IgG4-related kidney disease: experience from a Mexican cohort

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IgG4-related kidney disease: experience from a Mexican cohort Eduardo Martín-Nares 1 & Gabriela Hernandez-Molina 1 & Sonia Rodríguez-Ramírez 2 & Lemuel Rivera-Fuentes 2 & José Antonio Niño-Cruz 2 & Alicia Núñez-Abreu 3 & Ricardo Espinosa-González 3 & Norma Ofelia Uribe-Uribe 3 Received: 10 December 2019 / Revised: 22 April 2020 / Accepted: 1 May 2020 # International League of Associations for Rheumatology (ILAR) 2020

Abstract 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.

Keywords Hypocomplementemia . IgG4-related disease . IgG4-related kidney disease . Membranous glomerulonephritis . Tubulointerstitial nephritis

Introduction

* Norma Ofelia Uribe-Uribe [email protected] 1

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Belisario Domínguez Sección XVI, ZC 14080 Mexico City, Mexico

2

Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Belisario Domínguez Sección XVI, ZC 14080 Mexico City, Mexico

3

Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutric