IgG4-related disease: description of a case with pulmonary lesions, mediastinal lymphadenopathies and rapidly progressive renal failure

Nefrologia. 2015;35(2):218-23. doi: 10.1016/j.nefro.2015.05.023. Epub 2015 Jun 23.
[Article in English, Spanish]

Abstract

This is a case report of a 73-year-old man with new-onset acute renal failure while being investigated for pulmonary infiltrates and mediastinal lymphadenopathies. Urine tests showed tubular range proteinuria with no microhaematuria. Immunology tests showed elevated serum IgG and hypocomplementaemia (classical pathway activation). Renal biopsy and clinical-pathological correlation were crucial in this case, reinforcing their important role in the final diagnosis of acute kidney injury.

Keywords: Enfermedad relacionada con IgG4; IgG4 related disease; Immunology; Inmunología; Insuficiencia renal rápidamente; Rapidly progressive renal failure; progresiva.

Publication types

  • Case Reports

MeSH terms

  • Acute Kidney Injury / etiology*
  • Acute Kidney Injury / pathology
  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Autoimmune Diseases / etiology*
  • Autoimmune Diseases / pathology
  • Biopsy
  • Cachexia / etiology
  • Humans
  • Immunoglobulin G / blood*
  • Lung Diseases / etiology*
  • Lymphadenopathy / etiology*
  • Male
  • Mediastinum
  • Paraproteinemias / complications*
  • Paraproteinemias / diagnosis
  • Paraproteinemias / drug therapy
  • Plasma Cells / pathology

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulin G