A 38-year-old woman presented, at age 27, in April 1985,
with arthralgias, sinusitis, and episcleritis. She subsequently
experienced digital ischemia. She had no renal disease. She had smoked
one pack of cigarettes per day since age 17. Prednisone was begun for
possible polyarteritis nodosa, and she experienced improvement. Within
several months, while on prednisone, she had dyspnea and wheezing. A
chest radiograph showed an irregular 4-cm thick-walled cavity in the
left lower lobe. The erythrocyte sedimentation rate was 38 mm/h.
Spirometry revealed an FVC of 3.97 L (106% of predicted) and an
FEV1 of 1.84 L (58% of predicted). The diagnosis
of WG was confirmed by open lung biopsy, which showed necrotizing
granulomatous inflammation (Fig 1
), vasculitis (Fig 2
), and focal alveolar hemorrhage. There was no evidence of emphysema.
She was continued on prednisone and begun on cyclophosphamide with
resolution of systemic symptoms. Her dyspnea worsened over the next
several years. In 1987, a chest radiograph showed emphysema that was
most marked at the lung bases. By 1992, she was significantly limited
by dyspnea. Her α1-AT level was 32 mg/dL
(normal, 150 to 300 mg/dL), and her α1-AT
phenotype was PiZZ. She had no recurrence of WG.