A Case of Simultaneous Onset of Highly Active Systemic Lupus Erythematosus and IgG4-Related Renal Disease

    January 2022 in “ Modern Rheumatology Case Reports
    Yuya Fujita, S. Iwata, Kazuhisa Nakano, Shingo Nakayamada, Yusuke Miyazaki, Akio Kawabe, Hiroko Korekoda-Yoshinari, Aya Nawata, Yoshiya Tanaka
    TLDR Renal histology is crucial for diagnosing simultaneous SLE and IgG4RD.
    A 73-year-old woman experienced hair loss, purpura, and numbness for 1 year and was diagnosed with systemic lupus erythematosus (SLE) and IgG4-related disease (IgG4RD) based on various clinical and histological findings. She had high antinuclear antibodies, low white blood cell count, positive anti-cardiolipin and anti-ds-DNA antibodies, lupus retinopathy, high serum IgG4 levels, and renal histology showing endocapillary proliferative glomerulonephritis and storiform fibrosis. Treatment with high-dose glucocorticoids, hydroxychloroquine, and belimumab improved her symptoms. This case highlights the importance of renal histology in diagnosing simultaneous onset of SLE and IgG4RD.
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