• Jie Du, Hongyue Wang, Lili Zhang, Hongyu Li, Shuang Li, Chao Zhang, Fangfang Sun, and Lirong Zhao.
• Department of Electrical Diagnosis, the First Bethune Hospital of Jilin University, Changchun, China.
• Medicine (Baltimore). 2023 Jul 21; 102 (29): e34218e34218.

BackgroundThe anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are a collection of relatively rare autoimmune diseases characterized by the presence of ANCAs, predominantly against myeloperoxidase and proteinase 3. Multiple organs and systems are involved, but superficial lymph node involvement is rarely reported.Case PresentationA 31-year-old woman initially presented with bilateral axillary lymphadenopathy and that the hilums were not clear. We report the rare case of a patient who presented with an ANCA-associated systemic vasculitis whose initial manifestation was axillary lymphadenopathy. The axillary lymph node needle biopsy specimens had reactive hyperplasia. One year later, the bilateral inguinal lymph nodes had similar morphological and structural changes, and laboratory test results showed renal insufficiency. A renal biopsy revealed the presence of sclerotic glomeruli, crescentic glomeruli, and fibrous crescentic glomeruli, but no deposition of immunocomplex or complement. Finally, the patient was treated with prednisone and mycophenolate mofetil. As the laboratory indicators normalized, so did the sizes of the axillary lymph nodes. A subsequent laboratory examination showed that in addition to urine protein all indicators had normalized, ultrasonography showed slight enlargement of unilateral axillary lymph nodes and normal hilum structure.ConclusionsSuperficial lymphadenopathy is very rare in ANCA-associated systemic vasculitis. Studying this case improves our understanding of the initial manifestations of ANCA-associated vasculitis and may help provide accurate early diagnosis, thus allowing timely treatment and improved patient prognosis.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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