Asthma and orbital immunoglobulin G4-related disease

Background An association has been suggested between asthma and orbital immunoglobulin G4-related disease (IgG4-RD). Objective To explore this association, including asthma characteristics and risk factors. Methods A retrospective, computer-assisted search identified patients with orbital IgG4-RD seen at Mayo Clinic in Rochester, Minnesota from 1997 to 2014. Asthma prevalence and its related clinical and radiologic characteristics were studied. Results Thirty-one patients (17 men) with biopsy-proven orbital IgG4-RD were identified. Mean age at diagnosis was 54.3 years (SD 11.0 years). Median duration from onset of orbital symptoms to IgG4-RD diagnosis was 1.96 years (range 0.1-31.8 years). Twenty-two patients (71%) were not smokers, 6 (19%) were former smokers, and 3 (10%) were current smokers. Sixteen patients (52%) had asthma. Three patients had childhood asthma onset, and median age at asthma onset in the 7 patients with data available was 56 years (range 15-62 years). In this cohort, the most common findings at chest computed tomography were mediastinal and hilar lymphadenopathy (44%), linear scarring (20%), and nodules and bronchial wall thickening (16%). Bronchial wall thickening correlated with presence of asthma. Chronic sinusitis (94%) was most commonly associated with asthma. Serum IgG4 was markedly increased in patients with asthma (median 195.0 mg/dL, range 31.8-1,790.0 mg/dL) vs patients without asthma (median 78.9 mg/dL, range 7.7-166.0 mg/dL; P =.02). Treatment was commonly prednisone and then rituximab; rituximab helped control asthma in most cases. Two deaths were reported (median follow-up 4.2 years). Conclusion Asthma is commonly associated with orbital IgG4-RD and generally manifests as adult-onset bronchial wall thickening seen at computed tomography, increased serum IgG4 levels, and good rituximab response.