Temporal concurrence of vasculitis and cancer: a report of 12 cases

Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease.

Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis B or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness.

During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell arteritis (n = 2), polyarteritis nodosa (n = 2), and Wegener's granulomatosis (n = 1). The response of the vasculitis to glucocorticoid and cytotoxic therapy varied. Complete remission of vasculitis occurred in 4 of the patients, partial improvement occurred in 4 patients, and no improvement was noted in 4 patients. Complete remission occurred in 3 of the 4 patients in whom vasculitis and cancer were treated concurrently. Eight of 10 patients in whom followup was greater than 2 months demonstrated concordance of disease activity and treatment response for both cancer and vasculitis.

The close temporal relationship of cancer and vasculitis in our patients adds to circumstantial evidence of vasculitis at times being a paraneoplastic condition. Failure of a vasculitis to respond to conventional therapy should raise questions about underlying malignancy. Effective treatment of the cancer enhances the likelihood of improvement in vasculitis.