An Unusual Presentation of ANCA-Negative Granulomatosis With Polyangiitis (GPA) in the Gynecological Tract and Literature Review

Key messages & BULL; Gynecologic presentations of GPA are rare, especially in the uterus, fallopian tube, ovary, and vagina. & BULL; Diagnosis of GPA is difficult in the context of negative ANCA and unusual organ presentations. & BULL; It is important to maintain a clinical suspicion for GPA in the right clinical context. ObjectiveGranulomatosis with polyangiitis (GPA) is a small and medium vessel vasculitis that generally manifests in the upper and lower respiratory tracts and kidneys but can less frequently be seen in other organ systems. Approximately 5-10% of patients with GPA have negative anti-neutrophil cytoplasmic antibody (ANCA) titers; ANCA is detectable in 90% of patients with systemic GPA (generalized or severe) and 70-80% with limited GPA (localized or non-severe). We report an unusual case of ANCA-negative systemic GPA in the gynecological tract and review the literature.MethodsWe searched Ovid, Medline, and Pubmed using the search terms gyne and vasculitis. ResultsThis 83-year-old woman underwent total abdominal hysterectomy and salpingo-oophorectomy for an adnexal mass, and acutely post-operatively developed hypoxia and hemoptysis with previous leukocytoclastic vasculitis, chronic sinusitis, membranous nephropathy (but not glomerulonephritis), with negative ANCA titers. Pathology showed medium vessel vasculitis in the uterus, cervix, fallopian tubes, and ovaries. We identified 10 case reports of gynecologic involvement of GPA; all had positive ANCAs. Seven involved the cervix alone, one in the cervix and upper vagina, one in the vagina, and one in the cervix and ovary. Six had a known diagnosis of systemic GPA prior to gynecologic presentation, three presented with gynecologic and systemic manifestations, and one had limited GPA in the urethra and vagina.ConclusionThis current case of gynecologic GPA is unique as GPA in the uterus and fallopian tube has not been described and she had a negative ANCA.

Automated summary

Gynecologic presentations of GPA are rare, especially in the uterus, fallopian tube, ovary, and vagina. Diagnosis of GPA is difficult in the context of negative ANCA and unusual organ presentations. It is important to maintain a clinical suspicion for GPA in the right clinical context.