreactivity and guide therapy. A acute response to a vasodilator is defined as a fall in pulmonary artery hypertension of at least 10mm Hg to 40mm Hg or less, with an increased or unchanged cardiac output. Eysmann et al(30) proven that right heart catherization is required to perform presence of PH, for diagnosing purposes.
In a study conducted by Abdulaziz et al(20) in 2008 found that systolic pulmonary artery pressure was higher in cystic bronchiectasis with right and left ventricular dysfunction.
49
Study showed correlation between HRCT score and SPAP and FEV%.It showed SPAP could be a predictor of future pulmonary hypertension .Patient with cystic bronchiectasis have significantly higher impairement of pulmonary physiology as compared with cylindrical bronchiectasis patients.
Figure 13 :Echocardiography demonstrating right ventricular enlargement.
Figure : 14
50
Approach to therapy
Treatment goals for patients with pulmonary hypertension include reduction in clinical signs, and symptoms and improvement in exercise tolerance, hemodynamics, and quality of life, with decreased need for hospitalization and longer survival. Long term oxygen administration has been shown partially to reduce the progression of pulmonary hypertension.
Phosphodiesterase Type -5 Inhibitors
Pulmonary vasodilating effects of nitric oxide are mediated through cGMP.Nitric oxide activates guanylate cyclise,which increases cGMP production.Cyclic GMP causes vasorelaxation,but effects are attenuated by rapid degradation of cGMP by phoshodiesterase.Sildenafil and Tadalafil inhibit phosphodiesterase type 5,thus enhancing relaxation and growth inhibition of vascular smooth muscle cells..both have demonstrated improvement in exercise capacity and functional class.
Endothelin Receptor Antagonists
Endothelin -1 is a potent vasoconstrictor and smooth muscle mitogen, that is over expressed in the plasma and lung tissue of patients with pulmonary hypertension.
o Bosentan is a oral nonselective endothelin receptor antagonist,
o Ambrisentan is a oral selective endothelin type A receptor antagonist, both of these agents have been shown to improve exercise capacity, functional class, and time to clinical worsening.
51
Prostanoids
Prostacylin is a potent vasodilator and inhibitor of platelet activation and smooth muscle proliferation. Three prostanoids have been shown to improve exercise capacity, quality of life, hemodynamics are epoprostenol,treprostinil, and iloprost.
Thus the above mentioned studies shows that alveolar hypoxia in bronchiectasis is a potent stimulus for pulmonary vasoconstriction was leading to the development of pulmonary artery hypertension and bronchiectasis patients with pulmonary hypertension have worse survival than bronchiectasis subjects without pulmonary hypertension.Studies also found that extent and severity of bronchiectasis correlated strongly with severity of airflow obstruction. Clinical determinants of the 6-Minute Walk test in bronchiectasis provides a valuable information of the functional status in a group of patients with mild to moderate bronchiectasis and also indicates dynamic hyperinflation and increased work of breathing are responsible of disease.
52
RESULTS
We present our study results under following description 1. Distribution of study variables
2. Comparison of the variables within the study population 3. Correlation of variables in the study population
DISTRIBUTION OF VARIABLES IN STUDY POPULATION
A total of 40 patients were recruited for the study. In that 19 patients were cystic, 11 were cylindrical and 10 were varicose. Out of 40 patients 17 were male (42.5%) and 23 were female (57.5%). The mean age group of the study was 45-55 years. The average age was 5510 years. Among the study population 13 patients had history of smoking and 2 patients had history of alcohol intake.
Socio-demographic details N=40
Age 5510 years
Gender Male Female
17 (42.5%) 23(57.5%) BMI
Male Female
22 24
53
Table 1.a. DISTRIBUTION OF PATIENTS BASED ON GENDER Among the study population, out of 40 patients ,17 were male(42.5%) and were 23 female(57.5%).
Gender Numb
er of patien ts
Percent age
Male 17 42.5%
Female
23 57.5%
0 5 10 15 20 25
MALE FEMALE
DISTRIBUTION BASED ON GENDER
NUMBER
54
Table 1.b. DISTRIBUTION OF PATIENTS BASED ON AGE Among 40 patients, most of the patients in the age group of 40 to 60 year old.
The mean age of the study population was 5510 years. Distribution of patients in the age group of less than 40 years and more than 40 years as given below.
Age group Number of patients Percentage
<40 6 15%
40-60 20 50%
>60 14 35%
TABLE 1.c. DISTRIBUTION OF PATIENTS BASED ON BMI Among 40 patients the average BMI was 23.6 kg/m2. The mean BMI of male patients was 22 kg/ m2 and for female it was 24 kg/m2. There was no significant correlation between the BMI and extent of bronchiectasis.
BMI(mean)
Male 22
Female 24
55
TABLE IIa DISTRIBUTION OF PATIENTS BASED ON THE TYPE OF BRONCHIECTASIS
The extent of bronchiectasis analyzed and based on the involvement of number of chambers we classifies the patients into 3 different groups. The distribution of patients is demonstrated in the following table.
CLASS NUMBER OF PATIENTS TOTAL PERCENTAGE
MALE FEMALE
CYSTIC 6 13 19 47.5%
CYLINDRICAL 7 4 11 27.5%
VARICOSE 4 6 10 25%
56
II b . CORRELATION OF NUMBER OF SEGMENTS INVOLVED AND TYPE OF BRONCHIECTASIS
Among the 40 patients, we studied the number of lung segment involved in each patient . In that the patients in the cystic group showed more number of segment involvement than other groups.
The current study shows no significance in correlation between the radiological findings and extent of bronchiectasis. The patients in cystic group shows negative correlation of (r = -0.03), which shows the extent of bronchiectasis cant be predicated using radiological findings.
Group of patients No of segment involved
<4 4
Cystic 10 9
Cylindrical 4 6
Varicose 11 0
57
II c .CORRELATION OF HRCT SCORE WITH TYPE OF BROCHIECTASIS
The HRCT score done based 3 parameters
Bronchial dilation
Mucus thickness
Bronchial thickness
Among the parameters bronchial thickness was rare among the study population. Most of the patient had high HRCT score.
The patients in cystic group shows significant correlation with the HRCT findings (r=0.23) and extent of bronchiectasis. The positive correlation of HRCT score was statistically significant only in cystic group compared to others.
58
III. a .CORRELATION OF TYPE OF BROCHIECTASIS WITH FEV1/FVC RATIO
Among the 40 patients, based on extent of bronchiectsis patients were distributed into 3 different groups. In the 3 groups,cystic group shows the FEV1/FVC ratio was significantly low (p≤0.05). In cylindrical group also the ratio was low but it was not statistically significant.
Group of patients Mean FEVI/FVC P value
Cystic 34.815 0.04
Cylindrical 38.7114 0.3
Varicose 74.0716 0.68
59
III.b. CORRELATION OF 6 MINUTE WALK TEST AND FEV1/FEC IN STUDY POPULATION
In our study we observed a significant correlation between the 6 minute walk test and the pulmonary function test. Patients with altered PFT shows low score in six minute walk test .There was a positive correlation (r=0.25) in the study population with significant alteration in the FEV1/FVC . The 6 minute walk test was found to be useful in patients with bronchiectasis in predicting the severity.
According to the walking distance the patients were scored based on the following criteria
Distance Score
>350 0
250-349 1
150-249 2
≤149 3
We consider walking distance more than 400m as normal. Among the study population a total of 11 patients got score 3 , 5 got 2 , 7 got a score of 1 and 17 got score zero. There was no patient covered a walking distance of 400 m among the study participants. The significant correlation of FEV1/FVC ratio with 6 minute walk test shows p value (p<0.01)
60
III.c. CORRELATION OF MODIFIED MEDICAL RESEARCH COUNCIL DYSPNEA SCALE (MMRC) WITH TYPE OF
BRONCHIECTASIS
Among the study population while correlating the MMRC score , there was more patients coming under MMRC score of 2 . The patients in cystic group showed a high MMRC score as compared to the cylindrical but it was not statistically significant (p=0.8). the mean MMRC score in the study population was 2.
III.d. CORRELATION OF MODIFIED BORG DYSPNEA SCALE SCORE (BORG) WITH TYPE OF BRONCHIECTASIS
Among the study population while correlating the BORG score there was more patients coming under the score of 4 . In the current study there was no significant correlation between the BORG score and extent of bronchiectasis (p=0.98). The mean score of the entire study population was 4.
