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

Objective. Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of t emporal relationships, has led to skepticism about vasculitis being a paran eoplastic 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. Methods. Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using a n ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients w ith known chronic autoimmune disease or serologic evidence of hepatitis B o r C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of i llness. Results. During the 18.5 years of our study, more than 25 million inpatient s and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patien ts had vasculitis independent of cancer, more than 69,000 patients had canc er, and 69 patients had been identified who had both malignancies and syste mic vasculitis. Only 22 patients were identified in whom both vasculitis an d cancer occurred within the same 22 months. Mean age was 65 years (range 4 5-79). There was no gender preference (M = F). In 8 of the 12 cases, diagno ses were made within 3 months of each other. In 6 of the patients, the diag noses of both processes were made within 2 month. Ten of the 22 patients ha d vasculitis 1 to 3 months prior to or concurrent with the diagnosis of can cer. Six of the 22 patients had solid organ tumors, 4 had lymphoma, 2 had l eukemia, and 2 had multiple myeloma. The most common vasculitis was cutaneo us leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides include d giant cell arteritis In = 2), polyarteritis nodosa (n = 2), and Wegener's granulomatosis (n = 1). The response of the vasculitis to glucocorticoid a nd cytotoxic therapy varied. Complete remission of vasculitis occurred in 4 of the patients, partial improvement occurred in 4 patients, and no improv ement was noted in 4 patients. Complete remission occurred in 3 of the 4 pa tients in whom vasculitis and cancer were treated concurrently. Eight of 10 patients in whom followup was greater than 2 months demonstrated concordan ce of disease activity and treatment response for both cancer and vasculiti s. Conclusion. The close temporal relationship of cancer and vasculitis in our patients adds to circumstantial evidence of vasculitis at times being a pa raneoplastic condition. Failure of a vasculitis to respond to conventional therapy should raise questions about underlying malignancy Effective treatm ent of the cancer enhances the likelihood of improvement in vasculitis.