Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome

Background and objective: Constrictive bronchiolitis is a rare and potentially fatal manifestation of paraneoplastic autoimmune multi-organ syndrome (PAMS), also called paraneoplastic pemphigus. The current review aimed to assess the frequency and clinical features of constrictive bronchiolitis occurring in patients with PAMS. Methods: A computer-assisted search of medical records identified 17 patients with PAMS seen during the period 1994-2007. Medical records, radiological studies and biopsy results were reviewed. Results: There were 10 men and 7 women; the median age at diagnosis of PAMS was 60 years (range 33-72 years). Non-Hodgkin's lymphoma and chronic lymphocytic leukaemia were the underlying neoplasms in over two-thirds of these patients. Constrictive bronchiolitis was diagnosed in three patients who had respiratory symptoms, severe airflow obstruction on pulmonary function testing and evidence of air trapping on CT scanning with no other identifiable cause for these findings. In contrast to previous reports, two of the three patients with constrictive bronchiolitis presented with skin and respiratory manifestations in the absence of a known neoplasm. Seven patients with PAMS died (41%) after a median interval of 13 months (range 1-33 months) and the deaths included two of the constrictive bronchiolitis patients who continued to worsen despite immunosuppressive therapy. Conclusions: In a minority of patients with PAMS, constrictive bronchiolitis occurs and tends to cause progressive airflow obstruction that responds poorly to immunosuppressive therapy. Constrictive bronchiolitis in these patients may be manifest prior to the discovery of the underlying neoplasm and the diagnosis of PAMS.