Rapidly progressive Bronchiolitis Obliterans Organising Pneumonia presenting with pneumothorax, persistent air leak, acute respiratory distress syndrome and multi-organ dysfunction: a case report

<p>Abstract</p> <p>Introduction</p> <p>Bronchiolitis Obliterans Organising Pneumonia (BOOP) may often present initially as a recurrent spontaneous pneumothorax and then develop multi-system complications.</p> <p>Case presentation</p> <p>A 17-year-old boy presented with a pneumothorax, which developed into rapidly progressive Bronchiolitis Obliterans Organising Pneumonia (BOOP). He developed multi-organ dysfunction (including adult respiratory distress syndrome, oliguric renal failure, acute coronary syndrome, cardiac failure and a right atrial thrombus) which necessitated prolonged intensive care. Diagnosis was confirmed on open lung biopsy and he responded well to treatment with corticosteroids.</p> <p>Conclusion</p> <p>BOOP is exquisitely sensitive to oral corticosteroids but if the diagnosis is not considered in such patients and appropriate treatment instituted early, BOOP may often lead to prolonged hospital admission with considerable morbidity.</p

O espectro clínico e radiológico da pneumonia em organização: análise retrospectiva de 38 casos Clinical and radiographic spectrum of organizing pneumonia: retrospective analysis of 38 cases

A pneumonia em organização, acompanhada ou não de bronquiolite obliterante, é uma condição anatomopatológica pulmonar específica com uma grande variedade de apresentações clínicas e radiológicas. A pneumonia em organização pode ser idiopática ou secundária a várias doenças, incluindo infecções e drogas. Objetivos: Descrever as manifestações clínicas, dados radiológicos e espirométricos de pacientes com pneumonia em organização. Métodos: Análise retrospectiva de pacientes com o diagnóstico de pneumonia em organização. Resultados: 38 pacientes foram incluídos na análise. Quatorze apresentavam também condições clínicas relacionadas à pneumonia em organização, e estes mais freqüentemente apresentaram infiltração pulmonar difusa (três de quatro casos) e bronquiolite obliterante associada (57% vs. 20%, p = 0,05). Dos 13 pacientes com bronquiolite obliterante associada apenas um era assintomático e dois apresentavam lesões localizadas (15%). De oito pacientes assintomáticos com lesões localizadas, nenhum possuía bronquiolite obliterante associada, e a apresentação radiológica freqüentemente mimetizou carcinoma brônquico. A espirometria de modo geral não contribuiu sobremaneira para o diagnóstico, provavelmente devido à alta prevalência de tabagismo na amostra. Conclusões: A pneumonia em organização possui apresentação clínica e radiológica variada. Fatores que sugerem a ausência de bronquiolite obliterante são a ausência de sintomas e lesões radiológicas localizadas, e esta forma da doença mais freqüentemente deve ser diferenciada de carcinoma brônquico na prática clínica.<br>Organizing pneumonia, whether or not accompanied by bronchiolitis obliterans, is a specific anatomicopathological condition of the lungs that can present in a variety of clinical and radiographic ways. It can be either idiopathic or secondary to a number of diseases, including infection and drugs. Objectives: To describe the clinical manifestations, radiographic and spirometric data seen in patients with organizing pneumonia. Methods: Retrospective analysis of patients with a diagnosis of organizing pneumonia. Results: 38 patients were included in the analysis. Fourteen also had clinical conditions related to organizing pneumonia, and they presented more frequently with diffuse pulmonary infiltrates (three of four cases) and associated bronchiolitis obliterans (57% vs. 20%, p = 0,05). Of the 13 patients with bronchiolitis obliterans, only one was asymptomatic and two had localized lesions (15%). Of the eight asymptomatic patients with localized lesions, no one had associated bronchiolitis obliterans, and the radiographic appearance often resembled bronchial carcinoma. Spirometry was generally of little value to diagnosis, probably because of the high prevalence of smoking in the sample. Conclusion: The clinical and radiographic presentation of organizing pneumonia is variable. Factors that suggest the absence of coexisting bronchiolitis obliterans are the absence of symptoms and localized radiographic lesions, and this form of the disease has to be more often differentiated from bronchial carcinoma in clinical practice