Successful treatment of dissecting aortic aneurysm due to giant cell arteritis.

A 72-year-old women with polymyalgia rheumatica clinically controlled on maintenance steroid therapy presented with symptoms of chest pain and numbness in the right arm. A diagnosis of dissecting aortic aneurysm was confirmed at thoracotomy and the aorta was successfully resected. Histology revealed active giant cell aortitis. We suggest that a normal erythrocyte sedimentation rate in patients with treated temporal arteritis does not preclude large vessel involvement.     

Myalgien bei Polymyalgia rheumatica, Arteriitis temporalis und anderen Vaskulitiden

Myalgias most commonly occur in polymyalgia rheumatica (PMR). About 45% of patients with giant cell arteritis present with symptoms of PMR. Other vasculitides may also lead to arthralgia and myalgia. While shoulder and pelvic pain is characteristic for PMR pain often also occurs in the back of the neck and in the region of the thoracic spine. In addition, patients often present with malaise, morning stiffness and weight loss. CRP and ESR are elevated. Ultrasound and MRI delineate minor synovitis, tenosynovitis and bursitis in the shoulder. Hip joint synovitis and trochanteric bursitis are also commonly seen. PMR should be distinguished from rheumatoid arthritis. The initial treatment comprises a prednisolone dose of 15-25 mg/day, followed by a weekly decrease of 1-2.5 mg. Once 10 mg/day has been reached the dose should be reduced more slowly.     

Rheumatoid arthritis in the elderly, presenting as polymyalgia rheumatica.

Two elderly patients presented with generalized aches and pains (particularly in the shoulders and the pelvic girdle), stiffness, fatigue, anemia, and an elevated erythrocyte sedimentation rate, but there were no signs or symptoms directly referable to the joints. Two and five months later respectively, pain, swelling, and signs of synovitis appeared in several joints in a symmetrical pattern, and a diagnosis of rheumatoid arthritis was made. Rheumatoid arthritis in the elderly may resemble polymyalgia rheumatica. On the other hand, synovitis in many patients with polymyalgia rheumatica may resemble rheumatoid arthritis. In the elderly, the differentiation of these two entities may be difficult. Moreover, patients initially presenting with the signs and symptoms of polymyalgia may eventually manifest typical rheumatoid arthritis.     

Giant cell (temporal) arteritis diagnosed following upper limb claudication

Giant cell arteritis or temporal arteritis occurs almost exclusively in people over 50 years of age. It classically presents with new onset temporal headache, scalp tenderness and jaw claudication. Proximal muscle pain and stiffness is often present because of frequent association with polymyalgia rheumatica. In most cases, the erythrocyte sedimentation rate is markedly elevated. Uncommon presentations include systemic symptoms and symptoms related to large artery involvement. We report a case of giant cell arteritis without symptoms related to the temporal artery, diagnosed angiographically following upper limb claudication and confirmed by temporal artery biopsy.     

Temporal arteritis after normalization of erythrocyte sedimentation rate in polymyalgia rheumatica

We describe a 72-year-old woman with classic presentation of polymyalgia rheumatica and an elevated erythrocyte sedimentation rate. After treatment with low-dose corticosteroids and normalization of the sedimentation rate, hematocrit, and serum alkaline phosphatase, she developed symptoms and biopsy proved temporal arteritis. In patients with polymyalgia rheumatica and an initially elevated sedimentation rate, normalization of the sedimentation rate with corticosteroid therapy does not exclude the need for temporal artery biopsy if the symptoms warrant it.     

Presentación de un caso de derrame pleurocárdico en un paciente diagnosticado de polimialgia reumática

Polymyalgia rheumatica is an inflammatory rheumatic disease that presents with bilateral pain and stiffness affecting mainly proximal muscles. It affects individuals over 50 years of age and it is usually associated with a raised erythrocyte sedimentation rate. Classically, treatment with low-dose corticosteroids results in a dramatic improvement in both symptoms and laboratory findings.We report the case of an 80 years old patient presenting polymyalgia rheumatica coinciding with pleuropericardial effusion. The patient had a very good response to treatment with rapid improvement in the symptomatology and laboratory findings.Polymyalgia Rheumatica is a common disease but it is rarely associated to pleuropericardial effusion. It should be considered in the differential diagnostic in patients presenting with pericardial effusion over 50 of age years due to the good response to treatment.     

Serum amyloid A as a potent therapeutic marker in a refractory patient with polymyalgia rheumatica

We report a patient with polymyalgia rheumatica (PMR) who showed a relapse soon after tapering of oral prednisolone. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were quickly normalized after the re-increase in oral prednisolone, and muscle pain and stiffness gradually improved in parallel with a decrease in serum amyloid A (SAA). Flow cytometry simultaneously demonstrated an increase in CD8+CD25+ cells and a decrease in CD4+CD25+ cells and CD4+CD45RA+ cells. When clinical symptoms remain with negative results for CRP and ESR even after the start of corticosteroid treatment, SAA might be a potent therapeutic marker for disease activity in PMR.     

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.     

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.     

Polymyalgia rheumatica and rheumatoid arthritis of the elderly: a clinical, laboratory, and scintigraphic comparison.

Clinical, laboratory, and scintigraphic features of 16 patients with polymyalgia rheumatica and 23 patients matched for age presenting with classical or definite rheumatoid arthritis (American Rheumatism Association 1958 criteria) of the elderly were compared in order to define features that might distinguish between these two syndromes. The sensitivity of proposed diagnostic criteria for polymyalgia rheumatica was always higher in the group with polymyalgia rheumatica, though only significantly so for morning stiffness. A comparison of 27 different laboratory features showed few significant differences between the diseases, though correlation between laboratory variables within each of the disease groups differed, perhaps suggesting a fundamental pathogenetic difference between them. Scintigraphy of the shoulder joint proved of no value in differential diagnosis. It was concluded that polymyalgia rheumatica and rheumatoid arthritis of the elderly are probably discrete clinical entities. Bilateral upper arm tenderness, lack of positive rheumatoid factor, and a normal caeruloplasmin are the most valuable features for distinguishing polymyalgia rheumatica from rheumatoid arthritis of the elderly.     

Acute pseudogout of the neck��the crowned dens syndrome: 2 case reports and review of the literature

Acute pseudogout of the neck, also known as the crowned dens syndrome, is a rare cause of neck pain characterised by crystalline deposition in periodontoid articular tissues. It is typified clinically by severe cervical pain and stiffness, often in conjunction with pyrexia and raised inflammatory markers. As such, it is often misdiagnosed. We report 2 cases of crowned dens syndrome masquerading respectively as meningitis and polymyalgia rheumatica, and review the literature with particular attention to the clinical and radiological aspects of this under-recognised condition.     

Polymyalgia rheumatica and type 2 diabetes mellitus complicated with electromyographic abnormalities that responded well to corticosteroid therapy

A 62-year-old man who had a 14-year history of diabetes complained of low-grade fever, general malaise, pain of bilateral femurs and hip girdle, and was adniitted to our hospital. The diagnosis of polymyalgia rheumatica (PMR) was made from the clinical symptoms, elevated C-reactive protein and erythrocyte sedimentation rate. Electromyography revealed abnormalities that suggested diabetic peripheral neuropathy. However, the abnormalities were improved after starting treatment with corticosteroids (PSL). After stopping PSL, electric nerve conduction disturbance developed; therefore, it was suggested that peripheral nerve involvement due to PMR was improved by administration of PSL regardless of the existence of diabetic peripheral neuropathy.     

Unilateral polymyalgia rheumatica with controlateral sympathetic dystrophy syndrome. A case of asymmetrical involvement due to pre-existing peripheral palsy

SIR, We describe the case of a man with pre-existing peripheralpalsy who developed polymyalgia rheumatica (PMR); he was sparedarthritis in the affected limb but experienced reflex sympatheticdystrophy syndrome (rSDS) in the paretic arm. A 72-year-old man was admitted to our hospital because of nocturnalpain in his right shoulder and the pelvic girdle, with long-lastingmorning stiffness, mild fever and weight loss. The pain in hisbuttocks and thighs was so severe that it limited his abilityto stand and walk. His knee reflex responses     

Polymyalgia rheumatica: clinical picture and principles of treatment

Polymyalgia rheumatica (PMR) is a common disease of the elderly. It is characterized by pain and stiffness in the neck, shoulders and the pelvic girdle. In most cases erythrocyte sedimentation rate and C-reactive protein levels are highly elevated. Polymyalgia rheumatica is frequently associated with giant cell arteritis. Steroids are the standard treatment for PMR but their dosage requires adjustment depending on clinical picture, co-morbid conditions and adverse effects. The most prominent features of the disease as well as the main principles of treatment are presented.     

Polymyalgia Rheumatica Following COVID-19 Vaccination

We herein report a 71-year-old woman presented with a fever, arthralgia, general malaise and leg muscle stiffness following administration of the COVID-19 mRNA vaccine (Comirnaty, Pfizer-BioNTech). Laboratory findings showed an elevated C-reactive protein level and erythrocyte sedimentation rate. In addition, Gallium-67 scintigraphy demonstrated an increased uptake in multiple joints. Typing of human leukocyte antigen (HLA) revealed the presence of the DRB1*0404/*0803 allele. These findings met the diagnostic criteria for polymyalgia rheumatica (PMR), and when we started steroid treatment, her symptoms improved rapidly. This patient developed PMR after receiving a COVID-19 mRNA vaccine (Comirnaty, Pfizer-BioNTech). This case is considered to be valuable, as the HLA-DRB1 allele was also confirmed.     

From diagnosis to remission: place of MRI in eosinophilic fasciitis

Eosinophilic fasciitis is a rare disease, but it must be considered in patients with adult myalgia. Here, we report the case of 32-year-old man who presented with a 4-month history of bilateral myalgia of the lower limbs, which subsequently spread to the upper limbs. There was no specific medical history, no trauma, and no intense activity. Physical examination revealed induration of the skin with irreducible flexion of the fingers as observed in scleroderma. Laboratory examination showed peripheral eosinophilia associated with moderate elevation of both the erythrocyte sedimentation rate and C-reactive protein. No infectious, neoplastic, hemopathic, or immunological abnormality was detected. Magnetic resonance imaging (MRI) showed thickening of the muscular fascias of the thigh in high signal intensity on T2-weighted images, slightly enhanced after contrast agent injection on T1 images, without myositis. MRI-guided muscle biopsy demonstrated fascia infiltration characterized by mononuclear inflammatory cells and polynuclear eosinophils. A diagnosis of eosinophilic fasciitis was confirmed and the patient received prednisolone. One month later, he reported improvements in general health, pain, motion, joint mobility, and skin induration associated with normalization of both hypereosinophilia and biologic inflammation. After 12 months, clinical MRI and laboratory parameters were normal and the patient was considered to be in clinical remission.     

Polymyalgia rheumatica

Polymyalgia rheumatica is a syndrome that occurs in the elderly and is characterized by pain and stiffness involving the neck, the shoulder girdle, and the hip girdle. The aching should be present for greater than one month. Polymyalgia rheumatica may be more common than reported. The etiology remains unknown. There is generally little found pathologically in this disease. The physical examination is often not impressive. Synovitis may be a main contributing factor to many of the symptoms seen in patients with polymyalgia rheumatica. Symptoms often do not correlate with physical findings. Polymyalgia rheumatica must be differentiated from many conditions since the diagnosis remains entirely clinical. Osteoarthritis, flu syndromes, inflammatory myopathies, fibromyalgia, and depression all have features that may mimic polymyalgia rheumatica. Malignancies and infections may also be difficult to separate from polymyalgia rheumatica. Polymyalgia rheumatica may also be extremely difficult to differentiate from seronegative rheumatoid arthritis in patients older than 50 years. Although some patients with polymyalgia rheumatica have underlying giant cell arteritis, the majority apparently do not. The distinction between polymyalgia rheumatica and giant cell arteritis cannot be made on the basis of laboratory studies and relies solely on clinical symptoms and physical findings. Although nonsteroidal antiinflammatory medications may control symptoms in patients with mild disease, most patients with polymyalgia rheumatica require low-dose corticosteroids. The tapering schedule for the corticosteroids is contingent upon the response of symptoms and laboratory parameters. Polymyalgia rheumatica usually follows a benign course with almost complete response to an adequate treatment program. Recently, there have been several studies suggesting that the course of polymyalgia rheumatica may not be as short and simple as once proposed. Nevertheless, many patients may be completely weaned from corticosteroids. Other agents have been used in this disease, but for the most part their use remains somewhat controversial. Patients must be monitored carefully. Most patients do well, and treatment is effective.     

Correlation between 18-fluorodeoxyglucose accumulation in large vessels and serological markers of inflammation in polymyalgia rheumatica: a quantitative PET study

OBJECTIVE: To quantify 18-fluorodeoxyglucose (FDG) accumulation in large vessels in patients with polymyalgia rheumatica by positron emission tomography (PET), and to compare these data with serological markers of inflammation. METHODS: 13 untreated patients with active polymyalgia rheumatica underwent FDG positron emission tomography; eight were analysed in a second PET when in clinical remission. Six patients with other highly inflammatory conditions served as controls. For quantitative analysis, FDG uptake over nine defined vascular regions, divided by an individual background value, was expressed as a region of interest (ROI) index. These data were compared with the clinical status of the patient and with erythrocyte sedimentation rate (ESR), C reactive protein, haemoglobin, and platelet and leucocyte counts. RESULTS: By visual evaluation, 12 of the 13 patients showed an increased tracer uptake of the aorta or its major branches. By quantitative analysis, FDG uptake was significantly increased in polymyalgia rheumatica. In patients with active disease, the mean ROI index for all vascular regions exceeded that of controls by 70% (mean (SD): 1.58 (0.37) v 0.93 (0.12); p<0.001). In the eight patients who underwent follow up PET, the index declined substantially. In active polymyalgia rheumatica, FDG uptake was significantly correlated with C reactive protein (r = 0.8), ESR (r = 0.79), and platelet counts (r = 0.68). CONCLUSIONS: The observed FDG accumulation in the aorta and its branches and a strong correlation between tracer uptake and markers of inflammation is suggestive of large vessel arteritis. Quantitative ROI analysis appears to be a sensitive tool for detecting such inflammation.     

Temporal arteritis with low erythrocyte sedimentation rate: a review of five cases.

We compared 5 patients who had biopsy-proven temporal arteritis and erythrocyte sedimentation rates (ESR) less than 50 mm/hour with 25 patients who had temporal arteritis and high ESR and with 10 patients who had negative temporal artery biopsy results and low ESR. Patients with low-ESR temporal arteritis were similar to the other groups, except that they had a higher mean hemoglobin level than the high-ESR group and a significant increase in the percentage of patients (4 of 5) who had a previous diagnosis of polymyalgia rheumatica or had received steroid therapy compared with either of the other groups. The latter finding suggests that even low-dose steroid therapy can lower the ESR in patients with temporal arteritis. We conclude that the ESR is low in only a small percentage of patients with temporal arteritis, and that most of these patients have a history of polymyalgia rheumatica or steroid therapy.     

Relationship of the erythrocyte sedimentation rate to acute phase proteins in polymyalgia rheumatica and giant cell arteritis

We have compared the erythrocyte sedimentation rate (ESR) with other acute phase proteins (C-reactive protein (CRP), alpha(1) antitrypsin, orosomucoid, and haptoglobin) in 108 patients with polymyalgia rheumatica and/or giant cell arteritis. There was good correlation between CRP and ESR, but the ESR was also found to have the highest correlation with disease activity. The additional measurement of CRP or other acute phase proteins may be of value in a minority of cases.