Polymyalgia Rheumatica Temporal Arteritis and Occurrence of Malignant TYimors

Jozef Rovensky and Alena Tuchynova

Research Institute of Rheumatic Diseases, Piest'any, Slovak Republic

Polymyalgia rheumatica (PMR) and temporal arteritis (TA) are clinical syndromes characterized by their onset at advanced age. Little is known about the etiopathogenesis of these two nosological units. TA has recently been suggested to be an autoimmune syndrome brought on as a consequence of the immune response of the body against antigens localized in the walls of certain vessels [1]. Peptides of elastin are considered to be among the presumed targets of the autoimmune reaction [2],

The clinical picture of these syndromes is varied and thus their diagnosis rather difficult, due also to the fact that, unequivocal diagnostic tests are still lacking, which applies particularly to PMR. Differential diagnosis requires the exclusion of several other diseases with a symptomatology similar to that of PMR or TA. These include primarily infections and tumors, whose rate keeps increasing with advancing age, and they are frequently manifested as polymyalgia-like syndrome. Naschitz et al. [3] studied the incidence of cancer in 47 patients with PMR over a period of ten years. In five of these patients, polymyalgia-like syndrome was discovered 1-3 months before malignancy was diagnosed. In all these patients, scintigraphic examination detected metastases localized in bones and joints, while the primary tumor was in the lungs (1 patient), kidneys (1 patient), colon (2 patients), and in one patient the localization of the primary tumor could not be established. An interesting observation in this series was the atypical course of the polymyal-gic syndrome, which differed from classical PMR by the onset of complaints before the age of 50 years, by affecting only one typical site, asymmetrically affecting typical localizations, by pain in the joints and by partial or delayed effect of prednisone on the relief of symptoms. The authors assumed that patients with an atypical course of PMR are at a higher risk of having developed a malignancy metastasizing into bones or articulations.

On the other hand, cases of coexistence of PMR and/or TA with tumor diseases have also been reported. The interval between the manifestation of PMR and TA and diagnosis of malignancy was sufficiently long for the polymyalgic syndrome not to be considered a paraneoplastic one. As early as in 1969, Mackenzie [4] described the development of malignancy in one subject of a series of 76 patients with PMR. Several papers have appeared since addressing the potential association between PMR and/or TA and the incidence of malignant tumors (Table 1). Presumably the most detailed study was published by Haga et al. [5] who investigated the incidence of tumor diseases in 185 patients with PMR and/or TA in a prospective study covering the years 1978-1983. A series of 925 subjects randomly selected from the Central Population Registry of Norway served as controls. The data obtained from the patients and from the control subjects were compared with data from the Cancer Registry of Norway. By the end of the 5-year study, malignancy was established in 27 patients (14.6%) with PMR and/or TA and in 131 subjects (14.2%) from the control group. A higher occurrence rate of malignant tumor diseases was recorded in 16 patients with histologically verified TA (24.6%). In this subgroup of patients, the risk of developing malignancy was 2.25 times higher than in the control group and 4.4 times higher compared to the other patients with PMR and TA. In 13 patients of the series,

Table 1. Survey of published papers about the incidence of malignant tumors in patients with PMR and TA

Author [ref.]

No. of patients


No. of patients with malignancy

Localization/ type of tumor(s)

Kalra & Delamere [6]


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