IgG4-related kidney disease: experience from a Mexican cohort

• Published in: Clinical rheumatology Vol. 39; no. 11; pp. 3401 - 3408
• Main Authors: Martín-Nares, Eduardo, Hernandez-Molina, Gabriela, Rodríguez-Ramírez, Sonia, Rivera-Fuentes, Lemuel, Niño-Cruz, José Antonio, Núñez-Abreu, Alicia, Espinosa-González, Ricardo, Uribe-Uribe, Norma Ofelia
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.11.2020; Springer Nature B.V
• Subjects: Adult; Biopsy; Brief Report; Computed tomography; Creatinine; End-stage renal disease; Female; Glucocorticoids; Hematuria; Humans; Hypocomplementemia; Immunoglobulin G; Immunoglobulin G4-Related Disease - diagnosis; Kidney - diagnostic imaging; Kidney diseases; Laboratories; Lesions; Male; Medicine; Medicine & Public Health; Middle Aged; Nephritis; Nephritis, Interstitial; Patients; Phenotypes; Proteinuria; Remission; Rheumatoid factor; Rheumatology; Serum levels; Hypocomplementemia; Membranous glomerulonephritis; IgG4-related disease; Tubulointerstitial nephritis; IgG4-related kidney disease
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-020-05135-y

To evaluate the clinical/serological phenotype and outcomes of IgG4-related kidney disease. Case series of IgG4-related kidney disease from a cohort of 69 patients with IgG4-related disease. We defined kidney involvement as the presence of at least one of the following conditions: (A) laboratory parameters of kidney injury (proteinuria and/or elevated creatinine levels and/or hematuria); and/or (B) contrast-enhanced computed tomography features (multiple low-density lesions and/or nephromegaly and/or hypovascular solitary mass and/or renal pelvic lesion and/or perinephric lesions). We identified 17 patients with kidney involvement (24.6%), with a mean age of 53.6 ± 11.3 years; thirteen (76.5%) were male. Six patients fulfilled the laboratory criteria, six the imaging criteria, and five both. Five patients had a renal biopsy, the main histopathological diagnosis being IgG4 tubulointerstitial nephritis. Sixteen patients received glucocorticoids and 12 also immunosuppressors and/or biologics. Sixteen patients presented either total or partial renal remission at a median follow-up of 26 months, while one patient developed end-stage renal disease. Patients with kidney disease, as opposed to patients without kidney involvement, had a higher number of involved organs, higher IgG4-related disease responder index and IgG4 and IgG1 serum levels, higher prevalence of rheumatoid factor, and lower C3 and C4 levels. Our study emphasizes the systemic nature of IgG4-related disease, highlighting that renal involvement is usually present in a subset of patients with multisystemic disease, high IgG1 and IgG4 levels, and hypocomplementemia. Key Points • IgG4-RKD presents at a younger age in Mexican mestizo patients . • IgG4-RKD presents with proteinuria and kidney injury or as an asymptomatic imaging finding . • IgG4-RKD presents in the context of multisystemic disease, hypocomplementemia, and high IgG1 and IgG4 levels .