Purpose of review: To provide a critical analysis of a rare disorder, single-organ vasculitis, emphasizing those organs in which the excision of the vasculitic lesion can be curative. To recommend a rational approach to diagnosis, longitudinal follow-up and treatment.
Recent findings: Patients with focal single-organ vasculitis affecting abdominal and genitourinary organs, breast and aorta have been reported as individual cases and small series. Single-organ vasculitis differs from systemic forms of vasculitis in disease expression and prognosis. Occasionally, what appears to be a localized process evolves into a systemic disease. Depending on the organ affected, some clinical, serological and histopathologic features may be helpful in predicting the extent of the vasculitic process. With the exception of severe ischemic or hemorrhagic complications affecting the abdominal organs and dissection or rupture of the aortic arch, the prognosis of focal single-organ vasculitis tends to be excellent. Resection of the inflammatory lesion may be curative.
Summary: The diagnosis of focal single-organ vasculitis is always presumptive and requires exclusion of systemic illness at the time of diagnosis as well as throughout the period of continued care. Clues from clinical symptoms, laboratory tests and histopathologic features at the time of diagnosis may assist in devising surveillance strategies.