Should all patients with polymyalgia rheumatica have a vascular ultrasound assessment?

There is a growing appreciation that both giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely interrelated conditions that have significant overlap in aetiology, clinical characteristics and treatment regimens. Subclinical GCA in PMR is becoming increasingly recognised, and there is evolving evidence that this may be a more aggressive disease phenotype than PMR. Ultrasound (US) lends itself well as a screening tool for GCA in PMR; it is inexpensive, non-invasive, widely available, lacks ionising radiation, may be performed at the bedside and is recommended by EULAR as a first-line investigation for suspected GCA. There is insufficient evidence to currently recommend that all patients with PMR should have a US assessment for vascular involvement. However, as clinical and laboratory parameters alone do not accurately diagnose patients with subclinical GCA, we suggest that vascular US will be increasingly performed by rheumatologists in practice to identify these patients with PMR, preferably as part of larger prospective outcome studies.

Subclinical giant cell arteritis increases the risk of relapse in polymyalgia rheumatica.

OBJECTIVE: The aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its optimal treatment approach. METHODS: Patients with PMR who fulfilled the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology Provisional Classification Criteria for PMR, did not have GCA symptoms and were routinely followed up for 2 years and were stratified into two groups, according to their ultrasound results: isolated PMR and PMR with subclinical GCA. The outcomes (relapses, glucocorticoid use and disease-modifying antirheumatic drug treatments) between groups were compared. RESULTS: We included 150 patients with PMR (50 with subclinical GCA) with a median (IQR) follow-up of 22 (20-24) months. Overall, 47 patients (31.3 %) had a relapse, 31 (62%) in the subclinical GCA group and 16 (16%) in the isolated PMR group (p<0.001). Among patients with subclinical GCA, no differences were found in the mean (SD) prednisone starting dosage between relapsed and non-relapsed patients (32.4±15.6 vs 35.5±12.1 mg, respectively, p=0.722). Patients with subclinical GCA who relapsed had a faster prednisone dose tapering in the first 3 months compared with the non-relapsed patients, with a mean dose at the third month of 10.0±5.2 versus 15.2±7.9 mg daily (p<0.001). No differences were found between relapsing and non-relapsed patients with subclinical GCA regarding age, sex, C reactive protein and erythrocyte sedimentation rate. CONCLUSIONS: Patients with PMR and subclinical GCA had a significantly higher number of relapses during a 2-year follow-up than patients with isolated PMR. Lower starting doses and rapid glucocorticoid tapering in the first 3 months emerged as risk factors for relapse.

Diagnosis and Treatment of Calcium Pyrophosphate Deposition (CPPD) Disease: A Review.

Calcium Pyrophosphate Dihydrate (CPPD) crystal-related arthropathies are a common cause of acute and chronic arthritis caused by the deposition of calcium pyrophosphate crystals in joints and soft tissues, resulting in inflammation and joint damage. They present with a wide spectrum of clinical manifestations and often present challenges to diagnosis and management as they commonly affect older co-morbid patients. The challenges are compounded by a lack of a well-defined description of CPPD. However, an international expert-driven process is underway to develop CPPD classification criteria. Treatment is also problematic as unlike gout, there are no agents available that decrease the crystal burden. Treatment options have often been extrapolated from gout treatment pathways without having extensive trials or a solid evidence base. It is hoped the new CPPD classification guidelines will contribute to large multicentre studies, with well-defined patient cohorts, which will facilitate the production of high-quality evidence to guide the management of this condition. Here, we discuss the barriers and facilitators in diagnosing and treating CPPD-related arthropathy.

Multidisciplinary approach to anti-MDA5 antibody-positive dermatomyositis associated with rapidly progressive interstitial lung disease.

A man in his 40s was referred to our centre with rapidly progressive interstitial lung disease for lung transplant evaluation. Three months prior to his presentation he had developed periorbital oedema and discolouration, papules over the dorsal aspect of his metacarpophalangeal (MCP) joints and mucocutaneous ulcerations over the dorsum and palmar aspects of his MCPs. He had also been experiencing progressive shortness of breath. Based on the characteristic appearance of the cutaneous lesions, lack of muscle weakness on clinical examination, rapid progression of the interstitial lung disease together with presence of melanoma differentiation-associated gene 5 (MDA5) antibodies a diagnosis of anti-MDA5 dermatomyositis was made. Prompt treatment was initiated with aggressive combined immunomodulatory therapy that resulted in significant improvement in symptoms.

Dynamic platelet function: A novel biomarker in inflammatory arthritis?

BACKGROUND: Patients with inflammatory arthritis die prematurely of cardiovascular disease. Inflammation activates platelets. Since treatment of inflammatory arthritis is associated with reduced mortality, and decreased platelet reactivity reduces cardiovascular events, we hypothesised that platelet reactivity as measured by dynamic platelet function (DPF) would be increased in patients with inflammatory arthritis and that reactivity could be reduced with therapeutic intervention. OBJECTIVES: To characterise platelet function using a validated physiological assay in patients with inflammatory arthritis before and after disease improvement. METHODS: 22 patients were recruited and treated as per local protocol. DPF was measured at baseline and after clinical improvement. Video microscopy was utilised to measure dynamic platelet behaviour in microliters of blood perfused over von Willebrand factor (VWF) at arterial shear rates (1500 s-1). Motion-analysis software measured the number of platelets interacting with VWF, translocating across VWF, the speed and distance platelets travelled across VWF, and stably adhering to the surface. Platelet parameters at baseline and following improvement were compared using Wilcoxon signed rank test and paired student t-test. Changes in platelet function were correlated to inflammatory disease markers by Pearson Correlation. RESULTS: 18 patients completed the study. Platelet adhesion decreased and platelet motion increased following treatment. Tender joint count correlated with platelet adhesion (Pearson r = 0.616, p≤0.01) while CRP correlated with velocity of platelet movement (Pearson r = 0.563, p≤0.01). CONCLUSIONS: Improvement in clinical markers of inflammation is associated with a corresponding change in platelet function. Given the association between reduced mortality and decreased platelet reactivity our results suggest that an appropriate assay of platelet function could guide future therapy of patients with inflammatory arthritis.