Bronchiolitis obliterans organizing pneumonia in common variable immunodeficiency syndrome

A 26-year-old woman with common variable immunodeficiency syndrome (CVID) associated with frequent episodes of pneumonia underwent an open lung biopsy showing bronchiolitis obliterans organizing pneumonia (BOOP). Following corticosteroid therapy, there were no further episodes of pneumonia. In addition, there was roentgenographic and gas exchange improvement. This is the first case of BOOP in association with CVID. An immunologic basis of BOOP is postulated.     

Bronchiolitis obliterans organizing pneumonia

Bronchiolitis obliterans with organizing pneumonia (BOOP) is a pathological syndrome common to a variety of pulmonary inflammatory disorders. It is defined by the presence of buds of granulation tissue consisting of fibroblasts and collagen within the lumen of the distal airspaces. BOOP may be secondary to lung injury resulting especially from infection or drug toxicity or may develop in the context of connective tissue diseases or after lung or bone marrow transplantation. BOOP may also be idiopathic (and then preferentially called cryptogenic organizing pneumonia) and then is the hallmark of a distinct clinicoradiological syndrome of subacute pneumonia, with typical alveolar patchy and often migratory pulmonary opacities on imaging. Other imaging presentations consist of diffuse infiltrative opacities or focal pneumonia. Improvement with corticosteroids is usually spectacular, but relapses are common after stopping or while reducing treatment.     

A newly recognized syndrome--radiation-related bronchiolitis obliterans and organizing pneumonia. A case report and literature review.

Bronchiolitis obliterans and organizing pneumonia (BOOP) is a syndrome that has been associated with a variety of underlying disorders, including infection, collagen vascular diseases and toxic fume inhalation. Rarely, however, BOOP has been associated with radiation- or chemotherapy-induced pulmonary toxicity. Over the past 3 years, several case series have reported BOOP in the unique setting of radiation in breast cancer patients. This study describes our experience with this newly recognized syndrome and a review of the English-language literature on this syndrome.     

Influenza A-associated bronchiolitis obliterans organizing pneumonia mimicking Wegener's granulomatosis

We describe a patient with bronchiolitis obliterans organizing pneumonia (BOOP) requiring respiratory support and treated with corticosteroids and cytoxan for presumed Wegener's granulomatosis (WG). The diagnosis of WG was based on clinical presentation and strongly positive stains for anti-neutrophilic cytoplasmic antibodies (cANCA). The results of an open-lung biopsy were consistent with BOOP. Although BOOP has previously been described as one of the pulmonary manifestations of WG, other more specific histologic features of WG such as capillaritis or necrotizing vasculitis were lacking. Because influenza A virus was cultured from the patient's lung tissue, final assessment of the illness focused on this as the etiologic agent triggering the pulmonary syndrome. The presence of ANCA was considered to be nonspecific. The patient's condition improved with appropriate therapy for BOOP.     

A case of primary Sj?gren's syndrome with interstitial pneumonia showing bronchiolitis obliterans organizing pneumonia pattern and lymphofollicular formation]

A 46-year-old woman with a 2-year history of xerostomia, who had had an episode of suspected Sj?gren's syndrome in 1995, was admitted to our hospital because of a dry cough. Chest radiography on admission showed ground-glass infiltrates and reticular shadows in both lower lung fields. Primary Sj?gren's syndrome was diagnosed by lip biopsy. Video-assisted thoracoscopic lung biopsies revealed the co-existence of interstitial pneumonia with the BOOP pattern and follicular bronchiolitis. Treatment with oral prednisolone improved the symptoms, and reduced the abnormal chest shadows. This was an interesting case of the BOOP pattern in a lung lesion associated with primary Sj?gren's syndrome.     

Graft versus host-associated pulmonary disease and other idiopathic pulmonary complications after hematopoietic stem cell transplant

Noninfectious conditions are now the major pulmonary causes of morbidity and mortality after hematopoietic stem cell transplantation (HSCT). The idiopathic pneumonia syndrome (IPS) remains one of the more common and serious pulmonary complications within months after transplantation. Epidemiological data suggest that, although graft versus host disease (GVHD) reactions may play an etiological role, the major contributing factor is conditioning-related toxicity. Among lung conditions that are more closely associated with GVHD are both obliterative bronchiolitis (usually occurring months to years after HSCT) and bronchiolitis obliterans organizing pneumonia (BOOP). The former is an inexorably progressive condition, whereas BOOP behaves similarly to idiopathic BOOP seen in other populations. Also addressed in this article are engraftment syndrome, diffuse alveolar hemorrhage, and pulmonary veno-occlusive disease. Knowledge of these complications is now a part of the contemporary practice of pulmonary medicine, no longer isolated to the transplant pulmonologist.     

Clinicopathologic study of various lung diseases with bronchiolitis obliterans organizing pneumonia (BOOP) pattern in open lung biopsy]

The histologic findings of BOOP are nonspecific, and the diagnosis of idiopathic BOOP is one of exclusion. We studied the clinicopathologic features of various lung diseases with histopathological appearance of bronchiolitis obliterans organizing pneumonia (BOOP) pattern in open lung biopsy specimens. The 17 patients with BOOP pattern studied included idiopathic BOOP (n = 7), unclassified interstitial pneumonia (n = 1), collagen vascular disease (n = 3, RA 1, PM/DM 2), hypersensitivity pneumonitis (HP, n = 2), eosinophilic pneumonia (EP, n = 1), multiple lung abscesses (n = 1), limited form of Wegener's granulomatosis (n = 1), and pneumocystis carinii pneumonia associated with adult T cell leukemia (n = 1). There were no differences in clinical symptoms, laboratory data, respiratory function, and cytological findings in bronchoalveolar lavage fluid (BALF) between cases of idiopathic BOOP and other lung diseases. The duration of clinical symptoms was less than one year in 16 patients (one case had no symptoms). Chest X-rays showed bilateral patchy, nodular, or reticular shadows in all cases. Multiple patchy migratory shadows were only observed in cases of idiopathic BOOP or EP. Organizing pneumonia was recognized in the transbronchial lung biopsy specimens of all patients with idiopathic BOOP, HP or EP. Regarding prognosis, relapses occurred in idiopathic BOOP (n = 5), polymyositis, EP, limited form of Wegener's granulomatosis, and Pneumocystis carinii pneumonia. Four patients with idiopathic BOOP relapsed when steroid therapy was decreased or stopped. These findings indicate that idiopathic BOOP should be differentiated from other lung diseases, and these patients should be followed for a long period of time.     

Secondary bronchiolitis obliterans organizing pneumonia with primary Sj?gren's syndrome, resulting in acute respiratory distress syndrome: a case report]

A 71-year-old female who was taking 10 mg/day of prednisolone for Sj?gren's syndrome was admitted because of fever and dyspnea with multiple infiltrative shadows on chest radiography and computed tomography (CT), although she had been discharged only 4 days before. On the 1st and 2nd admissions, a BOOP pattern had been suspected, and she was treated by tapering the prednisolone dose from 40 mg/day to 10 mg/day, which resulted in the disappearance of the infiltrative lung shadows. This time we confirmed the BOOP pattern with Sj?gren's syndrome, because bronchoalveolar lavage showed an increase of total cells, with a high lymphocyte fraction, and a transbronchial lung biopsy revealed loose fibroblastic plugs in some alveolar ducts and alveoli. Also, there were intra-alveolar accumulations of foamy macrophages. Furthermore, we noticed migration of pulmonary opacity. Although the clinical symptoms of the patient improved, the response to the prednisolone therapy appeared to be poor. At 35 mg of prednisolone (which had been initiated at 40 mg/day), the disease became rapidly exacerbated by a common cold, and developed into ARDS on the 30th hospital day. In spite of intensive care, the patient died. Here we report a rare case in which the BOOP pattern based on Sj?gren's syndrome resulted in ARDS. In general, prednisolone is effective against the BOOP pattern, but we need to be aware of the possibility of a poor response to this BOOP pattern in Sj?gren's syndrome.     

Air leak syndrome as one of the manifestations of bronchiolitis obliterans organizing pneumonia

A 46-year-old man developed respiratory distress with air leak syndrome (ALS), including pneumothorax, pneumomediastinum, and subcutaneous emphysema. Open lung biopsy was performed and revealed the histopathologic evidence of bronchiolitis obliterans organizing pneumonia (BOOP), which responded well to steroid treatment. As far as we know, this appears to be the first case of BOOP presenting with ALS as one of its major complications.     

Recurrent bronchiolitis obliterans organizing pneumonia in a patient with limited cutaneous systemic sclerosis

Systemic sclerosis (SSc) is a generalized disorder characterized by fibrosis and vascular obliteration in the skin, lung, gastrointestinal tract, and kidney. One of its two subsets is a stable, limited cutaneous group (lSSc). Pulmonary involvement in scleroderma is common, and several types of pulmonary disorders are associated with SSc. Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare finding in lung disorders associated with SSc. We describe a case of lSSc with BOOP that was responsive to steroid therapy. Of interest is that the lung disorders appeared in different periods and areas. It might be important to diagnose abnormal shadows in lung fields before treatment of patients with SSc.     

Bronchiolitis obliterans organizing pneumonia. Clinical and roentgenological features in 26 cases

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.     

Bronchiolitis obliterans organizing pneumonia associated with polymyalgia rheumatica.

The association of bronchiolitis obliterans organizing pneumonia (BOOP) with polymyalgia rheumatica is rare, and only one case has previously been described. This study reports on the case of an 80 yr-old male who presented with malaise, nonproductive cough and exertional dyspnoea for several weeks, along with a history of bilateral shoulder and pelvic girdle pain of several months' duration. The chest radiograph revealed a pneumonic infiltrate in the right lower lobe, which was unresponsive to antibiotics. Bronchoscopy, bronchoalveolar lavage and a transbronchial lung biopsy established the diagnosis of BOOP. The patient improved consistently on steroids. As in other connective diseases, organizing pneumonia may be one of the early manifestations of polymyalgia rheumatica.     

Bronchiolitis obliterans organizing pneumonia in cancer: a case series

Bronchiolitis obliterans with organizing pneumonia (BOOP) is an infrequently encountered clinical condition that can mimic a number of other pathologic lung processes. The presentation of this treatable condition in cancer patients has not been described in any large series.We conducted a retrospective study of patients with BOOP at Memorial Sloan-Kettering Cancer Center, NewYork, NY, U.S.A. from January 1992 to December 1999. The type and treatment of primary cancer, clinical and radiographic features of initial BOOP presentation, and outcome following therapy were recorded. Forty-three patients with an underlying diagnosis of cancer were found on lung biopsy to have BOOP as an isolated entity. BOOP was encountered in patients with a variety of clinical presentations, and many types of malignancies. The symptom patterns were non-specific, as were the physiological abnormalities. The only clear relationship between the underlying malignancyand the diagnosis of BOOP at presentation was in the chest radiographic findings. Patients with solid organ tumors were more likely to have nodular or mass like radiographic abnormalities (81%) than to have diffuse infiltrates (19%).We observed the opposite pattern in patients with hematologic malignancies (22% vs.67%). The vast majority of patients recovered from this condition. In conclusion, For cancer patients, BOOP represents a treatable cause of lung disease with protean manifestations. BOOP can mimic pulmonary malignancy and pulmonary infection. In cancer patients, the evaluation of new pulmonary symptoms accompanied by radiographic changes should include a consideration of this diagnosis.     

Bronchiolitis obliterans organizing pneumonia and bronchogenic carcinoma coexisting in different parts of the lungs

Bronchiolitis obliterans organizing pneumonia (BOOP) coexisting with nonsmall cell lung cancer in a separate part of the lungs is rare. We report the case of a man diagnosed with BOOP by video-assisted thoracoscopic lung biopsy; the patient also had adenocarcinoma in a different part of the lungs. The BOOP was treated with corticosteroids and the carcinoma was surgically resected. Outcome and clinical course were good after both procedures. The possibility of BOOP associated with bronchogenic carcinoma at a distant part of the lungs, although rare, must be taken into account in the differential diagnosis of radiographic alterations that may present in either of the 2 diseases.     

Lesion with morphologic feature of organizing pneumonia (OP) in CT-guided lung biopsy samples for diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP): a retrospective study of 134 cases in a single center

Background

Small biopsy samples are generally considered inconclusive for bronchiolitis obliterans organizing pneumonia (BOOP) diagnosis despite their potential to reveal organizing pneumonia (OP) pathologically, necessitating risky invasive tissue biopsy during surgery for reliable confirmation.

Objective

OP by CT-guided lung biopsy was to evaluate the role in the diagnosis of BOOP.

Methods

A retrospective review of 134 cases with the OP feature in the CT-guided lung biopsy samples between 2004 and 2011 at a single center was conducted. Diagnostic accuracy of OP by CT-guided lung biopsy and clinical-radiographic data alone were compared.

Results

After exclusion of 11 cases due to pathology with others besides OP and 15 cases for loss to follow-up, 108 were included. Of these, 95 cases and 13 cases were classified as BOOP and non-BOOP group, respectively. Among BOOP group, only 30 were initially diagnosed as BOOP according to the typical clinical and radiographic features. The other 65 cases with atypical features were diagnosed as BOOP mainly based on OP by CT-guided lung biopsy. Among non-BOOP group, one was misdiagnosed as BOOP, and others were not BOOP according to clinical and radiographic findings. Thus, OP by CT-guided lung biopsy produced a diagnostic accuracy of 87.96% (95/108), much higher than 31.25% (30/96) observed using clinical and radiographic data alone. Combined, these techniques produced diagnostic accuracy of 98.96% (95/96).

Conclusions

OP by CT-guided lung biopsy can be effectively used as the pathological evidence for BOOP diagnosis and reducing unnecessary surgery.     

Bronchiolitis obliterans organizing pneumonia as a complication of allogeneic bone marrow transplantation.

We report a patient who underwent two allogeneic bone marrow transplants for chronic myelogenous leukemia, initially in 1984 and again after relapse in 1990, who developed an identical pulmonary syndrome at a similar interval following each transplant. The patient presented with a non-productive cough, bilateral inspiratory crackles, and multiple patchy infiltrates on chest X-ray. Pulmonary function testing revealed a restrictive abnormality but no obstructive defects. The appearance of this pulmonary disorder after each transplant coincided with the development of chronic graft-versus-host disease. In both instances, this pulmonary syndrome completely reversed with corticosteroid therapy. The patient's chest computed tomographic scan and lung biopsy specimens were consistent with the diagnosis of bronchiolitis obliterans with organizing pneumonia (BOOP). While bronchiolitis obliterans has been reported following allogeneic transplant, BOOP has not previously been reported in this setting.     

Bronchiolitis obliterans organizing pneumonia (BOOP): the cytological and immunocytological profile of bronchoalveolar lavage.

The cytological and immunocytological profile of bronchoalveolar lavage (BAL) was studied in 10 patients with idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) and compared with the data in idiopathic pulmonary fibrosis (IPF) (n = 22), chronic eosinophilic pneumonia (CEP) (n = 9), and extrinsic allergic alveolitis (EAA) (n = 24). Lymphocyte subsets were enumerated using an immunoperoxidase slide assay. The BAL pattern in BOOP patients was characterized by several features: 1) colorful cell differentials with an increase in all cell types, most markedly in lymphocytes, and more moderately in neutrophils, eosinophils and mast cells, as well as the presence of foamy macrophages and, occasionally, of plasma cells; 2) decreased CD4/CD8 ratio; 3) normal percentage of CD57+ cells; and 4) increase in activated T-cells in terms of human leucocyte antigen-DR (HLA-DR) expression, and occasionally also interleukin-2 receptor (CD25) expression. The findings were most similar to those in EAA except for the CD25 expression, which was always normal, and the CD57+ cells, which were increased in EAA. The increase in lymphocytes discriminated best between BOOP and IPF. The eosinophils were significantly higher in CEP than in BOOP with little overlap. In conclusion, BAL may be of value to distinguish between BOOP and other interstitial lung disease.     

Idiopathic bronchiolitis obliterans organizing pneumonia. Definition of characteristic clinical profiles in a series of 16 patients

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic finding common to various injuries to the lung of either definite or idiopathic etiology. Since the presentation of patients with idiopathic BOOP varies, we studied 16 patients with BOOP on pulmonary histology to define more distinct and homogeneous clinical and imaging profiles of idiopathic BOOP. We distinguished three groups of patients: group 1 (n = 4), with multiple patchy migratory pulmonary involvement of the pneumonia type. Their clinical course was subacute, with cough, fever, weight loss, mild dyspnea, and increased ESR. Chest x-ray film and CT scan showed multiple alveolar opacities. All patients completely recovered with corticosteroid therapy but relapsed when therapy was stopped too rapidly. Group 2 (n = 5) had solitary pulmonary involvement of the pneumonia type occurring in a similar clinical context. Since carcinoma was suspected, they underwent surgical excision of the pneumonic area and recovered without relapse. Group 3 patients (n = 7) presented with diffuse pulmonary involvement of the interstitial lung disease type. They had more progressive onset of more severe dyspnea, crackles heard over all lung surfaces, and interstitial opacities with or without alveolar opacities on chest imaging. Improvement with corticosteroid therapy was obtained in only three patients. In all three groups, lung function test results showed a restrictive pattern. The obstructive pattern characteristic of pure bronchiolitis obliterans was found in none. BAL showed a mixed pattern (increase of both lymphocytes and polymorphonuclear cells) in the patients of the first two groups. Thus, we distinguished three characteristic clinical and imaging profiles in patients with idiopathic BOOP: multiple patchy pneumonia, solitary pneumonia, and diffuse interstitial lung disease. These profiles are so different that they should be distinguished in clinical studies of idiopathic BOOP.     

Bronchiolitis obliterans organizing pneumonia associated with essential mixed cryoglobulinemia

Bronchiolitis obliterans organizing pneumonia (BOOP) is an uncommon and underrecognized pathologic finding observed in a variety of syndromes including connective tissue diseases. Although lung involvement in essential mixed cryoglobulinemia (EMC) has been reported, there are no previous reports of BOOP in EMC. We report herein a case of longstanding EMC associated with interstitial and cavitary lung disease histologically consistent with BOOP. A theory of a possible etiologic basis for the development of BOOP in patients with connective tissue diseases is discussed.     

Idiopathic bronchiolitis obliterans organizing pneumonia with peripheral infiltrates on chest roentgenogram

We report five patients with chest roentgenograms showing peripheral infiltrates similar to that described for chronic eosinophilic pneumonia (CEP). While lung biopsy specimens (transbronchial and open in two and transbronchial only in three cases) revealed typical changes of bronchiolitis obliterans organizing pneumonia (BOOP) as the predominant finding in all cases, two cases had changes consistent with a resolving CEP. We speculate that (1) it may be common for BOOP to present with peripheral infiltrates, (2) chest roentgenograms showing peripheral infiltrates are not diagnostic of any specific entity, (3) idiopathic BOOP may represent the evolution of untreated CEP, (4) a lung biopsy may be a more appropriate initial approach than the therapeutic trial of corticosteroids when peripheral infiltrates of unknown origin are present, and (5) transbronchial biopsy may be adequate to establish a working clinical diagnosis of BOOP.