Progressive obstructive lung disease associated with microscopic polyangiitis.
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.
Brugiere
O
O
Service de Pneumologie et Reanimation Medicale, Hopital Beaujon, Clichy, France.
Raffy
O
O
Sleiman
C
C
Groussard
O
O
Rothchild
E
E
Mellot
F
F
Jebrak
G
G
Mal
H
H
Roue
C
C
Pariente
R
R
Fournier
M
M
D000402:Airway Obstruction / Q000209:etiology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001992:Bronchoalveolar Lavage Fluid; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008173:Lung Diseases, Obstructive / Q000503:physiopathology*; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012129:Respiratory Function Tests; D014657:Vasculitis / Q000188:drug therapy / Q000503:physiopathology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone