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[Aortic involvement in patients with temporal arteritis and polymyalgia rheumatica].

In 4 patients with temporal arteritis or polymyalgia rheumatica, women aged 60, 57, 83 and 73 years respectively, signs of aortic involvement were established. The first patient presented with signs of systemic inflammation without signs of temporal arteritis or aortitis. In the second, an acute symptomatic thoracoabdominal aneurysm developed. In the third, temporal arteritis was associated with chronic progressive dilatation ofthe thoracic aorta. The fourth developed signs of intermittent claudication of the extremities. The clinical manifestations in all patients were attributed to chronic inflammation of the aorta caused by giant cell arteritis. Aortic giant cell arteritis frequently accompanies temporal arteritis, but is rarely diagnosed. Up to 75% of patients with temporal arteritis may have some degree of aortic involvement. Thoracic aneurysms, complicated by rupture or dissection, are the most serious complications. Aortic disease associated with signs of systemic inflammation should trigger the suspicion of giant cell arteritis. Corticosteroids are the most important part of treatment. Three patients recovered following treatment; the first two received an endoprosthesis; in the woman aged 83 years, this was not technically possible; she died after 1.5 years.

Adrenal Cortex Hormones↗

Polymyalgia rheumatica with normal erythrocyte sedimentation rate: clinical aspects.

Polymyalgia rheumatica is a relatively common syndrome of the elderly characterized by severe pain and stiffness in the neck shoulder and pelvic girdles, along with increased acute phase reactants. The current diagnostic criteria include as a requirement an erythrocyte sedimentation rate (ESR) higher than 30 or 40 mm/1 hr. Nevertheless, in several reports, a sizable proportion of patients with PMR, from 7% up to 22%, had an ESR that was either normal or slightly increased at the time of diagnosis, supporting the notion that an increased ESR should not be an absolute requirement for its diagnosis. This subset is characterized by a younger age, a less marked predominance of females, a longer diagnostic delay and a lower frequency of constitutional symptoms such as fever or weight loss. When determined, a majority of these patients had increased levels of C reactive protein. The recognition of this subset of patients with PMR and a low ESR can be very useful to the clinician, avoiding unnecessary suffering of the patients and the risks of ineffective therapy with nonsteroidal antiinflammatory drugs. To avoid the over-diagnosis of PMR in patients with a low ESR, we propose a set of criteria that can be easily applied in daily practice to patients with a clinical picture consistent with PMR but with a normal or slightly increased ESR.

Blood Sedimentation↗

Serum cytokines and steroidal hormones in polymyalgia rheumatica and elderly-onset rheumatoid arthritis.

BACKGROUND: Polymyalgia rheumatica (PMR) may create some difficulties in the differential diagnosis of elderly-onset rheumatoid arthritis (EORA) and of EORA with PMR-like onset (EORA/PMR). AIM: To investigate possible differences between three groups of patients, with regard to serum levels of inflammatory cytokines and steroidal hormones at baseline and after 1 month of treatment with glucocorticoids (prednisone 7.5-12.5 mg/day). PATIENTS AND METHODS: 14 patients with PMR, 15 with EORA and 14 with EORA/PMR, as well as 15 healthy, matched controls were analysed. Tumour necrosis factor alpha (TNFalpha), interleukin (IL)6, IL1 receptor antagonist (IL1Ra), cortisol, dehydroepiandrosterone sulphate (DHEAS) and 17-hydroxy-progesterone (PRG) were evaluated. RESULTS: Serum levels of both TNFalpha and IL6 were significantly higher in all three groups of patients than in controls (p<0.01). Serum IL6 levels were significantly higher in patients with both PMR and EORA/PMR than in patients with EORA (p<0.05). IL1Ra serum levels were significantly higher in patients with EORA than in controls (p<0.001) and in patients with PMR and EORA/PMR (p<0.05). DHEAS was significantly lower in patients with EORA/PMR than in those with EORA (p<0.05). PRG was significantly higher in all patient groups (p<0.05). After glucocorticoid treatment, serum TNFalpha and IL6 levels significantly decreased in all patient groups; IL1Ra significantly increased in patients with PMR and in those with EORA/PMR; cortisol, DHEAS, and PRG significantly decreased in patients with PMR and in those with EORA/PMR (p<0.05). CONCLUSIONS: Different cytokine and steroidal hormone patterns suggest that patients with PMR and those with EORA/PMR seem to be have a more intensive inflammatory reaction and are more efficient responders to glucocorticoid treatment than patients with EORA.

17-alpha-Hydroxyprogesterone↗

Corticotropin releasing hormone promoter polymorphisms in giant cell arteritis and polymyalgia rheumatica.

OBJECTIVE: Giant cell (temporal) arteritis (GCA) and polymyalgia rheumatica (PMR) are different but overlapping diseases of unknown etiology affecting the elderly. Corticotropin-releasing hormone (CRH) helps to regulate the immune response and maintain homeostasis during inflammatory stress. CRH promoter region polymorphisms in the 5'regulatory region of the CRH gene have been described. To investigate the possible implications of the CRH promoter polymorphisms in PMR and GCA susceptibility we have examined a series of patients with these conditions. METHODS: Sixty-two patients with biopsy-proven GCA, 86 patients with isolated PMR and 147 ethnically matched controls from the Lugo region of Northwest Spain were included in this study. Patients and controls were genotyped for CRH polymorphism in the 5' regulatory region of the gene at position 1273 (alleles A1 andA2) and at position 225 (alleles B1 and B2) by PCR-restriction fragment length polymorphism. Allele frequencies and genotype distribution were evaluated by the chi-square test. RESULTS: When GCA and PMR patients were examined for alleles and genotypes for each CRH polymorphism no significant differences in frequency were found compared with controls. A higher CRH-A2 allele frequency was observed in GCA patients with visual complications (21.4%) compared with controls (9.2%) and GCA cases without eye involvement [6.3%; p= 0.017, Pcorr = 0.034, O.R: 4.1 (95% CI 1.2- 13.9)], although this was based on a small sample of patients with ischemic visual complications (n = 14) and should be interpreted with caution. No differences in CRH allele or genotype frequencies were observed in isolated PMR patients stratified by relapses and recurrence of disease symptoms. CONCLUSION: Polymorphisms in the CRH gene regulatory region do not appear to be associated with increased susceptibility to PMR or GCA. The CRH-A2 allele may encode risk for the development of visual complications in GCA, although further studies to confirm this will be required.

Aged↗

Calcium pyrophosphate deposition disease mimicking polymyalgia rheumatica: a prospective followup study of predictive factors for this condition in patients presenting with polymyalgia symptoms.

OBJECTIVE: To assess the characteristics of calcium pyrophosphate deposition disease (CPDD) with proximal involvement mimicking polymyalgia rheumatica (PMR), and to identify the best predictive factors for the presence of a clinical pattern of CPDD in patients presenting with polymyalgia symptoms. METHODS: Patients diagnosed with either PMR or CPDD at the Rheumatology Division of Hospital Meixoeiro (Vigo, Spain) over a 7-year period (1997-2003) were prospectively followed for at least 12 months. RESULTS: The study group comprised 118 patients with PMR features and 112 patients with CPDD. Eighty-two of the 118 patients with PMR manifestations were diagnosed as having pure PMR, and 36 met the diagnostic criteria for both PMR and CPDD. Patients with CPDD mimicking PMR were older (P = 0.02) and had peripheral arthritis more frequently (P = 0.004) than those with pure PMR. Radiologic osteoarthritic changes in the hands and knees, including more advanced radiologic grade of knee osteoarthritis, and tendinous calcifications were more frequent in patients with PMR/CPDD (P < 0.001). The best predictive factors for the occurrence of this atypical pattern of CPDD in a patient presenting with PMR features were the age at diagnosis and the presence of tibiofemoral osteoarthritis, tendinous calcifications, and ankle arthritis. CONCLUSION: Involvement of proximal joints may be the clinical presentation of CPDD. CPDD should be included in the spectrum of diseases mimicking PMR. The presence of tibiofemoral osteoarthritis, tendinous calcifications, and ankle arthritis are clues that may alert the clinician to the presence of CPDD in an elderly patient presenting with PMR manifestations.

Aged↗

Clinical and pathologic studies of synovitis in polymyalgia rheumatica.

Clinically detectable joint swelling was found in 10 of 13 fully evaluated patients considered to have polymyalgia rheumatica. Five patients had some joint findings at disease onset. Knees were most commonly affected. Sternoclavicular involvement was seen in 3 patients. Joint effusions in 8 patients had 300-5,700 leukocytes/mm3 with a mean of 2,900. Six synovial biopsy specimens studied by light microscopy revealed mild to moderate synovial proliferation and chronic inflammation that was generally less severe than in typical rheumatoid arthritis. Electron microscopy identified microvascular changes and large amounts of vesicular and granular debris in lining cells. In 1 patient, a "fingerprint" pattern in the granular material was suggestive of the findings in some immune complexes. This still unexplained synovitis may, as previously suggested, be important in the pathogenesis of the polymyalgia rheumatica syndrome.

Adult↗

Polymyalgia rheumatica as a manifestation of a large hepatic cavernous hemangioma.

A 59-year-old woman presented with polymyalgia rheumatica which was refractory to conventional anti-inflammatory and steroid therapy. A full investigation for an underlying occult malignancy showed only the presence of a giant cavernous hepatic hemangioma. To our knowledge, polymyalgia rheumatica has never been described in association with giant cavernous hepatic hemangioma; resection of the latter lesion resulted in complete and, to this date, definite resolution of rheumatologic complaints in our patient.

Female↗

Fatal renal failure in polymyalgia rheumatica caused by disseminated giant cell arteritis.

A 62-year-old man with classical signs of polymyalgia rheumatica for one year suddenly developed fatal renal failure, despite treatment with steroids. Autopsy showed disseminated giant cell arteritis in the kidneys, pancreas, oesophagus, and larynx, with the most pronounced changes in the kidneys. All intrarenal arteries and arterioles were affected. The changes ranged from occlusive intimal proliferation to severe fibrinoid necrosis in the whole arterial wall. Fibrinoid necrosis was also found in the afferent and efferent arteries and several thromboses were seen in the glomeruli. It is not known why this apparently non-complicated case of polymyalgia rheumatica suddenly turned into a fatal case of disseminated giant cell arteritis. The only precipitating factor to be incriminated seems to be a reduction in the corticosteroid dose. This case adds further support to suggestions of the existence of a distinct type of systemic giant cell arteritis.

Acute Kidney Injury↗

[Immunohistological and histological findings in the temporal artery in polymyalgia rheumatica].

Immunofluorescent and light-microscopic investigations of the temporal artery were carried out in 36 patients with polymyalgia rheumatica. An arteritis was seen in 21 patients. Depositions of fibrin, immunoglobulins, and complement were found in the wall of the temporal artery and/or the vasa vasorum in 24 patients. 14 control patients showed neither an arteritis nor depositions of complement. Positive results were obtained in 91.6% of the patients with polymyalgia rheumatica when simultaneous light-microscopic and immunofluorescent examinations of the temporal artery were carried out.

Adult↗

HLA-DR locus antigens in polymyalgia rheumatica and giant cell arteritis.

HLA-DR locus antigens were determined in 69 patients with giant cell arteritis, polymyalgia rheumatica, or both. The frequencies of HLA-DR4 and HLA-DRW6 in these patients were increased above normals. But the differences were not significant in the total group nor when the patients were separated according to the presence or absence of polymyalgia rheumatica.

Aged↗

Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases.

BACKGROUND: The crowned dens syndrome, related to microcrystalline deposition in the peri-odontoid articular and abarticular structures, is mainly responsible for acute or chronic cervical pain. PATIENTS: We report eight cases of crowned dens syndrome with atypical presentations mimicking giant cell arteritis, polymyalgia rheumatica, meningitis or discitis. The clinical and radiological aspects of these cases are presented and discussed. RESULTS: For all patients, fever, cervical stiffness, headaches and biological inflammatory syndrome were reported. For three patients, impairment of general condition, occipito-temporal or mandible pain and weakness with inflammatory pain of the shoulder girdle was suggestive of giant cell arteritis and/or polymyalgia rheumatica, leading to temporal artery biopsy and/or long-term steroid treatment. Recurrence of clinical symptoms when tapering steroids was noted. In two cases, previous breast carcinoma led to the initial diagnosis of metastatic spondylitis. For three patients with vomiting, nausea and Kernig's and/or Brudzinski's sign, the first diagnosis was meningitis, leading to unhelpful lumbar puncture. In all cases, diagnosis of crowned dens syndrome once evoked, was confirmed by cervical CT scanning and dramatic improvement with non-steroidal anti-inflammatory drugs or colchicine. CONCLUSION: This under-recognized entity must be considered as a differential diagnosis of meningitis and discitis, but also of giant cell arteritis and polymyalgia rheumatica, as well as a possible aetiology for fevers of unknown origin. CT scanning is necessary for diagnosis. Clinicians should be aware of such misleading clinical presentations.

Aged↗

Serum C-reactive protein in polymyalgia rheumatica. A prospective serial study.

A prospective serial study of 13 well-documented, previously untreated cases of polymyalgia rheumatica was undertaken in order to assess the behavior of the nonspecific indices of disease activity, erythrocyte sedimentation rate and serum C-reactive protein (CRP) concentration, during induction of disease remission by prednisolone therapy. The clinical manifestations of all patients responded rapidly and completely to steroids, and the serum CRP value, which was raised in all patients at presentation, fell to normal at a rate which precisely reflected the clinical improvement. The erythrocyte sedimentation rate also fell, but did so much more slowly than the CRP concentration and, in half the patients, was still not normal after 14 days. These results indicate that assay of serum CRP provides a precise means of objectively assessing the course of polymyalgia rheumatica during initial therapy with steroids, and suggest that routine measurements of CRP may make a useful contribution to the management of the disease.

Aged↗

[Polymyalgia rheumatica and myelitis associated with anti-cardiolipin antibody].

A 78-year-old woman was admitted to our hospital on September 14, 1992, because of systemic myalgia and stiffness, joint pain, and gait disturbance. She had begun to feel headache and pain in the neck and shoulder in the middle of August, 1992. The pain became systemic, and was accompanied by a low-grade fever, which was unresponsive to NSAIDs. On admission, she had no joint swelling or deformities in the extremities. Neurological examination revealed weakness in the right leg, hypoalgesia below the left C4 level, hyperreflexia in the right extremities, and right Babinski's sign. The erythrocyte sedimentation rate was very high (100 mm/h). Levels of other acute phase reactants were also high. Tests for antinuclear antibody and anti-cardiolipin antibody were positive, but a test for rheumatoid factor was negative. Creatine kinase activity was within normal limits. A T1-weighted magnetic resonance image of the cervical spine at 0.5 T showed an intramedullary low signal. A T2-weighted image showed a borderless spindle-like high signal. Four nodules enhanced by Gd DTPA were seen at C1-C4. The age at onset, myalgia, stiffness, and erythrocyte sedimentation rate were considered to be consistent with a diagnosis of polymyalgia rheumatica. Glucocorticoid treatment was therefore started, and a dramatic clinical improvement was evident within a few days. The patient was discharged from hospital on November 30, 1992. To our knowledge, myelopathy complicated by polymyalgia rheumatica has never been reported previously. Recently, some patients with polymyalgia rheumatica have been reported to have anti-cardiolipin antibody in serum. In the present case anti-cardiolipin antibody may have played a role in the formation of microemboli or in angitis of the cervical spine.

Aged↗

Peripheral joint involvement in polymyalgia rheumatica: a clinical study of 56 cases.

A follow-up study of 56 patients with the provisional diagnosis of polymyalgia rheumatica showed that 12 developed peripheral synovitis during the course of the illness. Five of the 12 were noted to have synovitis on presentation which was characteristically mild, pauci-articular and cleared quickly after commencing prednisolone therapy. The remaining seven had persistent synovitis of peripheral small joints despite an initial brisk response to oral prednisolone. Five of these patients were found to satisfy the ARA criteria for rheumatoid arthritis after a mean follow-up period of 3.5 years. It is suggested that the persistence of peripheral synovitis in patients treated as polymyalgia rheumatica should alert the clinician to the possible development of rheumatoid arthritis.

Aged↗

Delayed onset of polymyalgia in polymyalgia rheumatica.

Four female patients presenting with systemic symptoms of polymyalgia rheumatica are described. They suddenly developed classical girdle pains up to a year after the onset of severe systemic symptoms, a course not often emphasized.

Aged↗

Giant cell arteritis and polymyalgia rheumatica.

The close relationship between giant cell arteritis and polymyalgia rheumatica has not been clearly explained. These disorders affect the same patient population and often coexist in the same person. Monitoring of the erythrocyte sedimentation rate is a useful tool in both diagnosis and treatment. Management with varying doses of prednisone has proved effective in resolving symptoms.

Diagnosis, Differential↗

Methotrexate for corticosteroid-resistant polymyalgia rheumatica and giant cell arteritis.

Three patients (one with polymyalgia rheumatica with jaw claudication and two with biopsy-proven giant cell arteritis) were initially treated using prednisone (40-60 mg daily). The response was good in all three, but each experienced exacerbation of symptoms and elevation of Westergren sedimentation rates (WSR) with dose reduction. The addition of methotrexate (7.5-12.5 mg/wk) resulted in diminished symptoms and lower WSR and proved to be steroid-sparing.

Aged↗

Giant cell arteritis (cranial arteritis, polymyalgia rheumatica).

Giant cell arteritis, which is probably due to disturbed immune mechanisms, has a spectrum of clinical symptoms in elderly people. In nearly all cases such general signs as loss of appetite, loss of weight and fever are present. The sedimentation rate is almost without exception about 100 mm in the first hour. The two most frequent and typical clinical syndromes are polymyalgia rheumatica and cranial arteritis. The polymyalgia rheumatica is characterized by periarticular pain which is mostly symmetrical and accentuated in the shoulder girdle. Increasingly severe temporal headache and ocular distrubances are found with cranial arteritis in more than 50% of cases. A combination of both diseases is frequent. Other arterial branches are rarely involved. The course of the disease is over a period of 1 1/2 to 2 years. Treatment with corticosteroids is indicated mainly because of the severe ocular complications with blindness. It should begin immediately, be intensive and last over a long period. Regular followup is necessary over several years in order to avoid relapses.

Age Factors↗