A case with good response to belimumab for lupus nephritis complicated by IgG4-related disease

Author:

Yamamoto M1ORCID,Aochi S2,Suzuki C1,Nakamura S3,Murakami R4,Ogawa Y5,Takahashi H1

Affiliation:

1. Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Japan

2. Department of Internal Medicine, Japan Self Defense Forces Sapporo Hospital, Sapporo, Japan

3. Department of Rheumatology, Hokkaido Orthopedic Memorial Hospital, Sapporo, Japan

4. Department of Rheumatology, JR Sapporo Hospital, Sapporo, Japan

5. Hokkaido Renal Pathology Center, Sapporo, Japan

Abstract

Immunoglobulin (Ig)G4-related disease (IgG4-RD) is an unusual complication of systemic lupus erythematosus (SLE). We report a case in which belimumab proved efficacious for not only SLE, but also IgG4-RD. A 58-year-old Japanese woman had suffered from photodermatosis and erythema on the limbs for 20 years. She presented in slight fever and fatigue since 2016. Laboratory data showed hypergammaglobulinemia, proteinuria and positive results for anti-nuclear antibody and anti-double-stranded DNA antibody. Furthermore, elevated levels of serum IgG4 were detected. Contrast-enhanced computed tomography disclosed multiple areas of poor enhancement in the kidneys. The patient was diagnosed with lupus nephritis and IgG4-RD from renal biopsy. Treatment was started with prednisolone at 40 mg/day and mycophenolate mofetil. Proteinuria and serological findings improved initially, but tapering the dose of glucocorticoid proved difficult. After treatment was started with belimumab, clinical symptoms and proteinuria resolved completely. The dose of glucocorticoid was successfully tapered and serum concentration of IgG4 fell further. This appears to represent the first report of a case in which both SLE and IgG4-RD were effectively treated using belimumab.

Publisher

SAGE Publications

Subject

Rheumatology

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