Temporal Arteritis with Occult Malignancy: Co-existence or Causal Relationship?
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To obtain the answers, we searched MEDLINE and PubMed for published literature.
A study published in 1999 reviewed MEDLINE data for literature dealing with cancer-associated rheumatic syndromes.1 The authors found that certain rheumatic syndromes have a definite association with underlying malignancy, such as asymptomatic polyarthritis presenting in the elderly with explosive onset, rheumatoid arthritis, Sjögren’s syndrome, hypertrophic osteoarthropathy, dermatomyositis, polymyalgia rheumatica (PMR) with atypical features, Lambert-Eaton myasthenic syndrome, palmar fasciitis and arthritis, eosinophilic fasciitis poorly responsive to steroids, erythema nodosum lasting more than 6 months, and onset of Raynaud’s phenomenon or cutaneous leukocytoclastic vasculitis over age 50 years. Second, they found substantial evidence that certain longstanding rheumatic syndromes, especially rheumatoid arthritis, Felty’s syndrome, Sjögren’s syndrome, dermatomyositis, systemic lupus erythematosus, and TA, could behave like premalignant conditions. Third, presence of mono- clonal gammopathy in rheumatoid arthritis and monoclonal antibodies 17-109 in Sjögren’s syndrome are reliable signs of malignant transformation. In conclusion, the authors suggested that the presence of specific rheumatic syndromes and certain clinical and laboratory findings may justify a work-up for hidden malignancy.
A retrospective study among 111 patients (89 PMR, 14 TA, 8 PMR + TA) from the Netherlands found that malignancies were diagnosed in 12 patients.2 Most malignancies were diagnosed long before or after the diagnosis of PMR. Another prospective, controlled study from Norway among 185 patients with PMR and TA and 925 matched controls between 1978 and 1983 followed patients up to 1987 and cross-checked data with registry files.3 Malignancy was registered in 16 (24.6%) patients with biopsy-proven TA.3 The hazard rate for developing malignancy in patients with biopsy-proven TA was 2.35 times higher than controls and 4.40 times higher than the rest of the patient population. The long interval between diagnosis of PMR and TA and registration of malignancy (mean, 6.5 years) was not consistent with a paraneoplastic mechanism.3
Isolated case reports of association of TA with various malignancies have been reported. The malignancies described in association with TA include pituitary tumor,4 adenocarcinoma lung,5 temporal lobe meningioma,6 carcinoma breast and carcinoma lung (2 cases),7 carcinoma cervix uterus, acute myeloid leukemia (3 cases),8,9 multiple myeloma,10,11 myelodysplastic syndrome, and chronic myeloid leukemia (2 cases).12
A 2002 population-based study from Norway studied patients with PMR and TA and matched controls for association with malignancy.13 No differences were found in frequencies or types of malignant neoplasms between patients with PMR or TA and population controls.
The exact etiopathologic relationship between inflammatory mechanism of TA and neoplastic proliferation of malignancy remains unclear. A study suggested that the PMR and TA-like picture could either be a direct consequence of tumor, effect of cancer therapy, or some other mechanism.14 Finally, it should be noted that, in general, both malignancies and TA occur at greater frequency during later life and are both considered to be age-prevalent illnesses. It is possible that the findings in this patient may not represent anything more than this age-related increase.
Several studies have been published that establish the association of TA and PMR with malignancy. Cases of TA have been described to have an underlying malignancy that may occur simultaneously or several years later. The rheumatic manifestations of malignancy include a wide spectrum of osteoarticular, muscular, glandular, endocrinologic, or systemic features that could be similar to TA and PMR and thereby pose a diagnostic challenge.
1. Naschitz JE, Rosner I, Rozenbaum M, et al. Rheumatic syndromes: Clues to occult neoplasia. Semin Arthritis Rheum 1999;29(1):43-55.
2. Mertens JC, Willemsen G, Van Sasse JL, et al. Polymyalgia rheumatica and temporal arteritis: A retrospective study of 111 patients. Clin Rheumatol 1995;14(6):650-655.
3. Haga HJ, Eide GE, Brun J, et al. Cancer in association with polymyalgia rheumatica and temporal arteritis. J Rheumatol 1993;20(8):1335-1339.
4. Papaioannou CC, Trautmann JC, Kazmier FJ, Hunder GG. Case report: Association of giant cell arteritis and pituitary tumor. Report of two cases. Am J Med Sci 1979;277(1):85-90.
5. Lie JT. Simultaneous clinical manifestations of malignancy and giant cell temporal arteritis in a young woman. J Rheumatol 1995;22(2):367-369.
6. Schattner A, Green L, Bentwich Z, Lifschitz B. Temporal lobe meningioma masked by polymyalgia rheumatica. Isr J Med Sci 1985;21(5):441-444.
7. Bachmann LM, Vetter W. Pitfalls in diagnosis of polymyalgia rheumatica/ temporal arteritis. Schweiz Rundsch Med Prax 2000;89(20):879-884.
8. Suzuki N, Ushiyama O, Ohta A, Yamaguchi M. Occurrence of malignancy in patients with biopsy-proven temporal arteritis. Nihon Rinsho Meneki Gakkai Kaishi 1995;18(1):104-109.
9. Warrington KJ, Scheithauer BW, Michet CJ. Acute myeloid leukemia associated with necrotizing temporal arteritis. J Rheumatol 2003;30(4):846-848.
10. Estrada A, Stenzel TT, Burchette JL, Allen NB. Multiple myeloma-associated amyloidosis and giant cell arteritis. Arthritis Rheum 1998;41(7):1312-1317.
11. Gelber M. Polymyalgia rheumatica as the presenting symptom of multiple myeloma. Harefuah 1995;128(9):544-545.
12. Espinosa G, Font J, Munoz-Rodriguez FJ, et al. Myelodysplastic and myeloproliferative syndromes associated with giant cell arteritis and polymyalgia rheumatica: A coincidental coexistence or a causal relationship? Clin Rheumatol 2002;21(4):309-313.
13. Myklebust G, Wilsgaard T, Jacobsen BK, Gran JT. No increased frequency of malignant neoplasms in polymyalgia rheumatica and temporal arteritis. A prospective longitudinal study of 398 cases and matched population controls. J Rheumatol 2002;29 (10):2143-2147.
14. Avina-Zubieta JA, Enkerlin HL, Galindo-Rodriguez G. Rheumatic manifestations of malignancy. Curr Opin Rheumatol 1996;8(1):47-51.