Concurrent temporal (giant cell) arteritis and malignancy: report of 20 patients with review of the literature.

Objective: To determine the frequency of occurrence of malignancy concurrently with temporal arteritis (TA), as well as features and outcome of the vasculitis in such cases.

Methods: In a series of 271 consecutive patients with TA (219 biopsy-proven), we retrospectively analyzed the frequency and type of malignancy concurrent with vasculitis (less than 1 year before or after), as well as the main features and outcome of TA in this setting. We also surveyed all cases published in the French-British literature.

Results: We observed 20 patients with TA and concurrent malignancy and reviewed 27 similar published reports. GCA was documented pathologically in 86% of the cases. The time between diagnosis of TA and that of malignancy averaged 3.5 months (synchronous diagnoses in 27 patients). Various locations of cancers were found, particularly the gastrointestinal tract (9 cases); blood malignancies accounted for 45% of cases (lymphoid disorder in 9, myelodysplastic syndrome in 11, chronic myelogenous leukemia in 1). In our patients, logistic regression analysis failed to demonstrate differences between those with and without malignancy, except for a higher frequency of rheumatic involvement in the former group (60% vs 30%; p = 0.01). The initial response to steroid treatment was good in 92% of 40 assessable patients, and the vasculitis course mirrored that of malignancy in only 2 patients. Regarding the outcome of TA, no differences were observed in our patients with and without malignancy.

Conclusion: Concurrent malignancy in TA is not a rare finding, being observed in up to 7.4% of the cases. Solid malignancies and hematological disorders, especially myelodysplastic syndromes, may represent precipitating factors for development of TA, which infrequently run a paraneoplastic course. Patients with and without malignancy seem almost indistinguishable regarding features and outcome of TA. Physicians who care for patients with TA should be mindful of this potential association, even in typical cases.