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IgG4-related kidney disease: experience from a Mexican cohort

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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.

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Authors and Affiliations

  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

    Eduardo Martín-Nares & Gabriela Hernandez-Molina

  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

    Sonia Rodríguez-Ramírez, Lemuel Rivera-Fuentes & José Antonio Niño-Cruz

  3. Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Belisario Domínguez Sección XVI, ZC 14080, Mexico City, Mexico

    Alicia Núñez-Abreu, Ricardo Espinosa-González & Norma Ofelia Uribe-Uribe

Authors
  1. Eduardo Martín-Nares
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  2. Gabriela Hernandez-Molina
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  3. Sonia Rodríguez-Ramírez
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  4. Lemuel Rivera-Fuentes
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  6. Alicia Núñez-Abreu
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  8. Norma Ofelia Uribe-Uribe
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Correspondence to Norma Ofelia Uribe-Uribe.

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We obtained approval from the Institutional Review Board and the study complies with the Declaration of Helsinki. Patients gave written informed consent.

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Martín-Nares, E., Hernandez-Molina, G., Rodríguez-Ramírez, S. et al. IgG4-related kidney disease: experience from a Mexican cohort. Clin Rheumatol 39, 3401–3408 (2020). https://doi.org/10.1007/s10067-020-05135-y

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  • DOI: https://doi.org/10.1007/s10067-020-05135-y

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