Clinical characteristics and treatment of IgG4-related diseases: a report of 48 cases / 中华全科医师杂志

ABSTRACT

Forty eight patients with IgG-related disease (IgG-RD) were treated in Beijing Luhe Hospital, Capital Medical University between January 2014 and January 2017. The clinical features, organ involvement, laboratory findings and treatment of patients were analyzed. The median age of patients was 59 (29-78) years old. The most common symptoms were submandibular gland enlargement (20.8%), abdominal pain (18.8%), eyelid mass or edema (18.8%) and jaundice (16.7%). The involved organs mainly were submandibular glands (62.5%), lymph nodes (54.2%), lacrimal glands (45.8%), parotid glands (41.7%) and pancreas (31.3%). PET/CT was performed in 9 cases (18.8%), the SUVmax value was not correlated with the number of affected organs (r=0.511, P=0.160) and IgG4 (r=0.385, P=0.306). Histopathology showed increased lymphocytes and plasma cells in 46 cases (95.8%), eosinophilia in 15 cases (31.3%), and fibrous hyperplasia in 26 cases (54.2%). Twenty-three patients (47.9%) were treated with single glucocorticoid, 20 patients (41.6%) were treated with glucocorticoid combined immunosuppressants, 3 patients (6.3%) received surgical treatment, and 2 (4.2%) were observed with watch and wait regimen. Forty (97.9%) patients responded well to the initial treatment. Ten out of 23 cases (43.5%) in corticosteroid monotherapy group relapsed, while 6 out of 20 cases (30.0%) in corticosteroids plus immunosuppressive group relapsed, for whom the full dose of corticosteroid and immunosuppressive agents were still effective. IgG4-RD is a chronic inflammatory and fibrous sclerotic disease with multiple organ involvement, increase serum IgG4 level and tissue infiltration with large number of IgG4+cells. Hormone and immunosuppressive therapy is effective, and the overall prognosis is good.