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[A case of idiopathic nonspecific interstitial pneumonia and autoimmune hemolytic anemia after complete cure of polymyalgia rheumatica].

In 1997, at the age of 68, a man was admitted with polymyalgia rheumatica, which was successfully treated with oral prednisolone. In 1999, a chest X-ray revealed that he had interstitial changes in both lung fields. Because there were no symptoms, he was observed without treatment. However, from around 2003, he began to experience gradual progression of severe dyspnea on exertion. He was admitted to our hospital in 2004, at the age of 75, and we found interstitial lung deterioration. Nonspecific interstitial pneumonia (NSIP) was diagnosed because of the increase in the number of lymphocytes in bronchoalveolar lavage fluid and CT findings. He was also found to have autoimmune hemolytic anemia (AIHA). Treatment of AIHA with 1mg kg prednisolone led to improvement of not only the anemia but also the interstitial pneumonia. The coexistence of both idiopathic interstitial pneumonia and autoimmune hemolytic anemia is very rare. Moreover, this is the first report of polymyalgia rheumatica occurring prior to these two diseases.

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Polymyalgia rheumatica and temporal arteritis: evidence and guidelines for diagnosis and management in older people.

Polymyalgia rheumatica and temporal arteritis commonly present for the first time in older people. Guidelines for diagnosis, investigation and management are available but have largely been developed by rheumatologists from studies where older people have been excluded. It is not clear whether geriatricians care for a group of patients with different clinical presentations compared to those under the care of other clinicians. There is a need for further prospective studies and randomised controlled trials to clarify a host of outstanding issues to improve the care of older people with polymyalgia rheumatica and temporal arteritis.

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Microheterogeneity of alpha 1-antichymotrypsin in the management of giant-cell arteritis and polymyalgia rheumatica.

1. Using crossed immunoaffinity electrophoresis with free concanavalin A in the first dimension, we studied the glycan microheterogeneity of alpha 1-antichymotrypsin in sera from patients with giant-cell arteritis and/or polymyalgia rheumatica, and its variation in the serum of several of these patients during induction of disease remission by prednisone therapy and possible further recurrence of giant-cell arteritis and/or polymyalgia rheumatica. 2. In the serum of patients with active disease we observed increased proportions of concanavalin A nonreactive and concanavalin A weakly reactive fractions. The results were expressed as the ratio of concanavalin A non-reactive fraction plus concanavalin A weakly reactive fraction to concanavalin A reactive fraction, called R alpha 1-ACT. An R alpha 1-ACT higher than 1.8 (upper normal value) was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 2/22 sera from patients with inactive disease (specificity 91%). 3. The erythrocyte sedimentation rate and the serum C-reactive protein level, common markers of biological inflammation, are usually elevated in active giant-cell arteritis and/or polymyalgia rheumatica. The two parameters are commonly used to guide the therapy during the course of the disease, but they have no specificity. An erythrocyte sedimentation rate of greater than 30 mm/h was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 5/22 sera from patients with inactive disease (specificity 77%).(ABSTRACT TRUNCATED AT 250 WORDS)

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[Carpal tunnel syndrome in polymyalgia rheumatica. Clinical and electromyographic response to systemic treatment].

The association between carpal tunnel syndrome and Polymyalgia Rheumatica has already been described. As in other systemic diseases the bilaterality of the syndrome is a common finding. Surgical treatment is usually necessary in carpal tunnel syndrome. Three cases of the syndrome associated with polymyalgia rheumatica are presented. Clinical remission and normalization of the electromyographic tracing were achieved by systemic corticosteroid treatment. The relationship between these two conditions is once more confirmed. A "wait and see" period which might eventually avoid unnecessary surgical intervention is strongly recommended.

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A follow-up study of polymyalgia rheumatica: evidence of chronic axial synovitis.

A follow-up study of 23 patients with polymyalgia rheumatica 3.2 yr after onset and 2.6 yr after diagnosis showed that (1) 15 of 16 still had abnormal joint scans; (2) 18 of 23 still needed antiinflammatory drugs; (3) 16 of 23 believed they still had the disease; (4) erythrocyte sedimentation rates were persistently elevated beyond the 90th percentile in 11 patients (47%); and (5) 7 of 18 patients with available roentgenograms had joint abnormalities. We suggest that polymyalgia rheumatica is a chronic arthritis affecting primarily the proximal joints.

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Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. A retrospective study of 63 patients.

The initial clinical symptoms, the course of the disease, and the effect of corticosteroid treatment have been analyzed in a retrospective study of 63 patients with temporal arteritis or polymyalgia rheumatica. The relationship between the physical examination of the temporal regions, the ophthalmological examination, and biopsy from the temporal artery with respect to the diagnostic value were examined. Histological examination of biopsy specimens from the temporal artery in 58 patients revealed arteritis in 46. Half of the patients had only local symptoms from the temporal regions; one fourth presented such symptoms as well as myalgias, and one fourth had myalgias only. Patients presenting local symptoms of temporal arteritis as well as of myalgias had always had myalgias as the initial symptom and developed local symptoms of temporal arteritis 1-24 months later. Permanent reduction of vision occurred in 20% of the patients. Symptoms of generalized arteritis were observed in several patients. The overlapping of the clinical symptoms, the positive biopsy findings in patients with polymyalgia rheumatica as the only local symptom and the identical reaction to corticosteroid treatment support the conception of temporal arteritis and polymyalgia rheumatica as two manifestations of the same disease. The physical and the ophthalmological examinations were of limited diagnostic value. Positive biopsy findings were seen in 25 patients with noraml palpatory findings, and in 46 patients without eye symptoms the ophthalmoscopic examination revealed no signs of arteritis. If the first biopsy from the temporal artery is negative, biopsy from the contralateral temporal artery should be performed. Correctly timed corticosteroid treatment in adequate doses can prevent reduction of vision in giant-cell arteritis. The treatment is a long-term therapy, its average duration in the present study being more than two years.

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Changes in bone mass during low dose corticosteroid treatment in patients with polymyalgia rheumatica: a double blind, prospective comparison between prednisolone and deflazacort.

OBJECTIVE: To compare the long term effects of low dosage prednisolone or deflazacort treatments on bone mass in patients with polymyalgia rheumatica. METHODS: Thirty patients with polymyalgia rheumatica were allocated on a random double blind basis to receive treatment with prednisolone or deflazacort. Bone mineral content (BMC) was measured in the lumbar spine and in the distal forearm before treatment and three, six, and 12 months after treatment. RESULTS: At three months the decrease in lumbar BMC and bone mineral density (BMD) was significantly greater in the deflazacort group than in the prednisolone group (p < 0.05), but at six and 12 months there was no difference between the two groups. In all patients after one year there was a significant loss of BMC: a 6.4% loss in lumbar BMC and a 1.8% loss in distal forearm BMC. Loss in lumbar BMC after six months was correlated to the cumulative dose of corticosteroid (r = 0.4; p < 0.05) and was significantly greater in the group of patients who had persisting symptoms of polymyalgia at six weeks, three months, or both, after treatment started (p = 0.05). CONCLUSION: This low dose study failed to reveal any calcium sparing properties of deflazacort compared with prednisolone. Possible explanations for this finding are discussed.

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Diagnostic errors in polymyalgia rheumatica and temporal arteritis.

Incomplete clinical response or persistence of a rapid ESR despite corticosteroid treatment of polymyalgia rheumatica or temporal arteritis should always arouse suspicion and prompt a search for other diagnoses. Lumbar spinal stenosis and Pancoast's tumor are two unusual entities that can complicate or compete for the diagnosis of polymyalgia rheumatica and temporal arteritis.

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Skeletal manifestations of polymyalgia rheumatica.

In a review of 39 patients with polymyalgia rheumatica (PMR), sternoclavicular and acromioclavicular arthropathy was noted in 15% and 10% of the patients, respectively. Diffuse clavicular swelling preceded the onset of PMR in one patient. Carpal tunnel syndrome was seen in four patients (10%).

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Temporal lobe meningioma masked by polymyalgia rheumatica.

An elderly woman who had a typical polymyalgia rheumatica (PMR) syndrome, associated with severe headache, was considered to have giant cell arteritis despite negative temporal artery biopsies and was treated with corticosteroids. At autopsy, no arteritis, but a large temporal lobe meningioma, was found as the cause of her headache. Both PMR and meningiomas are frequent in elderly patients, and overlapping symptoms may occur. Patients with PMR and headache should be fully investigated to exclude an occult meningioma or other disease, especially when no positive histologic evidence of temporal arteritis can be obtained.

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Secondary (AA-type) amyloidosis in patients with polymyalgia rheumatica.

Several cases of systemic amyloidosis associated with polymyalgia rheumatica (PMR) or giant-cell arteritis (GCA) have been described. Nevertheless, the type of amyloid deposit has not been characterized in most of them. Here we report on two patients with PMR (one with associated GCA) who developed nephrotic syndrome and end-stage renal failure caused by massive amyloid deposition. Immunohistochemical analysis showed that the amyloid deposits were of AA type (secondary amyloidosis) in both cases.

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Ultrastructural examination of the skeletal muscles in polymyalgia rheumatica.

Skeletal muscle biopsies of 13 patients with polymyalgia rheumatica (PMR) were examined systematically with the electron microscope. Ultrastructural changes in the PMR specimens have been classified according to 14 criteria including nucleus, myofilaments, mitochondria, T-system, as well as glycogen deposition, lipid, lipofuscin, and myelin figures. The described muscle changes are of both regressive and progressive character and non-specific in themselves. The systematic recording of these criteria shows an extraordinaryly high incidence in PMR. This constellation of characteristics gives the ultrastructural picture of the skeletal musculature in PMR a definite profile. Similarities with the changes in other muscular diseases suggest that in PMR also neurogenous mechanisms may play a pathogenetic role.

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Polymyalgia rheumatica: a comprehensive review of this debilitating disease.

Polymyalgia rheumatica (PMR) is a cause of moderate to severe musculoskeletal pain in older people. This debilitating disease is characterized by bilateral proximal aching and morning stiffness in the neck, shoulders, upper arms, lower back, hips, and thighs. This article includes specific assessments related to PMR and an associated disease, giant cell arteritis (GCA). A comprehensive review of the incidence, etiology, duration of illness, pertinent laboratory studies, and medical treatment is provided for health care practitioners. A patient teaching handout summarizes key concepts. Specific clues that help the practitioner recognize the disease in patients and associated treatments are emphasized.

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A seasonal pattern in the onset of polymyalgia rheumatica.

The seasonal distribution in the onset of polymyalgia rheumatica (PMR) was determined in 58 patients with the disease and compared with that in 44 patients affected by rheumatoid arthritis of elderly onset (EORA). Thirty six (62%) cases of PMR developed during May to August; by contrast, only 14 (31%) cases of EORA developed in the same months, this latter disease failing to show any seasonal clustering. The monthly distribution of PMR correlated with outside temperature and hours of sunshine. These data suggest that PMR might be triggered by such factors as actinic damage of superficial vessels or infective agents with a seasonal cycle. Finally, the summer clustering of PMR may be helpful in the differential diagnosis from EORA.

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Polymyalgia rheumatica and chronic lymphatic leukaemia.

A patient with polymyalgia rheumatica (PMR) is reported in whom, at the time of diagnosis, the number of lymphocytes in peripheral blood and bone marrow was normal. Lymphocytic infiltration typical of PMR was detected in renal and muscle biopsy specimens, and a liver fine-needle aspirate contained an abnormal lymphocytic infiltration, probably leukaemic. The number of lymphocytes gradually increased in bone marrow and peripheral blood until, almost 5 years after PMR had been diagnosed, a typical picture of chronic lymphatic leukaemia (CLL) was observable. The significance of the reported combination is discussed. The possibility is pointed out that a CLL in progress must be suspected when persistent lymphocytosis occurs in a patient with typical PMR, even if initially the response to treatment with corticosteroids suggests that PMR is the sole disorder.

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Peripheral blood lymphocyte subsets in polymyalgia rheumatica.

Peripheral blood lymphocytes from 23 patients with polymyalgia rheumatica (PMR) were characterized using monoclonal antibodies and flow cytometry in a two-year prospective study. There were no significant differences in absolute numbers or relative percentages of lymphocytes or CD3+, CD4+, CD8+ T cells or the CD4+ T cell functional subsets, virgin (CD4+CD45RA+) and memory (CD4+CD29+) T cells, in patients before or during corticosteroid treatment compared to controls. Previous reports on decreased levels of CD8+ T cells as a characteristic of PMR/giant cell arteritis was not confirmed. The absolute number and relative percentage of lymphocytes with natural killer cell activity, CD16+ CD56+ cells, were significantly lower in patients with active untreated PMR as well as during corticosteroid treatment compared to controls, but at the two-year follow-up the difference was less marked.

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Genetic epidemiology. Giant cell arteritis and polymyalgia rheumatica.

Giant cell arteritis (GCA) (temporal arteritis) and polymyalgia rheumatica (PMR) are common, frequently related conditions in people generally over 50 years of age. Most studies have shown an association of GCA with HLA-DRB1 *04 alleles. As regards isolated PMR, however, the HLA class II genetic susceptibility varies from one population to another. Besides associations with HLA, tumor necrosis factor appears to influence susceptibility to both conditions. Genetic polymorphisms have also been considered to be important candidates as factors of susceptibility to GCA and PMR. In this regard, gene polymorphisms for ICAM-1 (intercellular adhesion molecule 1), RANTES (regulated upon activation, normal T cell expressed, and presumably secreted), and interleukin (IL)-1 receptor antagonist seem to play a role in the pathogenesis of GCA and PMR in some populations. However, additional studies are required to clarify the genetic influence on susceptibility to these conditions.

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