Features Of Polymyalgia Rheumatica Research Articles

Clinical features of polymyalgia rheumatica patients in Japan: Analysis of real-world data from 2015 to 2020.

To assess clinical features in patients with polymyalgia rheumatica (PMR) in Japan by the International Classification of Disease (ICD)-10 code assignment. Demographics, treatment patterns, and concomitant diseases (identified using ICD-10 code only) in patients who were assigned the PMR ICD-10 code M35.3 at least once between 1 January 2015 and 31 December 2020 were aggregated from a nationwide medical information database owned by the Health, Clinic, and Education Information Evaluation Institute. The cumulative number of patients with PMR was 6325 (mean [standard deviation] age, 74.3 [11.4] years; male:female, 1:1.3). Most patients were >50 years (96.5%) with >33% between 70 and 79 years. Glucocorticoids were prescribed in ∼54% of patients within 30 days of PMR code assignment. All other drug types were prescribed in <5% of patients. Hypertension, diabetes mellitus, rheumatoid arthritis, and osteoporosis were noted in >25% and giant cell arteritis in 1% of patients. During the study period, 4075 patients were newly assigned the PMR code and 62% were prescribed glucocorticoids within 30 days. This is the first retrospective real-world data analysis describing the clinical features of PMR in a large patient population from Japan. Further studies of prevalence, incidence, and clinical features are warranted in patients with PMR.

Predictive Factors of Giant Cell Arteritis in Polymyalgia Rheumatica Patients.

Polymyalgia rheumatica (PMR) is an inflammatory rheumatism of the shoulder and pelvic girdles. In 16 to 21% of cases, PMR is associated with giant cell arteritis (GCA) that can lead to severe vascular complications. Ruling out GCA in patients with PMR is currently a critical challenge for clinicians. Two GCA phenotypes can be distinguished: cranial GCA (C-GCA) and large vessel GCA (LV-GCA). C-GCA is usually suspected when cranial manifestations (temporal headaches, jaw claudication, scalp tenderness, or visual disturbances) occur. Isolated LV-GCA is more difficult to diagnose, due to the lack of specificity of clinical features which can be limited to constitutional symptoms and/or unexplained fever. Furthermore, many studies have demonstrated the existence-in varying proportions-of subclinical GCA in patients with apparently isolated PMR features. In PMR patients, the occurrence of clinical features of C-GCA (new onset temporal headaches, jaw claudication, or abnormality of temporal arteries) are highly predictive of C-GCA. Additionally, glucocorticoids' resistance occurring during follow-up of PMR patients, the occurrence of constitutional symptoms, or acute phase reactants elevation are suggestive of associated GCA. Research into the predictive biomarkers of GCA in PMR patients is critical for selecting PMR patients for whom imaging and/or temporal artery biopsy is necessary. To date, Angiopoietin-2 and MMP-3 are powerful for predicting GCA in PMR patients, but these results need to be confirmed in further cohorts. In this review, we discuss the diagnostic challenges of subclinical GCA in PMR patients and will review the predictive factors of GCA in PMR patients.

P044 Giant cell arteritis in an immunocompromised patient with rheumatoid arthritis

Abstract Background/Aims Giant cell arteritis (GCA) is a common medium-large vessel vasculitis with an approximate incidence of 10 cases per 100,000 people over 50 years old. First line management of GCA is with high dose glucocorticoids followed by steroid sparing agents, namely methotrexate with tocilizumab for resistant cases. Here we describe a patient established on methotrexate for rheumatoid arthritis, receiving rituximab with intravenous methylprednisolone (IVMP) who went on to develop GCA. Methods A 65-year-old Caucasian female with seropositive rheumatoid arthritis (RA) established on 20mg weekly subcutaneous methotrexate and low dose 5mg prednisolone was escalated to her tenth cycle of rituximab with 100mg IVMP for an arthritic flare. The day after her second infusion, two weeks apart, the patient presented with headaches. These were severe temporal pains with scalp tenderness for the past four weeks. She denied visual changes, jaw claudication or polymyalgia rheumatica features. Examination identified bilateral thickened and tender temporal arteries. Ultrasound confirmed bilateral frontal temporal artery halo signs, with intimal thickening of 0.9mm and 0.4mm of the left and right respectively. Blood tests showed a rise in C-reactive protein to 46mg/L from 13mg/L. In view of these findings, procedural risks and recent immunosuppression biopsy was not sought. The patient was commenced on 40mg prednisolone before weaning by 5 mg 2-3 weekly. Her headache improved, without relapse and her CRP normalised within 2 weeks. Given tocilizumab had previously been ineffective in the patient’s RA management, she declined transition to this biologic and her methotrexate was increased to 25mg. Results Development of GCA in a patient with pre-established immunotherapy is rare. This report represents only the second case noted in the literature of the vasculitis occurring in a patient whilst on rituximab. Excluding tocilizumab (anti-IL6), a meta-analysis of other biological agents including infliximab, adalimumab, etanercept (anti-TNF) and abatacept (anti-CTLA4) have not been proven to have clinical significance over placebo in GCA. However, evidence of rituximab and its role in GCA is limited with no clinical trials. Contrary to this case of GCA, rituximab (anti-CD20) therapy has a well-recognised role in ANCA associated vasculitis and there are growing calls for trials in large vessel vasculitides. Recent case series of rituximab in Takayasu’s arteritis have presented contrasting outcomes of persistent disease activity versus clinical and laboratory remission. Robust clinical trials are therefore required to evaluate the long-term impact of rituximab on large vessel vasculitis. Conclusion Although rare, clinicians should be vigilant for the development of GCA despite established immunosuppression. Disclosure N. Chauhan: None. A. Kinder: None.

AB0589 Non-Infectious Aortitis: Still an Underdiagnosed Entity. Report of 32 Cases from A Single Center

BackgroundNon infectious aortitis may present as an idiopathic isolated condition or associated with a wide spectrum of diseases. Aortitis often presents with nonspecific symptoms leading in many cases to an...

AB0905 Late-onset spondyloarthropathy mimicking polymalgia rheumatica: Evidence from MRI

BackgroundPolymyalgia rheumatica (PMR) is a clinical syndrome, typically affecting older people, presenting with inflammatory musculoskeletal symptoms. Late onset rheumatoid arthritis is a well-known PMR mimic, but spondyloarthropathy (SpA) can also...

Polymyalgia Rheumatica as the First Presentation of Metastatic Lymphoma

A 48-year-old HIV-positive woman presented with progressive pain and stiffness of both shoulders and hips. She was given the diagnosis of polymyalgia rheumatica (PMR) due to high erythrocyte sedimentation rate. However, a 1-week course of prednisolone failed to improve her symptoms. She later discovered a breast lump of which histopathological tissue was consistent with a diffuse large B-cell lymphoma. Whole body bone scan revealed multiple bony metastases. The presence of atypical features of PMR and lack of dramatic response to steroids should prompt physicians to raise the probability of differential diagnoses other than PMR, and in particular, malignancy.

Distinct clinical features of polymyalgia rheumatica

Distinct clinical features of polymyalgia rheumatica

Polymyalgia rheumatica in primary care: a cohort study of the diagnostic criteria and outcome

Polymyalgia rheumatica (PMR) is common and is usually diagnosed and managed in primary care. There are no generally accepted primary care criteria for diagnosis. To identify what features are used to diagnose PMR, to benchmark these against diagnostic criteria and to identify features at diagnosis with prognostic significance. This was a retrospective cohort study of all patients diagnosed with PMR in three UK general practices between January 1994 and December 2003. The medical records were examined for features of PMR. The duration of steroid treatment was used as a proxy for duration of disease. Analysis of prognostic predictors was by Cox proportional hazards models. One hundred and eighty-three patients were identified, giving an overall annual incidence of 11.3 per 10 000 patients aged 50 or over. The median age at diagnosis was 75 (interquartile range 69, 79) years: 138 (75%) were female. The most common diagnostic features were proximal muscle pain in 151 (82%), raised inflammatory markers in 160 (87%), clinical response to corticosteroids in 166 (91%) and normalization of inflammatory markers in 147 (81%). Twenty (11%) had normal inflammatory indices. The median duration of treatment was 1.4 years (interquartile range 0.8, 2.4). Female sex and raised inflammatory markers were independently associated with longer treatment: female hazard ratio 1.5 (1.0, 2.2) P = 0.047 and raised inflammatory markers 2.0 (1.2, 3.2) P = 0.01. Primary care practitioners do not use established criteria to diagnose PMR and sometimes diagnose the condition even when inflammatory markers are normal. This exposes patients to a risk of inappropriate steroid use.

Late onset undifferentiated spondyloarthritis presenting with polymyalgia rheumatica features: description of seven cases

To underline the importance of considering a diagnosis of undifferentiated spondyloarthritis (uSpA) in patients presenting polymyalgia rheumatica (PMR) features. All patients with late onset uSpA meeting criteria for PMR at the onset of their disease seen in the Rheumatology Division of Xeral-Calde Hospital of Lugo, Spain during a 5 year period, and in the Rheumatology Department of Lucania, Italy in a two and a half year period, were studied. Six patients with late onset uSpA showing PMR symptoms at the onset were seen during the study periods in the two centres. Another patient had previously been observed in Lugo in a study dealing with the spectrum of conditions mimicking PMR. Of the seven patients, five had manifestations of SpA at the beginning of the disease and two developed these in the following 6 months. All seven met the Amor and/or the ESSG criteria for classifying and diagnosing SpA. The possibility that late onset uSpA may have PMR-like features at the beginning of the disease should be taken into account. The diagnosis is not difficult if the entire clinical spectrum of SpA is considered.

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

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. 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. 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. 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.

Large Vessel Vasculitides (including Polymyalgia Rheumatica)

Abstract The most important large vessel vasculitides are giant cell arteritis (GCA) and Takayasu's arteritis. These are similar but distinct idiopathic diseases characterized by constitutional symptoms with raised inflammatory markers in the blood and underlying granulomatous large vessel vasculitis. The two conditions are distinguished predominantly by the nature of the vessels involved and the resultant clinical presentation. Polymyalgia rheumatica (PMR) is a syndrome that may precede GCA. There is clinical and histological overlap between these two conditions, which can be considered opposite ends of a disease spectrum, but their management differs and thus they are discussed separately in this contribution. Pathology – both GCA and Takayasu's arteritis are associated with panarteritis of large and medium vessels involving lymphocytes, macrophages and multinucleate giant cells. The inflammation starts with infiltration of the adventitia and progresses inwards, involving the media and resulting in extensive fragmentation of the internal elastic lamina. Intimal proliferation is found but fibrinoid necrosis is uncommon. Healing with replacement of elastic tissue by fibrosis, obliteration of the vasa vasorum and irregular thickening of the intima occurs particularly in Takayasu's arteritis. This can result in stenosis, dilatation and/or aneurysm formation. Recanalization of occluded vessels is common. Differential diagnosis – distinguishing GCA from simple PMR can be difficult because the conditions overlap and can coexist. Focal symptoms and signs suggesting vessel ischaemia in association with nonspecific PMR features and a high acute phase response strongly suggest GCA and the need for high-dose corticosteroids to prevent irreversible ischaemic damage. Distinguishing GCA from Takayasu's arteritis is helped by differences in age group affected and expression of disease. GCA is usually more corticosteroid responsive and has a less protracted course than Takayasu's arteritis. Other differential diagnoses are listed in Figure 1.

A prospective study of 287 patients with polymyalgia rheumatica and temporal arteritis: clinical and laboratory manifestations at onset of disease and at the time of diagnosis.

A prospective study of 287 patients with giant cell arteritis (GCA), including polymyalgia rheumatica (PMR) and temporal arteritis (TA), was conducted during 1987-1994. All patients were evaluated prior to the start of drug treatment. During the same period, 31 patients with GCA, of whom 12 cases had TA, were admitted to other departments in the hospital. At onset of disease, all patients were > or = 50 yr of age. Peripheral arthritis was found in 24.4% of patients with PMR, while none of the patients with TA exhibited such manifestations. Clinical features at onset of disease differed from those appearing at presentation to the hospital. Thus, the gradual development of a full-blown clinical picture may be responsible for the delay in diagnosis of GCA. The majority of cases (80%) presented with "pure' PMR without clinical signs or symptoms of concomitant TA. In a random sample of 68 patients with "pure' PMR, histological examinations of biopsy specimens of the temporal artery revealed inflammatory changes in three patients only (4.4%). Consequently, arterial biopsy in patients with clinical features of PMR only, appears to be unnecessary. Among patients with TA referred to the department of internal medicine, general malaise, loss of weight and sustained fever were prominent manifestations. Such features may thus necessitate a diagnostic arterial biopsy even in the absence of clinical arteritis or myalgia. Both ESR and CRP were within normal levels in 1.2% of the cases. Further clinical and laboratory examinations performed at diagnosis of GCA disclosed only one case of malignancy. Routine chest X-rays did not reveal unexpected pathological findings. Permanent and complete blindness due to arteritis was observed in one patient only. No association between GCA and thyroid dysfunction was detected.

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.