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[A case of temporal arteritis associated with polymyalgia rheumatica and subclinical Sjögren's syndrome].

A case of temporal arteritis (TA) associated with polymyalgia rheumatica (PMR) and subclinical Sjögren's syndrome (sub SjS) was presented in this paper. A 76 year-old-male was admitted with headache, fever and weight loss in April 1987. Myalgia of upper extremities and of thighs developed during the past two months before admission. He also had noticed bilateral wrist pain. Physical examination revealed slight cord-like thickening of left temporal artery with tenderness. Cerebral angiography disclosed narrowing of frontal and parietal branches of left temporal artery. Temporal artery biopsy was consistent with TA. Diagnosis of PMR was made by Bird's diagnostic criteria. Although sicca symptoms were not seen, sialography revealed moderate sialectasis. Pathological finding of salivary gland was compatible with sub SjS. Possibility of occult lymphoma was eliminated by CT scanning or myelogram. Laboratory evaluation disclosed slight anemia, leukocytosis and thrombocytosis. Blood chemistry showed no abnormal finding except for hyperfibrinogenemia. Serological studies indicated positive C-reactive protein and slight elevation of alpha 2 and beta globulin fractions of serum protein. Either rheumatoid factors, antinuclear antibodies, anti-smooth muscle antibodies, cryoglobulin or circulating immune complexes were not detected. The HLA-B8 and DR3, frequently detected in TA and SjS, were not identified. Tuberculin test was negative. These results suggested that immunological aberration not caused by genetic factors but by senescence would induce presence of TA associated with both PMR and sub SjS.

Aged↗

Polymyalgia rheumatica and seronegative rheumatoid arthritis may be the same entity.

Of 159 patients with either polymyalgia rheumatica, seronegative rheumatoid arthritis (RA) or an undifferentiated syndrome with features of both who were followed for at least 30 months, synovitis recurred in 57. Twenty of the 57 patients had one episode of polymyalgia and another that looked like RA. Recurrences responded to prednisone and no joint destruction was seen. Temporal arteritis was seen with both diagnoses. These observations suggest that a benign symmetric synovitis occurs in older patients and may present as polymyalgia or as a polyarthritis that resembles RA.

Arthritis, Rheumatoid↗

IL-6 promoter polymorphism at position -174 modulates the phenotypic expression of polymyalgia rheumatica in biopsy-proven giant cell arteritis.

OBJECTIVES: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are related diseases in which diverse genetic and environmental factors are implicated. Both GCA and PMR are characterized by an intense acute phase reaction. In these sYndromes the increased production of IL-6 has been observed. To investigate further the genetic influence of IL-6 in GCA and PMR we have examined the IL-6 promoter polymorphism (G to C) at position -174 in the 5' region in a series of patients from Northwest Spain diagnosed with GCA and/or PMR. METHODS: Sixtxy-two biopsy-proven GCA patients (30 of them with associated PMR) and 84 patients with isolated PMR were studied. Patients and ethnically matched controls (n = 124) were from the Lugo region (Galicia, Northwest Spain). Patients and controls were genotyped for HLA-DRB1 and IL-6 polymorphism at position -174 by molecular methods. RESULTS: IL-6-174 allele C was marginally increased infrequency in GCA patients with PMR manifestations compared with isolated GCA (Pcorr 0.06; OR = 2.3). The increase in the frequency of the CC genotype in GCA patients with PMR versus those with isolated GCA was statistically significant (Pcorr 0.02). The increased frequency of allele C in GCA patients with PMR was more commonly observed in HLA-DRBI *04 negative patients. However, this polymorphism was not associated with a higher risk of ischemic events in GCA or with relapses in PMR. CONCLUSION: Allele C at position -174 in the 5' promoter region of the IL-6 gene may be associated with PMR in biopsy-proven GCA patients not carrying HLA-DRBI *04 alleles.

Alleles↗

Clinical outcome of 149 patients with polymyalgia rheumatica and giant cell arteritis.

OBJECTIVE: To assess the clinical outcome of patients with polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). METHODS: All charts of consecutive patients with a diagnosis of PMR and/or GCA attending a tertiary referral center from June 1989 to February 1996 were reviewed following a predetermined protocol. Subsequently, the majority of patients (90%) were assessed clinically or by telephone interview. Registered variables included demographic data, disease characteristics, prednisone dosage and duration, comorbidities, and clinical outcomes. RESULTS: There were 149 patients (133 with PMR alone, 7 with GCA alone, 9 with both); 94 (63%) were females; the mean age was 68 +/- 9 years, and the mean disease duration from the first symptom to the rheumatology consultation was 13 +/- 12 weeks (1-99). Typical clinical features of PMR were present in patients with PMR. Synovitis was observed in 26 patients. The presenting symptoms for GCA were typical features in 13 patients and blindness in 3 (2%) patients. Mean followup was 3.7 +/- 2 years. Comorbid conditions were present in 71 patients: 12 patients had hypertension, 13 had fractures, 8 diabetes, 29 cataract, 8 major infection, and 37 had other complications. Cancer was diagnosed in 4 patients and 6 patients had died. Prednisone was prescribed in 148 patients (mean dose 23 +/- 14 mg) for a mean time of 28 +/- 29 mo. Nonsteroidal antiinflammatory drugs were prescribed in 51 (34%) patients and methotrexate in 2. Disease remission was achieved in 81 (54%) patients (72 remissions, 9 presumed remissions) in whom steroid therapy had been stopped. Another 54 (36%) patients were still taking prednisone at the time of the interview, all were in clinical remission. Seventeen patients developed rheumatoid arthritis subsequent to the diagnosis of PMR. CONCLUSION: PMR and GCA should not necessarily be considered diseases with favorable outcome. In many of our patients, steroids were required for a prolonged period. Some patients developed significant complications attributable to steroid therapy. A significant number of patients progressed to rheumatoid arthritis.

Aged↗

Effect of hemiparesis on polymyalgia rheumatica.

Central nervous system insults with secondary hemiparesis appears to influence the symptom complex of polymyalgia rheumatica. Symptoms are reported here not to develop in established hemiparesis.

Aged↗

Sternoclavicular erosions in polymyalgia rheumatica.

The incidence of erosive arthropathy of the sternoclavicular joints in 25 consecutive cases of polymyalgia rheumatica was studied by means of sternoclavicular tomography. Definite erosions were found in 11 patients. Erosions were most likely to be found in patients whose symptoms had been present for more than 6 months.

Female↗

Polymyalgia rheumatica. Abrupt and gradual withdrawal of prednisolone treatment, clinical and laboratory observations.

Eighteen patients with polymyalgia rheumatica had corticosteroid treatment withdrawn abruptly under close observation. In each case polymyalgic symptoms reappeared but were controlled rapidly when prednisolone was reintroduced. Prednisolone withdrawal was then started by slow decrements of dose. In no patient was it possible to withdraw prednisolone treatment, after using either method, during the period of observation.

Aged↗

Polymyalgia rheumatica as an unusual cause of pleural and pericardial effusion.

We report a patient with polymyalgia rheumatica (PMR) who initially presented with chest pain pleural and pericardial effusions. A history was then obtained of shoulder girdle aching and stiffness, suggestive of PMR, and laboratory investigations supported the diagnosis. A rapid response to 15 mg prednisolone once daily was noted, with resolution of effusion and symptoms. Although pericardial effusion has previously been reported, this is the first reported case of pleuropericardial effusions in PMR. Rheumatologists should be aware that PMR may occasionally be the explanation for pleuropericardial effusion in the elderly.

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Systemic amyloidosis AL with temporal artery involvement revealing lymphoplasmacytic malignancy in a man presenting as polymyalgia rheumatica.

A 68 year old man presented with a clinical and biological picture that suggested polymyalgia rheumatica. Temporal artery biopsy disclosed no inflammatory change but massive light chain amyloid deposits in the media. Further exploration showed a malignant lymphoplasmacytic haemopathy with a triclonal gammopathy and a muscular, rectal, and probable cardiac amyloidosis. Cryoglobulinaemia and high concentrations of soluble interleukin 2 receptor (sIL-2R) were also found. This is the fifth case with confirmed involvement of the temporal artery. The especially high sIL-2R concentration was thought to reflect the tumour mass rather than lymphocyte activation.

Aged↗

A 67-year-old woman with polymyalgia rheumatica and left hemispatial neglect.

A 67-year-old woman with a diagnosis of polymyalgia rheumatica presented initially with periods of confusion and incontinence. A CT scan of the brain was normal and she was treated with tapering doses of corticosteroids and clinical improvement. After a brief period off steroids, the patient presented with a progressive dementia, left-sided clumsiness, gait disturbances and left hemispatial neglect. An MRI at this time demonstrated a large area of edema over the right parietal lobe and intense cortical enhancement. A chest CT demonstrated multiple nodules. Biopsies of the lung and brain failed to identify any infectious organisms or malignant tissue. The leptomeningeal biopsy revealed multiple granulomatous areas with central necrosis and hystiocytic cells consistent with idiopathic hypertrophic pachy-meningitis.

Aged↗

[Polymyalgia rheumatica in patients of a rheumatologic specialty practice. Clinical signs and etiological events].

Manifestations of polymyalgia rheumatica in 100 ambulatory patients of an internistic, rheumatological specialist's practice were investigated. In this group of patients, both the clinical course of the disease and its prognosis were found to be more benign than is commonly reported in the literature. Thus, for example, temporal arteritis was found in only three of the one hundred patients. Not all of the patients were found to have the markedly elevated ESR normally considered to be typical. For this reason, to establish inflammatory activity, in addition to ESR, C-reactive protein (CRP) should also be determined in order to ensure that atypical forms with less marked signs of inflammation are not overlooked. Thirty of the 100 patients reported possible triggering life events.

Aged↗

Serological and immunohistochemical determination of von Willebrand factor antigen in serum and biopsy specimens from patients with arteritis temporalis and polymyalgia rheumatica.

Von Willebrand factor antigen (vWF-Ag) levels in serum from patients with untreated polymyalgia rheumatica (PMR) and arteritis temporalis (AT) were measured using an ELISA technique. The level in patients with AT was significantly higher (median 229%, range 182-358%) than in patients with PMR (median 190%, range 131-390%) and in controls (median 179%, range 65-296%). Using an immunoperoxidase technique and biopsy specimens from 21 temporal arteries (7 patients with AT, 7 with PMR and 7 with other diseases), vWF-Ag was localized in the luminal endothelium in all cases. VWF-Ag was also present in many new vessels along the lamina elastica of the artery wall in all patients with AT, but not in patients with PMR. We relate the higher vWF-Ag level in these patients to an increased vWF-Ag production by endothelian cells in new, proliferating vessels in the artery wall.

Aged↗

Skeletal muscle mitochondrial function in polymyalgia rheumatica and in giant cell arteritis.

OBJECTIVE: To ascertain whether mitochondrial function is impaired in polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). PATIENTS AND METHODS: Thirteen patients suffering from isolated PMR, 19 from GCA (eight with and 11 without PMR) and 25 healthy people submitted to orthopaedic surgery were included. Skeletal muscle was obtained from the quadriceps by open biopsy. Mitochondrial histological abnormalities were assessed on Gomori's trichrome staining and on cytochrome c oxidase and succinic dehydrogenase reactions. Biochemical studies consisted of polarographic measurement of oxidative activity using complex I, II, III and IV substrates, and spectrophotometric determination of individual enzymatic activity of such complexes. RESULTS: We did not find differences among groups either with respect to the percentage of histological or histochemical abnormalities [P = not significant (NS) for all stainings and reactions], oxidative capacity (P = NS for all substrates) or individual enzymatic activities (P = NS for all complexes). CONCLUSION: Skeletal muscle mitochondria remain histologically and functionally unaffected in PMR and in GCA.

Aged↗

Cancer in association with polymyalgia rheumatica and temporal arteritis.

In our prospective controlled study, a total of 185 patients with polymyalgia rheumatica (PMR) and temporal arteritis (TA) diagnosed during 1978-83 and their 925 matched controls were cross checked with the data files at the Cancer Registry of Norway at the end of 1987. Malignancy was registered in 27 patients (14.6%) and 131 controls (14.2%) between 1953 and the end of 1987. Malignancy was registered in 16 (24.6%) of the patients with biopsy demonstrating arteritis temporalis. The hazard rate for developing malignancy after diagnosis for the whole patient population was not significantly different from the controls. The hazard rate for developing malignancy in patients with positive biopsy, however, was 2.35 times higher than in the controls (p = 0.036) and 4.40 times higher than the rest of the patient population (p = 0.007) (Cox proportional hazards model). The general long interval between diagnosis of PMR and/or TA and registration of malignancy (mean 6.5 years) is not consistent with a paraneoplastic mechanism.

Adult↗

[Polymyalgia rheumatica and temporal arteritis. Observations on prognosis and treatment].

1. There is still controversy about polymyalgia rheumatica (PMR) and temporal arteritis (TA), either expressions of a single disease or two different conditions with overlapping. Nearly 50% of TA present with a PMR syndrome and 5% of PMR have a positive temporal artery biopsy. 2. Biopsy is useful for diagnostic purposes but does not seem to have any prognostic value in the management of PMR and TA. 3. The best symptomatic treatment is represented by prednisone. Prednisone has to be continued over a large period of time creating adverse effects in elderly people unless minimal doses are used. 4. TA visual or neurological complications are often observed in the first weeks of the disease thus leading many authors to recommend high doses (0.5 mg to 1 mg/kg/day) to patients with TA and even with PMR. To us starting high doses are to be used in severe clinical conditions of TA, particularly those presenting visual symptoms (nevertheless lower doses may be successful as well). Concerning other patients with TA and with PMR a starting dose of 10 to 30 mg depending on the clinical picture then a follow up dosage of 10 and even less, is suggested.

Biopsy↗

Fat suppression magnetic resonance imaging in shoulders of patients with polymyalgia rheumatica.

OBJECTIVE: To evaluate the sites of inflammatory process in the shoulders of patients with polymyalgia rheumatica (PMR) using fat suppressed magnetic resonance imaging (MRI). METHODS: Six consecutive, untreated new patients with PMR were investigated. Five patients with early rheumatoid arthritis (RA) and 4 patients with early psoriatic arthritis (PsA) with bilateral shoulder symptoms served as a control group. Bilateral shoulder fat-suppressed MRI sequences were performed in all patients and controls. We evaluated the presence of joint synovitis, bursitis, tenosynovitis, and bone and soft tissue edema. RESULTS: Bilateral subacromial/subdeltoid bursitis was found in all patients with PMR, in 1/5 (20%) patients with RA (p < 0.05), and in none with PsA (p < 0.02). Glenohumeral synovitis was present in all case and controls. Biceps tenosynovitis was observed in 4/6 (67%) patients with PMR, in 4/5 (80%) with RA (not significant, NS), and in all 4 patients with PsA (NS). No evidence of bone edema adjacent to the joint capsule and entheseal insertions or in the soft tissues was present in either cases or controls. CONCLUSION: The absence of extracapsular abnormalities in the early shoulder disease of PMR does not confirm the hypothesis of a capsular-based disorder.

Aged↗

Polymyalgia rheumatica in patients with a normal erythrocyte sedimentation rate.

OBJECTIVE: To ascertain the frequency of polymyalgia rheumatica (PMR) with a normal erythrocyte sedimentation rate (ESR; < or = 30 mm/hour) and to determine any defining clinical characteristics. METHODS: A retrospective chart review study of all patients meeting the clinical criteria for PMR seen over a 5-year period in a hospital and an office-based rheumatology practice. RESULTS: We evaluated 117 patients; 26 (22.2%) had a pretreatment ESR of < or = 30 mm/hour (mean +/- SD 19.8 +/- 7.5 versus 74.4 +/- 30.3 mm/hour for elevated ESR group; P<0.0001). Of the 26 normal ESR patients, 15 (58%) were female compared with 74 of the 91 elevated ESR patients (81%) (P<0.02, by Fisher's exact test). The mean hemoglobin concentration was significantly lower in the elevated ESR population (mean +/- SD 1.23 +/- 0.15 gm/liter versus 1.38 +/- 0.11 gm/liter; P<0.0001). The duration of symptoms prior to treatment with prednisone was significantly longer for patients with a normal ESR (149 +/- 95 days versus 103 +/- 95 days for elevated ESR patients; P<0.04). CONCLUSION: In our series, PMR with a normal ESR accounted for approximately one-fifth of all PMR patients, more commonly in men. The lack of characteristically abnormal laboratory findings may result in a delay in the proper diagnosis and management of this condition.

Aged↗

Methotrexate in polymyalgia rheumatica: preliminary results of an open, randomized study.

OBJECTIVE: Steroids are the only treatment of polymyalgia rheumatica (PMR). We report the effects of methotrexate (MTX) plus prednisone versus prednisone alone in PMR. METHODS: Twenty-four patients with recent onset PMR were studied in a randomized prospective study lasting one year. Patients were given MTX (MTX arm) 10 mg intramuscularly plus prednisone every week, or prednisone alone (Pred arm). After 6 months an attempt was made to stop prednisone, and to use the lowest possible dose over the next 6 months. RESULTS: At the 12th month, all patients were in clinical remission, acute phase reactants were in the normal range in both arms of the study, 6 patients were no longer taking steroids in the MTX arm versus 0/12 in the Pred arm, and the amount of prednisone in the 2 groups was statistically different (1.84 versus 3.2 g; p < 0.0001). In addition, bone mineral density was significantly decreased in the Pred arm, but not in the MTX arm. CONCLUSION: The MTX regimen allowed the use of much less prednisone over one year to obtain full control of PMR with no loss of efficacy. It also allowed sparing of bone in elderly patients at increased risk of osteoporotic fractures.

Aged↗