Constrictive bronchiolitis obliterans in patient with Castelman's disease

A 37-year-old woman with hialin-vascular type Castelman's disease (CD) localised in the retroperitoneal region, incompletely resected, developed progressive dyspnoea. The chest radiograph taken 3 months before the operation was normal. The chest CT scan revealed diffused bronchiectases, hyperinflation and air trapping. Pulmonary function tests disclosed severe obstructive impairment with hyperinflation. The bronchoscopic examination of the bronchial tree was normal. Cultures of sputum, bronchial washing and blood were negative. No pemphigus antibodies were found. Mycoplasmal, chlamydial and viral infections were excluded. Histological examination of specimens obtained by open lung biopsy revealed bronchiolar inflammation, submucosal bronchial fibrosis with obliteration of bronchiolar lumen. Constrictive bronchiolitis obliterans (CBO) was diagnosed. Despite slight clinical and spirometric improvements that were achieved due to corticosteroid therapy, one year later she died as a result of respiratory failure. It is widely known that patients with CD develop CBO during the course of paraneoplastic pemphigus. However, we present the case of CBO and CD but without any symptoms of this condition.