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Overlapping Tubulointerstitial Lupus Nephritis and Immunoglobulin G4-Related Disease: A Case Report.

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Citation: Cureus. 15(6):e40664, 2023 Jun.PMID: 37366478Institution: MedStar Union Memorial HospitalDepartment: Internal Medicine ResidencyForm of publication: Journal ArticleMedline article type(s): Case ReportsSubject headings: Year: 2023 ISSN:

2168-8184

Name of journal: CureusAbstract: Immunoglobulin G4-related disease (IgG4-RD) in conjunction with systemic lupus erythematosus (SLE) is a rare occurrence. In this case report, we present the case of a 50-year-old female who was diagnosed with SLE based on clinical and laboratory criteria. The patient exhibited pericardial effusion necessitating pericardiocentesis, pleural effusion requiring thoracentesis, and impaired renal function necessitating dialysis. Renal biopsy revealed findings consistent with tubulointerstitial lupus nephritis and IgG4-related disease. Additionally, elevated levels of serum IgG4 were detected. The patient received intravenous pulse dose steroids and oral steroids, which were tapered gradually, followed by daily hydroxychloroquine treatment and two doses of rituximab every two weeks. Consequently, the patient experienced an improvement in renal function and no longer needed dialysis. To our knowledge, only a few reports of this overlap exist. This late diagnosis of SLE could be explained by the fact that IgG4 is associated with milder renal disease in lupus patients, due to its inability to activate the classical complement pathway. IgG4-RD/SLE overlap patients usually respond well to a combination of steroids and other immunosuppressants used to treat SLE. However, our experience with treating this disease overlap remains limited due to its extreme rarity. Copyright © 2023, Karageorgiou et al.All authors: Acharya I, Karageorgiou I, Karageorgiou S, Pokharel A, Pokharel AFiscal year: FY2023 Digital Object Identifier:

https://dx.doi.org/10.7759/cureus.40664

Date added to catalog: 2023-07-21

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Item type	Current library	Collection	Call number	Status	Date due	Barcode
Journal Article	MedStar Authors Catalog	Article	37366478	Available		37366478

Immunoglobulin G4-related disease (IgG4-RD) in conjunction with systemic lupus erythematosus (SLE) is a rare occurrence. In this case report, we present the case of a 50-year-old female who was diagnosed with SLE based on clinical and laboratory criteria. The patient exhibited pericardial effusion necessitating pericardiocentesis, pleural effusion requiring thoracentesis, and impaired renal function necessitating dialysis. Renal biopsy revealed findings consistent with tubulointerstitial lupus nephritis and IgG4-related disease. Additionally, elevated levels of serum IgG4 were detected. The patient received intravenous pulse dose steroids and oral steroids, which were tapered gradually, followed by daily hydroxychloroquine treatment and two doses of rituximab every two weeks. Consequently, the patient experienced an improvement in renal function and no longer needed dialysis. To our knowledge, only a few reports of this overlap exist. This late diagnosis of SLE could be explained by the fact that IgG4 is associated with milder renal disease in lupus patients, due to its inability to activate the classical complement pathway. IgG4-RD/SLE overlap patients usually respond well to a combination of steroids and other immunosuppressants used to treat SLE. However, our experience with treating this disease overlap remains limited due to its extreme rarity. Copyright © 2023, Karageorgiou et al.

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