Currently, only 25% of all polymyalgia rheumatica (PMR) patients are referred to specialists. An expert committee has recently recommended confirmation of diagnosis by specialist care. This can help to avoid misdiagnoses and hospital stays and can result in lower glucocorticoid doses.Using ultrasound, magnetic resonance imagining (MRI), or positron emission tomography-computed tomography (PET-CT), typical periarticular inflammatory changes are observed, especially in the shoulder and pelvic girdle area. However, for clinical use, ultrasound is usually sufficient.In 20-25% of newly diagnosed PMR patients without symptoms of giant cell arteritis (GCA), GCA can be detected through vascular ultrasound. These patients require higher glucocorticoid doses in analogy to GCA therapy. There is growing awareness of a joint GCA-PMR spectrum disease.Glucocorticoids remain the primary treatment. The interleukin-6 inhibitor Sarilumab has recently been approved in the USA for patients with recurrent PMR. Studies have also demonstrated the effectiveness of Tocilizumab in PMR.
INTERDISZIPLINäRE ZUSAMMENARBEIT: : Aktuell werden nur 25% aller PMR-Patienten (PMR: Polymyalgia rheumatica) fachärztlich-rheumatologisch überwiesen. Eine Expertenkommission empfahl kürzlich die fachärztliche Diagnosebestätigung. Dadurch können Fehldiagnosen und Krankenhausaufenthalte vermieden sowie niedrigere Glukokortikoiddosen erreicht werden.
UNASSIGNED: Mithilfe der Sonografie, MRT oder PET-CT lassen sich typische periartikuläre entzündliche Veränderungen finden, insbesondere im Schulter- und Beckengürtelbereich, wobei für den klinischen Alltag die Sonografie meist ausreichend ist.
UNASSIGNED: Bei 20–25% aller neu diagnostizierten PMR-Patienten ohne RZA-Symptome lässt sich sonografisch eine RZA nachweisen. Diese Patienten mit „stiller“ RZA benötigen dieselbe Therapie wie andere RZA-Patienten. Zunehmend wird von einer RZA-PMR-Spektrum-Erkrankung ausgegangen.
UNASSIGNED: Glukokortikoide sind weiterhin die Hauptsäule in der Therapie. Der Interleukin-6-Inhibitor Sarilumab wurde kürzlich in den USA für rezidivierende PMR zugelassen. Studien konnten auch eine Wirksamkeit von Tocilizumab zeigen.

OBJECTIVE: To perform a systematic literature review on definitions and instruments used to measure remission, relapse, and disease activity in polymyalgia rheumatica (PMR), to inform an OMERACT project to endorse instruments for these outcomes.
METHODS: A search of Pubmed/MEDLINE, EMBASE, CINAHL, Cochrane, and Epistemonikos was performed May 2021 and updated August 2023. Qualitative and quantitative studies published in English were included if they recruited people with isolated PMR regardless of treatment. Study selection and data extraction was performed independently by two investigators and disagreement was resolved through discussion. Data extracted encompassed definitions of disease activity, remission and relapse, and details regarding the instruments used to measure these outcomes.
RESULTS: From the 5,718 records, we included 26 articles on disease activity, 36 on remission, and 53 on relapse; 64 studies were observational and 15interventional, and none used qualitative methods. Some heterogeneity was found regarding definitions and instruments encompassing the domains pain, stiffness, fatigue, laboratory markers (mainly acute phase reactants), and patient and physician global assessment of disease activity. However, instruments for clinical signs were often poorly described. Whilst measurement properties of the polymyalgia rheumatica activity score (PMR-AS) have been assessed, data to support its use for measurement of remission and relapse is limited.
CONCLUSIONS: Remission, relapse, and disease activity have been defined heterogeneously in clinical studies. Instruments to measure these disease states still need to be validated. Qualitative research is needed to better understand the concepts of remission and relapse in PMR.
BACKGROUND: PROSPERO identification: CRD42021255925.

BACKGROUND: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are frequently overlapping conditions. Unlike in GCA, vascular inflammation is absent in PMR. Therefore, serum biomarkers reflecting vascular remodelling could be used to identify GCA in cases of apparently isolated PMR.
METHODS: 45 patients with isolated PMR and 29 patients with PMR/GCA overlap were included. Blood samples were collected before starting glucocorticoids for all patients. Serum biomarkers reflecting systemic inflammation (interleukin-6 (IL-6), CXCL9), vascular remodelling (MMP-2, MMP-3, MMP-9) and endothelial function (sCD141, sCD146, ICAM-1, VCAM-1, vWFA2) were measured by Luminex assays.
RESULTS: Patients with GCA had higher serum levels of sCD141 (p=0.002) and CXCL9 (p=0.002) than isolated PMR. By contrast, serum levels of MMP-3 (p=0.01) and IL-6 (p=0.004) were lower in GCA than isolated PMR. The area under the curve (AUC) was calculated for sCD141, CXCL9, IL-6 and MMP-3. Separately, none of them were >0.7, but combinations revealed higher diagnostic accuracy. The CXCL9/IL-6 ratio was significantly increased in patients with GCA (p=0.0001; cut-off >32.8, AUC 0.76), while the MMP-3/sCD141 ratio was significantly lower in patients with GCA (p<0.0001; cut-off <5.3, AUC 0.79). In patients with subclinical GCA, which is the most difficult to diagnose, sCD141 and MMP-3/sCD141 ratio demonstrated high diagnostic accuracy with AUC of 0.81 and 0.77, respectively.
CONCLUSIONS: Combined serum biomarkers such as CXCL9/IL-6 and MMP-3/sCD141 could help identify GCA in patients with isolated PMR. It could allow to select patients with PMR in whom complementary examinations are needed.

BACKGROUND: The objective of this systematic review was to provide an overview of current developments and potentially available therapeutic options for polymyalgia rheumatic (PMR) and giant cell arteritis (GCA), in the coming years.
METHODS: We conducted a systematic review of 17 national and international clinical trial databases for all disease-modifying anti-rheumatic drugs (DMARDs) for PMR and GCA that are already marketed, in clinical development or withdrawn. The search was performed on January 2024, with the keywords \"polymyalgia rheumatica\" and \"giant cell arteritis\". For each molecule, we only considered the study at the most advanced stage of clinical development.
RESULTS: For PMR, a total of 15 DMARDs were identified: 2 conventional synthetic DMARDs (csDMARDs), 11 biologic DMARDs (bDMARDs) and 2 targeted synthetic DMARDs (tsDMARDs). For GCA, 18 DMARDs were identified: 2 csDMARDs, 14 bDMARDs and 2 tsDMARDs. Currently, there are only 2 approved corticosteroid-sparing therapies in these diseases, which both target the IL-6 signaling pathway, namely tocilizumab in GCA and sarilumab in PMR. Most of the molecules in current development are repurposed from from other conditions and clinical research in PMR/GCA seems to be mostly driven by the potential to repurpose existing treatments rather than by translational research.
CONCLUSIONS: This systematic review identified 23 DMARDs evaluated for PMR and GCA: 3 csDMARDs, 17 bDMARDs and 3 tsDMARDs. Several promising treatments are likely to be marketed in the coming years.

BACKGROUND: The study aimed to determine the efficacy and safety of biological disease-modifying antirheumatic drugs (bDMARDs) in the treatment of polymyalgia rheumatica (PMR) complicated by rheumatoid arthritis (RA).
METHODS: Patients with PMR which could be classified as RA and who were treated with bDMARDs were included in the analysis. The primary endpoint was the clinical Polymyalgia Rheumatica Activity Score (Clin-PMR-AS) after 26 weeks of treatment, and the secondary endpoint was adverse events during the observation period.
RESULTS: A total of 203 patients with PMR which was resistant or intolerant to glucocorticoids and could be classified as RA were receiving bDMARDs and were enrolled in the study. There were 83, 82, and 38 patients in the tumor necrosis factor inhibitor (TNFi), interleukin-6 receptor inhibitor (IL-6Ri), and cytotoxic T lymphocyte-associated antigen-4-immunoglobulin (CTLA4-Ig) groups, respectively. Twenty-six weeks after bDMARD initiation, Clin-PMR-AS levels were significantly lower in the IL-6Ri group as compared to other groups. Multiple regression analysis was performed with Clin-PMR-AS as the objective variable. Body mass index (BMI), history of bDMARDs, and IL-6Ri use were identified as factors involved in Clin-PMR-AS. After adjustment for group characteristics using inverse probability of treatment weighting with propensity scores, the Clin-PMR-AS score at 26 weeks was significantly lower in the IL-6Ri group (9.0) than in both the TNFi (12.4, p = 0.004) and CTLA4-Ig (15.9, p = 0.003) group.
CONCLUSIONS: IL-6Ri may potentially improve the disease activity of PMR compared to other bDMARDs.

BACKGROUND: An altered immune tolerance disturbed by immune checkpoint inhibitors (ICIs) may contribute to new-onset polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). This systematic literature review (SLR) examines the characteristics of PMR and GCA-like syndromes following anticancer treatment with ICIs, summarizing their demographic, clinical and treatment-related features to provide insights whether they differ from the idiopathic forms.
METHODS: The SLR was conducted in Medline and EMBASE databases from inception to July 2024, and in the EULAR/ACR abstract database (2021-2023). ICI-induced PMR and GCA syndromes were compared to the primary forms of the diseases using data from studies that included both groups as comparators. For manuscripts lacking direct comparisons, we summarized the main findings and discussed the differences using systematic reviews or large observational studies on the primary forms.
RESULTS: From 1237 screened abstracts, 46 met the inclusion criteria, involving 358 patients (314 with ICI-PMR and 44 with ICI-GCA). ICI-PMR had an estimated pooled prevalence of 0.1% [95% CI: 0.07%, 0.14%] among ICI recipients and 15.9% [95% CI: 12.6%, 19.9%] among patients experiencing rheumatic immune-related adverse events. Patients with ICI-PMR had a male-to-female ratio of 1.7:1 and a mean age of 71 ± 4 years. Most cases were associated with PD1/PDL1 blockers (87%). Clinical features included inflammatory pain in the girdles (100%), though pelvic girdle involvement was under-reported in some cases (3/28 studies). Peripheral arthritis was present in 35% of patients. Laboratory tests showed normal or slightly elevated inflammatory markers in 26% of cases. Glucocorticoids (GCs) led to symptom improvement in 84% of cases although 20% required immunosuppressive treatment and 14% experienced relapses. ICI-GCA had a prevalence of 0.06% among ICI recipients, with equal gender distribution and a mean age of 71 ± 5 years. Most patients received anti-PD1/PDL1 blockers (57%). Clinical manifestations included cephalic symptoms (75%), permanent visual loss (23%) and symptoms related to large-vessel involvement (54%). High-dose GCs were effective, with 96% achieving remission, though 17% experienced relapses.
CONCLUSIONS: ICI-induced PMR and GCA may have distinct clinical profiles compared to idiopathic forms, with potentially milder symptoms and better treatment responses. Further studies are needed to confirm these findings and better understand the long-term outcomes and pathophysiology of these conditions.

Seven of the 11 newer medications recently or soon to be approved to treat rheumatologic diseases discussed in this article are biologic agents and reflect the current ability of science to target specific components of the immune system. The other agents are molecules that are directed against specific immune pathway targets as well. All have shown superiority to placebo and in some cases have been compared to currently accepted therapies. Safety issues are generally centered around infections due to the immune-interrupting nature of these therapies.

Polymyalgia rheumatica (PMR) is an inflammatory disease common in people aged 50 years and older. This condition is characterized by the presence of pain and stiffness involving mainly the shoulder and pelvic girdle. Besides the frequent association with giant cell arteritis (GCA), several conditions may mimic PMR or present with PMR features. Since the diagnosis is basically clinical, an adequate diagnosis of this condition is usually required. Positron emission tomography/computed tomography (PET-CT) has proved to be a useful tool for the diagnosis of PMR. The use of 18F-FDG-PET imaging appears promising as it provides detailed information on inflammatory activity that may not be evident with traditional methods. However, since PET-CT is not strictly necessary for the diagnosis of PMR, clinicians should consider several situations in which this imaging technique can be used in patients with suspected PMR.

OBJECTIVE: In routine care, clinicians may employ 2-[18F]fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) computed tomography (CT) to validate their initial clinical diagnosis of polymyalgia rheumatica (PMR). Nevertheless, the diagnostic utility of combining FDG-PET/CT findings with clinical presentation has not been explored. Therefore, this study aimed to investigate whether the diagnostic accuracy for PMR could be enhanced by combining FDG-PET/CT findings with the clinical baseline diagnosis or the 2012 ACR/EULAR clinical classification criteria for PMR.
METHODS: An investigation and a validation cohort were included from two countries, encompassing 66/27 and 36/21 PMR/non-PMR patients, respectively. The cohorts comprised treatment-naïve patients suspected of PMR, who initially received a clinical baseline diagnosis and underwent FDG-PET/CT scans. The FDG-PET/CT Leuven-score was applied to classify patients as either PMR or non-PMR and combined with the clinical baseline diagnosis. Final diagnoses were established through clinical follow-up after twelve or six months in the investigation and validation cohorts, respectively.
RESULTS: In the investigation cohort, a clinical baseline diagnosis yielded a sensitivity/specificity of 94%/82%, compared with 78%/70% using the ACR/EULAR criteria. Combining the clinical baseline diagnosis with a positive Leuven-score showed a sensitivity/specificity of 80%/93%, compared with 80%/82% for an ACR/EULAR-Leuven-score. In the validation cohort, the baseline diagnosis revealed a sensitivity/specificity of 100%/91%, compared with 92%/76% using the ACR/EULAR criteria. Combining FDG-PET/CT with the baseline diagnosis demonstrated a sensitivity/specificity of 83%/95% compared with 89%/81% for the ACR/EULAR-Leuven-score.
CONCLUSIONS: Combining FDG-PET/CT findings with the clinical baseline diagnosis or ACR/EULAR clinical classification criteria can improve the diagnostic specificity for PMR.

Remitting seronegative symmetrical synovitis with pitting oedema is a rare rheumatological condition, predominating in the elderly male. It is characterised by the abrupt onset of marked pitting oedema, symmetrical distal synovitis, absence of rheumatoid factor and an excellent response to glucocorticoids. RS3PE may be the harbinger of a malignancy so the diagnosis should prompt evaluation and exclusion of such condition; in these cases, the response to glucocorticoids is only partial and treating the neoplasia is essential. The differential diagnosis includes late-onset rheumatoid arthritis, polymyalgia rheumatica and calcium pyrophosphate crystal-related arthritis. We present the case of a patient with remitting seronegative symmetrical synovitis with pitting oedema associated with clear cell renal cell carcinoma.