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A case of recurrent IgG4-related disease successfully treated with belimumab after remission of systemic lupus erythematosus

Yu Katayama, Takayuki Katsuyama, Kenta Shidahara, Shoichi Nawachi, Yosuke Asano, Keiji Ohashi, Yoshia Miyawaki, Eri Katsuyama, Mariko Narazaki, Yoshinori Matsumoto, Ken‐ei Sada, Jun Wada

2022Lara D. Veeken12 citationsDOI

Abstract

Dear Editor, IgG4-related disease (IgG4-RD) is a chronic systemic disease characterized by the elevation of serum IgG4, infiltration of lymphocytes and IgG4-positive plasma cells, and fibrosis [1]. IgG4-RD affects any organs such as salivary glands, orbits, pancreas, kidneys, aorta, retroperitoneum and mimics malignancy, infection, and other autoimmune diseases including SLE. SLE is also a chronic inflammatory disease that presents variable symptoms, and IgG4-RD and SLE rarely coexist. The first-line treatment of IgG4-RD is glucocorticoids (GCs). Although IgG4-RD usually shows a good response to GCs, it often relapses. When GC treatment fails, there is no evidence on what immunosuppressive agents should be added next. We herein report a case of IgG4-RD complicated with SLE, successfully treated with belimumab for the relapse of IgG4-RD, not SLE, possibly leading to novel insights into the treatment of IgG4-RD. A 76-year-old man with fatigue and 4 kg weight loss was admitted to...

Topics & Concepts

MedicineBelimumabIgG4-related diseaseMalignancyInternal medicineGastroenterologyAutoimmune diseaseSystemic diseaseDiseaseFibrosisRituximabImmunologyAntibodyB cellB-cell activating factorIgG4-Related and Inflammatory DiseasesNeuroendocrine Tumor Research AdvancesPlatelet Disorders and Treatments