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      Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh

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          Abstract

          BACKGROUND

          Because reports of bronchiolitis obliterans organizing pneumonia (BOOP) are lacking from the Middle East, we conducted a retrospective review of of all histopathologically proven cases of BOOP over a 10-year period at three tertiary care hospitals in Riyadh and describe the clinical features and outcome.

          METHODS

          Charts at the three hospitals were searched using a specific code for BOOP or cryptogenic organizing pneumonia (COP). Lung specimens had to show histological proof of BOOP with a compatible clinical picture. Chest radiographs and high-resolution CT scans were reviewed.

          RESULTS

          Twenty cases of biopsy-proven BOOP had well-documented clinical and radiographic data. There were 11 males and 9 females (mean age, 58 years; range, 42–78). The clinical presentation of BOOP was acute or subacute pneumonia-like illness with cough (85%), fever (70%) dyspnea, (85%) and crackles (80%). The most frequent radiological pattern was a bilateral alveolar infiltrate. The most common abnormality on pulmonary function testing (n=14) was a restrictive pattern (11 patients). Most patients (70%) had no underlying cause (idiopathic BOOP). Other associations included thyroid cancer, rheumatoid arthritis, syphilis and Wegner's granulomatosis. Ten patients (50%) had a complete response to steroids, 6 (30%) had a partial response and 3 (15.8%) with secondary BOOP had rapid progressive respiratory failure and died.

          CONCLUSION

          The clinical presentation of BOOP in our patients is similar to other reported series. A favorable outcome occurs in the majority of cases. However, BOOP may occasionally be associated with a poor prognosis, particularly when associated with an underlying disease.

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          Most cited references17

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          Bronchiolitis obliterans organizing pneumonia.

          In 50 of 94 patients with bronchiolitis obliterans we found no apparent cause or associated disease, and the bronchiolitis obliterans occurred with patchy organizing pneumonia. Histologic characteristics included polypoid masses of granulation tissue in lumens of small airways, alveolar ducts, and some alveoli. The fibrosis was uniform in age, suggesting that all repair had begun at the same time. The distribution was patchy, with preservation of background architecture. Clinically, there was cough or flu-like illness for 4 to 10 weeks, and crackles were heard in the lungs of 68 per cent of the patients. Radiographs showed an unusual pattern of patchy densities with a "ground glass" appearance in 81 per cent. Physiologically, there was restriction in 72 per cent of the patients, and 86 per cent had impaired diffusing capacity. Obstruction was limited to smokers. The mean follow-up period was four years. With corticosteroids, there was complete clinical and physiologic recovery in 65 per cent of the subjects; two died from progressive disease. This disorder differs from bronchiolitis obliterans with irreversible obstruction. It was confused most often with idiopathic pulmonary fibrosis. In view of the benign course and therapeutic response, a histologic distinction is important.
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            Cryptogenic organizing pneumonia. Characteristics of relapses in a series of 48 patients. The Groupe d'Etudes et de Recherche sur les Maladles "Orphelines" Pulmonaires (GERM"O"P).

            Cryptogenic organizing pneumonia (COP) is a clinicopathologic syndrome characterized by rapid resolution with corticosteroids, but frequent relapses when treatment is tapered or stopped. We retrospectively studied relapses in 48 cases of biopsy-proven COP. One or more relapses (mean 2.4 +/- 2.2) occurred in 58%. At first relapse, 68% of patients were still under treatment for the initial episode. Compared with the no-relapse group, nine patients with multiple (>/= 3) relapses had longer delays between first symptoms and treatment onset (22 +/- 17 versus 11 +/- 8 wk, p = 0.02), and elevated gamma-glutamyltransferase (124 +/- 98 versus 29 +/- 13 IU/L, p = 0.001) and alkaline phosphatase (190 +/- 124 versus 110 +/- 68 IU/L, p = 0.04) levels. Relapses did not adversely affect outcome. Corticosteroid treatment side effects occurred in 25% of patients. Standardized treatment in 14 patients allowed a reduction of prednisone cumulated doses (p < 0.05) without affecting outcome or relapse rate. We conclude that: (1) delayed treatment increases the risk of relapses; (2) mild cholestasis identifies a subgroup of patients with multiple relapses; (3) relapses do not affect outcome, and prolonged therapy to suppress relapses appears unnecessary; (4) a standardized treatment allows a reduction in steroid doses.
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              Cryptogenic organizing pneumonia: CT findings in 43 patients.

              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. 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. 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 .25). Nodules were present in seven (22%) of 32 immunocompetent patients and six (55%) of 11 immunocompromised patients (p < .025). 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.
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                Author and article information

                Journal
                Ann Saudi Med
                Ann Saudi Med
                Annals of Saudi Medicine
                King Faisal Specialist Hospital and Research Centre
                0256-4947
                0975-4466
                Jan-Feb 2007
                : 27
                : 1
                : 32-35
                Affiliations
                [* ]Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
                []Department of Medicine, King Fahd National Guard Hospital, Riyadh, Saudi Arabia
                []Department of Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia
                [§ ]Department of Pathology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
                Author notes
                Correspondence and reprint requests: Abdullah Al-Mobeireek, MD, Department of Medicine King Faisal Specialist Hospital and Research Centre Riyadh, Saudi Arabia, T: +966-1-442 7493, F: +966-1-442 7499, mobeireek@ 123456yahoo.com
                Article
                asm-1-32
                10.5144/0256-4947.2007.32
                6077020
                17277498
                6de2245c-6629-48fc-a43c-c886ee170e92
                Copyright © 2007, Annals of Saudi Medicine

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 01 November 2006
                Categories
                Original Article

                Medicine
                Medicine

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