Gynecologic vasculitis (GynV) has been reported as part of systemic vasculitis (SGynV) and as single-organ (isolated gynecologic) vasculitis (IGynV). In the current study, we analyzed the clinical and histologic characteristics of patients with GynV and sought to identify features that differentiate the isolated from the systemic forms of the disease. We used pathology databases from our institution and an English-language literature search (PubMed) to identify affected patients with biopsy-proven GynV. Using a standardized format for data gathering and analysis, we recorded clinical manifestations, laboratory and histologic features, and surgical and medical therapies. Patients were analyzed as 2 subsets: IGynV and SGynV.A total of 163 patients with GynV were included (152 from the literature and 11 from the Cleveland Clinic pathology database). The incidence of vasculitis among all gynecologic surgeries in our institution over 16 years was 0.15%. Half of the patients presented with vaginal bleeding. Other less common presentations included the finding of an asymptomatic abdominal mass, uterine prolapse, atypical cervical smear, and pelvic pain. Constitutional and musculoskeletal symptoms were reported in 24% of patients. One hundred fifteen (70.6%) patients had IGynV, and 48 (29.4%) had SGynV. Compared to patients with SGynV, those with IGynV were younger (median age, 51 yr; range, 18-80 yr vs. median, 68 yr; range, 32-83 yr; p = 0.0001) and presented more often with vaginal bleeding (57% vs. 25%; p = 0.0002) and less frequently with asymptomatic pelvic masses (6% vs. 35%; p = 0.0001). IGynV was less often associated with constitutional or musculoskeletal symptoms (7% vs. 74%; p = 0.0001). Patients with IGynV were much less likely to have abnormal erythrocyte sedimentation rates (26% vs. 97%; p = 0.0001) and anemia (17% vs. 80%; p = 0.0001) than patients with SGynV. None of the patients with IGynV received corticosteroids, whereas almost all patients with SGynV received corticosteroids and about one-third also received cytotoxic therapy. In IGynV, the site most often involved was the uterus, particularly the cervix, whereas in SGynV lesions were more often multifocal, affecting mainly ovaries, fallopian tubes, and myometrium. Nongranulomatous inflammation occurred in most patients with IGynV, while the predominant histologic pattern noted in SGynV was granulomatous.While vasculitis was the only lesion in 32% of the resected specimens, leiomyomas (18.4%) and endometrial carcinoma (8.3%) were the most frequent concomitant benign and malignant (nonvasculitic) lesions, respectively. Except for benign ovarian abnormalities, which were more frequent in SGynV than in IGynV (21% vs. 4%; p = 0.001), other benign (50%) and malignant (18%) conditions were similarly present in both groups. Among SGynV patients, giant cell arteritis was diagnosed in 29 of the 48 (60.4%) patients, and one-third presented without symptoms of vascular involvement or polymyalgia rheumatica. In summary, GynV is rare and most often occurs as a single-organ disease. It is usually an incidental finding in the course of surgery. The isolated form is associated with the absence of systemic symptoms and normal acute phase reactants, and does not require systemic therapy. Among systemic vasculitides, giant cell arteritis is the most frequently reported form of systemic vasculitis with gynecologic involvement.