Isolated vasculitis involving the female genital tract: clinicopathologic spectrum and phenotyping of inflammatory cells.

Isolated vasculitis involving the female genital tract (IVF) is rare. Although both giant cell arteritis (GCA) and polyarteritis nodosa (PAN) types of involvement have been documented, several clinicopathologic features of IVF are not clearly understood. We wish to report two cases of IVF (one GCA and one PAN) and compare them with previously reported cases. Including our two cases, we found a total of 33 reported cases of IVF, which included 18 GCA and 15 PAN. In cases of GCA, all parts of the female genital tract were involved with roughly comparable frequency, whereas in the case of PAN, the cervix is uniformly involved, with the vulva and myometrium being affected once and twice, respectively. In all cases of GCA and most cases of PAN, the vasculitis represented an incidental finding upon microscopic examination of the female genital tract removed for unrelated problems; however, in one case of PAN, vaginal bleeding probably related to vasculitis-induced cervical ulcer was reported. In either category, no clearcut pattern of predisposing factors was identified and, at the time of diagnosis, none of the patients had any diseases known to be associated with vasculitis, such as connective tissue disease, rheumatoid arthritis, or drug hypersensitivity. Although antineutrophilic cytoplasmic antibodies were recently described as sensitive and specific markers for systemic vasculitis, tests for these antibodies were not done on previously reported cases and were negative for both current cases. Long-term follow-up in IVF indicates that even without any specific treatment for vasculitis, systemic involvement did not occur.(ABSTRACT TRUNCATED AT 250 WORDS)