Progressive obstructive lung disease associated with microscopic polyangiitis

• Published in: American journal of respiratory and critical care medicine Vol. 155; no. 2; p. 739
• Main Authors: Brugiere, O, Raffy, O, Sleiman, C, Groussard, O, Rothchild, E, Mellot, F, Jebrak, G, Mal, H, Roue, C, Pariente, R, Fournier, M
• Format: Journal Article
• Language: English
• Published: United States 01.02.1997
• Subjects: Airway Obstruction - etiology; Antibodies, Antineutrophil Cytoplasmic - blood; Bronchoalveolar Lavage Fluid; Cyclophosphamide - therapeutic use; Female; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Lung Diseases, Obstructive - physiopathology; Middle Aged; Prednisone - therapeutic use; Respiratory Function Tests; Vasculitis - drug therapy; Vasculitis - physiopathology
• ISSN: 1073-449X
• DOI: 10.1164/ajrccm.155.2.9032221

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.