Introduction

Vasculitides have been linked to several processes, including infections, drugs and neoplastic disease. Their occurrence in association with malignancies is a rare, and therefore less known, phenomenon. Because of its rarity, this phenomenon has been described mostly in case-reports and small series. In these reports, two categories can be distinguished based on the different underlying pathogenetic processes. (1) Paraneoplastic vasculitides. These are usually considered as vasculitis-associated malignancies. The development of vasculitis is thought to be induced by distant tumor via mediators. The paraneoplastic vasculitides may precede (as a rule by no more than two years), appear concomitantly with, or follow the diagnosis of cancer. The clinical course of a paraneoplastic vasculitis usually parallels that of the tumor. Thus, the cure of the neoplasia is usually, but not invariably, accompanied by regression of the paraneoplastic disorder [1], Conversely, recurrence of vasculitis several years after diagnosis of the malignancy can be a sign of relapse of the tumor. Paraneoplastic vasculitides are usually classified as secondary vasculitides to distinguish them from the primary vasculitides. Nonetheless, they may fulfill the classification criteria for the primary vasculitides. Screening procedures for cancer should therefore be included in the diagnostic procedure for primary vasculitides to exclude an underlying malignant disease. Most cases of vasculitides associated with malignancy have been reported to occur simultaneously with, or a few months before or after, diagnosis of the underlying malignancy. Cutaneous leukocytoclastic vasculitis is the most common type of vasculitis in patients with malignancy [2, 3], (2) Malignancies occurring several years after onset of vasculitis. Cryoglobulinemic vasculitis may be the best example for this group. It is now widely accepted that cryoglobulinemic vasculitis is usually associated with hepatitis C virus. After a latency period of several years, hepatitis C virus can induce malignant lymphoma. The basic defect underlying the disorders in this category may be a deficiency in suppressor T-cell function, which allows unopposed proliferation of B lymphoid cells and the subsequent transition from polyclonal lymphoid hyperplasia to monoclonal proliferative disease. Such a transition may take some years to develop. Thus, the interval between the diagnosis of vasculitis and the diagnosis of the associated malignant disease can amount to several years. Cryoglobulinemic vasculitis is sometimes regarded as a benign lymphoproliferative disease because the cryoglobulins are produced as a result of monoclonal or polyclonal B lymphocyte proliferation. The benign lymphoproliferationmay evolve into malignant non-Hodgkin's lymphoma [4].

These two categories of pathogenetic association between the two diseases have to be distinguished from three other groups mimicking such an association: (1) pseudovasculitides (malignancies mimicking vasculitis); (2) pseudomalignancies (vasculitides mimicking malignancy); and (3) vasculitides as adverse reactions to anticancer therapy in patients with neoplasm (Table 1).

Table 1. Categories of vasculitides associated with malignant disease

Pathogenetic association

Processes mimicking and association

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