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Vol. 72, No. 3, 2005
Issue release date: May–June 2005
Respiration 2005;72:254–262
(DOI:10.1159/000085366)

Bronchiolitis obliterans Organizing Pneumonia

Clinical and Roentgenological Features in 26 Cases

Oymak F.S.a · Demirbaş H.M.a · Mavili E.b · Akgun H.c · Gulmez I.a · Demir R.a · Ozesmi M.a
Departments of aChest Disease, bRadiology and cPathology, Erciyes University Medical Faculty, Kayseri, Turkey
email Corresponding Author

Abstract

Background: Bronchiolitis obliterans organizing pneumonia (BOOP) may be classified as cryptogenic (idiopathic) and secondary. There are no clear clinical and radiological features distinguishing between idiopathic and secondary BOOP. Objectives: To analyze the etiologic factors, clinical and radiological features, diagnostic approach and response to therapy at onset and outcome in subjects with BOOP. Methods: The medical files of Erciyes University Hospital from 1995 to 2003 were retrospectively reviewed. Patients with biopsy-proven BOOP were selected for evaluation. The etiology and initial features of BOOP, treatment, resolution, relapse, and survival were obtained from medical records, and a follow-up patient questionnaire. Results: We have diagnosed 26 cases (13 males /13 females) with BOOP syndrome (mean age 54 ± 15 years, range 14–93). More than half the patients (58%) were classified as idiopathic BOOP. Patients presented with cough (92%), dyspnea (70%), pleuritic chest pain, hemoptysis and fever (50%). The biopsy specimens had been obtained by transbronchial and/or transthoracic lung biopsy in 18 cases (69%). At radiological evaluation, there were bilateral patchy alveolar and/or interstitial infiltrates in 16 patients (62%), and solitary pneumonic involvement in 10 patients (38%). Three patients recovered spontaneously, 5 remained cured after resection of the focal lesion. Corticosteroid therapy was given in 17 patients (65%). Apart from four patients who died (death was attributable to BOOP in only 1 patient) and three patients who relapsed, the prognosis was good in all patients. Conclusions: The etiology of BOOP is usually idiopathic. We observed that hemoptysis and pleuritic chest pain were a relatively frequent symptom in BOOP in the present series, in contrast to previous observations. The diversity of radiological and clinical presentations including hemotysis and pleuritic chest pain should prompt consideration of the diagnosis in patients with persisting pulmonary symptoms and radiological findings.


 Outline


 goto top of outline Key Words

  • Bronchiolitis obliterans organizing pneumonia
  • Organizing pneumonia
  • Cryptogenic organizing pneumonia

 goto top of outline Abstract

Background: Bronchiolitis obliterans organizing pneumonia (BOOP) may be classified as cryptogenic (idiopathic) and secondary. There are no clear clinical and radiological features distinguishing between idiopathic and secondary BOOP. Objectives: To analyze the etiologic factors, clinical and radiological features, diagnostic approach and response to therapy at onset and outcome in subjects with BOOP. Methods: The medical files of Erciyes University Hospital from 1995 to 2003 were retrospectively reviewed. Patients with biopsy-proven BOOP were selected for evaluation. The etiology and initial features of BOOP, treatment, resolution, relapse, and survival were obtained from medical records, and a follow-up patient questionnaire. Results: We have diagnosed 26 cases (13 males /13 females) with BOOP syndrome (mean age 54 ± 15 years, range 14–93). More than half the patients (58%) were classified as idiopathic BOOP. Patients presented with cough (92%), dyspnea (70%), pleuritic chest pain, hemoptysis and fever (50%). The biopsy specimens had been obtained by transbronchial and/or transthoracic lung biopsy in 18 cases (69%). At radiological evaluation, there were bilateral patchy alveolar and/or interstitial infiltrates in 16 patients (62%), and solitary pneumonic involvement in 10 patients (38%). Three patients recovered spontaneously, 5 remained cured after resection of the focal lesion. Corticosteroid therapy was given in 17 patients (65%). Apart from four patients who died (death was attributable to BOOP in only 1 patient) and three patients who relapsed, the prognosis was good in all patients. Conclusions: The etiology of BOOP is usually idiopathic. We observed that hemoptysis and pleuritic chest pain were a relatively frequent symptom in BOOP in the present series, in contrast to previous observations. The diversity of radiological and clinical presentations including hemotysis and pleuritic chest pain should prompt consideration of the diagnosis in patients with persisting pulmonary symptoms and radiological findings.

Copyright © 2005 S. Karger AG, Basel


goto top of outline Introduction

Organizing pneumonia is a nonspecific response to various forms of lung injury and is the pathological hallmark of the distinct clinical entity named ‘bronchiolitis obliterans organizing pneumonia’ (BOOP) [1, 2]. BOOP is an important cause of respiratory morbidity. It has been classified as cryptogenic (idiopathic) or secondary. Histologic findings include plugs of granulation tissue lying within small airways, alveolar ducts and alveoli [1, 2]. These histologic features are a common pattern of repair. BOOP may be secondary to a number of clinical settings including inhalation of toxic fumes, immunologic and connective tissue disorders, reaction to viral, bacterial, or fungal infections; inflammatory bowel disease, human immunodeficiency virus infection, common variable immune deficiency (CVID), radiation therapy, myelodysplastic syndrome, drug reactions, malignant diseases, and bone marrow or solid organ transplantation [1, 2, 3, 4, 5]. The disease in other patients is idiopathic, and idiopathic BOOP has been named ‘cryptogenic organizing pneumonia’ [1, 2, 3, 4].

BOOP is characterized by subacute illness with shortness of breath, fever, malaise, and weight loss present for a period ranging from 3 to 6 months [1, 3, 4]. Its full clinical spectrum and course are variable. With steroid therapy, BOOP has a good prognosis [1, 2, 3, 4]. However, some patients may relapse when the glucocorticoid dose is reduced [2, 6], and some cases may progress rapidly and have a poor prognosis [7].

The aim of this study was to analyze the etiologic factors, clinical and roentgenological features at onset and outcome in patients with BOOP. Over a 7-year period we have diagnosed 26 patients of biopsy-proven BOOP and we present here detailed clinical and radiographic data of this cohort.

 

goto top of outline Patients and Methods

We reviewed the medical files of patients admitted to the Department of Chest Diseases at Erciyes University Hospital between1995 and 2003. There were 26 cases of biopsy-proven BOOP. All the patients were investigated for the presence of connective tissue disease, antineutrophil cytoplasmic antibodies, serological assays for Mycoplasma pneumoniae, Chlamydia trachomatis, Legionella pneumophila, adenovirus, cytomegalovirus, herpes simplex virus, and hepatitis virus (HBV, HCV). Clinical evaluation included immunological status, drug consumption, symptoms at presentation, routine biochemical data, medication requirement and outcome. The radiographs were reviewed by at least two experienced radiologists.

goto top of outline Clinical Analysis

We reviewed the clinical records, chest X-ray films, lung tissue sampling, routine laboratory tests including erythrocyte sedimentation rate (ESR) and full blood cell counts of all patients. We also reviewed pulmonary function tests (PFTs), chest tomography, high resolution computerized tomography (HRCT), and bronchoalveolar lavage (BAL) when available.

The diagnosis of BOOP was based on the following criteria: (1) Abnormal chest radiograph ranging from multiple acinar/nodular shadows to solitary pneumonia-like or nodular shadows; (2) histopathologically, the presence of intraluminal fibrotic buds within the alveoli and alveolar ducts with or without bronchiolar involvement, and infiltration of chronic inflammatory cells in the alveolar septa with preservation of the alveolar structure; (3) negative microbiological analysis on BAL fluid; (4) a well-documented improvement that was either spontaneous, or after exclusive steroid treatment; (5) in cases with progressive respiratory failure and increasing radiographic shadows, an open lung biopsy or autopsy that excluded other entities.

Patients with conditions diagnosed as ‘suspected’ BOOP at discharge who did not undergo open lung biopsy, surgical resection, transbronchial lung biopsy (TBLB) or transthoracic lung biopsy (TTLB) were excluded, as were many with necrosis or aggregates of neutrophils in specimens suggesting the healing stage of bacterial pneumonia or abscess.

 

goto top of outline Results

goto top of outline Etiologic Inquiries

The clinical variants of BOOP are reported in table 1. Fifteen cases (58%) had no associated condition and were reported as idiopathic; 11 cases had associated diseases such as immunologic and connective tissue disorders [1 rheumatoid arthritis (RA), one Wegener’s granulomatosis 2 systemic lupus erythematosus (SLE)]. One patient with SLE also had adrenal carcinoma. In 2 patients, BOOP was possibly related to drugs; 1 was taking amiodarone and the other was on interferon therapy. In 1 case, non-Hodgkin lymphoma was diagnosed 3 months after the treatment of BOOP with steroids. In 1 patient with chronic myelogenous leukemia, BOOP developed 1 year after autologous bone marrow transplantation. This patient had manifestations of graft-versus-host disease. One with secondary BOOP had accidentally inhaled welding vapors before he developed the current pulmonary disease. An occasional exposure to birds was disclosed in 1 patient with secondary BOOP. One with secondary BOOP diagnosed by open lung surgery was admitted to the hospital three times with the diagnosis of late resolution pneumonia. His immunoglobulin levels and white cell immunology were consistent with CVID.

TAB01

Table 1. Clinical variants of BOOP in the population study (n = 26)

The search for bacteria, viruses, and parasites was negative in sputum, bronchial and alveolar washings. All patients except 2 had negative serology for various microorganisms. One patient with BOOP had hepatitis C infection. The other patient with CVID had a positive serology for mycoplasma and adenovirus.

One patient in the idiopathic BOOP group had had arthralgias without arthritis for 7 years before the onset of pulmonary illness. Rheumatoid factor was negative in this patient. One patient with focal BOOP has a love-bird at home, but his clinical presentation clearly differed from bird-fancier’s lung. Two patients with idiopathic BOOP had gastroesophagial reflux symptoms.

goto top of outline Clinical Data

There were 13 males and 13 females (mean age 54 ± 15 years, range 14–93 years). Four patients (15%) were current smokers, 15 patients (58%) never smoked and 7 patients (28%) were ex-smokers (they had stopped smoking at least 1 year before the diagnosis).

Presenting signs, symptoms and physical findings of the patients with BOOP are summarized in table 2. A persistent cough and dyspnea were the most common presenting symptoms and a flue-like illness with fever occurred in half of the subjects. Pleuritic chest pain was the most prominent symptom in the patients with large nodules or masses near the pleura. Half of the patients suffered from hemoptysis and pleuritic chest pain. Five patients (38%) with hemoptysis had focal BOOP and 4 of them had cavitating nodules. Hemoptysis was slight in 11 out of 13 patients (85%). Two patients had moderate hemoptysis (total 200 ml). There was no asymptomatic patient. The interval between the onset of the disease and the diagnosis of the patients was between 1 month and 7 years (mean 7 ± 16 months). Crackles were heard at a high rate (81%). Wheezes were present in 10 cases (38%). Clubbing was detected in only 5 patients (19%).

TAB02

Table 2. Symptoms and physical findings of patients with BOOP (n = 26)

goto top of outline Laboratory Findings

The erythrocyte sedimentation rate was elevated (>20 mm/h) in 25 patients (96%) and the leukocyte count was increased (>9,000/mm3) in half of the patients (table 3). Three patients (12%) had mild to moderate eosinophilia on initial presentation (eosinophil counts ≥1,000/mm3). Thrombocytosis was observed in 3 patients (12%). Two patients with SLE had moderate thrombocytopenia which was (<100,000/mm3). Seven patients (30%) had significant resting hypoxemia (PaO2 <60 mm Hg).

TAB03

Table 3. Laboratory data of 26 patients with BOOP

PFTs were available for 24 patients (table 3). They were within normal limits in 3 patients (13%), while 13 patients (54%) demonstrated obstructive or mixed ventilatory defects; of these, 5 (38%) never smoked and 8 patients (62%) had smoked >20 pack-years. Only 8 patients (30%) had isolated restrictive defects. Diffusing capacity for carbon monoxide was reduced (<80%) in 11 of 15 cases (73%).

BAL was performed in 25 patients. An increase in the percentage of lymphocytes was seen in 72% of 25 patients, an increase in the percentage of neutrophils in 11 patients (44%), and increase in the percentage of eosinophils in 3 patients (12%). The percentage of eosinophils exceeded that of lymphocytes in 1 patient (4%).

Pulmonary tissue was obtained by surgery in 7 patients (27%), TBLB in 12 patients (46%), CT guided TTLB in 7 patients (7%), and postmortem biopsy in 1 patient (4%).

goto top of outline Pathologic Findings

Polypoid masses of fibrous connective tissue consisted of plugs or buds of inflammatory cells, fibroblasts, and connective tissue filling respiratory bronchioles, alveolar ducts and alveoli. The most conspicuous cells were foamy and/or hemosiderin-laden alveolar macrophages occupying free alveolar airspaces in the patients with hemoptysis. Some neutrophils, eosinophils, lymphocytes and plasma cells were occasionally found within the airspaces. Rare multinucleated giant cells were found in 1 patient with idiopathic BOOP. In the interstitium, inflammatory cells (mainly lymphocytes and plasma cells) were found in all. Histologic features were identical for the idiopathic, secondary and focal BOOP groups.

goto top of outline Chest Roentgenographic Findings

Sixteen patients (62%) had a radiological presentation consistent with the typical pattern of alveolar and/or interstitial infiltrates (fig. 1, 2, 3) usually described for BOOP, and 10 patients (38%) had atypical radiological findings with focal pneumonic infiltration (fig. 4), which is rarely described in BOOP. Chest radiography, CT scan and HRCT findings are summarized in table 4. Distribution of the infiltrates on radiological investigations was more frequent in the lower zones.

FIG01

Fig. 1. Typical imaging pattern of organizing pneumonia with patchy alveolar opacities on chest radiography.

FIG02

Fig. 2. Typical imaging pattern of organizing pneumonia with patchy consolidation in the lower lobes containing air bronchograms on CT scan.

FIG03

Fig. 3. Diffuse infiltrative pattern of organizing pneumonia with interstitial and small alveolar opacities on CT scan.

FIG04

Fig. 4. Solitary focal mass with cavitation in the right upper lobe. This pattern may be diagnosed as lung cancer.

TAB04

Table 4. Chest Radiography, CT scan and HRCT findings in 26 patients with BOOP

Patterns of opacities were bilateral patchy alveolar infiltrates (fig. 1 and 2) in 7 patients (27%), interstitial infiltrates (reticulonodular opacities) in 4 patients (15%), and both alveolar and interstitial infiltrates (fig. 3) in 5 patients (19%). Solitary pulmonary infiltration was seen in 10 patients (38%). Half of them were located in the upper lobes and the other half in the lower lobes. Bilateral shadows were seen in 16 patients (62%). Migration of opacities was seen in 7 patients (27%) on serial roentgenograms. Follow-up radiographs were available in all.

Areas of ground glass attenuation were detected in 3/26 on HRCT scan, in which the lesion appeared to be minimal or focal on chest radiography. None had honeycomb changes. Ancillary findings identified on CT were an air bronchogram (fig. 2) in 15 patients (58%), mediastinal lymphadenopathy in 8 patients (31%), small pleural effusion in 9 patients (35%), pleural thickening in 7 patients (27%), unilateral hyperinflation in 5 patients (19%), cavitating nodules in 4 patients (15%), and small discrete nodules near the main lesion (satellite lesions) in 4 patients (15%).

goto top of outline Outcome

Treatment results are shown in table 5. Corticosteroid therapy was administered to 17 patients (65%) and 4 patients were followed up without corticosteroid therapy. Five patients (19%) had surgery (lobectomy, segmentectomy). Three patients (13%) improved spontaneously. One patient, an elderly male with focal BOOP, has been followed up without therapy. Clinical follow-up was available in all patients. Mean duration of follow-up was 22 ± 26 months (range: 1 month to 7.5 years).

TAB05

Table 5. Treatment and prognosis on 26 patients with BOOP

Prednisolone therapy was given orally; the initial dose varied from 40 to 100 mg/day in 17 patients, and resulted in complete clinical recovery with a normal chest film except few remaining shadows in 13 patients. Clinical improvement was seen within several days, and the response was dramatic in some cases. Only 3 patients (12%) had relapse when glucocorticoids were discontinued after 1–3 months. Two patients with drug-associated BOOP improved after cessation of their drugs and treatment with steroids.

Four patients died in the following period but only 1 death (4%) was related to BOOP, whereas the other 3 deaths were unrelated to BOOP. One patient with RA died due to acute respiratory failure within 1 month despite steroid treatment. She was severely ill and required invasive mechanical ventilation. Another patient demonstrated radiographic relapse of BOOP that was attributed to steroid intolerance. This patient, classified as idiopathic, died from the progression of BOOP. The other patient with idiopathic BOOP died from iatrogenic pneumothorax due to TBLB. One patient with non-Hodgkin lymphoma died due to sepsis and neutropenia after chemotherapy.

 

goto top of outline Discussion

BOOP may be classified into three categories according to its etiology: organizing pneumonia of determined cause; organizing pneumonia of undetermined cause but occurring in a specific and relevant context, and cryptogenic (idiopathic) organizing pneumonia [4]. The first two groups were classified as secondary BOOP in our series. More than half of the patients in our series were classified as having idiopathic. Some predisposing factors have been identified in the patients with secondary BOOP. Several possible causes and associated disorders coexisted in some patients in our series.

In a retrospective review of 74 patients with pathologically proven organizing pneumonia, Lohr et al. [8] found no distinguishing clinical, radiologic, or pathologic features between the patients with idiopathic and secondary BOOP. Secondary BOOP was observed in 42% of our patients. Their clinical profile and radiological patterns were similar to those observed in the patients with idiopathic BOOP. The present study findings were in agreement with the findings of Lohr et al. [8] and Cazzato et al. [9].

BOOP is commonly diagnosed during the fifth decade, although occasional cases have been reported in adolescents [10]. Only one of our patients with idiopathic BOOP was a 14-year-old girl. There was no correlation between smoking status and BOOP [3, 4]. Most patients in our series were nonsmokers or ex-smokers.

In the BOOP syndrome, three clinical patterns are recognized [11]. The first pattern is the most common and occurs in patients with a preceding upper respiratory tract infection (URTI) that proceeds to cough and progressive dyspnea after several weeks or months. The second pattern simulates idiopathic pulmonary fibrosis (IPF) but progresses over a shorter period. The third pattern develops rapidly with acute presentation [4, 7, 11]. The clinical findings of our patients were consistent with these patterns.

Pleuritic chest pain commonly associated with large nodules near the pleura and hemoptysis occurred in half of our patients. Both symptoms in our series were more frequent than previously reported [1, 8, 9, 12].

Hemoptysis is uncommon in BOOP [1, 4, 9, 12], and when it occurred, very small quantities of blood were generally expectorated [1, 7]. Blood-streaked sputum has been reported in patients with idiopathic BOOP [13], in 1 case associated with RA [14], a case of influenza-A-associated BOOP [15] and in a 70-year-old man with pulmonary nodular lesions [16]. Epler et al. [1] noted slight hemoptysis in only 1 of 50 patients. Hemoptysis was also described in 3 cases with solitary nodules out of 16 patients in the series of Cordier et al. [12], in only 1 of 78 patients in the series of Cazzato et al. [9], and in 10 of 70 patients in the series of Lohr et al. [8]. In addition to these studies, hemoptysis was reported in 3 cases with cavitating nodules out of 5 patients with solitary BOOP [17]. However, the quantity of blood was not described in these patients. Severe hemoptysis as the cardinal presenting manifestation of BOOP is extremely rare and has only been reported in 2 cases by Mroz et al. [18]. Two of our patients had moderate hemoptysis and the others had minimal hemoptysis. In summary, in the present series, in contrast to previous observations, hemoptysis was a relatively frequent symptom in BOOP [8, 9, 12].

Pleuritic chest pain is uncommon in patients with BOOP [1, 9, 12] although it was reported in up to 23% of 61 patients [8], 58% of 12 patients with pleurally based infiltration in the series of Spiteri et al. [19] and 50% of 12 patients with multiple large nodules near the pleura [20]. Chest pain was also reported in few patients including 3 with solitary BOOP out of 16 patients in the series of Cordier et al. [12] and 2% of 78 patients in the series of Cazzato et al. [9]. Pleuritic chest pain was more common when opacities where located in the subpleural region in the patients with BOOP. This interesting feature is one characteristic of multifocal organizing pneumonia which preferentially involve the subpleural regions [3, 19, 20]. Pleuritic chest pain in our series was more common than the other series [1, 8, 9, 12] and associated with pleurisy, pleural thickening and nodules near the pleura.

Although the imaging pattern of BOOP is heterogeneous, radiographic findings can suggest the diagnosis [4]. Plain chest radiography remains the first diagnostic approach to patients with BOOP but has limited diagnostic sensitivity and specificity as in the other diffuse infiltrative lung diseases [21]. The chest radiography varies depending on initial clinical manifestations [11]. Patients with a preceding URTI have patchy, mixed alveolar-interstitial infiltrates, often with a bilateral peripheral predominance and a migratory pattern [3, 4, 11, 12]. Our results confirm that bilateral alveolar and/or interstitial infiltrates are the most commonly seen radiographic findings. Patients presenting with a pattern similar to IPF have progressive lower lung zone infiltrates [3, 4, 11, 12]. Interstitial infiltrates were seen in 4 patients (14%) in our series. Patients with an acute fulminating presentation have diffuse and extensive alveolar infiltrates [3, 4, 7]. One patient had a fulminating variant of BOOP in our series.

BOOP may also be seen as a solitary lesion which usually occurs in the upper lobes and may cavitate [12]. It may be seen with both secondary and cryptogenic BOOP and resection results in a cure [4]. Focal solitary BOOP was seen in 36% of our patients, similar to the series of Cordier et al. [12], Cazzato et al. [9] and Lohr et al. [8].

HRCT is actually the recommended imaging technique in the diagnosis, assessment and follow-up of BOOP; it also allows the evaluation of treatment effectiveness and the selection of the type and the location of the biopsy when required, as in the other diffuse infiltrative lung diseases [21]. In agreement with Cazzato et al. [9], HRCT scans were more sensitive in detecting ground glass infiltrates, subpleural distribution, nodules with/without cavitation in our series. In the series of Müller et al. [22], all 14 patients had areas of air-space consolidation, small nodules, or both on CT. Bouchardy et al. [23] found focal nodular opacities in 5 patients (42%), and areas of consolidation with air bronchograms in 4 patients (33%). Chest imaging findings in our series were similar to the previously reported data [22, 23].

Less common radiological findings in patients with BOOP include mediastinal lymphadenopathy, pleural effusion, or pleural thickening. Pleural effusion is generally uncommon, although it has been reported in 22% of 38 patients [24] and 27% of 14 patients [22]. In two other studies, small pleurisy and pleural thickening were respectively reported in 13 and 8% [25], and in 17 and 33% [20] on CT scan. Pleural effusion was also present in 5% of 78 patients in the series of Cazzato et al. [9], and in 5% of 42 patients in the series of Epler et al. [1]. Pleurisy and pleural thickening were more frequent in our series (35 and 27%, respectively) than that reported by others [1, 9].

There are no specific laboratory findings in BOOP. The ESR and C-reactive protein levels are increased [4, 8, 26]. An ESR >20 mm/h was seen in 96%, and leukocytosis >9,000/ml in 50% of the patients at presentation. Thrombocytosis was observed in 3 patients (12%); however, none of these laboratory data help to diagnose BOOP.

The most common finding on PFT in patients with BOOP is a mild or moderate restrictive ventilatory defect. Airflow obstruction may be present in smokers but is not a characteristic of BOOP [3, 4]. Fifty-four percent of our patients demonstrated obstructive or mixed ventilatory defects; of these, 5 patients (38%) had never smoked. The mixed or obstructive physiology in nonsmokers was also reported previously and included patients with BOOP and cancer [27]. The presence of airflow obstruction without smoking in our patients may be due to narrowed rather than occluded bronchioles and therefore participating in expiratory airflow in some patients. Additionally, spirometry and diffusing capacity are useful to monitor the response of patients to therapy as in the other diffuse infiltrative lung diseases [28]. Mild hypoxemia at rest and/or on exercise is common and severe hypoxemia may reflect severe pulmonary disease or shunting in more limited lesions of BOOP [3, 4].

BAL findings in BOOP have been characterized previously. An increase in all cell types is usually found, lymphocytes being the most prevalent [4, 29, 30]. BAL was used to exclude other disorders or causes of BOOP, particularly infections in our series.

In the present study, the pathologic diagnosis of BOOP was made by using TBLB and TTLB in most cases. Although an open lung biopsy is the best way to obtain a representative lung specimen, TBLB may be sufficient unless the clinical features, including radiographic findings and the response to antibiotics or steroids, are not consistent with BOOP [9, 30, 31]. The use of TBLB and TTLB for diagnosing focal BOOP is controversial, because BOOP may exist along with a malignant neoplasm. TBLB for cryptogenic BOOP was reported by Poletti et al. [30] to have a sensitivity of 64% and a specificity of 86%, although the number of patients with focal disease in that study was not specified. Focal BOOP was diagnosed by TTLB and/or TTLB in 4 cases in the present study.

BOOP constitutes a distinct part of the spectrum of diffuse infiltrative lung diseases. This disorder initially needed to be distinguished from usual interstitial pneumonia (UIP) or IPF if pathologists interpret extensive intra-airway organization as interstitial fibrosis although distinct pathologic and clinic findings are made in the two diseases. BOOP has a better prognosis and often responds well to steroids [4, 25, 32]. Five of our cases were initially diagnosed as UIP by pathologists based on TBLB alone. All 5 cases later underwent open lung biopsy or TTLB to exclude UIP, and improved completely with steroid therapy. The mixed or restrictive physiology, no honey-comb pattern, and response to treatment were consistent with the diagnosis of BOOP in our cases.

BOOP is characterized by an indolent course and good prognosis. Untreated BOOP tends to progress gradually over weeks, although rapid deterioration [7] and spontaneous remissions have been described [3, 4, 6]. In our series, 3 patients improved spontaneously and only 1 death (4%) was due to progressive BOOP, whereas the other 3 deaths were unrelated to BOOP. Cohen et al. [7], have demonstrated that the prognosis is poor when BOOP is associated with a chronic disease or with exposure to drugs. Respiratory mortality in patients with idiopathic and secondary BOOP were the same in our series.

Although BOOP responds well to corticosteroids, the effective dose is not established yet. The minimal dose for a minimal period is recommended for steroid treatment [6, 26]. Steroid therapy was given orally, the initial dose varied from 40 to 100 mg/day prednisolone for 3–6 months.

Three patients relapsed when corticosteroids were decreased to low-dose within 1 year after the initial episode similar to study of Lazor et al. [6]. Two of them had idiopathic and one had secondary BOOP. The frequency of relapses in our series was evidently lower than in the other studies [6, 33]. There is no apparent explanation for this observation.

In conclusion, BOOP is usually idiopathic. We observed that hemoptysis and pleuritic chest pain due to pleural involvement were a relatively frequent symptom in BOOP in the present series, in contrast to previous observations. The relapse rate among the patients in our series was lower than in previous studies. The other findings containing clinical symptoms, chest imaging findings, diagnostic methods, BAL results, and prognosis were the same as previous observations.

The diversity of radiological and clinical presentations including hemoptysis and pleuritic chest pain should prompt consideration of the diagnosis in patients with persisting pulmonary symptoms and radiological findings. In view of the benign course and therapeutic response, a histologic diagnosis is important.


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  25. Chandler PW, Shin MS, Friedman SE, Friedman SE, Katzenstein A-L: Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR 1986;147:899–906.
  26. Izumi T, Kitaichi M, Nishimura K, Nagai S: Bronchiolitis obliterans organizing pneumonia. Clinical features and differential diagnosis. Chest 1992;102:715–719.
  27. Mokhtari M, Bach PB, Tietjen PA, Stover DE: Bronchiolitis obliterans organizing pneumonia in cancer: A case series. Respir Med 2002;96:280–286.
  28. Chetta A, Marangio E, Olivieri D: Pulmonary function testing in interstitial lung diseases. Respiration 2004;71:209–213.
  29. Costabel U, Teschler H, Guzman J: Bronchiolitis obliterans organizing pneumonia (BOOP): The cytological and immunocytological profile of bronchoalveolar lavage. Eur Respir J 1992;5:791–797.
  30. Poletti V, Castrilli G, Romagna M, Colaste A, Aiello FB, Baruzzi G, Musiani P: Bronchoalveolar lavage, histological and immunohistochemical features in cryptogenic organizing pneumonia. Monaldi Arch Chest Dis 1996;51:289–295.
  31. Bartter T, Irwin RS, Nash G, Balikian JP, Hollingsworth HH: Idiopathic bronchiolitis obliterans organizing pneumonia with peripheral infiltrates on chest roentgenogram. Arch Intern Med 1989;149:273–279.
  32. Muller NL, Guerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC: Differential diagnosis of BOOP and usual interstitial pneumonia: Clinical, functional, and radiologic findings. Radiology 1987;162:151–156.
  33. Watanabe K, Senju S, Wen FQ: Factors related to the relapse of bronchiolitis obliterans organizing pneumonia. Chest 1998;114:1599–1606.

 goto top of outline Author Contacts

Assistant Professor F. Sema Oymak
Erciyes University Medical Faculty
Department of Chest Disease
TR–38039 Talas/Kayseri (Turkey)
Tel. +90 352 4374937/21906, Fax +90 352 4375807, E-Mail fsoymak@yahoo.com


 goto top of outline Article Information

Received: August 20, 2003
Accepted after revision: August 26, 2004
Number of Print Pages : 9
Number of Figures : 4, Number of Tables : 5, Number of References : 33


 goto top of outline Publication Details

Respiration (International Journal of Thoracic Medicine)

Vol. 72, No. 3, Year 2005 (Cover Date: May-June 2005)

Journal Editor: C.T. Bolliger, Cape Town
ISSN: 0025–7931 (print), 1423–0356 (Online)

For additional information: http://www.karger.com/res


Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Abstract

Background: Bronchiolitis obliterans organizing pneumonia (BOOP) may be classified as cryptogenic (idiopathic) and secondary. There are no clear clinical and radiological features distinguishing between idiopathic and secondary BOOP. Objectives: To analyze the etiologic factors, clinical and radiological features, diagnostic approach and response to therapy at onset and outcome in subjects with BOOP. Methods: The medical files of Erciyes University Hospital from 1995 to 2003 were retrospectively reviewed. Patients with biopsy-proven BOOP were selected for evaluation. The etiology and initial features of BOOP, treatment, resolution, relapse, and survival were obtained from medical records, and a follow-up patient questionnaire. Results: We have diagnosed 26 cases (13 males /13 females) with BOOP syndrome (mean age 54 ± 15 years, range 14–93). More than half the patients (58%) were classified as idiopathic BOOP. Patients presented with cough (92%), dyspnea (70%), pleuritic chest pain, hemoptysis and fever (50%). The biopsy specimens had been obtained by transbronchial and/or transthoracic lung biopsy in 18 cases (69%). At radiological evaluation, there were bilateral patchy alveolar and/or interstitial infiltrates in 16 patients (62%), and solitary pneumonic involvement in 10 patients (38%). Three patients recovered spontaneously, 5 remained cured after resection of the focal lesion. Corticosteroid therapy was given in 17 patients (65%). Apart from four patients who died (death was attributable to BOOP in only 1 patient) and three patients who relapsed, the prognosis was good in all patients. Conclusions: The etiology of BOOP is usually idiopathic. We observed that hemoptysis and pleuritic chest pain were a relatively frequent symptom in BOOP in the present series, in contrast to previous observations. The diversity of radiological and clinical presentations including hemotysis and pleuritic chest pain should prompt consideration of the diagnosis in patients with persisting pulmonary symptoms and radiological findings.



 goto top of outline Author Contacts

Assistant Professor F. Sema Oymak
Erciyes University Medical Faculty
Department of Chest Disease
TR–38039 Talas/Kayseri (Turkey)
Tel. +90 352 4374937/21906, Fax +90 352 4375807, E-Mail fsoymak@yahoo.com


 goto top of outline Article Information

Received: August 20, 2003
Accepted after revision: August 26, 2004
Number of Print Pages : 9
Number of Figures : 4, Number of Tables : 5, Number of References : 33


 goto top of outline Publication Details

Respiration (International Journal of Thoracic Medicine)

Vol. 72, No. 3, Year 2005 (Cover Date: May-June 2005)

Journal Editor: C.T. Bolliger, Cape Town
ISSN: 0025–7931 (print), 1423–0356 (Online)

For additional information: http://www.karger.com/res


Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

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    External Resources

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  27. Mokhtari M, Bach PB, Tietjen PA, Stover DE: Bronchiolitis obliterans organizing pneumonia in cancer: A case series. Respir Med 2002;96:280–286.
  28. Chetta A, Marangio E, Olivieri D: Pulmonary function testing in interstitial lung diseases. Respiration 2004;71:209–213.
  29. Costabel U, Teschler H, Guzman J: Bronchiolitis obliterans organizing pneumonia (BOOP): The cytological and immunocytological profile of bronchoalveolar lavage. Eur Respir J 1992;5:791–797.
  30. Poletti V, Castrilli G, Romagna M, Colaste A, Aiello FB, Baruzzi G, Musiani P: Bronchoalveolar lavage, histological and immunohistochemical features in cryptogenic organizing pneumonia. Monaldi Arch Chest Dis 1996;51:289–295.
  31. Bartter T, Irwin RS, Nash G, Balikian JP, Hollingsworth HH: Idiopathic bronchiolitis obliterans organizing pneumonia with peripheral infiltrates on chest roentgenogram. Arch Intern Med 1989;149:273–279.
  32. Muller NL, Guerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC: Differential diagnosis of BOOP and usual interstitial pneumonia: Clinical, functional, and radiologic findings. Radiology 1987;162:151–156.
  33. Watanabe K, Senju S, Wen FQ: Factors related to the relapse of bronchiolitis obliterans organizing pneumonia. Chest 1998;114:1599–1606.