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PubMed1981

Prognosis and management of polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is considered to be a benign disease by some, while others think it is a more serious illness which required similar treatment to giant cell arteritis (GCA). The progress of 85 patients with PMR who presented to a district general hospital has been studied in an attempt to study this relationship. Thirty-eight patients had PMR alone, and 14 developed PMR and GCA within 1 month. Five patients presented with GCA and then developed PMR, and 28 patients developed symptoms of GCA after presenting with PMR (PMR leads to GCA). Arteritis and complications developed up to 9 years after the onset of PMR (mean 1 year). Twenty-two patients (26%) developed some cerebral or visual complication. Fifteen of these patients were in the PMR leads to GCA group. All 6 patients with permanent loss of vision were in this group. Seven patients developed complications while on corticosteroids. 97% of patients required corticosteroids for at least 1 year; 32% of patients still required 10 mg of prednisone or more after 1 year. PMR is not a benign disease.

Giant Cell Arteritis↗
PubMed2004

Polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology characterized by aching and stiffness in the shoulder and in the pelvic girdles and neck. In the past, PMR was considered a manifestation of giant cell arteritis (GCA) or a variant of elderly-onset rheumatoid arthritis (EORA). The current diagnostic criteria for PMR were empirically formulated by clinical experts who had studied the disease extensively. Arthroscopic, radioisotopic and magnetic resonance imaging studies all have indicated the presence of a synovitis in proximal joints and periarticular structures. The synovitis is probably responsible for the musculoskeletal symptoms in PMR. The prominence assigned to the proximal symptoms has probably overshadowed the less well recognized and more variable distal musculoskeletal manifestations which are present in about half of the cases. A normal erythrocyte sedimentation rate does not exclude a diagnosis of PMR. C-reactive protein and interleukin-6 seem to be more sensitive indicators of disease activity both at diagnosis and during relapse/recurrence. Corticosteroids are the drugs of choice for treating PMR. A course of treatment of 1-2 years is often required. However, some patients have a chronic, relapsing course and require low doses of corticosteroids for several years. Large, multicenter, double-blind, placebo-controlled studies are required to define the role of methotrexate and anti-TNF-alpha agents as corticosteroid-sparing drugs in PMR.

Aged↗
PubMed1997

Synovial expression of cell adhesion molecules in polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is a common disorder of the elderly: the pathogenesis of the syndrome is still debated, though active synovitis of the shoulder has recently been confirmed. To investigate the pathogenesis of this synovitis we evaluated cell adhesion molecule (CAM) expression in shoulder synovial tissue from patients with PMR, correlated synovial expression with the serum levels of soluble forms, and assessed the changes associated with corticosteroid treatment. Arthroscopic synovial biopsies were obtained from 12 untreated and seven corticosteroid (CS)-treated cases. CAM expression was evaluated by MoAb staining on frozen sections and computerized image analysis. Soluble CAM were quantified by ELISA. Endothelial cells expressed intercellular adhesion molecule-1 (ICAM-1), E- and P-selectins. Infiltrating cells were ICAM-1 and beta1-integrin-positive, while L-selectin expression was limited to intravascular leucocytes. Synovial lining cells strongly expressed vascular cell adhesion molecule-1 (VCAM-1), and less intensely ICAM-1. Only the soluble form of ICAM-1 (sICAM-1) was elevated in untreated patients. CS treatment was associated with a decrease in ICAM-1, VCAM-1 and E- and P-selectin expression. sICAM-1 levels were in the normal range in treated patients. VLA-5 and 6 expression was widely distributed among cell types, and was not CS-sensitive. Active shoulder synovitis is associated with different CAM expression in PMR. ICAM-1 expression is widely distributed and correlates with elevated levels of the soluble form; it is significantly lower in CS-treated asymptomatic cases.

Aged↗
PubMed2006

Diagnosis, differential diagnosis and treatment of polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is a common disorder in the elderly population. The diagnosis is based upon recognition of a clinical syndrome, consisting of pain and stiffness in the shoulder and pelvic girdle, muscle tenderness of the upper and lower limbs and nonspecific somatic complaints. In addition, in most cases the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) concentration are highly elevated. Although PMR and giant cell arteritis (GCA) are commonly regarded as two clinical variations of the same disease, their clinical picture is quite different. Whilst in PMR the musculoskeletal symptoms predominate, the major features of GCA are arterial inflammation and its consequences, which suggests clinical and pathological discrepancies between the two syndromes and important differences with respect to morbidity and mortality. The prognosis of correctly diagnosed PMR is excellent. It is well known that corticosteroid therapy in PMR usually leads to rapid and dramatic improvement of patients' complaints and returns them to previous functional status. However, prolonged corticosteroid treatment, sometimes for several years, may be necessary to maintain clinical improvement. Despite all the knowledge about the beneficial effects of corticosteroid treatment, data concerning the optimal dosage regimen are lacking. Long-term corticosteroid use can be associated with various adverse events, of which induction of osteoporosis, diabetes mellitus and infection among the worst. A Corticosteroid Side Effect Questionnaire has been shown to dose-dependently detect adverse effects perceived by patients. The European League Against Rheumatism (EULAR) response criteria for PMR comprise a core set of markers for monitoring therapeutic responses in PMR, namely ESR or CRP, the visual analogue scale of patient's pain and physician's global assessment, as well as morning stiffness and the ability to elevate the upper limbs. The PMR-disease activity score has been developed on the basis of EULAR response criteria as a means of expressing disease activity as an absolute number. A score <7 indicates low disease activity, scores 7-17 suggest medium activity, and a score >17 is indicative of high disease activity. The PMR-disease activity score has been proven to be highly correlated with patient's global assessment, patient satisfaction and ESR. It provides an easily applicable and valid tool for disease activity monitoring in patients with PMR. Improved knowledge of disease activity processes, exact monitoring of disease activity and treatment responses, and increased risk-estimation of treatment schedules should ultimately improve the care of patients with PMR.

Aged↗
PubMed2002

[Polymyalgia rheumatica revisited].

Polymyalgia rheumatica is an inflammatory disorder that usually affects persons over the age of 50 causing proximal muscle pain and stiffness, and an elevated erythrocyte sedimentation rate. Although increasingly recognized in this age group, it remains a diagnosis of exclusion and although several diagnostic criteria have been proposed, none has been clearly accepted. While polymyalgia rheumatica is associated with giant cell arteritis, obtaining a temporal artery biopsy is not recommended in patients with polymyalgia rheumatica without symptoms of giant cell arteritis. Early diagnosis and low dose corticosteroid therapy improve patients' clinical features and functional status. Treatment usually lasts between 12 and 24 months and the majority of patients manage to discontinue treatment completely.

Administration, Oral↗
PubMed2004

[Polymyalgia rheumatica (arteritica)].

Polymyalgia rheumatica occurs mainly in patients older than 60 years of age. Differential diagnostically, the condition can be identified on the basis of a number of laboratory parameters (ESR, CRP, CK, autoantibodies) and, if the findings are not unequivocal, the additional use of electromyography and muscle biopsy. Some 40-50% of the patients have concomitant giant cell arteritis. Treatment of polymyalgia rheumatica is glucocorticoids are applied, usually for a period of at least one year. The initial and the tapered dose is dependent on whether an associated arteritis is present.

Aged↗
PubMed1999

Corticosteroid requirements in polymyalgia rheumatica.

BACKGROUND: Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of unknown cause that affects older individuals. Clinical symptoms respond promptly to corticosteroids, but treatment is often required for several years, frequently resulting in adverse drug effects. Guidelines for the optimal use of corticosteroids that maximize relief of symptoms but minimize adverse effects of the therapy are needed. OBJECTIVE: To determine whether clinical or laboratory parameters in PMR could be identified that allow for stratifying patients into subsets with differences in corticosteroid requirements. PATIENTS AND METHODS: We studied 27 patients with PMR treated with a standardized schedule of prednisone. Patients were reevaluated at monthly intervals for pain scores and physician and patient assessments. Plasma interleukin 6 level and the erythrocyte sedimentation rate were measured at each visit. The duration of steroid therapy and the cumulative steroid dose were calculated. RESULTS: Based on the initial response to therapy and the duration of disease, the 27 patients could be subdivided into 3 distinct groups. Eight with low erythrocyte sedimentation rates responded rapidly and required corticosteroids for less than 1 year with rare disease flares on tapering of prednisone. Twelve others responded well initially but did not tolerate reduction to lower doses and had remitting disease of more than 1 year. Seven patients had only a partial response to the initial steroid regimen. After 4 weeks of therapy, the erythrocyte sedimentation rates improved, but levels of interleukin 6 remained elevated. Pretreatment pain scores were also higher in these partial responder patients (P = .05). CONCLUSIONS: Polymyalgia rheumatica is a heterogeneous disease with variations in the treatment duration and dose of corticosteroids required for suppression of symptoms. Pretreatment erythrocyte sedimentation rate and nonresponsiveness of interleukin 6 to steroid therapy are helpful in dividing patients into subsets with different treatment requirements.

Acute Disease↗
PubMed1997

Molecular analysis of HLA-DR polymorphism in polymyalgia rheumatica. Swiss Group for Research on HLA in Polymyalgia Rheumatica.

OBJECTIVE: To analyze by molecular typing possible associations of HLA-DRB1 alleles with polymyalgia rheumatica (PMR) compared to controls and patients with rheumatoid arthritis (RA) in Switzerland. METHODS: In a multicenter survey, we recruited 100 patients with PMR with and without signs of giant cell arteritis (GCA), 198 with RA, and 200 controls (volunteer bone marrow donors). HLA-DR generic typing was performed by microtiter plate oligotyping and DR4 subtypes analyzed by dot blot hybridization with sequence specific oligonucleotides or by polymerase chain reaction sequence specific primers. RESULTS: DR4 and DR1 tended to be increased in PMR, compared to controls (36 vs 30%, p = 0.30; and 19 vs 12%, p = 0.16, respectively). Frequencies of all RA associated DR4 and DR1 subtypes tended to be increased in PMR as well. Frequency of the HLA-DR beta 1 70-74 shared motif (QK/RRAA) was significantly higher in PMR than in controls [50 vs 36%, odds ratio (OR) = 1.8, p = 0.018], although lower than in RA (77 vs 36%, OR = 6.0, p < 0.0001), and slightly out of the range of significance if a Bonferroni correction was applied (p = 0.1). At double dose, this epitope was also increased in PMR, but not significantly (5 vs 2%, OR = 2.6, p = 0.17), while it was markedly augmented in RA (22 vs 2%, OR = 14, p = < 0.0001). In patients with the shared epitope, the frequency of clinical signs of GCA tended to be increased (19 vs 10%, p = 0.25). Frequency of the HLA-DR beta 1 DRYF 28-31 motif was identical in PMR (95%) and controls (93%). CONCLUSION: PMR may be associated with the HLA-DR beta 1 70-74 shared epitope. This association, however, would be much weaker for PMR than for RA, particularly with the shared epitope at double dose. PMR is clearly not associated with the HLA-DR beta 1 DRYF 28-31 motif.

Aged↗
PubMed2003

EULAR response criteria for polymyalgia rheumatica: results of an initiative of the European Collaborating Polymyalgia Rheumatica Group (subcommittee of ESCISIT).

OBJECTIVE: To develop response criteria for polymyalgia rheumatica (PMR) for monitoring treatment and comparing alternative treatments regimens. METHODS: 76 patients, mean (SD) age 68.7 (7.7) years, were enrolled. Corticosteroids, and non-steroidal anti-inflammatory drugs (NSAIDs) were the only drugs allowed during the observation period. Erythrocyte sedimentation rate (ESR), C reactive protein (CRP), alpha(2) globulin, serum iron, pain, physician's global assessment (PGA), morning stiffness (MST), muscle tenderness (MT), myalgia, and the elevation of upper limbs (EUL) were determined regularly. The daily corticosteroid and NSAID doses as the corticosteroid response time were recorded. To ensure evaluation of an adequate number of patients (n = 57) week 24 was chosen for final analysis. RESULTS: ESR, CRP, alpha(2) globulin, pain, PGA, MST, myalgia, MT, and EUL showed significant improvement (p<0.0001) at week 24 compared with week 0. Multiple regression analysis showed that changes of ESR (p = 0.08), CRP (p = 0.41), alpha(2) globulin (p = 0.13), MST (p = 0.1), and MT (p = 0.07) were independent of pain, but myalgia (p<0.001) and EUL (p = 0.003) were pain dependent. Consequently, a core set of PMR response criteria, comprising ESR or CRP, pain, PGA, MST, and EUL was established. Assessment of treatment responses with this core set resulted in 90%, 70%, 50%, and 20% improvement in 31/57 (54%), 46/57 (81%), 51/57 (89%), and 54/57 (95%) of the patients, respectively. CONCLUSION: These PMR response criteria are a promising tool for better monitoring of disease activity and treatment in PMR. It is proposed that these criteria should be used in clinical trials in the near future to explore alternative treatment options for PMR.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Distal musculoskeletal manifestations in polymyalgia rheumatica.

In polymyalgia rheumatica (PMR) the marked and distinctive symptoms of proximal aching and stiffness have tended to draw attention away from the distal musculoskeletal manifestations which also occur in this syndrome. Peripheral manifestations are present in about half of all cases of PMR and include joint synovitis, diffuse swelling of the distal extremities with or without pitting edema, tenosynovitis and carpal tunnel syndrome. Awareness of these findings will help to facilitate the proper diagnosis and institution of appropriate therapy for this disease.

Humans↗
PubMedDate not supplied

Synovial expression of vasoactive intestinal peptide in polymyalgia rheumatica.

OBJECTIVE: Polymyalgia rheumatica (PMR) is an inflammatory disease that typically affects elderly people. Its clinical hallmark is the severity of pain in the shoulder and pelvic girdle. Mild to moderate synovitis and/or bursitis of the joints involved has been described. Neuropeptides are involved in nociception and modulation of inflammatory reaction. To evaluate whether neuropeptides have a role in PMR pathophysiology, we studied the expression of substance P (SP), calcitonin gene-related peptide (CGRP), vasoactive intestinal peptide (VIP) and somatostatin (SOM) in shoulder synovial tissues of PMR patients. METHODS: Synovial expression of neuropeptides was investigated by immunohistochemical analysis, in two groups of PMR patients: the first one at the onset of disease and the second one after corticosteroid treatment, and in other joint diseases, rheumatoid arthritis (RA) and osteoarthritis (OA). RESULTS: The only significant expression of VIP was found in PMR and, to a lesser extent, in RA synovial tissue. In PMR, we observed VIP immunostaining both in the lining layer and in the sublining area. In patients on corticosteroid treatment VIP lining layer expression was not significantly different while VIP positive cells in the sublining area were almost absent. CONCLUSION: Local VIP production in PMR synovial tissue might contribute to the typical musculoskeletal discomfort and it may have a role in the immunomodulation of synovial inflammation.

Aged↗
PubMed2004

Bone turnover in untreated polymyalgia rheumatica.

BACKGROUND: Polymyalgia rheumatica (PMR) is a common condition in the elderly. A previous study demonstrated that it is associated with an increase in bone resorption. This effect was ameliorated by steroids, implying that inflammation is the cause of increased bone resorption and that this can be reduced by steroids. This is in keeping with accumulating evidence that systemic inflammation is associated with bone resorption and bone loss. We studied bone formation and resorption markers in 53 patients with PMR prior to any therapeutic intervention. METHODS: Bone resorption was measured by estimating urinary free pyridinoline (fPYD) and deoxypyridinoline (fDPD). Bone formation was estimated by measuring serum concentrations of procollagen type 1 N-terminal propeptide (P1NP). Disease activity was assessed using inflammatory markers (erythrocyte sedimentation rate and C-reactive protein). Patients had a baseline dual-energy X-ray absorptiometer scan to assess bone mineral density. RESULTS: Bone resorption markers were significantly increased and bone formation markers significantly decreased in PMR patients prior to treatment, compared with a control population matched for gender and age. CONCLUSIONS: This implies that bone turnover is uncoupled in PMR. This may lead to a decrease in skeletal mass in the long term due to the disease process alone. However, no significant loss of bone mineral density was detected. It is possible that, due to the acute onset of PMR, increased bone resorption is not present long enough to result in a detectable decrease in bone mineral density. The effects of steroid treatment on bone metabolism and the subsequent long-term outcome need to be investigated.

Aged↗
PubMed1997

Effects of inflammation and treatment on bone turnover and bone mass in polymyalgia rheumatica.

OBJECTIVE: Polymyalgia rheumatica (PMR) has an abrupt onset of inflammatory symptoms, making it a useful model for studying the effects of inflammation in bone. PMR requires corticosteroid treatment, which may itself have a detrimental effect on bone. This study used serially measured biochemical markers of bone turnover and bone density to address the relative contributions of systemic inflammation and corticosteroid therapy to bone loss. METHODS: Fifty untreated patients with PMR were randomized to receive oral prednisolone or intramuscular methylprednisolone. Biochemical bone markers (pyridinoline [PYR], deoxypyridinoline [DPYR], procollagen type I carboxy-terminal peptide [PICP]) and bone mineral density (BMD) were measured at baseline and at 6, 12, and 24 months. RESULTS: The median disease duration at presentation was 12 weeks (range 5-32 weeks). Levels of urinary crosslinks were increased in patients with untreated PMR compared with controls (PYR 74.9 +/- 30.0 nmoles/mmole creatinine, DPYR 14.6 +/- 6.4 nmoles/mmole creatinine [mean +/- SD]; P = 0.0001); the PICP level was normal (115.0 +/- 39.0 microg/liter). With treatment, the crosslinks levels fell and PICP levels rose within 6 months (P = 0.01). Bone resorption (PYR) correlated with untreated disease activity (erythrocyte sedimentation rate [ESR]) (r = 0.5, P = 0.003) and with interleukin-6 levels (r = 0.48, P = 0.05). There was a significant reduction in BMD of both the hip and the spine after 12 months of treatment (P = 0.0002), with no difference between treatment groups. As the steroid dosage was reduced, bone mass improved. Initial ESR influenced the percent change in BMD at 1 year (r = 0.35, P = 0.05), while cumulative steroid dose, mean ESR, and type of steroid used did not. CONCLUSION: Inflammation in PMR increases bone resorption and appears to have a more detrimental effect on bone than does low-dose corticosteroid. If corticosteroids can be tapered and discontinued, bone loss in PMR can be a transient phenomenon.

Adrenal Cortex Hormones↗
PubMed1998

Polymyalgia rheumatica in biopsy proven giant cell arteritis does not constitute a different subset but differs from isolated polymyalgia rheumatica.

OBJECTIVE: To assess clinical and laboratory features that may be useful in differentiating isolated polymyalgia rheumatica (PMR) from PMR associated with biopsy proven giant cell arteritis (GCA); and in differentiating biopsy proven GCA associated with PMR from GCA without manifestations of PMR. METHODS: Clinical records of patients with PMR and biopsy proven GCA diagnosed at Hospital Xeral, Lugo, Spain from January 1987 through May 1997 were reviewed. Patients with a positive temporal artery biopsy were categorized into 2 different subgroups according to the presence or absence of associated PMR. The patients with biopsy proven GCA associated with PMR were compared with a group of patients with isolated PMR (not associated with GCA). RESULTS: From a total of 108 biopsy proven patients with GCA, 45 had associated PMR. Apart from a predominance of women and a longer delay to diagnosis, patients with PMR associated with GCA did not differ from the patients with GCA without PMR manifestations. In comparing patients with isolated PMR (n=117) with patients with PMR associated with GCA, we observed that PMR associated with GCA was a more severe disease, with significant abnormality in most laboratory variables, including constitutional syndrome, higher elevation of erythrocyte sedimentation rate and platelet counts, and lower values of hemoglobin. CONCLUSION: In both isolated PMR and PMR associated with GCA we observed a predominance of women. While there are no differences in the type of polymyalgia symptoms in patients with isolated PMR versus PMR associated with GCA, severe abnormalities associated with the inflammatory response in PMR may have prognostic value for more severe disease, which may be linked to the presence of GCA.

Adult↗
PubMed1996

[Carpal tunnel syndrome as initial manifestation of polymyalgia rheumatica].

A case of polymyalgia rheumatica with atypical manifestations, including carpal tunnel syndrome, distal myalgias and a low eritro sedimentation is reported. The association of polymyalgia rheumatica and carpal tunnel syndrome is controversial. Although it has been reported previously in a recent and large review of carpal tunnel syndrome in Rochester, USA, there was not any more cases of polymyalgia rheumatica associated with carpal tunnel syndrome than in the general population. In our patient, the symptoms of polymyalgia rheumatica and of carpal tunnel syndrome were present at the beginning, and both responded satisfactorally to the treatment with methylprednisone. This form of presentation and its response to treatment suggest that the symptoms of carpal tunnel syndrome are caused by the inflammation of the carpal synovial, which is an unfrequent manifestation of polymyalgia rheumatica. We report this case for the purpose of altering to an unusual form of presentation of polymyalgia rheumatica in order to avoid unnecessary surgery for the carpal tunnel syndrome.

Aged↗
PubMed2005

Acute onset of polymyalgia rheumatica in pregnancy.

BACKGROUND: Polymyalgia rheumatica is uncommon in young women and remains a diagnostic challenge for pregnant women. CASE: A 28-year-old pregnant woman developed polymyalgia rheumatica in the third trimester. Laboratory investigations revealed elevated erythrocyte sedimentation rate and C-reactive protein levels with normal muscle enzyme levels and seronegativity for rheumatoid factor. Although her symptoms deteriorated as pregnancy progressed, she drastically improved by treatment with prednisone. She underwent cesarean delivery at 39 weeks. She was relapse-free of polymyalgia rheumatica after discontinuation of prednisone on the 50th postoperative day. CONCLUSION: The diagnosis of polymyalgia rheumatica is important to properly manage pregnancy.

Acute Disease↗
PubMed2000

Polymyalgia rheumatica and temporal arthritis.

Polymyalgia rheumatica and temporal arteritis are closely related inflammatory conditions that affect different cellular targets in genetically predisposed persons. Compared with temporal arteritis, polymyalgla rheumatica is much more common, affecting one in 200 persons older than 50 years. Temporal arteritis, however, is more dangerous and can lead to sudden blindness. The diagnosis of polymyalgia rheumatica is based on the presence of a clinical syndrome consisting of fever, nonspecific somatic complaints, pain and stiffness in the shoulder and pelvic girdles, and an elevated erythrocyte sedimentation rate. Temporal arteritis typically presents with many of the same findings as polymyalgia rheumatica, but patients also have headaches and tenderness to palpation over the involved artery. Arterial biopsy usually confirms the diagnosis of temporal arteritis. Early diagnosis and treatment of polymyalgia rheumatica or temporal arteritis can dramatically improve patients' lives and return them to previous functional status. Corticosteroid therapy provides rapid and dramatic improvement of the clinical features of both conditions. Therapy is generally continued for six to 24 months. Throughout treatment, clinical condition is assessed periodically. Patients are instructed to see their physician immediately if symptoms recur or they develop new headache, jaw claudication or visual problems.

Age of Onset↗