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PubMed2004

Cryptogenic organizing pneumonia.

Cryptogenic organizing pneumonia is a rare, distinct disorder that is sufficiently different from the other diseases in the group of idiopathic interstitial pneumonias to be designated as a separate entity. In its most typical presentation, it is characterized by dyspnea and cough, with multiple patchy alveolar opacities on pulmonary imaging. Definite diagnosis is obtained by the finding of buds of granulation tissue in the distal airspaces at lung biopsy. No cause (as infection, drug reaction, or associated disease as connective tissue disease) is found. Corticosteroid treatment is rapidly effective, but relapses are common on reducing or stopping treatment.

Cryptogenic Organizing Pneumonia↗
PubMedDate not supplied

[Cryptogenic organizing pneumonia -- case report].

Cryptogenic organizing pneumonia (COP) is one of the new recognized histological patterns of idiopathic interstitial pneumonias, in witch no cause or association is identified. Idiopathic bronchiolitis obliterans-organizing pneumonia (BOOP) is a synonymous. This rare entity is characterized by involvement of alveoli and bronchioles, which are filled by intraluminal polyps of fibroblastic tissue (Masson bodies). The clinical presentation often mimics that of community-acquired pneumonia. Persistent non-productive cough, dyspnoea with exertion and weight loss are common features, and in one-half of the cases the onset is heralded by a flu-like syndrome. Definitive diagnosis depends on histological data, and video-assisted thoracoscopic (VAT) has become the established technique. Spontaneous recovery is rare; corticosteroid therapy provides cure in two thirds of cases. The authors present a case of a patient whose lung biopsy by VAT confirmed the diagnosis. She was treated with corticoids without recurrence in a two year follow-up.

Cryptogenic Organizing Pneumonia↗
PubMed2001

Cryptogenic organizing pneumonia.

The terms cryptogenic organizing pneumonia (COP) and idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP) are synonymous. Typical COP should be viewed as a clinicopathological syndrome, consisting of respiratory and systemic symptoms (usually low-grade), patchy consolidation on chest radiography and computed tomography, a restrictive defect on pulmonary function testing, and the presence of buds of granulation tissue within the distal airways and alveolar spaces. In the correct clinical context, the diagnosis can be secured by transbronchial biopsy, with typical bronchoalveolar lavage features providing useful diagnostic support. In typical COP, corticosteroid therapy is highly effective; an early trial of withdrawal of treatment is appropriate because relapse of COP is not associated with a poor long-term outcome. Atypical forms of COP are outlined in this article, including focal, explosive, progressive fibrotic, and nonprogressive fibrotic variants. The occasional evolution of COP into a progressive fibrotic disorder is associated with a poor long-term outcome and poses particular therapeutic difficulties.

Journal Article↗
PubMed2003

Organizing pneumonia: cryptogenic and disease associated.

Organizing pneumonia is defined by the presence of buds of granulation tissue (fibroblastic cells and connective matrix) within the alveolar lumen, usually associated with proliferative bronchiolitis obliterans. It may result from several causes, but cryptogenic organizing pneumonia is a distinct clinicopathologic entity with characteristic clinical and imaging features. Typical COP is thus easily recognized by the clinician, and the diagnosis is made by the finding of organizing pneumonia on lung biopsy specimen. The response to corticosteroids is rapid and complete usually without sequelae, but relapses occur frequently and prolonged corticosteroid treatment is often necessary.

Journal Article↗
PubMedDate not supplied

A case of cryptogenic organizing pneumonia occurring in Crohn's disease.

A 29 year-old-man with Crohn's disease, who developed diffuse pulmonary infiltrates and hypoxemia two months following oral administration of mesalazine, was examined. Clinical findings and computed tomography were suggestive of, and lung histology was diagnostic of, bronchiolitis obliterans organizing pneumonia, also known as cryptogenic organizing pneumonia. Although the data did not allow for definitive conclusions, they did suggest that the pulmonary disease was an extraintestinal manifestation of Crohn's disease, rather than an adverse reaction to mesalazine. In fact, the patient showed clinical, radiological and functional improvements, despite the treatment with mesalazine and the withdrawal of steroid therapy.

Adult↗
PubMed1993

The histological diagnosis of clinically documented cases of cryptogenic organizing pneumonia: diagnostic features in transbronchial biopsies.

Eleven cases of clinically diagnosed cryptogenic organizing pneumonia were examined in order to establish the histological features found at transbronchial biopsy and to correlate this with open lung biopsy which followed in six cases. The essential pathological feature was the presence of buds of granulation tissue (Masson bodies) indicating organization of a persistent exudate by fibroblasts and capillaries within alveoli, with preservation of the alveolar architecture. In addition, both acute and chronic inflammatory cells were present in the interstitium. These features, combined with the clinical history, were sufficient for a diagnosis in seven of the 11 cases on transbronchial biopsies. Four biopsies lacked these features. Patients proceeded to open lung biopsy in addition to the two with histological features of cryptogenic organizing pneumonia on transbronchial biopsy, but where the clinician wanted to eliminate other pathology because of rapid clinical deterioration. Five of the six cases coming to open lung biopsy confirmed cryptogenic organizing pneumonia with Masson bodies within alveoli, but changes were focal in three with very few Masson bodies in one. One case which had the features on transbronchial biopsy lacked them in the open lung biopsy. Transbronchial biopsy, therefore, can yield diagnostic material in the majority of patients with cryptogenic organizing pneumonia while open lung biopsy, which is considered the gold standard for interstitial lung disease, may yield negative results because of sampling error and the rapid evolution and changing pattern of the disease.

Adult↗
PubMed2003

Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications.

OBJECTIVE: The aim of our study was to evaluate the usefulness of the reversed halo sign on high-resolution CT in the diagnosis of cryptogenic organizing pneumonia. MATERIALS AND METHODS: Between 1996 and 2001, we saw 31 patients with biopsy-proven cryptogenic organizing pneumonia. During the same period, we also saw 30 patients with non-cryptogenic organizing pneumonia diseases, from which cryptogenic organizing pneumonia should be differentiated: Wegener's granulomatosis (n = 14), diffuse bronchioloalveolar carcinoma (n = 10), chronic eosinophilic pneumonia (n = 5), and Churg-Strauss syndrome (n = 1). Two independent observers analyzed CT findings and recorded how frequently the so-called reversed halo sign (central ground-glass opacity and surrounding air-space consolidation of crescentic and ring shape) was seen on high-resolution CT. RESULTS: The most common patterns of parenchymal abnormalities of cryptogenic organizing pneumonia were ground-glass opacity (28/31 patients, 90%) and consolidation (27/31, 87%). The ground-glass opacity pattern showed random distribution, and the consolidation pattern showed subpleural or peribronchovascular (20/27 patients, 74%) distribution with predominance in the middle or lower lung zone. The reversed CT halo sign was seen in six (19%) of 31 patients with cryptogenic organizing pneumonia and in none of the patients with the diseases that needed to be differentiated from cryptogenic organizing pneumonia on CT. CONCLUSION: The reversed halo sign, although seen only in one fifth of patients with the disease, appears relatively specific to make a diagnosis of cryptogenic organizing pneumonia on CT.

Adult↗
PubMed2005

Cryptogenic organizing pneumonia: variety of radiologic findings.

BACKGROUND: Cryptogenic organizing pneumonia is increasingly being recognized as a major cause of diffuse infiltrative lung disease. The differential diagnosis of non-infectious diseases that resemble pneumonia should include this entity. Understanding the radiologic features of this entity will help in defining the correct diagnosis, although lung biopsy is needed to provide histopathologic confirmation. Treatment with steroids achieves an excellent response. OBJECTIVES: To present a variety of radiologic findings on high resolution computerized tomography in eight sequential patients with COP, together with clinical and pathologic correlation. METHODS: Sequential HRCT examinations of eight patients (four males) aged 53-80 years (mean 65.5 years) with pathologcally proven COP were retrospectively analyzed by a consensus of two experienced chest radiologists for the existence and distribution of airspace consolidation, ground-glass opacities, nodular thickening along bronchovascular bundles and small (<1 cm) and large (>1 cm) nodules. The distribution of radiologic findings was classified as unilateral or bilateral, located in the upper, lower or middle lobe, and central or peripheral. Also recorded was the presence or absence of mediastinal lymphadenopathy and pleural effusion. Correlation with clinical symptoms was analyzed. RESULTS: All eight patients had bilateral airspace consolidations. in two cases consolidations were limited to central fields, in four they were peripheral, and in the remaining two cases they were both central and peripheral. Small nodules were noted in six cases and large nodules in three. Ground-glass opacities were found ln four cases. All patients had enlarged lymph nodes (1-1.5 cm) in the mediastinum. Radiologic abnormalities resolved or improved after steroid treatment in all patients. CONCLUSIONS: HRCT findings of bilateral multiple heterogenic lung infiltrates and nodules associated with mild mediastinal lymphadenopathy in a patient with non-specific clinical symptoms are suggestive of COP; in such cases lung biopsy is indicated. Radiologic resolution of abnormalities correlates well with clinical improvement under adequate steroid treatment.

Aged↗
PubMed1996

Neutrophil chemotactic activity in cryptogenic organizing pneumonia and the response to erythromycin.

The present study was aimed at elucidating the role of inflammatory cells in the pathogenesis of cryptogenic organizing pneumonia, and the mode of action of erythromycin in inhibiting the progression of the disease. Bronchoalveolar lavage fluid was obtained from 16 patients with cryptogenic organizing pneumonia and 4 control subjects. Neutrophil chemotactic activity was determined in relation to the concentration of two cytokines, interleukin-8 and tumor necrosis factor-alpha. Eight patients with cryptogenic organizing pneumonia, 4 with bronchoalveolar lavage fluid neutrophilia and 4 without, received low dose oral erythromycin daily (600 mg) for 2 to 3 months. Bronchoalveolar lavage fluid was obtained before and after treatment. In the bronchoalveolar lavage fluid of cryptogenic organizing pneumonia patients with bronchoalveolar lavage fluid neutrophilia, the levels of neutrophil chemotactic activity, interleukin-8, and tumor necrosis factor-alpha were significantly increased compared with levels measured in control subjects and in cryptogenic organizing pneumonia patients without bronchoalveolar lavage fluid neutrophilia. The level of interleukin-8 correlated with the percent of neutrophils and neutrophil chemotactic activity of bronchoalveolar lavage fluid, while the level of tumor necrosis factor-alpha did not. Furthermore, the levels of interleukin-8 and neutrophil chemotactic activity in the bronchoalveolar lavage fluid of cryptogenic organizing pneumonia patients with bronchoalveolar lavage fluid neutrophilia were significantly decreased following treatment with erythromycin. In contrast, the level of tumor necrosis factor-alpha was not affected by treatment with erythromycin. It is possible that cryptogenic organizing pneumonia is caused by neutrophil-mediated inflammation, and that the favorable clinical effect of erythromycin is due to inhibition of neutrophil accumulation in the peripheral airways through a biological activity other than bacteriostasis, e.g., local suppression of interleukin-8 production.

Aged↗
PubMed2006

[Radiological features of cryptogenic organizing pneumonia].

OBJECTIVE: To explore the radiological features of cryptogenic organizing pneumonia (COP). METHODS: Five patients with COP were pathologically identified. The chest CT scanning was performed in all patients. The radiological features of COP were classified into four types according to pulmonary consolidation, ground grass opacity (GGO), nodule, and liner or reticular lesion. The distribution of lesions were classified according to the single or double lung fields, upper or lower lung field, or the surrounding bronchovascular bundle or subpleural area. The radiological types and distributions of the lesions were analyzed. RESULTS: Among these 5 patients, pulmonary consolidation was found in 4 patients, ground grass opacity in 3 patients, nodules in 2 patients, and reticulation in one patient. Various radiological features were also observed at the same time. The pulmonary consolidation was distributed along the bronchovascular bundle or subpleural area. The distribution of GGO and pulmonary nodules were nonspecific. CONCLUSIONS: The most common radiological features of COP were pulmonary consolidation and GGO. The distribution of pulmonary consolidation is specific. It extends along the bronchovascular bundle or located in the subpleural area.

Adult↗
PubMed1994

Cryptogenic organizing pneumonia: CT findings in 43 patients.

OBJECTIVE: Description of the CT findings of cryptogenic organizing pneumonia has been limited to a small number of cases. This study was performed to characterize the CT findings of this disease in a larger number of cases and to compare the findings in immunocompetent and immunocompromised patients. MATERIALS AND METHODS: The CT scans of 43 (32 immunocompetent and 11 immunocompromised) patients who had biopsy-proved cryptogenic organizing pneumonia were reviewed. The scans were obtained by using contiguous 8- or 10-mm collimation and selected thin (1.5 or 2.0 mm) section (n = 23), thin-section collimation at 10-mm intervals (n = 12), or 8- or 10-mm collimation only (n = 8). The scans were analyzed by three observers, and final decisions were reached by consensus. RESULTS: The most common pattern seen was consolidation, which was present alone or as part of a mixed pattern in 34 cases (79%). The consolidation had a predominantly subpleural and/or peribronchovascular distribution in 27 cases (63%). Ground-glass attenuation and nodules were seen in 26 patients (60%) and 13 patients (30%), respectively, and were usually random in distribution. Consolidation was present in 91% (29/32) of immunocompetent and 45% (5/11) of immunocompromised patients (p < .01). Ground-glass attenuation was present in 56% (18/32) of immunocompetent and 73% (8/11) of immunocompromised patients (p > .25). Nodules were present in seven (22%) of 32 immunocompetent patients and six (55%) of 11 immunocompromised patients (p < .025). CONCLUSION: We conclude that in immunocompetent patients the CT findings in cryptogenic organizing pneumonia most commonly consist of bilateral areas of consolidation involving mainly the subpleural and/or peribronchovascular regions. In the immunocompromised patient, the CT findings are variable.

Adult↗
PubMed2004

[Interstitial fibrosis in cryptogenic organizing pneumonia could be a predictive factor of relapses].

INTRODUCTION: Cryptogenic organizing pneumonia (COP) is a clinicopathologic syndrome characterized by a good prognosis with steroid therapy, but frequent relapses when the dose of corticosteroid is reduced or stopped. CASE REPORT: The purpose of this study was to identify histopathologic features related to the relapse of COP. Six cases of COP that had been diagnosed using open lung biopsy were selected for evaluation. The 6 cases were put into two groups composed of 3 patients who relapsed and 3 who did not relapse. Their pathologic features were examined and compared. CONCLUSIONS: Interstitial fibrosis of the lung parenchyma could correspond to histopathologic characteristics of relapses in COP.

Adult↗
PubMed1994

Cryptogenic organizing pneumonia with atypical histopathological features.

A case report of cryptogenic organizing pneumonia (COP); also known as bronchiolitis obliterans organizing pneumonia (BOOP) is presented. The histopathologic findings of COP are well documented in the literature and typically consist of organizing pneumonia of uniform appearance. This case report describes, in addition to the classic findings, more acute exudative inflammation not usually associated with this condition. Variation in the evolution of the pneumonic process is one of the reasons for reporting this case. The promotion of awareness of this treatable condition is the other reason for reporting this case. When multifocal areas of consolidation are demonstrated radiologically, particularly when peripheral and basal, and in the correct clinical setting, the possibility of COP should be entertained. This condition responds dramatically to steroid therapy, and has a good prognosis.

Bronchiolitis Obliterans↗
PubMed2006

[Clinicopathologic study of cryptogenic organizing pneumonia].

OBJECTIVE: To study the clinicopathologic features and differential diagnosis of cryptogenic organizing pneumonia (COP). METHODS: The clinical, radiologic and pathologic features of 11 patients with COP confirmed by open or video-assisted thoracoscopic (VATS) lung biopsy were analyzed. Treatment information and follow-up data were also obtained. RESULTS: COP usually affected female patients over 40 years of age. Clinical presentations included cough, sputum and exertional dyspnea. High-resolution computerized tomography showed scattered speckled, patchy and trabecular shadows over both lung fields. Honeycomb changes were not found. Histologically, polypoid growths of granulation tissue were noted within respiratory bronchioles, small airways and alveolar spaces. These lesions had a patchy and peribronchiolar distribution and were uniform in appearance. The overall response rate to glucocorticoid was 81.8% (P < 0.01). The duration of follow up ranged from 6 to 134 months. Apart from one patient who developed aggravation of symptoms, the disease pursued a relatively stable clinical course. CONCLUSIONS: In general, COP responds well to glucocorticoid therapy. Open or VATS lung biopsy is important for arriving at a definitive diagnosis, especially for those cases presenting with atypical clinical and radiographic manifestations. Multiple biopsies with larger samples are preferred in order to avoid misdiagnosis.

Adult↗
PubMed2006

[Clinicopathologic features of 18 cases of cryptogenic organizing pneumonia].

OBJECTIVE: To analyze the characteristics of pathologically proved cases of cryptogenic organizing pneumonia (COP) including manifestation of CT scan, bronchoalveolar lavage (BAL) fluid cytology, pulmonary histopathological appearance. METHODS: Twenty-three consecutive patients from April 1994 to October 2005 in Peking Union Medical College Hospital with pathologic features of organizing pneumonia were enrolled in the study. RESULTS: Eighteen patients were diagnosed as COP. Percutaneous lung biopsy/transbronchial lung biopsy was performed in 10 patients, while open lung biopsy/video-assistant thorascopic biopsy was performed in 8 patients. There were 8 males and 10 females, aged (53.5 +/- 11.0) yr. Dyspnea, cough, inspiratory crackles were the most common symptoms and signs. Lung function tests showed a restrictive ventilatory defect (11/18), and impairment of carbon monoxide transfer factor (T(LCO), 18/18). Chest computed tomography scan in 18 cases mainly showed ground glass opacities (4/18), patchy areas of alveolar consolidation (13/18) with air bronchograms (3/18), and pleural effusion (4/18). BAL fluid cytological analysis showed the percentage of lymphocyte was 0.40 +/- 0.16, neutrophil 0.15 +/- 0.08, eosinophil 0.05 +/- 0.03; the CD(4)/CD(8) ratio was 0.43 +/- 0.21. All patients were treated with corticosteroids. Sixteen were alive and clinically improved or stabilized at the last follow up (8.7 +/- 6.2 months after diagnosis). Two patients died, one of respiratory failure, and another of pulmonary infection. CONCLUSIONS: The diagnosis of COP is usually suggested by clinical-radiological findings, but needs to be confirmed histopathologically. The combination of BAL fluid cytological findings and lung histological appearance obtained during a bronchoscopic procedure appears to be an effective method for diagnosing this disease.

Adult↗
PubMed1996

Bronchoalveolar lavage, histological and immunohistochemical features in cryptogenic organizing pneumonia.

This study describes bronchoalveolar lavage (BAL), histological and immunohistochemical features in a series of 10 patients with cryptogenic organizing pneumonia (COP). The histological diagnosis was performed by transbronchial biopsy in seven cases and by open lung biopsy in three cases. All patients showed a marked increase in lymphocytes and a mild increase in neutrophils and eosinophils in BAL fluid. The number of T-lymphocytes expressing human leucocyte antigen-DR (HLA-DR) surface antigen was increased (p < 0.002). The majority of lymphocytes expressed the CD8 phenotype, so that the CD4/CD8 ratio was markedly decreased. Masson bodies were present in the lung specimens of all patients. Most of the epithelial cells surrounding the Masson bodies were immunoreactive with an anti-granulocyte/macrophage colony-stimulating factor (GM-CSF) monoclonal antibody. The great majority of mononuclear cells in the lung specimens showed immunoreactivity with anti-CD3, anti-CD8 and anti-CD45R0 monoclonal antibodies. In the Masson bodies, spindle cells were immunoreactive with anti-alpha smooth muscle (alpha-sm) actin monoclonal antibody. Glucocorticoid treatment (the therapy of choice in COP) downregulated GM-CSF messenger ribonucleic acid (mRNA) expression in lung epithelial cell lines. These findings indicate that the combination of bronchoalveolar lavage cell profile with histological evidence is a valuable means of corroborating a clinical diagnosis of cryptogenic organizing pneumonia, and that granulocyte/macrophage colony-stimulating factor may be one of the cytokines involved in the pathogenesis.

Biopsy↗
PubMed1995

Cryptogenic organizing pneumonia in the immunocompromised patient: radiologic findings and follow-up in 12 patients.

OBJECTIVE: To assess the radiographic, computed tomography (CT) and pathologic findings and response to therapy of cryptogenic organizing pneumonia in immunocompromised patients. PATIENTS AND METHODS: The study group consisted of 12 consecutive immunocompromised patients (seven men and five women), examined between July 1988 and July 1993, who had pathologically proven cryptogenic organizing pneumonia and for whom chest radiographs and CT scans had been obtained within 9 days of diagnosis. The 12 patients were treated with corticosteroids and underwent follow-up radiography. RESULTS: The findings of chest radiography included consolidation (in 10 patients), reticulation (in 3) and small nodules (in 2). None of the radiographic abnormalities were localized to particular zones of the lung. The CT findings included ground-glass attenuation (in nine patients), consolidation (in six), small nodules (in five) and reticulation (in two). In 8 of the 12 patients (67%) these abnormalities were most marked in the subpleural and peribronchovascular regions. The follow-up radiographs showed improvement in 11 (92%) of the patients. CONCLUSIONS: The most common radiographic abnormality associated with cryptogenic organizing pneumonia in these immunocompromised patients was consolidation, and the most common CT finding was ground-glass attenuation with or without associated consolidation or nodules. Most of the patients responded to corticosteroid therapy, irrespective of the initial radiologic pattern.

Adult↗