The EuroMyositis registry: an international collaborative tool to facilitate myositis research

Aims: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. Methods: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Results: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%). Smoking was more frequent in connective tissue disease overlap cases (45%, OR 1.44, 95% CI 1.09 to 1.90, p=0.012). Smoking was associated with interstitial lung disease (ILD) (OR 1.32, 95% CI 1.06 to 1.65, p=0.013), dysphagia (OR 1.43, 95% CI 1.16 to 1.77, p=0.001), malignancy ever (OR 1.78, 95% CI 1.36 to 2.33, p<0.001) and cardiac involvement (OR 2.40, 95% CI 1.60 to 3.60, p<0.001). Dysphagia occurred in 39% and cardiac involvement in 9%; either occurrence was associated with higher Health Assessment Questionnaire (HAQ) scores (adjusted OR 1.79, 95% CI 1.43 to 2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95% CI 2.52 to 5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, OR 2.06, 95% CI 1.65 to 2.57, p<0.001). ILD occurred in 30%, most frequently in antisynthetase syndrome (71%, OR 10.7, 95% CI 8.6 to 13.4, p<0.001). Rash characteristics differed between adult-onset and juvenile-onset DM cases ('V' sign: 56% DM vs 16% juvenile-DM, OR 0.16, 95% CI 0.07 to 0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71% and azathioprine in 51%. Conclusion: This large multicentre cohort demonstrates the importance of extramuscular involvement in patients with IIM, its association with smoking and its influence on disease severity. Our findings emphasise that IIM is a multisystem inflammatory disease and will help inform prognosis and clinical management of patients

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: Results from the MYONET registry.

OBJECTIVES To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations, and malignancy, between adults with anti-synthetase syndrome (ASyS) and dermatomyositis (DM). METHODS Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1ɣ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V sign, erythroderma, and/or periorbital rash). RESULTS In total 1,054 patients were included (DM, n = 405; ASyS, n = 649). In ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease, and cardiac involvement differentiated ASyS-DMskin from DM (all p< 0.001), whereas higher frequency of any of four DM-type rashes: heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%) differentiated DM from ASyS-DMskin (all p< 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both p< 0.001). CONCLUSION DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management

Treatment of large-vessel vasculitis: where do we stand?

Editorial on the new strategies for the treatment of large vessels vasculitis

Role of 18F-fluorodeoxyglucose positron emission tomography in the workup of retroperitoneal fibrosis

Retroperitoneal fibrosis is a syndrome characterised by the presence of fibrosclerotic tissue in the retroperitoneum, often encasing the ureters. In most cases, retroperitoneal fibrosis is idiopathic, but may also be associated with large vessel vasculitis at distant sites, with the so-called IgG4-related sclerosing disease, as well as with exposure to some medications, infections, malignancies, surgery, or radiation. 18-Fluorodeoxyglucose (FDG) positron emission tomography (PET) is a nuclear medicine technique which is able to accurately identify in vivo areas characterised by elevated glucose metabolism, such as inflammatory, infective, and neoplastic lesions. There is mounting evidence suggesting that FDG-PET may have a role in assessing disease activity in idiopathic retroperitoneal fibrosis, but the role of FDG-PET in secondary retroperitoneal fibrosis is less established. Herein, we present four patients with retroperitoneal fibrosis of different etiology (isolated idiopathic, associated with large-vessel involvement, associated with carcinoid tumour, and secondary to pergolide) who underwent FDG-PET as part of their workup. The implications of FGD-PET results in the diagnosis and treatment of retroperitoneal fibrosis of different etiology are discussed

Imaging findings in primary central nervous system vasculitis

Primary central nervous system vasculitis (PCNSV) is a rare primary vasculitis limited to the brain and spinal cord. It can affect any age group, but has a predilection for subjects aged 40 to 60 years without clear gender predominance. Clinical manifestations are nonspecific, including headache, non-focal neurological features and, less frequently, focal neurological signs. Brain biopsy is the diagnostic gold standard, but may be falsely negative when unaffected tissue is sampled. In addition, brain biopsy carries a small but significant risk of serious complications. Imaging procedures are a key part of the workup of PCNSV patients. They can be used to document the extent and type of lesions, to gauge response to treatment, and sometimes as surrogates for brain biopsy. Magnetic resonance is extremely sensitive but non-specific. The most common findings are multiple bilateral ischaemic lesions often involving white and grey matter. Conventional or magnetic resonance angiography (MRA) typically shows segmental narrowing and dilation in multiple cerebral arteries. However, atypical findings have also been described both with magnetic resonance and angiography. This review discusses the state-of-the-art of current imaging techniques in the workup of PCNSV patients and highlights future prospects

18F-Fluorodeoxyglucose positron emission tomography for the assessment of myositis: a case series

To establish whether 18FFluorodeoxyglucose (FDG) positron emission computerised tomography (FDG-PET/CT) might reveal active disease in patients with myositis

Isolated vasculitis of the lower extremities in a patient with polymyalgia rheumatica and giant cell arteritis

Case report: Isolated vasculitis of the lower extremities in a patient with polymyalgia rheumatica and giant cell arteriti

Immunosuppressive and immunomodulatory therapies for idiopathic inflammatory myopathies

Objectives: This is a protocol for a Cochrane Review (intervention). The objectives are as follows:. This protocol is for two separate reviews to assess the effects (benefits and harms) of immunosuppressant and immunomodulatory treatments for the idiopathic inflammatory myopathies. Targeted treatments To assess the effects (benefits and harms) of targeted immunosuppressant and immunomodulatory treatments for the idiopathic inflammatory myopathies: dermatomyositis (DM, including juvenile dermatomyositis, jDM), immune mediated necrotising myopathy (IMNM), anti-synthetase syndrome (ASS), overlap-myositis (OM) and polymyositis (PM). We will also include cancer-related myositis and amyopathic dermatomyositis. Non-targeted treatments To assess the effects (benefits and harms) of non-targeted immunosuppressant and immunomodulatory treatments for the idiopathic inflammatory myopathies: dermatomyositis (DM, including juvenile dermatomyositis, jDM), immune mediated necrotising myopathy (IMNM), anti-synthetase syndrome (ASS), overlap-myositis (OM) and polymyositis (PM). We will also include cancer-related myositis and amyopathic dermatomyositis