Abstract: The patient was a male aged 64 year old with the following symptoms: rash and multiple enlarged lymph nodes. Laboratory examination results showed proteinuria, renal dysfunction, hemolytic anemia, antinuclear antibodies (ANAs) positivity, antidsDNA antibodies (AdsDNA) positivity, hypocomplementemia, and elevated serum IgG4 levels. Renal biopsy showed IgG4positiveplasma cell infiltration in renal interstitium. Based on the clinical and pathological manifestations, the patient was final diagnosed as IgG4 related disease with SLElike manifestations.

Key words: hemolytic anemia, IgG4-related disease, systemic lupus erythematosus