An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review

被引:1
作者
Iwamoto, Megumi [1 ]
Asashima, Hiromitsu [1 ]
Sugita, Toshiki [1 ]
Kawashima, Fumina [1 ]
Sugita, Naoki [1 ]
Rai, Akiyoshi [1 ]
Kuroda, Yuki [1 ]
Kawashima, Akira [1 ]
Tabuchi, Daiki [1 ]
Akao, Satoshi [1 ]
Sato, Ryota [1 ]
Nishiyama, Taihei [1 ]
Toko, Hirofumi [1 ]
Honda, Fumika [1 ]
Ohyama, Ayako [1 ]
Kitada, Ayako [1 ]
Abe, Saori [1 ]
Miki, Haruka [1 ]
Hagiwara, Shinya [1 ]
Kondo, Yuya [1 ]
Tsuboi, Hiroto [1 ]
Matsumoto, Isao [1 ]
机构
[1] Univ Tsukuba, Inst Med, Dept Rheumatol, 1-1-1 Tennodai, Tsukuba, Ibaraki 3058575, Japan
关键词
IgG4-related disease; Systemic lupus erythematosus; Belimumab; DIAGNOSIS; LYMPHADENOPATHY; PREDICTORS; MANAGEMENT;
D O I
10.1007/s00296-023-05510-3
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
IgG4-related disease (IgG4-RD) is a systemic condition in which IgG4+ plasma cell infiltration and fibrosis cause organ swelling and lead to diverse clinical manifestations. Although IgG4-RD typically responds to glucocorticoids (GCs), relapse during tapering occurs and an early GC-sparing approach might therefore be beneficial. Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiple symptoms that is also treated with GCs as a first-line therapy. Recently, belimumab, a recombinant human IgG-1 lambda monoclonal antibody that inhibits B-cell activating factor, was approved, but reports of use for IgG4-RD are scarce. Here, we present a rare case of IgG4-RD complicated with SLE which was successfully treated with belimumab. A 67-year-old man was diagnosed with IgG4-RD based on a high serum IgG4 level and histopathological findings. Furthermore, he had pericardial effusion on echocardiography, and laboratory tests revealed thrombocytopenia, autoimmune hemolysis, positive anti-nuclear antibodies, positive anti-DNA antibodies, and hypocomplementemia. These data led to an SLE diagnosis. Treatment was started with prednisolone at 40 mg/day, plus hydroxychloroquine, which initially improved both the SLE and IgG4-RD symptoms. During the GC tapering, belimumab was added and clinical symptoms resolved completely. Our case and the literature review summarize reported rare overlapping cases of IgG4-RD and SLE and suggest that belimumab is a promising candidate for the treatment of IgG4-RD.
引用
收藏
页码:549 / 556
页数:8
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