A case of simultaneous onset of highly active systemic lupus erythematosus and IgG4-related renal disease

Mod Rheumatol Case Rep. 2022 Jun 24;6(2):178-182. doi: 10.1093/mrcr/rxac002.


The patient was a 73-year-old woman who had hair loss, purpura, and numbness of the soles for past 1 year. Three months prior, she was diagnosed with interstitial lung disease (ILD) and was admitted to our department. She was diagnosed with systemic lupus erythematosus (SLE) based on positive antinuclear antibodies 1280× (speckled type), hair loss, low white blood cell count, positive anti-cardiolipin and anti-ds-DNA antibodies, and lupus retinopathy. In addition, the patient was also diagnosed with immunoglobulin G (IgG)4-related disease (IgG4RD) based on high serum IgG4 levels, ILD, urine occult blood, protein, and cast, and renal histological findings showed endocapillary proliferative glomerulonephritis, increased IgG4 positive plasma cells, and characteristic storiform fibrosis. High-dose glucocorticoid therapy, hydroxychloroquine, and belimumab were administered, which improved the SLE symptoms of lupus retinopathy and peripheral neuropathy, as well as the IgG4RD symptoms of ILD and urinary findings. Herein, we report a rare case of simultaneous onset of IgG4-related nephropathy with active glomerular lesions and SLE, in which renal histology, including fluorescent antibodies, was crucial for diagnosis.

Keywords: IgG4-related disease; Systemic lupus erythematosus; glomerulonephritis; interstitial nephritis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Alopecia
  • Female
  • Humans
  • Immunoglobulin G
  • Immunoglobulin G4-Related Disease* / complications
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Immunoglobulin G4-Related Disease* / pathology
  • Kidney Diseases*
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / drug therapy
  • Retinal Diseases*


  • Immunoglobulin G