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PubMed2006

Polymyalgia rheumatica and temporal arteritis: the endocrine relations and the pathogenesis. Review.

Polymyalgia rheumatica (PMR) and giant cell or temporal arteritis (TA) are related chronic inflammatory conditions which typically affect the aged subjects. Typical features of PMR include general symptoms of inflammation as well as severe muscle pain in shoulder and pelvic girdle. The manifestation of TA depends on localization of affected artery, usually branch vessels of the aortic arch. Etiology of PMR and TA remains unclear. The association with HLA system and characteristics of the inflammatory response are discussed in this paper. Age related changes in neuroendocrine system could also represent a pathogenic factor in genetically disposed individuals. Complex bi-directional neuroendocrine-immune relations are further modified by ongoing chronic inflammation. Good clinical response to glucocorticoids in PMR patients supports the assumption that the actual levels of cortisol are lower than would be expected during ongoing inflammation. Moreover, decreased levels of adrenal androgens have been observed in PMR and thus possible adrenal androgens supplementation during glucocorticoid treatment sketches new prospects for the treatment of PMR and TA.

Aged↗
PubMedDate not supplied

TNF-alpha blockade induce clinical remission in patients affected by polymyalgia rheumatica associated to diabetes mellitus and/or osteoporosis: a seven cases report.

Polymyalgia rheumatica (PMR) is a chronic inflammatory condition of the elderly, characterized by aching and morning stiffness in the cervical region, shoulders and pelvic girdles. A steroid treatment course of 6-24 months is often required, but, due to important side effects, it is troublesome if the PMR patient is also affected by diabetes mellitus (DM) and/or osteoporosis. Aim of our study is to test anti-TNF alpha treatment as a steroid sparing tool in PMR patients affected by DM or osteoporosis. In particular, we hypothesise that TNF alpha blockade can be useful not only in remission maintaining, but also in the induction of clinical remission without corticosteroids in this kind of patients. In a six months follow up, patients had clinical improvement, confirmed by physical medical examination, and a statistically significant reduction in ESR and CRP mean values. Anti-TNF alpha treatment was well tolerated by all patients. These preliminary data suggest than Infliximab can be useful in the treatment of PMR patients, not only for steroid sparing purposes, but also as first line therapy in PMR patients with severe comorbidity, such as diabetes mellitus or osteoporosis.

Aged↗
PubMed2001

Seronegative rheumatoid arthritis in elderly and polymyalgia rheumatica have similar patterns of HLA association.

OBJECTIVE: To examine whether polymyalgia rheumatica (PMR) and late onset rheumatoid arthritis have identical or similar HLA-DRB1 genetic associations. METHODS: Seventy five PMR cases without evidence of giant cell arteritis were available for study. One hundred seven RA cases were investigated, of which 62 had disease onset after age 60 years. All cases were compared with 145 ethnically matched controls. All cases and controls originated from Lugo, NW Spain. HLA-DRB1 typing was performed on DNA samples using PCR based molecular methods. RESULTS: Early onset RA (< or =40 yrs) was strongly associated with DRB1*04 (DRB1*0401 and *0404). In contrast late onset RA (> or = 60 yrs) was not associated with DRB1*04 but was associated with DRB1*01. Stratification of late onset RA cases by rheumatoid factor status revealed that DRB1*01 was only increased in seropositive RA cases. Late onset seronegative RA cases exhibited increased frequency of DRB1*13/*14; this was also observed in PMR cases where coexistence of GCA had been excluded. CONCLUSION: These data indicate that (1) HLA associations with RA differ with respect to age at disease onset; and (2) seronegative late onset RA and "isolated" PMR have a similar HLA-DRB1 association and may have an identical etiological basis.

Adolescent↗
PubMed1993

[Giant cell arteritis and polymyalgia rheumatica presenting as subclavian artery obstruction].

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common diseases in the elderly. The arteritis usually affects medium sized vessels, but large vessel involvement can also occur leading to arm claudication, bruits, loss of pulses and pallor of the upper extremities. The differential diagnosis of large vessel arteritis includes atherosclerosis and Takayasu's disease. Atherosclerosis, which affects patients of similar age to GCA is usually confined to the lower limbs and can be differentiated on the basis of the clinical setting and investigations such as the ESR, arteriography and temporal artery biopsy. Takayasu's arteritis' although histologically and arteriographically indistinguishable from GCA, is predominantly a disease of young women. A patient is described who presented with upper limb ischemia. A clinical examination revealed absence of right radial pulses and presence of murmurs at level of the carotids. The blood pressure was unrecordable in the upper right limb. The ESR was 102 mm/hr and the C-reactive protein was 11.66 mg/dl. A selective arteriography of the aortic arch and its branches revealed a right subclavian artery obstruction with good collateral circulation and a left subclavian artery stenosis. The biopsy of left temporal artery showed a typical GCA in acute stage. Treatment with prednisolone 30 mg/day was started and four weeks later, the ESR had fallen to normal. In addition this case confirms that PMR implies a systemic arteritis.

Arterial Occlusive Diseases↗
PubMed2003

Hypothalamic-pituitary-adrenocortical axis function in patients with polymyalgia rheumatica and giant cell arteritis.

BACKGROUND: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are 2 closely related syndromes affecting elderly people. One of the most striking features of these conditions is the development of the disease in an almost exclusive manner in people older than 50 years. Despite this close association with age, the pathogenic mechanisms that could explain this age-related predisposition are unknown. Aging is accompanied by a number of quantitative and qualitative changes in the endocrine system that may predispose to several pathologic conditions that occur in the elderly. OBJECTIVE: To explore the hypothalamic-pituitary-adrenal axis in PMR and GCA. METHODS: Basal levels of adrenal hormones as well as the response to low-dose adrenocorticotropin hormone (ACTH) were investigated in 20 patients with active untreated disease and compared with levels in 16 healthy age-matched controls. RESULTS: Male patients with active disease had low basal levels of androstenedione compared to the controls. After low-dose ACTH challenge, cortisol and dehydroepiandrosterone reached higher levels in patients than in healthy subjects, indicating that the adrenal gland function was not suppressed. Furthermore, the authors did not find a clear relationship between the levels of acute phase reactants and adrenal hormones in the patient population. CONCLUSIONS: The authors' findings are probably more compatible with the hypothesis that the abnormalities found in the patient group are the consequences of chronic illness rather than a crucial factor contributing to the pathogenesis of the disease.

17-alpha-Hydroxyprogesterone↗
PubMed1987

Breast arteritis in polymyalgia rheumatica.

A 79-year-old woman with the clinical presentation of polymyalgia rheumatica (PMR) also had breast cancer. Histopathologic evidence of arteritis was found in mastectomy tissue and in a temporal artery, neither of which had produced symptoms before surgery. Previously reported cases are reviewed in the context that breast vasculitis may be more common than in generally recognized in patients with PMR.

Aged↗
PubMedDate not supplied

Giant cell arteritis and polymyalgia rheumatica. Review for the otolaryngologist.

Patients suspected of giant cell arteritis or polymyalgia rheumatica are often referred to the otolaryngologist for temporal artery biopsy. These patients may initially present to the otolaryngologist with symptoms referable to the head and neck. A comprehensive review of these two interrelated disorders is provided, with emphasis on head and neck manifestations and features of obtaining the temporal artery biopsy specimen.

Biopsy↗
PubMed1980

The clinical pictures of giant cell arteritis. Temporal arteritis, polymyalgia rheumatica, and fever of unknown origin.

In a prospective study, 68 hospitalized patients were diagnosed as having giant cell arteritis. Temporal artery biopsy was performed in all patients and showed histologic evidence of arteritis in 42 (62%). Twenty-six patients had a negative biopsy but met the clinical criteria for the diagnosis. Four different clinical pictures were recognized. Thirteen patients (19%) had symptoms of localized temporal arteritis without muscular discomfort. The polymyalgia rheumatica syndrome without signs of localized arteritis was seen in 33 patients (49%). Seventeen (25%) had symptoms of both polymyalgia rheumatica and temporal arteritis. Five patients (7%) had general symptoms only, such as fever, anorexia, and fatigue, without muscular or arteritic symptoms.

Aged↗
PubMed1979

Temporal arteritis and polymyalgia rheumatica: two aspects of one disease.

Temporal (cranial, giant cell) arteritis and polymyalgia rheumatica are probably different expressions of the same inflammatory disease. The disease occurs mainly in the elderly and is characterized in the early stage by vague, nonspecific symptoms. Tenderness of the temporal arteries is diagnostic but rarely is present early. Unfortunagely, loss of vision usually occurs before the diagnosis is made. A high index of suspicion and frequent testing of the ESR in elderly patients with vague complaints may lead to earlier diagnosis and appropriate therapy and thus may prevent blindness and even death.

Blindness↗
PubMed1982

In vitro cytotoxicity of human endothelial cells in polymyalgia rheumatica and giant cell arteritis.

We studied sera from 20 patients with polymyalgia rheumatica (PMR)/giant cell arteritis (GCA), 15 patients with systemic lupus erythematosus (SLE), 15 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangietasia) and 33 age and sex matched controls for cytotoxicity against human endothelial cells using a 51Cr release assay. We observed increased levels of endothelial cytotoxic activity in the PMR/GCA sera compared with controls (P less than 0.001). This cytotoxicity was predominantly found in the GCA group, where 7 out of 10 patients (70%) demonstrated significant cytotoxicity. Sequential studies showed that the cytotoxic activity returned to normal when the disease was under control. Although 7 SLE and 3 CREST sera had significant cytotoxic activity, as a group they did not differ from controls (P less than 0.05). Pre-incubation with soybean trypsin inhibitor suppressed the cytotoxic activity in the positive sera suggesting the cytotoxicity is mediated via a protease mechanism. Our results demonstrate the presence of a cytotoxic factor in the serum of patients with PMR/GCA which may play an important role in the pathogenesis of the vascular lesions observed in these disorders.

Aged↗
PubMed1990

Subclavian and axillary involvement in temporal arteritis and polymyalgia rheumatica.

PURPOSE: We describe 10 female patients with temporal arteritis (TA) and/or polymyalgia rheumatica (PMR) who presented with upper-extremity ischemia. PATIENTS, METHODS, AND RESULTS: Arm claudication or Raynaud's phenomenon was the initial manifestation of the disease in four cases, appeared with classical symptoms in one case, or occurred during decreasing corticosteroid therapy in five cases. Temporal artery biopsy was performed in nine patients and showed typical giant-cell granulomatous arteritis in seven cases. Angiograms in all cases showed multiple bilateral smooth stenoses, or obliterations of postvertebral subclavian and/or axillary arteries, or both. Symptoms always improved with corticosteroid treatment and none of the patients required reconstructive surgery, although angiography performed after stabilization did not show revascularization of occluded vessels. CONCLUSION: We conclude that large-artery involvement in TA and PMR affects most commonly the subclavian and axillary arteries, with a female predominance comparable to that in Takayasu's arteritis. Both these disorders should be considered in elderly women with occlusive disease of the upper extremities. Although response to steroid therapy was sufficient in our series to avoid surgery, we believe it is preferable to recognize large-artery involvement as early as possible and recommend performance of ultrasonic Doppler examination when any sign of oncoming ischemia or stenosis is observed.

Aged↗
PubMed1987

Isolation and analysis of immune complexes from sera of patients with polymyalgia rheumatica and giant cell arteritis.

Serum samples were obtained from patients with polymyalgia rheumatica (PMR: n = 10) or giant cell arteritis (GCA; n = 7), or both. Samples were taken either before treatment or within one week of starting prednisolone. Immune complexes (IC) were concentrated by polyethylene glycol (PEG) precipitation then purified with either IgG anti-C1q-Sepharose or IgG anti-C3c-Sepharose. Complex components were separated by sodium dodecyl sulphate (SDS) gradient polyacrylamide gel electrophoresis then transferred to nitrocellulose by Western blotting. Identification of proteins was carried out using specific antisera. All the IC contained IgM (mu chain), some contained IgA (alpha chain), and IgG (gamma chain). C1r, C1s, C1q, C3, C4, and C reactive protein (CRP), where tested, were found in most but not all IC. The occurrence of properdin, factor B, alpha 2 macroglobulin (alpha 2M), factor H (beta 1H), C1 esterase inhibitor, and C4 binding protein was also investigated. Immune complexes in PMR and GCA differed from those previously characterized in rheumatoid arthritis (RA)1 purified by anti-C1q-Sepharose which contained immunoglobulins and C1q only. No properdin or factor B were detected in RA IC purified with either anti-C1q-Sepharose or anti-C3c-Sepharose.

Aged↗
PubMed1999

Giant cell arteritis and polymyalgia rheumatica: are pregnancies a protective factor? A prospective, multicentre case-control study. GRACG (Groupe de Recherche sur l'Artérite à Cellules Géantes).

OBJECTIVE: To assess the potential role of allo-immunization, either by former pregnancies, or by a history of blood transfusion, in the pathogenesis of giant cell arteritis and polymyalgia rheumatica. METHODS: Two hundred and eighty-five incident female cases and 186 age-matched, population-based female controls were prospectively included in a multicentre case-control study. RESULTS: The number of pregnancies was significantly lower in cases than in controls (nulliparous: 21.55% vs 12.90%; > or =4 pregnancies: 16.25% vs 27.42%; Wilcoxon rank sum test: P = 0.0019) in biopsy-proven or negative temporal arteritis and, to a lesser extent, in polymyalgia rheumatica. No difference was found for history of blood transfusion. Pregnancies remained negatively associated with the disease in a multivariate analysis including cardiovascular risk factors such as smoking or a pre-existing peripheral vascular disease. CONCLUSION: Former pregnancies are not a risk factor for giant cell arteritis. Pregnancies may be protective thanks to an effect of the associated hyperoestrogenic state against alterations of the artery wall, as suggested in animal models.

Aged↗
PubMed2002

Renal cell carcinoma mimicking polymyalgia rheumatica. Clues for a correct diagnosis.

Proximal musculoskeletal symptoms mimicking the clinical picture of polymyalgia rheumatica may herald the onset of solid malignancies. We report on three patients presenting with polymyalgia-like symptoms, who had renal cell carcinoma. The review of the literature and our cases suggest that the absence of prolonged morning stiffness, the atypical clinical findings, the inefficacy of corticosteroids, and the absence of shoulder sonographic pathologic findings may help to facilitate the proper diagnosis.

Aged↗
PubMedDate not supplied

Malignant boutonneuse fever and polymyalgia rheumatica: a coincidental association?

Mediterranean spotted fever is a tick-borne disease that is endemic in the Mediterranean basin from spring to autumn. Usually mild, the disease can be severe in some cases, especially when risk factors are encountered in patients or when treatment is delayed. The correlation between these malignant forms and patients' immunological disorders remains unclear, while the pathophysiology of the disease seems well known. A case of a malignant form of Mediterranean spotted fever is reported which occurred 2 months prior to the diagnosis of polymyalgia rheumatica. Evidence of immunological disorders consisted only in an antiphospholipid antibody associated with a transient lupus anticoagulant. No underlying risk factors other than the primary undiagnosed phase of polymyalgia rheumatica has been observed.

Aged↗
PubMedDate not supplied

Von Willebrand factor in polymyalgia rheumatica and giant cell arteritis.

Patients with giant cell arteritis (GCA) and with polymyalgia rheumatica (PMR) have increased plasma levels of von Willebrand factor (vWF) even during corticosteroid therapy. The association between vWF, subclinical inflammation and cardiovascular complications in GCA/PMR remains to be investigated.

Giant Cell Arteritis↗
PubMed1986

Corticosteroids and bone mass in asthma: comparisons with rheumatoid arthritis and polymyalgia rheumatica.

Bone mass has previously been shown to be reduced at peripheral bone sites in patients with bronchial asthma receiving corticosteroids. To assess whether total bone mass is reduced in asthma total body calcium was measured by in vivo neutron activation analysis in patients receiving various treatments for asthma and compared with results from normal controls and patients with rheumatoid arthritis and polymyalgia rheumatica. Compared with controls total body calcium was reduced by 13.6% (p less than 0.001) in patients with asthma receiving daily oral corticosteroids but by only 9.0% (p less than 0.005) in a similar group of patients who had received oral calcium supplements at the start of their corticosteroid treatment. Total body calcium was also reduced in a group of patients receiving only inhaled corticosteroids (8.8%; p less than 0.001) but not significantly reduced in a small group of patients with asthma who had never received these drugs. When compared with controls a group of patients matched for age and for dose of corticosteroids given for rheumatoid arthritis had a similar reduction in total body calcium to the patients with asthma receiving daily oral treatment (17.7%; p less than 0.001), but no such reduction was shown in patients with polymyalgia rheumatica. These findings suggest that the risk of bone loss with low dose oral corticosteroids in similar in asthma and rheumatoid arthritis. Further work is required to assess the clinical relevance of small losses of bone associated with the use of inhaled corticosteroids.

Administration, Oral↗