DOI 10.1378/chest.108.4.955 1995;108;955-961Chest

 Shepherd and Rick CarterM. Brooke Nicotra, Manuel Rivera, Andrew M. Dale, Robert Bronchiectasis in an Aging CohortMicrobiologic Characterization of Clinical, Pathophysiologic, and

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Clinical, Pathophysiologic, andMicrobiologic Characterization ofBronchiectasis in an Aging Cohort"M. Brooke Nicotra, MD, FCCP; Manuel Rivera, MD, FCCP;Andrew M. Dale, MD; Robert Shepherd, MD;and Rick Carter, PhD, FCCP

Study objective: Awareness of bronchiectasis on thepart of clinicians has been low in recent years, al¬though it was previously well recognized. We believethat bronchiectasis is underdiagnosed, and that cur¬

rent literature is skewed toward the esoteric etiologiesof bronchiectasis.Design: We reviewed the clinical, radiologic, microbi¬ologic, and physiologic findings in 123 well-studiedpatients with proved bronchiectasis.Setting: The University ofTexas Health Center at TylerHospital and Clinics.Measurements and results: There were 38 men and 85women with a mean (±SD) age of 57.2±16.7 years;55% were lifetime nonsmokers. Diagnosis was con¬

firmed with CTs ofthe chest in 56%, by bronchogramin 28%, and surgery with the remainder. Seventy per¬cent of patients gave a history of an antecedentpotentially causative event for the bronchiectasis,usually pneumonia. Symptoms of bronchiectasis in¬cluded chronic cough with the production of purulentsputum, hemoptysis, recurrent fever, and pleurisy.

The finding of crackles on chest examination was therule (70%) with wheezing present in 34% ofthe group.Pulmonary function studies documented airway ob¬struction to be present in 54% of the lifetime non-

smokers. The chest radiographs were abnormal in91.3%, showing fibrotic stranding and infiltrates. Avariety of pathologic microbial flora, particularlyPseudomonas aeruginosa and other opportunistic or¬

ganisms, were isolated from the sputum. Patients whohad smoked had much the same picture as nonsmok¬ers, although they had a greater degree of airway ob¬struction.Conclusions: A characteristic clinical picture of bron¬chiectasis emerges after reviewand evaluation ofthesedata. Knowledge of this picture should allow readyrecognition of the disease.

(CHEST 1995; 108:955-61)

Key words: abnormal chest radiograph; bronchiectasis;chronic cough; clinical diagnosis; COPD

"Dronchiectasis was well recognized in the preanti-¦*"* biotic era.1 Osier2 lists factors associated with thedevelopment of bronchiectasis as bronchial wall in¬flammation and obstruction ofthe lumen by secretions.Conditions leading to these abnormalities includedchronic bronchitis and emphysema, chronic phthisis,the catarrhal pneumonias ofchildren, and the presenceof foreign bodies in the air tubes.More recently, bronchiectasis has been described as

"an orphan disease" with a prevalence estimated to be"probably low and decreasing."3 Emphasis has beenplaced on genetic abnormalities as likely etiologies forbronchiectasis. Over the last 10 years, and in the course

ofobserving a large population ofpatients with chronicpulmonary diseases, we have become convinced thatbronchiectasis is not a rare disease and that geneticdisease is not the usual etiology. We have beenimpressed further with the frequency with which thisprocess still goes unrecognized, even when character-

*From the Departments of Medicine, Radiology, and Physiology,The University of Texas Health Center at Tyler.Reprint requests: Dr. Nicotra, PO Box 2003, The University ofTexasHealth Center at Tyler, Tyler, TX 75710

istic features are present, and with diagnostic tech¬niques readily available. We reviewed the records of allpatients with a diagnosis of bronchiectasis seen at theUniversity of Texas Health Center at Tyler since 1985.We have integrated the historic information, clinical,radiographic, spirometric, and microbiologic featuresof this condition to define a set of "typical" findings,enabling prompt diagnosis.

MethodsA retrospective review ofpatients seen at the University ofTexas

Health Center at Tyler from 1985 to 1993 with a diagnosis ofbronchiectasis was carried out. In each case, the diagnosis had beenmade by the patient's primary physician on the basis of an assess¬

ment of the overall clinical picture. Because of the retrospectivenature of this report, it was not possible to determine whatdiagnostic criteria were being used for the original diagnosis by theprimary physician.The charts were analyzed for demographic data, clinical, spiro¬

metric, and microbiologic information. The information was ana¬

lyzed from the time of presentation, even if the diagnosis of bron¬chiectasis was not evident initially. All subsequent data were alsoanalyzed; in many cases, information from a several-year period wasavailable. Pathology and radiology reports, including those ofbronchograms and CTs, were reviewed. A case was considered to

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Table 1.Demographic and Smoking Data

Data No.

No. of patientsAge, yr, at studySex, %MaleFemale

Race/ethnicity, %WhiteBlackHispanic

Smoking history, pack-yrAll smokers

Male*Female

Lifetime nonsmokers, %

12357.2±16.7

30.969.1

92.76.50.8

29.8±23.730.5±28.48.9±17.754.5

*Smoking history differs between men and women at p <0.0001.

<20 20-29 30-39 40-49 50-59 60-69 70-79 >80AGE (YEARS)

Figure 1. Age distribution in 10-year increments of the 123patients with bronchiectasis.

be proved if one or more of these demonstrated evidence of bron¬chiectasis.A review was carried out of all available chest radiographs using

the chest radiographs closest in time to the diagnosis of bron¬chiectasis, unless the radiographic reports documented the pres¬ence of a pneumonia that subsequently cleared. The plain chestradiographs from the time of diagnosis of bronchiectasis and themost recent radiographs were independently reviewed by a radiol¬ogist using the criteria outlined by Gudbjerg4 for the diagnosis ofbronchiectasis; these criteria included the presence of increasedpulmonary markings, honeycomb-like structures, atelectasis, andpleural changes. The extent of lung involvement was estimated byrecording the number of lobes that exhibited one or more of thecriteria of Gudbjerg for the diagnosis. CTs were performed usingthe high-resolution technique.56 The presence of bronchiectasiswas confirmed with the finding of abnormally dilated bronchi nearthe periphery of the lung fields, failure of bronchi to taper with in¬

creasing distance from the hilum, or the presence of cystic dilata¬tions of the bronchi with or without fluid levels.

All spirometric studies performed in these subjects were obtainedby searching pulmonary function laboratory computer disks, on

which were recorded all pulmonary function tests performed since1984. Linear regression equations for the change in FEV! were

generated for patients who had more than three spirometric stud¬ies performed over at least a 1-year period. Spirograms were per¬formed using a rolling seal spirometer (P.K. Morgan; Dallas). Lungvolumes were measured by body plethysmography (Cardiopulmo¬nary Instruments; Houston). The normal values ofCrapo et al7 wereused to calculate predicted spirometric values. Normal predictedlung volumes were derived from the equations of Goldman andBecklake8 (women) and Boren et al9 (men). All calculations were

made by personal computer (EBS Software and IBM PC; Dallas).The complete spirometric findings were then stored digitally on a

computer disk. Lung function testing was performed according toAmerican Thoracic Society criteria.10

Descriptive statistics (mean±SD) were computed (Quattro Pro4.0 and the Statistical Analysis System [SAS]). Correlations were

used to determine statistical relationships. Nonparametric andparametric statistics were used to detect significant differences be¬tween groups after adjusting for unequal sample size. Statisticalsignificance was set at p less than 0.05 or less.

ResultsA total of 363 patients with a diagnosis of bron¬

chiectasis were found by a search of the medical

records database. In 97% of patients, we were able toreview the charts. The diagnosis was incorrect due to

miscoding or insufficient clinical data were availablefor review in 40 (8%) of the patients. In 200 (55%) ofthe patients, the diagnosis was unproved. The remain¬ing 123 patients form the basis for this report.

In 69 (56%) ofthe patients, the clinical diagnosis wasconfirmed on the basis of CT, in 35 (28.5%) by bron¬chography, in 23 (18.7%) by surgery (5 of whose con¬

ditions had been previously diagnosed), and 1 patientby autopsy. In the earlier patients, a bronchogram or

surgery was a more frequent diagnostic tool, while inthe later patients CT was routinely used for diagnos¬tic confirmation.

Demographic and smoking data are shown in Table1. Although the mean age of the group was 57.2 years,the modal age was 61 years. Figure 1 demonstrates thatthe highest frequency of patients was in the 60- to 80-year-old age group. Only seven patients (5.7% of thegroup) were younger than 30 years old. Table 1 showsthat 70% of the group were women, mostly white. Ofinterest, the patient population at our institution is 30%nonwhite, predominantly black. More than half of thepatients, 54%, were lifetime nonsmokers while theaverage smoker had a 29.8±23.7 pack-year history.There was a striking difference in the smoking historybetween men and women (p<0.0001).

As may be seen in Table 2, 86 patients (69.9%) gavea history ofa significant pulmonary event preceding theonset of the respiratory symptoms. Despite the factthat the symptoms had often been present for manyyears prior to presentation to this hospital, 84 ofthe 123patients (60%) were able to give a definite time atwhich they felt the injury had occurred. The mean ageof injury was 20.1±23.1 years. Almost all the patients(98%) knew when the symptoms started, with an

average onset at age 38.6±24.8 years. The averageduration of time between injury and onset of symp¬toms, which could be determined only in the 81

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Table 2.Etiology ofBronchiectasis Table 3.Symptoms and Signs ofBronchiectasis

Etiology No.

Patient-stated injury as cause, % 69.9

Age, yr, atinjury 20.1±23.1 (n=84)Age, yr, at onset of symptoms 38.6±24.8 (n=120)Time between injury and onset symptoms, yr 13.6± 19.2 (n=81)Type of injury, % of patientsNo known injury 30.1Pneumonia 35.0Genetic disease* 4.1Pertussis 7.3Other childhood diseases 3.2Granulomatous diseasef 9.8Miscellaneous* 10.6

*ai-Antitrypsin deficiency, immotile cilia syndrome, cystic fibrosis.*Pulmonary tuberculosis, sarcoidosis.* Radiation pneumonitis, hypogammaglobulinemia, aspiration, trauma,foreign body obstruction.

patients who dated both events, was 13.6±19.2 years.In 35% of the patients the antecedent lung injury

was described as a "pneumonia," frequently one thathad occurred in childhood. Other specific childhoodillnesses, particularly pertussis, were also cited. Bron¬chiectasis following mycobacterial diseases, predomi¬nantly Mycobacterium tuberculosis, usually developedin older individuals. Infrequently, a variety of otherconditions were identified as antecedent etiologicevents. Most patients did not have specific studiesaimed at excluding a genetic disease such as cystic fi¬brosis as an etiology of the bronchiectasis. In allpatients younger than 30 years, however, more exten¬sive evaluation was carried out. In only five patients ofthe entire group was a contributing genetic diseasefound, (ie, ai-antitrypsin deficiency, cystic fibrosis,immotile cilia syndrome). Assessment of the levels ofimmune globulins was not systematically performed.Still, almost half of the patients had such studies, andin only five patients was hypogammaglobulinemia or

agammaglobulinemia present.As shown in Table 3, cough was the most common

symptom, affecting 90% of the cohort. Of the 30 pa¬tients (24.4%) who did not produce daily sputum, 24produced significant quantities of sputum in the pres¬ence of an upper respiratory tract infection. Hemop¬tysis was a frequent phenomenon (51.2%), with theepisodes tending to recur, and often at least one epi¬sode in which hemoptysis exceeded a tablespoon ofblood. Of interest, ten of the patients who did not

produce daily sputum did have significant hemoptysison at least one occasion, and three of these individu¬als had gross hemoptysis without any antecedent spu¬tum production.A history of recurrent episodes of fever, frequently

accompanied by pleuritic chest pain, was present inmost patients. Dyspnea was also a common complaint.None of these findings were statistically different

No. (%) of Patients With FindingAll Patients Nonsmokers Smokersn= 123 (100) n=67(54.4) n=55 (44.7)

SymptomsCough 111 (90.2) 59 (88.1) 52 (94.5)

Daily sputum 93 (75.6) 52 (77.6) 41 (74.5)Sputum with URI* only 24 (19.5) 10 (14.9) 14 (25.5)Hemoptysis 63 (51.2) 37 (55.2) 26 (47.3)Recurrent fever 86 (69.9) 50 (74.6) 36 (65.5)Recurrent pleurisy 57 (46.3) 34 (50.7) 23 (41.8)Dyspnea 88 (71.5) 45 (67.2) 43 (78.2)

SignsCrackles 86(69.9) 47(70.1) 39(58.2)Wheezes 42(34.1) 17(25.4) 25(45.5)Squeaks/rhonchi 54 (43.9) 30 (44.8) 24 (43.6)Clubbing 4 (3.2) 2 (3.0) 2 (3.6)

*URI=upper respiratory tract infection.

(p>0.05) between nonsmokers and smokers.The usual abnormality on chest examination was the

presence of crackles by auscultation. Wheezing was

less common, and it was no more frequent in thesmokers (20.3% of smokers vs 13.8% of nonsmokers,p>0.05). Another abnormality noted in many of thepatients was the finding of high-pitched inspiratorysqueaks. The presence of finger clubbing was rare.

Abnormalities on expiratory spirometry, which was

performed in 96 of the patients, were present in boththe smoking and the nonsmoking groups (Table 4).The FVC was reduced at least moderately for allgroups, suggesting the possibility of restrictive lungdisease. However, in those nonsmoking patients whohad determination oflung volumes (n=27), the restric¬tion was confirmed by a reduction in total lung capac¬ity (<90% predicted) in only 9 individuals. Interest¬ingly, a reduced FEVi/FVC ratio less than 70%,documenting airway obstruction, was present in thenonsmokers as well as in those who had smoked. In

Table 4.Pulmonary Function*

Parameter SexWholeGroup Nonsmokers Smokers

FVC, % pred Male

Female

FEVi, % pred Male

Female

FEVi/FVC, % Male

Female

63.9(21.0)70.8(18.5)49.3(24.6)60.2(22.4)59.0(18.7)65.8(13.5)

59.1(17.7)74.7(19.1)47.3(18.7)66.2(22.3)63.5(11.2)68.4(11.7)

65.3(21.6)62.2(13.4)48.9(26.0)46.6(15.8)57.7(20.1)59.9(15.4)

*Figures in parentheses represent standard deviation.

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fact, a normal FEVi/FVC ratio (FEV1/FVC >70%) was

present in only 24 of the 52 nonsmokers (46%) withstudies available.

In 21 nonsmoking patients, adequate studies wereavailable to calculate the slope ofthe FEVi change peryear (minimum of three studies over 3 or more years).A mean decrease of 0.054±0.089 L/yr was noted. Insome patients, progressive disease leading to respira¬tory failure and death did occur. Since all patients werenot followed throughout the course of disease, we

cannot give an exact figure for the proportion ofpatients who developed marked deterioration, as thiswould require a prospective study.

Plain chest radiographs were reviewed for 104patients at presentation using the criteria described byGudbjerg. In only nine patients were the findingsentirely normal. Changes consistent with increasedpulmonary markings were present in all patients whohad abnormal radiographs. These changes are oftendescribed on routine chest radiograph as scarring or

chronic infiltrates. Honeycombing of the lung was

present in 47% of the cases reviewed, even though theclinical interpretations rarely described this finding.Volume loss and pleural abnormalities were less fre¬quent (53.7 and 32.6%, respectively) than parenchymalfibrotic changes. The severity of the changes was no

different in the smoking and nonsmoking patients.The distribution of the abnormalities within the

lungs was approximately equal except that the rightupper lobe was often uninvolved. Multilobar involve¬ment was common. Only 22.1% of patients had a sin¬

gle lobe involved, with 24.2% having two, 18.9% hav¬ing three, and 15.8% having four lobes involved. Alllobes were involved in 15.8% of the patients.

In 65 patients, serial plain chest radiographs span¬ning an interval of at least 2 years were available forreview and comparison. The mean length of time be¬tween radiographs was 4.04±1.71 years. Fifty-fourpatients (83.1%) had no radiographic deterioration or

actual improvement over this period. In 13.8%, wors¬

ening of radiographic abnormalities in the same area

had occurred; in only 3.1% was a new area of abnor¬mality evident on the radiograph.One or more sputa cultures was available for review

in all but 18 patients. Given the chronicity of the spu¬tum production and respiratory complaints, it was not

always possible to determine if the patient was judgedto be acutely ill at the time of culture. In 88% of thepatients, a pathogenic organism was recovered fromthe sputum at least once. As can be seen in Table 5, a

wide variety of microbes was cultured from thesputum. The number of pathogens present over timein individual patients was widely variable, with 39% ofthe patients having a single pathogen, 29% with two,10% with three, and 8.5% with four or more pathogens.Gram-positive cocci represented 18% of the organ-

Table 5.Microbiology*Whole Group Nonsmokers Smokersn=123* (%) n-67 (%) n=55 (%)

Gram-positive cocci

Staphylococcus aureus 9 (7.3) 4 (6) 5 (9)Streptococcus pneumoniae 13(10.6) 7(10) 6(11)P-Hemolytic streptococci 9 (7.3) 5 (8) 4 (7)

Gram-negative bacteriaHaemophilus influenzae 37 (30.1) 19 (28) 18 (33)Pseudomonas aeruginosa 38 (30.9) 19 (28) 19 (35)Mucoid strain 17(13.8) 11(16) 6(11)

Gram-negative bacilli* 16(13.0) 10(15) 6(11)Moraxella catarrhalis 3(2.4) 1(1.5) 2(3.6)Anaerobic organisms 2 (1.6) 1(1.5) 1(1.8)Nocardia 4(3.3) 3(4.5) 1(1.8)Mycobacteria 28(22.8) 17(25) 11(20)Avium-intracellulare 21 (17.1) 14 (21) 7 (13)

Aspergilli6(4.9) 2 (3)4 (7)*Number (%) of patients in whom each organism was isolated fromsputum. Repetitive cultures in the same patient were not counted.

^Klebsiella pneumoniae, Escherichia coli, Citrobacter, Serratia marce¬

scens, Proteus, P multocida, Morganella, Alcaligenes, Acinetobacter.*One patient out of total of 123 had unknown smoking status.

isms. Pseudomonas aeruginosa was cultured in 38 pa¬tients, representing 23% of the total organisms; in 17patients, the mucoid strain of Pseudomonas, a markerof severe structural damage in the lung,11 was culturedat least once. Haemophilus influenzae was the secondmost common isolate, while other Gram-negative ba¬cilli were often present. Many of the patients had re¬

petitive cultures with the same organisms, particularlyH influenzae and P aeruginosa; in particular, once themucoid Pseudomonas was found, it tended to persistthereafter.Of note, and in line with recent observations in the

literature,12 Mycobacterium avium-intracellulare was

a common isolate, found in 21 patients. Nocardia andaspergilli were found in ten patients. Growth of allthree of these organisms tended to occur repetitively,but only intermittently with low colony numbers,sometimes with several years between positive cul¬tures. In no instance could a direct pathogenic role beattributed to these organisms and no specific therapywas directed at them.

DiscussionBronchiectasis as a primary pulmonary diagnosis

seems to be uncommon, judging by a review ofcurrentliterature,3 although we are not aware ofany incidenceor prevalence data for bronchiectasis for the UnitedStates. The reason for the apparent underdiagnosis ofthis debilitating lung disease may reside in the fact thatfew textbooks in pulmonary medicine portray bron¬chiectasis as a significant and/or common lung prob¬lem. This may be partly due to the lack of recent de¬scriptive data in patients with this disorder who are

older and who do not have a genetic bases forbronchiectasis.

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We have described 123 patients with an establisheddiagnosis of bronchiectasis. These patients share com¬mon clinical features; in contrast to patients with em¬

physema and chronic bronchitis.1 '14 54% of our

patients never smoked and 70% are women. The agegroup is older than the usual patient described with a

genetic disorder, and in most, there is no history of anysystemic immune deficiency. Bronchiectasis appearsto result from anatomic damage caused by prior bac¬terial or viral infection of bronchi. The age at presen¬tation with bronchiectasis averages 10 to 20 years afterthe causative incident, although since a detailed historyfocused on childhood pneumoniawas not always taken,the causative event may have occurred earlier than our

data suggest. Chronic purulent sputum production,recurrent fever, and hemoptysis occur in the majority,with recurrent bouts ofpleurisy present in almost half.The overwhelming number (91.3%) of plain chest ra¬

diographs are abnormal, showing chronic fibroticstranding and infiltrates, with honeycombing oftenseen as well. Microbiologic studies demonstrate colo¬nization or infection by a wide variety of organisms.Pulmonary function studies, considering only thenonsmokers, indicate primarily obstructive changes.

In the presence of these findings, it should not bedifficult to establish a diagnosis ofbronchiectasis. A CTof the chest done with the high-resolution techniquewill confirm the diagnosis. Bronchograms should not

routinely be necessary. The diagnosis should particu¬larly be considered in nonsmokers with airway ob¬struction, patients with chronic cough or recurrentbouts of purulent bronchitis, and in patients with he¬moptysis of uncertain etiology. Fiberoptic bronchos¬copy is not routinely performed. However, in thepresence of localized disease, particularly with volumeloss, the possibility of foreign body obstruction or tu¬mor should be considered and the airway investigatedby bronchoscopy. In a patient with diffuse disease,adequate culture material is generally obtainable withexpectorated sputum. In a patient unable to producesputum where infection is suspected or in the presenceof significant hemoptysis, bronchoscopy should beperformed.The demographics of our cohort are in contrast to

earlier studies that documented a slight male predom¬inance.115 Since the overall age of our population isolder than in the earlier publications, early mortality inmen may have had a selective effect. Smokers may bemore likely to be classified as having chronic bronchi¬tis rather than bronchiectasis; in the older individualswho made up most of our population, men were more

likely than women to have smoked. Finally, there is thepossibility that women may be more subject to persis¬tent lung damage after an insult than are men.16The presumed etiology of the bronchiectasis in our

patients was postinfectious in the majority; prior severe

infection was cited by 71 ofthe 86 patients who relateda prior event to the bronchiectasis. Our study was ret¬

rospective and therefore a thorough evaluation forunderlying genetic or acquired disease was not alwaysperformed. However, our findings are in agreementboth with earlier studies of populations with bron¬chiectasis17"19 and with a recent very meticulous andthorough prospective study of adults with bronchiecta¬sis.20 In this latter study, of 38 patients with diffusebronchiectasis with a mean age at study of 46 years,only 5 were found with primary ciliary dyskinesia and5 with underlying mucus disorders; none had hypo-globulinemia or ai-antiproteinase deficiency. Of thefive patients with ciliary dyskinesia, four individualswere from the Moslem Magreb population of NorthAfrica where a high degree of consanguinity exists;primary ciliary dyskinesia was present in only one

"European" patient in the group. This is in definitecontrast to opinions stated in recent textbooks of pul¬monary disease21,22 in which underlying systemic im¬mune defects are suggested as the usual cause ofbronchiectasis.

It might be questioned whether we missed geneticdisease because the patients excluded from furtherstudy because of a lack of documentation of anatomicbronchiectasis had more obvious clinical disease. Wedo not believe that this is the case, as the excludedgroup ofpatients had a mean age of63 years, which wasactually older than the group that we report in detail(Table 1). Further, only eight of these patients were

younger than 40 years old.We believe that the older age of our group is strong

evidence against inherited disease, particularly cysticfibrosis, the prime mucus disorder. A recent carefulreview of patients with cystic fibrosis with genotypicconfirmation of the disease found even the patientswith the most mild disease had a mean age at diagno¬sis of 11 ±6 years, and the oldest group, heterozygousfor the gene and without any evidence of pancreaticinsufficiency, averaged 26±9 years at the time ofstudy.23 However, because our patients did not un¬

dergo gene typing, we cannot conclusively rule out a

contribution of a cystic fibrosis gene in the cause ofthedisease.Our data document a lag time between the age at

injury and the age at onset of symptoms. We believethat the initial insult results in structural damage to theairway, which in itself may not cause symptoms; intime, however, the superimposition of chronic inflam¬mation on the anatomic damage leads to furtherabnormalities, and symptoms occur.124Our review ofthe symptoms and signs in this patient

group indicates the presence of a typical syndrome,characterized by cough, usually productive of sputum,which is purulent but not copious or foul smelling.Hemoptysis is frequent and tends to recur; the bleed-

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ing may be moderate or severe in amount. Dyspnea is

present in most (71%) patients. Recurrent bouts offever and pleurisy are common. Physical examination

typically reveals the presence of inspiratory crackles;wheezing is found in 34% of patients, whether smok¬ers or not. In comparing our findings with those ofPerry and King,1 published in 1940, it is striking thatvery little has changed with the exception of a decreasein the purulence, foul odor, and amount ofthe sputum.We have limited ourselves to nonsmokers in evalu¬

ating the physiologic data, so that the effects of smok¬ing will not confuse the picture as has occurred inearlier publications.25,26 Given the underlying abnor¬mality, any combination of physiologic abnormalitiesmay be expected; expiratory airflow obstruction was

the most common abnormality. The obstruction maybe due to structural damage to the airways, the pres¬ence of secretions, or even bronchial hyperexeitabili-ty.26,27 Restrictive changes were seen less often despitethe destruction and scarring that occurs; it is possiblethat restriction was masked by the obstruction. Pul¬monary function did deteriorate based on the yearlydecrement in FEVi, although the fall was not markedlygreater than would be expected in a normal populationofthis age.28 In some patients, however, progression to

respiratory failure did occur.

The characteristic plain chest radiographic featuresof bronchiectasis, outlined by Gudbjerg4^in 1955 andreinforced by Fraser and his colleagues, were presentin 91% of our population as well. It is the abnormalradiograph that may lead to consideration of bron¬chiectasis rather than chronic bronchitis since "theplain roentgenogram reveals changes highly suggestiveofbronchiectasis in the great majority ofpatients."29 Inchronic bronchitis, however, the same authors statethat "it is emphasized that chronic bronchitis is not a

roentgenologic diagnosis' (italics those of the originalauthor).29 The abnormalities on the chest radiographmay cause confusion with other diseases involving thelung parenchyma, but interpretation of these abnor¬malities in the clinical context should minimize thisconfusion.The development of techniques for obtaining and

interpreting CT of the airways and lung parenchymahas markedly simplified confirmation of the diagnosisof bronchiectasis.0,6 We do not believe that a diagnos¬tic CT is always needed. In many of our patients, thediagnosis was evident based on clinical features plusevidence of chronic infiltrates, honeycombing, and attimes, thickened bronchial walls containing air-fluidlevels. High-resolution CT should be helpful to con¬

firm a clinical diagnosis that is in doubt. Whether a CTis adequate for preoperative evaluation before lungresection is not established. Further, pneumonia mayshorten bronchi and increase bronchial diameter. Forthis reason, a time lapse of several weeks after resolu¬

tion of the pneumonia is suggested prior to obtainingCT of the chest for confirmation of bronchiectasis.

Microbiologic evaluation of this group of patientsresulted in the identification of a wide variety oforganisms, many of which are unlikely inhabitants ofthe respiratory tract in patients with chronic bronchi¬tis.30 The occurrence of the mucoid strain of P aerug¬inosa in the sputa of patients with bronchiectasis hasbeen documented previously.11 The presence of thisstrain of Pseudomonas, as well as organisms such as

Nocardia, atypical mycobacteria, and aspergilli most

likely reflects the alteration in local immune mecha¬nisms due to the underlying bronchopulmonary dam¬age and should alert the clinician to the possibility ofunderlying bronchiectasis.

Finally, what initial evaluation for an underlyingsystemic abnormality is appropriate for a patient inwhom a diagnosis of bronchiectasis is being consid¬ered? If the individual is young, particularly whenparenthood is being considered, or if there is a familyhistory suggestive oflung disease, it maybe worthwhileto evaluate for cystic fibrosis or other underlyinggenetic abnormality. Since mucoid P aeruginosa is of¬ten associated with cystic fibrosis, it is also worthwhileto evaluate this diagnostic possibility in young individ¬uals whose secretions harbor this organism. If recur¬

rent severe infections occur, then evaluation of im¬mune status is in order. In the more usual older patient,these studies are unlikely to be of value.

Clearly, one problem in making the diagnosis ofbronchiectasis in older patients lies in separatingpatients with it from patients with chronic bronchitis.We believe that the patient populations do differ on

clinical grounds. Most of our patients had never

smoked. The mean age at onset of symptoms occurredin the fourth decade, earlier than in patients withchronic bronchitis. Although several of the symptomsof bronchiectasis such as fever, pleurisy, and hemop¬tysis may occur in patients with chronic bronchitis, theyappear to be less frequent manifestations of disease,whereas in patients with bronchiectasis, they are

common. Avery significant difference between chronicbronchitis and bronchiectasis is found in the chest ra¬

diographs, which are generally abnormal in the lattergroup and generally not so in the former. Finally, themicrobiology of the two groups is contrasting; at one

point in the past, mucoid P aeruginosa was thought tobe pathognomonic of cystic fibrosis as it was so

intimately associated with anatomic airway damageprobably resulting from increased proteolytic enzymesreleased by the bacteria. The other low-grade patho¬gens such as the fungi and atypical mycobacteria thatwere found in these patients with bronchiectasis are

rarely reported in patients with chronic bronchitis. Weare in the process of doing a comparative studybetween patients with bronchiectasis and patients with

960 Clinical Investigations

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bronchitis, but we believe that there are clinical datato separate patients with the two processes in mostcases.

The prognosis of patients with bronchiectasis is not

clearly established by this study. Based on the radio-graphic data, deterioration in radiologic appearancebased on either worsening of findings in the initiallyabnormal area or development of new areas of abnor¬malities was the exception rather than the rule, but theradiographic findings may not mirror clinical function.Based on limited serial pulmonary function data, theyearly decrement in FEVi was small, but some patientswent on to respiratory failure. It will require a large,prospective study to establish the liklihood of respira¬tory deterioration and risk factors for such deteriora¬tion in a given patient.

This study clearly leaves many questions unan¬

swered, primarily because of its retrospective nature.Our purpose has been to redefine bronchiectasis as itnow exists, and in doing so, point out its unique featuresand its differences from chronic bronchitis at presen¬tation. The elucidation of its natural history in contrastto chronic bronchitis was not carried out in this studyand is best approached prospectively. We hope tobetter define these differences in further studies andperhaps to begin evaluating both prophylactic andtherapeutic interventions that may be of benefit to

patients with bronchiectasis. Furthermore, genetictyping using the latest techniques in evaluating thepresence of mild variances of cystic fibrosis in our ag¬ing cohort is planned.

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CHEST /108 / 4 / OCTOBER, 1995 961

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DOI 10.1378/chest.108.4.955 1995;108; 955-961Chest

and Rick CarterM. Brooke Nicotra, Manuel Rivera, Andrew M. Dale, Robert Shepherd

Bronchiectasis in an Aging CohortClinical, Pathophysiologic, and Microbiologic Characterization of

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