Polymyalgia rheumatica is a common inflammatory rheumatic disease in subjects aged 50 years or older and classically presents with shoulder and/or pelvic girdle pain and prolonged morning stiffness. Glucocorticoids represent the standard of treatment; glucocorticoid therapy is usually required for 1-2 years and often results in significant glucocorticoid-related side effects, especially in the elderly.

In this review, we aimed to provide a comprehensive overview of the management of polymyalgia rheumatica, with a particular focus on adjunctive therapies to the standard glucocorticoid treatment.

Given the high frequency of disease relapses (one-third of patients) and the adverse events related to prolonged glucocorticoid use, the need for glucocorticoid-sparing agents remains an important issue in the management of polymyalgia rheumatica. In selected patients, who are at risk for glucocorticoid-related side effects or in those with glucocorticoid-refractory disease, the addition of a glucocorticoid-sparing agent, either a synthetic or biologic disease-modifying anti-rheumatic drug, may represent a reasonable and effective therapeutic approach.

Polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA) with symptoms of PMR share some pathophysiologic features. Interleukin 6 (IL-6) levels are elevated in both groups. We investigated the effect of tocilizumab (TCZ), an IL-6 inhibitor, in both populations and whether there were any differences regarding effectiveness and safety between them. We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines by searching the following databases: PubMed, PMC, Medline, Scopus, Cochrane Library, and ClincalTrials.gov. We found eight articles including one systematic review, one randomized controlled trial (RCT), one posthoc analysis of an RCT, and five observational studies. A total of 668 patients were included in this study. After a comprehensive analysis, we can only infer that there is insufficient evidence to suggest TCZ as monotherapy. Nevertheless, using TCZ in combination with glucocorticoid can be an effective therapeutic option.

Giant cell arteritis (GCA), frequently associated with polymyalgia rheumatica (PMR), and Takayasu's arteritis (TAK) are characterized by extensive vascular remodeling that results in occlusion and stenosis. The pathophysiological mechanisms underlying the onset of GCA/PMR and TAK are still hypothetical. However, similarities and differences in the immunopathology and clinical phenotypes of these diseases point toward a possible link between them. The loss of tolerance in the periphery, a breakdown of tissue barriers, and the development of granulomatous vasculitis define a disease continuum. However, statistically powered studies are needed to confirm these correlations. In addition to glucocorticoids, inhibition of the interleukin-6 axis has been proposed as a cornerstone in the treatment of GCA/PMR and TAK. Novel biologic agents targeting the pathogenic pathway at various levels hold promise to achieve glucocorticoid-free sustained remission.

To determine a safe timeframe and parameters for performing cataract surgery after diagnosis and treatment of giant cell arteritis (GCA).

Single institution in the United States.

Retrospective chart review.

This retrospective study used ICD-9/10 and Current Procedural Terminology codes to identify all patients with biopsy-proven GCA who underwent cataract surgery from 2005 to 2019 at a single institution. Excluded from the study were patients whose date of biopsy diagnosis or dose of corticosteroids at the time of cataract surgery was unknown.

Chart review identified 15 eyes of 10 patients that met inclusion criteria; 80% of patients were women, and mean age was 74.4 years. Two patients had a history of arteritic ischemic optic neuropathy. There were no perioperative or postoperative complications in the 15 eyes that underwent cataract surgery with varying doses of prednisone at the time of surgery (1 to 25 mg daily prednisone ± 10 to 25 mg weekly methotrexate; median prednisone dose of 10.75 mg) and varying time from biopsy diagnosis of GCA to surgery of at least 7 months (median 13.75 months).

Cataract surgery seemed safe for patients with GCA on varying doses of prednisone at the time of surgery at least 7 months from time of biopsy diagnosis. There is a need for a larger cohort of data from neuro-ophthalmologists and cataract surgeons nationally to establish guidelines for safe cataract surgery in patients with GCA.

Colour Doppler sonography (CDS) is becoming ever more important in the diagnosis of GCA. Data on cut-off values for intima-media complex thickness (IMT) that can be used in clinical practice to distinguish between normal and inflamed arteries are limited. We aimed to derive potential cut-off values for IMT of seven preselected arteries by comparing IMT between GCA patients and a control group.

We performed CDS of the preselected temporal, facial, occipital, carotid, vertebral, subclavian and axillary arteries in consecutive newly diagnosed GCA patients between October 2013 and September 2019. A 'halo' with positive compression sign was considered a positive finding. We measured the maximum IMT in the preselected arteries and compared it with the maximum IMT of the control group.

We were able to demonstrate a halo sign in at least one of the examined arteries of 244/248 (98.4%) GCA patients. Temporal arteries were the most commonly affected vessels, involved in 192 (77.4%) patients. We found extracranial large vessel involvement in 87 (35.1%) patients. The following cut-off values showed high levels of diagnostic accuracy: ≥0.4 mm for temporal, facial and occipital arteries, ≥0.7 mm for vertebral arteries, and ≥1 mm for carotid, subclavian and axillary arteries.

The involvement of a large array of arteries is easily and commonly detected by CDS and provides a high diagnostic yield in patients with suspected GCA. Proposed IMT cut-off values might further improve the diagnostic utility of CDS in these patients.

Self-report of musculoskeletal conditions is often used to estimate population prevalence and to determine disease burden and influence policy. However, self-report of certain musculoskeletal conditions is frequently inaccurate, suggesting inadequate communication to the patient of their diagnosis. The aim of this study is to determine the association between functional health literacy (FHL) and self-reported musculoskeletal conditions in a representative population survey. FHL was measured using Newest Vital Sign in 2824 randomly selected adults. Participants also self-reported medically diagnosed arthritis, gout, and osteoporosis. Multiple logistic regression was adjusted for age and sex. The prevalence of self-reported arthritis, gout, and osteoporosis was 25.2%, 4.9%, and 5.6%, respectively. The prevalence of those at risk for inadequate FHL was 24.0% and high likelihood of inadequate FHL was 21.0%. However, over 50% of respondents with arthritis or gout had at risk/inadequate FHL, increasing to 70% of those self-reporting osteoporosis. After adjustment for age and sex, respondents in the arthritis subgroup of "don't know" and self-reported osteoporosis were significantly more likely to have inadequate FHL than the general population. This study indicates a substantial burden of low health literacy amongst people with musculoskeletal disease. This has implications for provider-patient communication, individual healthcare, population estimates of musculoskeletal disease, and impact of public health messages.

Glucocorticoids remain the cornerstone of medical therapy in giant cell arteritis (GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Imaging techniques (e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with large-vessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.

Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage-T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever, and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell-macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy.

Polymyalgia rheumatica is an inflammatory disease of unknown etiology affecting individuals aged fifty years and older, mainly of Caucasian ethnicity. Polymyalgia rheumatica is associated with giant cell arteritis more frequently than expected by chance alone. In both conditions, females are affected two to three times more often than males. The clinical hallmark manifestations of polymyalgia rheumatica are aching and morning stiffness in the shoulder girdle and often in the pelvic girdle and neck. Serum inflammatory markers are typically elevated, while the most consistent abnormal finding on imaging studies is bursitis in the symptomatic areas. A dramatic response to glucocorticoids is characteristic of polymyalgia rheumatica. Many patients are able to discontinue glucocorticoids six months to two years after the onset of clinical symptoms, but some patients may require longstanding glucocorticoid treatment. Glucocorticoid-sparing agents may be helpful in patients with chronic relapsing courses and those at high risk of glucocorticoid-related adverse events.

Objective. To determine the relationship between FCGR3B gene copy number variation (CNV) and biopsy proven giant cell arteritis (GCA). Methods. FCGR3B CNV was determined in 139 Australian biopsy proven GCA patients and 162 population matched controls, using a duplex qPCR assay and RNase P as the reference gene. Copy number was determined using Copy Caller software (v.1.0, Applied Biosystems, USA). CNV genotypes were classified into 3 groups (<2, 2, 3+) for analysis purposes, and analysis was performed using logistic regression. Results. All GCA patients had a positive temporal artery biopsy, and the most common presenting symptoms were visual disturbance and temporal headache. The mean age of patients at biopsy was 74 years (range 51-94) and 88/139 (63%) were female. The frequency of low (<2) FCGR3B copy number was comparable between GCA patients (9/139 = 6.5%) and controls (10/162 = 6.2%), as was the frequency of high (3+) FCGR3B copy number (15/130 (10.8%) in GCA patients versus 13/162 (8.0%) in controls). Overall there was no evidence that FCGR3B CNV frequencies differed between GCA patients and controls (χ (2) = 0.75, df = 2, P = 0.69). Conclusion. FCGR3B CNV is not associated with GCA; however, replicate studies are required.

Giant cell arteritis is the most common vasculitis in Caucasians. Acute visual loss in one or both eyes is by far the most feared and irreversible complication of giant cell arteritis. This article reviews recent guidelines on early recognition of systemic, cranial, and ophthalmic manifestations, and current management and diagnostic strategies and advances in imaging. We share our experience of the fast track pathway and imaging in associated disorders, such as large-vessel vasculitis.

Interleukin (IL)-6 is a cytokine with multiple biological activities. It contributes to host defense against pathogens, whereas accelerated production of IL-6 plays a significant pathological role in various diseases. Clinical trials have demonstrated the efficacy of tocilizumab, a humanized anti-IL-6 receptor antibody, for patients with rheumatoid arthritis, Castleman's disease or juvenile idiopathic arthritis, leading to approval of this innovative drug for the treatment of these diseases. Since IL-6 has been demonstrated to play a significant role in the development of various other autoimmune and inflammatory diseases, tocilizumab can be expected to become a novel drug for such diseases as well.