Malignancy Risk in Vasculitis

Abstract

The vasculitides encompass a rare subset of autoimmune diseases. Reports of the concurrent association of malignancies with some forms of vasculitis raise the possibility that patients with certain types of vasculitis may be at increased risk of cancer. Conversely, some forms of vasculitis may be a manifestation of malignancy. We review cancer risk in patients with large vessel vasculitis (giant cell arteritis and Takayasu arteritis), polyarteritis nodosa, and the circulating antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. In addition we discuss vasculitis as a paraneoplastic phenomenon, highlighting polyarteritis nodosa in association with hairy cell leukemia and reviewing the most common vasculitic manifestation of cancer, cutaneous vasculitis.

Vasculitides associated with haematological malignancies: a case-based review

“…While testing for malignancy in every leukocytoclastic vasculitis (LCV) would not be cost effective, based on our case and other studies,8 20 we suggest that if vasculitis persists despite treatment with steroids or recurs after initial response, it mandates search for malignancy with more complex imaging or invasive techniques.…”

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Learning points

While the exact mechanism still remains elusive, the fact that vasculitis can be a harbinger of hairy cell leukaemia needs to be considered. Patients presenting with vasculitis should be evaluated thoroughly with a history, physical examination and pertinent laboratory studies to exclude aetiologies including malignancy.

While testing for malignancy in every leukocytoclastic vasculitis (LCV) would not be cost effective, based on our case and other studies,8 20 we suggest that if vasculitis persists despite treatment with steroids or recurs after initial response, it mandates search for malignancy with more complex imaging or invasive techniques. Also, as exemplified by our case, if vasculitis is accompanied by atypical features like weight loss and unremitting constitutional signs and symptoms, it should raise a clinician's suspicion for cancer. In addition, physicians involved in the care of patients with cutaneous LCV must ensure adequate age appropriate screening.

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Giant cell arteritis and polymyalgia rheumatica as first manifestation of typical pulmonary carcinoid tumor

“…In biopsy-proven cases, however, GCA has been shown to increase the malignancy risk (13). Taken together, existing data seem to indicate that GCA does not increase the risk of neoplasia (14). On the other hand, reports of GCA in close association with tumors, including lung, breast, cervical, multiple myeloma, and chronic myelogenous leukemia, indicate that GCA may occur as a paraneoplastic disorder (15).…”

Kaposi’s sarcoma concurrent with granulomatosis polyangiitis

“…Vasculitis occasionally develops secondarily to malignancy and may occur in patients with previous or concomitant cancer because of risk factors predisposing to both conditions. In addition, inflammatory responses provoked by the underlying neoplasm contribute to the pathogenesis of malignancy-associated vasculitis (10).…”