Granulomatosis with polyangiitis with primary vaginal involvement and ureteral obstruction: A case report and systematic review of the literature

Background: Granulomatosis with polyangiitis (GPA) is a vasculitis with multi-organ involvement. Some cases of gynecologic involvement have been described, but there are no systematic reviews in the literature. Objectives: to provide a systematic review of the literature of GPA patients with gynecological involvement. Search Strategy: PubMed and Embase, were searched from inception to July 2012 for GPA patients with gynecological involvement Medical Subject Headings (MeSH) and free-text terms. Selection Criteria: Patients with GPA and primary gynecologic involvement, and/or genital recurrence. Exclusion criteria were other language, review articles, pregnancy, fertility or male patients. Data Collection and Analysis: Data were extracted on clinical evolution, symptoms, examinations findings, diagnosis delay, treatment, outcome, patient status, and follow-up. The main limitation of the studies was the high heterogeneity, which resulted in a narrative review and could lead to publication bias. Main Results: Seventeen studies included data from patients with GPA and primary or relapsed gynecological involvement. In GPA patients, the genitourinary system is affected in <1%. 61% of the authors of this review thought the patient had cancer. The main gynecological symptom is bleeding, but exclusive gynecologic symptomatology is rare (ENT: 63%, lungs: 44%, kidneys-urinary tract: 53%). GPA could affect all areas of the genital tract, but the most frequent location is the uterine cervix. Medical treatment for GPA is effective. Conclusions: GPA of the female genital tract must be considered when biopsies of an ulcerated malignant-appearing cervical or vaginal mass are negative for malignancy even when they are unspecific. Rheumatology consultation is indicated.