Executive summary on the optimization of the multidisciplinary and integrated approach to polymyalgia rheumatica and giant cell arteritis in Madrid region.

Polymyalgia rheumatica and giant cell arteritis can be a medical emergency in which a delay in correct diagnosis and therapeutic management can cause serious complications. With the aim of improving the care of patients with these pathologies in the Community of Madrid, a study was designed to identify the causes and possible solutions to address the problems related to the diagnosis of these pathologies. After the analysis, 11 areas of improvement related to four different aspects of the care process were identified: coordination and protocols, equipment, training and awareness of pathologies, and patient experience. Of all the areas identified, it was considered a priority to resolve those related to the generation of protocols for the comprehensive management of the pathologies, which include all the specialties and levels of care involved. Another crucial aspect is the increase in the degree of clinical suspicion of these pathologies.

What non-pharmacological treatments do people with polymyalgia rheumatica try: results from the PMR Cohort Study.

Polymyalgia rheumatica (PMR) is common. The mainstay of treatment, glucocorticoids, are associated with significant adverse effects and many patients remain on high doses for a number of years. Little is known about the use of other, non-pharmacological therapies as adjuncts in PMR. The PMR Cohort Study is an inception cohort study of patients diagnosed with PMR in primary care. This analysis presents data on the use and perceived impact of non-pharmacological therapies from a long-term follow-up survey. Non-pharmacological treatments were classified as either diet, exercise, or complementary therapies. Results are presented as adjusted means, medians, and raw counts where appropriate. One hundred and ninety-seven participants completed the long-term follow-up questionnaire, of these 81 (41.1%) reported using non-pharmacological therapy. Fifty-seven people reported using a form of complementary therapy, 35 used exercise and 20 reported changing their diet. No individual non-pharmacological therapy appeared to be associated with long-term outcomes. The use of non-pharmacological therapies is common amongst PMR patients, despite the paucity of evidence supporting their use. This suggests that people perceive a need for treatment options in addition to standard glucocorticoid regimens. Further research is needed to understand patients' aims when seeking additional treatments and to strengthen the evidence base for their use so that patients can be guided towards effective options.

Update on the Treatment of Giant Cell Arteritis and Polymyalgia Rheumatica.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are considered 2 diseases on the same spectrum due to their many underlying similarities. In recent years, both diseases have witnessed both diagnostic and treatment advances, which shaped the way we manage them. In this article, the authors focus on different diagnostic modalities in GCA as well as the presence of different clinical phenotypes and the role of screening for aortic involvement. The authors also discuss traditional treatments and the role of evolving steroid-sparing agents in the management of both GCA and PMR.

EULAR points to consider for the diagnosis and management of rheumatic immune-related adverse events due to cancer immunotherapy with checkpoint inhibitors.

BACKGROUND: Rheumatic and musculoskeletal immune-related adverse events (irAEs) are observed in about 10% of patients with cancer receiving checkpoint inhibitors (CPIs). Given the recent emergence of these events and the lack of guidance for rheumatologists addressing them, a European League Against Rheumatism task force was convened to harmonise expert opinion regarding their identification and management. METHODS: First, the group formulated research questions for a systematic literature review. Then, based on literature and using a consensus procedure, 4 overarching principles and 10 points to consider were developed. RESULTS: The overarching principles defined the role of rheumatologists in the management of irAEs, highlighting the shared decision-making process between patients, oncologists and rheumatologists. The points to consider inform rheumatologists on the wide spectrum of musculoskeletal irAEs, not fulfilling usual classification criteria of rheumatic diseases, and their differential diagnoses. Early referral and facilitated access to rheumatologist are recommended, to document the target organ inflammation. Regarding therapeutic, three treatment escalations were defined: (1) local/systemic glucocorticoids if symptoms are not controlled by symptomatic treatment, then tapered to the lowest efficient dose, (2) conventional synthetic disease-modifying antirheumatic drugs, in case of inadequate response to glucocorticoids or for steroid sparing and (3) biological disease-modifying antirheumatic drugs, for severe or refractory irAEs. A warning has been made on severe myositis, a life-threatening situation, requiring high dose of glucocorticoids and close monitoring. For patients with pre-existing rheumatic disease, baseline immunosuppressive regimen should be kept at the lowest efficient dose before starting immunotherapies. CONCLUSION: These statements provide guidance on diagnosis and management of rheumatic irAEs and aim to support future international collaborations.

The Italian Society of Rheumatology clinical practice guidelines for the management of polymyalgia rheumatica.

OBJECTIVE: to provide evidence-based up-to-date recommendations for the management of patients with a definite diagnosis of polymyalgia rheumatica (PMR). METHODS: A systematic literature review was performed to find the existing clinical practice guidelines (CPGs) on PMR and the framework of the Guidelines International Network Adaptation Working Group was used to appraise (AGREE II), synthesize, and customize the recommendations according to the needs of the Italian healthcare context. Rheumatologists on behalf of the Italian Society of Rheumatology (SIR) and from the SIR Epidemiology Unit joined the working group and identified the key health questions on PMR to guide the systematic literature review. Physicians, including general practitioners and specialists, and health professionals who manage PMR in the clinical practice were the target audience. The final recommendations were rated externally by a multi-disciplinary and multi-professional group of stakeholders. RESULTS: From the systematic search in databases (Medline, Embase) and grey literature, 3 CPGs were identified and appraised by two independent raters. Combining the statements and the evidence from these CPGs, 9 recommendations were developed by endorsement or adaptation in response to the initial key health questions. The quality of evidence was graded and the working group discussed the final recommendations in view of their implementation in the Italian healthcare context. CONCLUSIONS: In absence of national guidelines so far, these recommendations are the first to provide guidance for the management of patients with a diagnosis of PMR in Italy and they are expected to ensure the best evidence-based clinical practice for this disease.

Treat to Target: A Valid Concept for Management of Polymyalgia Rheumatica and Giant Cell Arteritis?

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common inflammatory diseases of the elderly. They have variable clinical courses and are usually treated with glucocorticoids (GCs). Relapses are frequent in both conditions. Physicians should balance the tradeoff of treatment-related adverse events and risk of relapse. The ultimate goal of treatment is control of the disease while maintaining patient well-being. A treat-to-target approach may achieve the aim of controlling inflammation and preserving patient's functioning and quality of life, and would require pursuit and evaluation of clinical, laboratory, imaging, and structural targets to tackle the different manifestations of GCA and PMR.

Giant cell arteritis: is the clinical spectrum of the disease changing?

BACKGROUND: Giant cell arteritis is a vasculitis of large and middle-sized arteries that affects patients aged over 50 years. It can show a typical clinical picture consisting of cranial manifestations but sometimes nonspecific symptoms and large-vessel involvement prevail. Prompt diagnosis and treatment is essential to avoid irreversible damage. DISCUSSION: There has been an increasing knowledge on the occurrence of the disease without the typical cranial symptoms and its close relationship and overlap with polymyalgia rheumatica, and this may contribute to reduce the number of underdiagnosed patients. Although temporal artery biopsy is still the gold-standard and temporal artery ultrasonography is being widely used, newer imaging techniques (FDG-PET/TAC, MRI, CT) can be of valuable help to identify giant cell arteritis, in particular in those cases with a predominance of extracranial large-vessel manifestations. CONCLUSIONS: Giant cell arteritis is a more heterogeneous condition than previously thought. Awareness of all the potential clinical manifestations and judicious use of diagnostic tests may be an aid to avoid delayed detection and consequently ominous complications.

Toward a Core Outcome Measurement Set for Polymyalgia Rheumatica: Report from the OMERACT 2018 Special Interest Group.

OBJECTIVE: To report the progress of the Outcome Measures in Rheumatology (OMERACT) Polymyalgia Rheumatica (PMR) Working Group in selecting candidate instruments for a core outcome measurement set. METHODS: A systematic literature review identified outcomes measured and instruments used in PMR studies, and a respondent survey and raw data analysis assessed their domain match and feasibility. RESULTS: Candidate instruments were identified for pain [visual analog scale/numerical rating scale (VAS/NRS)], stiffness (VAS/NRS and duration), and physical function (Health Assessment Questionnaire-Disability Index/modified Health Assessment Questionnaire). Domain match and feasibility assessments were favorable; however, validation in PMR was lacking. CONCLUSION: Further assessment of candidate instruments is required prior to recommending a PMR core outcome measurement set.

The availability of health information to patients with newly diagnosed polymyalgia rheumatica: results from the Polymyalgia Rheumatica (PMR) Cohort study.

AimThe aim of this study was to assess the provision of information to, and seeking of information by, patients newly diagnosed with polymyalgia rheumatica (PMR) in primary care. BACKGROUND: PMR is an inflammatory rheumatological condition of older people that can be treated with long-term oral glucocorticoids. Management usually requires the patient to understand the potential complications of treatment and the disease, as well as involvement in reducing treatment dose. This may be complex for patients to understand. METHOD: Data are taken from the baseline phase of the PMR Cohort study, which recruited newly diagnosed patients with PMR from UK primary care. Participants provided information on their PMR symptoms, general health and sociodemographics. They also completed items regarding information provision by their doctor at diagnosis, its usefulness and their own search for information.FindingsA total of 652 people responded to the baseline survey. In all, 399 (62.7%) had received written information from their doctor; 237 (98%) found it useful; 265 (42.9%) would have liked more information; and 311 (48.4%) sought out more information. Those who were not given information and did not seek it out tended to be older and have poorer internet access.Information provided at diagnosis to patients with PMR is useful, but more than a third did not receive any. This is concerning when PMR requires self-management and vigilance for red flags. Doctors should make use of the resources already available to them to support patients and should specifically ensure that these are available to more elderly patients and those without internet access.

Support available for and perceived priorities of people with polymyalgia rheumatica and giant cell arteritis: results of the PMRGCAuk members' survey 2017.

Polymyalgia rheumatica and giant cell arteritis are relatively common, but under research inflammatory rheumatological conditions. This survey aimed to ascertain the matters in which patients feel they need support with these conditions and appraise how the Charity PMRGCAuk currently supports these needs and could do so in the future. PMRGCAuk members (n = 910) were invited to complete an on-line survey. The survey requested the respondent's history of PMR and or GCA, their perceived priorities for support for people with PMR and or GCA and views on the services already provided by the Charity. A total of 209 people completed the survey. Less than 24% had heard of either PMR or GCA before their diagnosis. Priorities in supporting people with PMR and or GCA included: being on and tapering off glucocorticoids (76.6%), specifically, length of treatment and the risks versus benefits and managing side effects. Respondents generally reported satisfaction with the services currently provided by PMRGCAuk. The support provided by PMRGCAuk is very helpful to members and fills an important gap in provision for people with PMR and or GCA. The areas in which the greatest proportions of participants requested support do not have an evidence base to underpin them. It is incumbent on the research community to address patients' concerns and provide an evidence base where it is required by those affected.

[S3 guidelines on treatment of polymyalgia rheumatica : Evidence-based guidelines of the German Society of Rheumatology (DGRh), the Austrian Society of Rheumatology and Rehabilitation (ÖGR) and the Swiss Society of Rheumatology (SGT) and participating medical scientific specialist societies and other organizations]. / S3-Leitlinie zur Behandlung der Polymyalgia rheumatica : Evidenzbasierte Leitlinie der Deutschen Gesellschaft für Rheumatologie (DGRh), der Österreichischen Gesellschaft für Rheumatologie und Rehabilitation (ÖGR) und der Schweizerischen Gesellschaft für Rheumatologie (SGR) und der beteiligten medizinisch-wissenschaftlichen Fachgesellschaften und weiterer Organisationen.

Polymyalgia rheumatica (PMR) occurs almost exclusively in persons aged 50 years or older and it is the second most common inflammatory rheumatic disease in older people after rheumatoid arthritis. Since there are no specific tests for PMR, the exclusion of clinically similar differential diagnoses is essential to ascertain the diagnosis. These recommendations for the management of PMR assume an already established diagnosis of PMR. It is recommended to initiate treatment with glucocorticoids immediately after diagnosis and to provide appropriate patient information and education about the impact of the disease and its treatment. Methotrexate should be considered in patients at high risk for relapse and/or glucocorticoid-related adverse events. These guidelines have been elaborated because there is significant heterogeneity in the management of PMR in clinical practice in Germany (but also Europe and worldwide), despite the large number of patients with this disease. These guidelines are primarily based on the 2015 EULAR-ACR recommendations for the management of PMR, which were updated by the guideline committee and adapted to the German speaking countries.

Incidence of rheumatoid arthritis, psoriatic arthritis and polymyalgia rheumatica in an inland area of central Italy: results of the CAMPO-RHE study.

OBJECTIVE: The aim of the CAMPO-RHE study was to determine the incidence of rheumatoid arthritis (RA), psoriatic arthritis (PsA) and polymyalgia rheumatica (PMR) in patients attending a rheumatologic outpatient's clinic of a new institution in Campobasso, Italy. METHODS: Campobasso is a small town of approximately 50,000 inhabitants located in the inland territory of central Italy (Molise), and Public Health is managed from a single health authority. In Italy, all citizens are registered with a National Health System of General Practitioner (GP) Physicians. Between the 1st of June 2014 and the 31st of May 2016, all consecutive adult patients, sent by a GP, of Campobasso with any diagnosis of musculoskeletal symptoms/signs/complaints were evaluated in a single rheumatology outpatient clinic of our Academic Unit. The clinic represents the first and unique reference for GPs about rheumatic diseases in the territory. Subjects were classified using the 2010 EULAR criteria for RA, the CASPAR criteria for PsA and the 2012 ACR classification criteria for PMR. RESULTS: 1003 adult patients, sent by GPs, with articular or musculoskeletal complaints visited our clinic. Of these, 409 inhabitants of the municipality of Campobasso were evaluated for the study. During the 2-year study period we diagnosed 18, 19 and 12 new cases of RA, PsA and PMR respectively, with a new incident cases rate of 21.4, 22.59 and 27.43/100,000/year on the population at risk. CONCLUSION: The results of our study could contribute to better define the incidence of these rheumatic diseases classified with the new classification criteria.

[Polymyalgia rheumatica: diagnostic and therapeutic issues of an apparently straightforward disease.] / La polimialgia reumatica: difficoltà diagnostiche e terapeutiche per una malattia apparentemente "banale".

Polymyalgia rheumatica (PMR) is an inflammatory disease characterized by aching and stiffness in the girdles, which affects typically people over 50 years old and could overlap with giant cell arteritis (GCA) in about 15-20% of cases. Although the diagnosis of PMR is usually considered straightforward, clinicians facing this disease should be aware of its atypical manifestations, which can hamper the correct identification of PMR and, conversely, should be aware of other diseases which may present with polymyalgic features. The aim of this review is to synthetize current knowledge about clinical presentations of PMR, the differential diagnoses, the relationship with cancer, the clues to the presence of a concomitant GCA, the role of ultrasonography at the onset and in the follow-up and, finally, treatment approaches. Besides evidence from the literature, this review will highlight some "tips&tricks" useful in everyday clinical practice. The awareness of the different presentations and pitfalls of PMR could improve patients' management and avoid complications consequent upon unrecognized diseases or, conversely, overtreatment.

[Large vessel vasculitis : Giant cell arteritis and Takayasu arteritis]. / Großgefäßvaskulitiden : Riesenzellarteriitis und Takayasu-Arteriitis.

According to the Chapel Hill Classification, large vessel vasculitides encompass giant cell arteritis (GCA) and the histologically related Takakaysu arteritis (TAK). The two diseases lack autoantibodies and present with a systemic inflammatory response. GCA typically shows a sudden onset with profound sickness, loss of appetite and of body weight, and temporal headache. Due to the substantial risk of sudden blindness, diagnostic work-up has to be performed immediately and treatment started without delay. A close association between polymyalgia rheumatica (PMR) and GCA is well established. Takayasu arteritis very often begins in adolescence. In contrast to GCA, the general symptoms are much less pronounced and aside from occasional carotidodynia there is a lack of diagnostic symptoms. TAK is often diagnosed in late stages due to exercise-induced claudication.

The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease.

GCA and PMR are conditions of older persons that frequently overlap. The traditional concept of GCA has focused on cranial symptoms such as headache and visual disturbance, but extra-cranial manifestations such as constitutional symptoms, polymyalgia and limb claudication have also long been recognized. These symptoms may coincide with cranial GCA, occur as an independent clinical subset [large-vessel (LV) GCA] or overlap with PMR. Imaging studies have demonstrated that up to one-third of patients with PMR have subclinical LV inflammation at disease outset. The implication of this finding for PMR management is unclear. Pathophysiological studies have emphasized the pivotal role of dendritic cells (DCs) and T cells in the pathogenesis of GCA, and the activation of certain pattern recognition receptors on DCs may determine the clinical subset of GCA. In patients with only PMR clinically, it is conceivable that transmural arterial inflammation has either not yet started or is prevented by unexplored regulatory pathways. This concept is supported by vasculitis of peri-adventitial small-vessels and activated DCs in the adventitia of temporal arteries, in the absence of media-infiltrating T cells. This review examines the clinical and pathophysiological spectrum of GCA and its subsets with PMR, the role of newer imaging techniques for GCA diagnosis and the management of these diseases.

[Diagnostics and treatment of polymyalgia rheumatica]. / Diagnostik und Therapie der Polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is the most common autoimmune inflammatory disease in older persons with an average age of onset of 73 years. Typical symptoms include acute or subacute bilateral shoulder pain with severe stiffness and often neck and bilateral hip pain. Giant cell arteritis (GCA) occurs in approximately 20 % of cases and up to two thirds of patients with GCA have symptoms of PMR. There are many disease which mimic PMR, elderly onset rheumatoid arthritis is frequently misdiagnosed as PMR. Although there are no specific laboratory tests, C­reactive protein and erythrocyte sedimentation rates are elevated in over 90 % of patients. The diagnosis may be aided by imaging, especially ultrasonography and magnetic resonance imaging (MRI). Treatment currently consists of glucocorticoids at an initial dose of 12.5-25 mg prednisone equivalent daily. Treatment duration is typically 2­3 years but may be longer. Under certain conditions low-dose methotrexate can be used as adjuvant therapy.

Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review.

IMPORTANCE: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are related inflammatory disorders occurring in persons aged 50 years and older. Diagnostic and therapeutic approaches are heterogeneous in clinical practice. OBJECTIVE: To summarize current evidence regarding optimal methods for diagnosing and treating PMR and GCA. EVIDENCE REVIEW: MEDLINE, EMBASE, and Cochrane databases were searched from their inception dates to March 30, 2016. Screening by 2 authors resulted in 6626 abstracts, of which 50 articles met the inclusion criteria. Study quality was assessed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool or American College of Cardiology Foundation/American Heart Association methodology. FINDINGS: Twenty randomized clinical trials for therapy (n = 1016 participants) and 30 imaging studies for diagnosis and/or assessing response to therapy (n = 2080 participants) were included. The diagnosis of PMR is based on clinical features such as new-onset bilateral shoulder pain, including subdeltoid bursitis, muscle or joint stiffness, and functional impairment. Headache and visual disturbances including loss of vision are characteristic of GCA. Constitutional symptoms and elevated inflammatory markers (>90%) are common in both diseases. Ultrasound imaging enables detection of bilateral subdeltoid bursitis in 69% of PMR patients. In GCA, temporal artery biopsy remains the standard for definitive diagnosis. Ultrasound and magnetic resonance imaging (MRI) of large vessels revealing inflammation-induced wall thickening support the diagnosis of GCA (specificity 78%-100% for ultrasound and 73%-97% for MRI). Glucocorticoids remain the primary treatment, but the optimal initial dose and tapering treatment regimens are unknown. According to consensus-based recommendations, initial therapy for PMR is prednisone, 12.5 to 25 mg/day or equivalent, and 40 to 60 mg/day for GCA, followed by individualized tapering regimens in both diseases. Adjunctive methotrexate may reduce cumulative glucocorticoid dosage by 20% to 44% and relapses by 36% to 54% in both PMR and GCA. Use of tocilizumab as additional treatment with prednisone showed a 2- to 4-fold increase in remission rates of GCA in a randomized clinical trial (N = 30). CONCLUSIONS AND RELEVANCE: Diagnosis of PMR/GCA is made by clinical features and elevated inflammatory markers. In PMR, ultrasound imaging may improve diagnostic accuracy. In GCA, temporal artery biopsy may not be required in patients with typical disease features accompanied by characteristic ultrasound or MRI findings. Consensus-based recommendations suggest glucocorticoids as the most effective therapy for PMR/GCA. Methotrexate may be added to glucocorticoids in patients at risk for relapse and in those with glucocorticoid-related adverse effects or need for prolonged glucocorticoid therapy.