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An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review.
- Source :
-
Rheumatology International . Mar2024, Vol. 44 Issue 3, p549-556. 8p. - Publication Year :
- 2024
-
Abstract
- 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-1λ 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. [ABSTRACT FROM AUTHOR]
- Subjects :
- *TALL-1 (Protein)
*BELIMUMAB
*PLASMA cells
*DNA antibodies
*ANTINUCLEAR factors
Subjects
Details
- Language :
- English
- ISSN :
- 01728172
- Volume :
- 44
- Issue :
- 3
- Database :
- Academic Search Index
- Journal :
- Rheumatology International
- Publication Type :
- Academic Journal
- Accession number :
- 175459302
- Full Text :
- https://doi.org/10.1007/s00296-023-05510-3