• Internal medicine · Nov 2023

    A Diagnostic and Therapeutic Dilemma Concerning Exostosin 1/Exostosin 2-associated Lupus-like Membranous Nephropathy with Positive Anti-nuclear Antibody in an Elderly Man with Various Immune Abnormalities.

    • Mika Kawagoe, Kohei Odajima, Shinichiro Asakawa, Michito Nagura, Shigeyuki Arai, Osamu Yamazaki, Yoshifuru Tamura, Ryuji Ohashi, Takamasa Iwakura, Hideo Yasuda, Shigeru Shibata, and Yoshihide Fujigaki.
    • Department of Internal Medicine, Teikyo University School of Medicine, Japan.
    • Intern. Med. 2023 Nov 1; 62 (21): 318731943187-3194.

    AbstractExostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. The patient exhibited various other immune abnormalities. Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of the Systemic Lupus International Collaborating Clinics (SLICC) 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.

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