Am. J. Respir. Crit. Care Med., Vol 155, No. 2, 02 1997, 739-742.
Progressive obstructive lung disease associated with microscopic polyangiitis
O Brugiere, O Raffy, C Sleiman, O Groussard, E Rothchild, F Mellot, G Jebrak, H Mal, C Roue, R Pariente and M Fournier
Service de Pneumologie et Reanimation Medicale, Hopital Beaujon, Clichy, France.
Small airway involvement and progressive severe airflow obstruction are
unexpected features in patients with microscopic polyangiitis. We report
the case of a patient with microscopic polyangiitis and circulating
anti-neutrophil cytoplasmic antibodies (ANCA), who developed pulmonary
hyperinflation and airflow obstruction over a 7-yr period. Systemic
manifestations of this vasculitis improved under corticosteriods and
cyclophosphamid therapy, a treatment that did not influence either the very
high level of anti-myeloperoxidase antibodies or the ventilatory
impairment. Small airway involvement was suspected on the basis of
pathologic small airway lesions and a mild emphysematous pattern on
computed tomography (CT) scan, which was out of proportion with the
severity of the obstructive lung disease.
