Granulomatosis with polyangiitis (Wegener's) (GPA) is a systemic necrotizing vasculitis in which pulmonary nodules are a common manifestation. Our study examined whether pulmonary nodules, and nodule type (solid versus cavitary), are associated with different disease manifestations and outcomes.
Demographic, clinical, biologic, and radiologic data at diagnosis and during followup and treatments of GPA patients followed at the Mount Sinai Hospital (Canada) Vasculitis Clinic were analyzed. Patients were separated by the absence of lung nodules, presence of solid nodules only, and presence of cavitary nodules (+/- solid nodules). The study outcomes included followup lung imaging, relapses, and deaths.
Of 225 patients with GPA, 46 had solid nodules only and 44 had cavitary nodules at diagnosis. Demographic and clinical manifestations were similar in the patient subgroups at diagnosis. Cyclophosphamide (CYC) was used for induction after diagnosis in 76.7% of patients with cavitary nodules, compared with 64.7% of patients without nodules and 51.1% of patients with solid nodules (P = 0.04). The mean ± SD followup after diagnosis was 106.6 ± 92.6 months, and 6 of the patients died. In multivariable analysis, diagnosis before 2000 or pulmonary nodule cavitation at diagnosis were associated with relapse, with a hazard ratio of 0.38 (95% confidence interval [95% CI] 0.22-0.65; P < 0.001) and 1.53 (95% CI 1.00-2.33; P = 0.05), respectively, after adjustment for CYC use.
The presence of cavitary nodules led to increased use of CYC but had no impact on survival. Relapse occurred more often, however, in patients with cavitary nodules than in those with solid nodules or no nodules, and should be studied in other cohorts.