Clinical features of IgG4-related rhinosinusitis

Abstract Purpose IgG4-related disease is a systemic disease that affects various organs of the body. Aim of this study is to elucidate the clinical characteristics of IgG4-related rhinosinusitis. Material and methods Clinical features, laboratory findings, radiological and endoscopic findings, associated disease, treatment and prognosis were retrospectively examined in 10 patients with IgG4-related rhinosinusitis. Results The age was 59.1 ± 11.3 years old and male-to-female ratio was 1:1. The chief nasal complaints were hyposmia (n = 4), nasal obstruction (n = 3), and nothing (n = 3). Serum IgG4 levels were elevated in all patients and the value was 740.4 ± 472.4 mg/dl. Other IgG4-related diseases were associated in all 10 patients, including IgG4-related sialadenitis (n = 6), IgG4-related dacryoadenitis (n = 5), and autoimmune pancreatitis (n = 5). Imaging findings on CT/MRI were obstruction of the way of elimination (n = 10), thickening of the sinus mucous membrane (n = 10), and fluid in the sinus (n = 6). All of the cases had bilateral findings. Nasal endoscopic findings were chiefly deviated nasal septum (n = 5), polyps (n = 4), edema of the mucous membrane (n = 3). Histologically, abundant infiltration of IgG4 positive plasma cell and lymphocyte and an elevated IgG4+/IgG+ cell ration was detected in all 8 patients and 5 patients, respectively. Endoscopic sinus surgery was performed in 8 patients. Eight patients were treated with steroid therapy for other associated IgG4-related diseases. Symptoms improved in all 6 patients after an initial treatment (endoscopic surgery (n = 5) and steroids (n = 1)), but one patient suffered relapse. Conclusions IgG4-related rhinosinusitis is a distinct entity of IgG4-related disease, and is associated in patients with multiple IgG4-related diseases.