The spectrum of conditions mimicking polymyalgia rheumatica in Northwestern Spain

OBJECTIVE: To examine the spectrum and the main clinical data of patients presenting with polymyalgia symptoms who have conditions other than polymyalgia rheumatica (PMR) or PMR associated with giant cell arteritis (GCA) during a 10 year period in Northwestern Spain. METHODS: Clinical records of patients presenting with polymyalgia symptoms diagnosed at the Hospital Xeral-Calde Lugo from 1987 to 1996 were reviewed by rheumatology staff members. Patients were considered as having a condition suggestive of PMR if they met the following criteria: (1) Age > or =50 years at the onset of symptoms; (2) severe bilateral pain associated with morning stiffness for > 1 mo in at least 2 of 3 areas: neck, shoulder, and/or pelvic girdle; (3) erythrocyte sedimentation rate at the time of diagnosis > or =40 mm/h. Patients with pure PMR or with PMR associated with GCA were excluded from study. RESULTS: Twenty-three of the 208 patients (age 67.8 +/- 9.0 yrs) presenting with PMR symptoms were finally diagnosed as having conditions different from PMR and GCA. Men outnumbered women (61%). Malignancies and rheumatic diseases, especially seronegative symmetrical polyarthritis (SSP), were the most common entities. Elderly patients with solid malignancies had a poor response to low doses of prednisone. In patients with hematologic malignancies atypical symptoms of PMR such as lack of accentuation of symptoms with movement and a more diffuse continuous aching were observed. During followup 5 patients developed episodes of SSP (median duration 13 months, range 5 to 24), particularly in both hands, satisfying the American College of Rheumatology 1987 criteria for rheumatoid arthritis. However, arthritis responded promptly to corticosteroids with no disease progression. No cortical erosions or new episodes of PMR were seen in these patients after a followup of 6.8 +/- 2.6 years. With the exception of these 5 patients, duration of polymyalgia symptoms was not longer than 3 months from the onset of polymyalgia symptoms until a specific diagnosis was made. CONCLUSION: Polymyalgia symptoms are not uncommon as presenting manifestations of a wide spectrum of conditions. Special concern about the presence of diseases different from PMR or GCA must be considered in patients presenting with atypical symptoms of PMR. Also, the possibility of developing a SSP has to be considered during the followup of these patients.     

Polymyalgia rheumatica and colon malignacy: case report

Polymyalgia rheumatica (PMR) is a relatively common disorder in the elderly. Whereas the relationship between polymyalgia rheumatica and giant cell arteritis (GCA) is well recognized, there is still controversy about PMR and malignancy. We are presenting a patient with PMR and adenocarcinoma of the sygmoid colon and hypothesize a paraneoplastic relationship.     

Primary systemic amyloidosis presenting as giant cell arteritis and polymyalgia rheumatica

Primary systemic amyloidosis may present with features suggesting a vasculitis, including giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). In this report, we describe the clinical characteristics, temporal artery biopsy findings, and the response of vascular and musculoskeletal symptoms to corticosteroid therapy in 4 patients with primary systemic amyloidosis who presented with manifestations of GCA or PMR.     

Serious infections in people with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA): a time-trend national US study

Clinical Rheumatology - To assess incidence, time-trends, and outcomes of serious infections in people with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA). We examined the...     

Acute dissecting thoracic aortic aneurysm in a patient with polymyalgia rheumatica

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related disorders found in older patients, and vasculitis has been proposed as a part of the pathogenesis of PMR. We describe a female patient with PMR plus aortitis, both of which were well controlled on maintenance steroid therapy. Six months after the onset of her condition, however, she suddenly presented with chest pain. A diagnosis of dissecting aortic aneurysm was confirmed, and the aorta was successfully resected. Histology revealed infiltration of mononuclear cells including giant cells around the vaso vasorum with disruption of elastic lamina of the resected aorta. PMR or GCA may indicate an increased risk for aortic dissection in patients with normal erythrocyte sedimentation rate or C-reactive protein, and prompt recognition and therapy, not only during the active disease but also after symptoms of PMR have resolved, are needed.     

Anticardiolipin antibodies in giant cell arteritis and polymyalgia rheumatica: a study of 40 cases

The occurrence and clinical value of anticardiolipin antibodies (aCL) were studied in 33 patients with giant cell arteritis (GCA) and in seven patients with polymyalgia rheumatica (PMR), at onset and during follow-up. aCL were present in 19/40 (47.5%) GCA/PMR cases, most of them of the IgG isotype, whereas all controls (21 subjects) were aCL negative. The presence of aCL was not associated with inflammatory parameters or clinical signs of arteritis; however, they disappeared in a significant percentage (56%) of patients during steroid therapy. No correlation was found between ischaemic events and aCL, suggesting that they are not important for the development of vascular complications in GCA/PMR patients. Moreover, a retrospective evaluation of our data showed a correlation between aCL positivity and anaemia, whose significance remains to be elucidated.      

The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment.

OBJECTIVES--To examine the clinical course of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in a prospective study, after the initial two months. METHODS--Seventy four patients with PMR/GCA were followed for a median of 60 weeks. Detailed clinical and laboratory records were made on each visit. RESULTS--Twenty per cent of patients with PMR developed GCA and 24% of patients with GCA developed PMR from the onset of symptoms. After two months, most patients experienced at least one relapse. Relapses and persistence of abnormal symptoms and signs were most common in patients with both PMR and GCA and least common in those with GCA alone. Relapses were most common in the first year and 54% occurred in association with steroid reduction. Major complications were rare. Laboratory parameters and temporal artery histology were not helpful in predicting relapse. Only 24% of patients were able to stop steroid treatment after two years. CONCLUSIONS--Clinicians should consider more frequent review in patients at times of steroid reduction and especially within the first six months of treatment.     

The relation of polymyalgia rheumatica to rheumatoid arthritis

Sixty-four patients with the onset of rheumatoid arthritis (RA) after age 60 were followed for at least three years (mean 6.3 years); 33 patients had rheumatoid factor and 31 did not. Twenty-five of the 31 seronegative patients had an excellent response to low dose prednisone and did not require any additional medication. Six of these patients also had an episode diagnosed as polymyalgia rheumatica (PMR). These findings suggest that the synovitis currently diagnosed as seronegative RA in many older patients may not be the same disease as seropositive RA, but may be more closely related to or identical with PMR.     

Maladie de Horton et pseudopolyarthrite rhizomélique : critères diagnostiques

Giant cell arteritis still results in high morbidity related to systemic complications involving the eyes, the central nervous system, the heart and the aorta. Therefore, reliable diagnostic criteria are required in giant cell arteritis and polymyalgia rheumatica (PMR), to detect and manage at early stages patients with giant cell arteritis and PMR. Indeed, diagnostic criteria of giant cell arteritis and PMR have been validated. Taken together, first we propose to review the diagnostic criteria of giant cell arteritis (ACR, GRACG) and PMR (Bird, Jones-Hazleman, Chuang-Hunder, Dasgupta). Second, more recent diagnostic criteria of GCA and PMR are further reviewed, including biochemical/immunological data (especially anti-ferritin antibodies), temporal artery biopsy and imaging findings (artery ultrasonography, angio-CT-scan, angio-MRI, PET-scan, joint echography, and MRI).     

Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months.

Thirty nine patients with polymyalgia rheumatica (PMR) and 35 with giant cell arteritis (GCA) were treated with high or low dose steroid regimens in a prospective study of the first two months of treatment. Patients with PMR needed 15-20 mg prednisolone initially; 13/20 (65%) relapsed on an initial dose of 10 mg/day. All but two patients with GCA were successfully treated with 40 mg/day prednisolone initially but relapsed on a reduction to 20 mg/day. One patient with GCA receiving 30 mg/day relapsed after four weeks. Six patients with PMR developed GCA during the first two months and required an increased prednisolone dose to control symptoms. The erythrocyte sedimentation rate or C reactive protein did not predict relapse.     

Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in northern Italy

We studied the epidemiology of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) in a Mediterranean population. Ninety-nine patients with PMR and/or GCA were identified over a 9-year period (1980-1988) in Reggio Emilia, Italy. The average annual incidence of PMR and GCA was 12.7/100,000 and 6.9/100,000, respectively, in a population aged 50 years or older. Frequencies of HLA antigens were determined in 49 patients with PMR and/or GCA who were followed by staff at our rheumatology unit during the 1980-1988 period. When compared with HLA findings in 242 healthy controls, DR4 was not found to be significantly associated with PMR (24% in PMR patients versus 14% in controls). Patients with GCA also showed an increased frequency of DR4 compared with controls (36% versus 14%), but this difference was also not statistically significant. The immunogenetic features of PMR and GCA and the relationship between the immunogenetic and epidemiologic patterns in different populations are discussed.     

Polymyalgia rheumatica: myalgic syndrome or occult vasculitis?

Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder of unknown etiology which typically presents with symmetric myalgias in the shoulder and pelvic girdles. Other clinical signs include the rapid onset of symptoms and the almost exclusive manifestation in the elderly population. In around 20% of cases, PMR is associated with giant cell arteritis (GCA). However, new imaging techniques suggest that the prevalence of subclinical GCA (e. g. aortitis) in PMR is probably higher. Acute phase reactants like erythrocyte sedimentation rate and c-reactive protein are usually elevated. Myalgias are accompanied by synovitis and bursitis of the large proximal joints and can be visualized by ultrasound or magnetic resonance imaging. While the diagnosis of GCA can be verified by temporal artery biopsy, pathognomonic findings for PMR like specific autoantibodies are lacking. Typical for PMR is the rapid response to corticosteroids. Usually the therapy needs to be continued for at least 2 years. Due to adverse events in many cases a corticosteroid saving therapy like methotrexate is needed.     

Polymyalgia rheumatica

Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder of unknown etiology which typically presents with symmetric myalgias in the shoulder and pelvic girdles. Other clinical signs include the rapid onset of symptoms and the almost exclusive manifestation in the elderly population. In around 20% of cases, PMR is associated with giant cell arteritis (GCA). However, new imaging techniques suggest that the prevalence of subclinical GCA (e. g. aortitis) in PMR is probably higher. Acute phase reactants like erythrocyte sedimentation rate and c-reactive protein are usually elevated. Myalgias are accompanied by synovitis and bursitis of the large proximal joints and can be visualized by ultrasound or magnetic resonance imaging. While the diagnosis of GCA can be verified by temporal artery biopsy, pathognomonic findings for PMR like specific autoantibodies are lacking. Typical for PMR is the rapid response to corticosteroids. Usually the therapy needs to be continued for at least 2 years. Due to adverse events in many cases a corticosteroid saving therapy like methotrexate is needed.     

Cytidine deaminase in polymyalgia rheumatica and elderly onset rheumatoid arthritis

Serum cytidine deaminase (CD) as a marker of inflammatory disease was assessed in 44 patients and 47 controls to differentiate polymyalgia rheumatica (PMR) from elderly onset rheumatoid arthritis (EORA). The patients were divided into four groups: PMR with and without synovitis and seropositive and seronegative EORA. No statistically significant differences were found when serum CD levels of seropositive EORA patients were compared with serum CD of PMR patients without synovitis, neither when serum CD levels of all PMR patients were compared with a seronegative EORA group, nor when serum CD levels of PMR patients with synovitis were compared with those with EORA. Nevertheless, statistically significant differences were detected between EORAs serum CD levels and the control group (p=0.023). This difference was 10% when comparing CD levels of PMR patients with the control group (p=0.070). We did not demonstrate that serum CD levels could be a useful tool to differentiate PMR from EORA, but these findings could nevertheless reflect the presence of an inflammatory disease.     

Erythrocyte sedimentation rate and C reactive protein in the assessment of polymyalgia rheumatica/giant cell arteritis on presentation and during follow up.

The erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) were measured in 74 patients with polymyalgia rheumatica (PMR)/giant cell arteritis (GCA) on presentation, in the first month of treatment, and at long term follow up (up to 177 weeks). Before treatment the ESR was raised (greater than 30 mm/h) in all cases and the CRP was raised (greater than 6 mg/l) in 49/55 cases. The ESR was a better indicator of clinical disease activity except in patients who felt completely well at week 1. 'False positive' increases of ESR or CRP were rare. During relapses ESR was normal in 37/77 (48%) of cases and CRP in 41/73 (56%). It is suggested that ESR is the most useful laboratory parameter in assessing PMR/GCA.     

Anti-neutrophil cytoplasm antibodies in patients with giant cell arteritis and/or polymyalgia rheumatica.

Anti-neutrophil cytoplasm antibodies (ANCA) were detected at low titers by indirect immunofluorescence in 1 out of 13 patients with giant cell arteritis (GCA) alone, 7 out of 30 with polymyalgia rheumatica (PMR) alone and 4 out of 15 with GCA plus PMR (8, 23 and 27%, respectively). Anti-myeloperoxidase antibodies were also demonstrated by an enzyme-linked immunosorbent assay in 4 patients with GCA alone (31%), 13 with PRM alone (42%) and 5 with GCA plus PRM (33%). The C-reactive protein response was significantly higher (p < 0.05) in ANCA-positive than in ANCA-negative patients.     

Cellular immunity in polymyalgia rheumatica and giant cell arteritis: lack of response to muscle or artery homogenates.

Peripheral blood lymphocyte functions were evaluated in 20 patients with active polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA) by determining the percent of E-rosette-forming cells and by measuring the uptake of tritiated thymidine by peripheral blood lymphocytes after exposure to common infectious antigens and to homogenates of homologous and heterologous artery, muscle, and elastin. Although lymphocytes from patients with PMR and/or GCA were stimulated slightly by artery and muscle homogenates, no differences in lymphocyte responses were found when the results were compared with 22 normal controls and 16 patients with rheumatoid arthritis. The hypothesis that GCA results from a cellular immune reaction to normal or diseased arterial wall antigens is not supported by these studies.     

Cellular immunity in polymyalgia rheumatica and giant cell arteritis

Peripheral blood lymphocyte functions were evaluated in 20 patients with active polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA) by determining the percent of E-rosette-forming cells and by measuring the uptake of tritiated thymidine by peripheral blood lymphocytes after exposure to common infectious antigens and to homogenates of homologous and heterologous artery, muscle, and elastin. Although lymphocytes from patients with PMR and/or GCA were stimulated slightly by artery and muscle homogenates, no differences in lymphocyte responses were found when the results were compared with 22 normal controls and 16 patients with rheumatoid arthritis. The hypothesis that GCA results from a cellular immune reaction to normal or diseased arterial wall antigens is not supported by these studies.     

Giant cell arteritis and polymyalgia rheumatica after influenza vaccination: report of 10 cases and review of the literature

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are inflammatory rheumatic diseases common in people over the age of 50 years. Herein, we report 10 cases of previously healthy subjects who developed GCA/PMR within 3 months of influenza vaccination (Inf-V). A Medline search uncovered additional 11 isolated cases of GCA/PMR occurring after Inf-V. We discuss the role of individual susceptibility, the potential function of immune adjuvants as triggers of autoimmunity post-vaccination, and the correlation of our observation with the 'ASIA' syndrome, i.e. autoimmune/inflammatory syndrome induced by adjuvants and including post-vaccination phenomena.