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We sought to determine the prevalence of additional connective tissue diseases (CTDs) in patients with idiopathic inflammatory myopathies (IIM), and to study the muscle biopsy patterns in various clinico-serologic subsets of myositis. We undertook a retrospective cohort study of 648 patients with a histological diagnosis of IIM. The following was determined from the South Australian Myositis Database: presence of associated CTDs, histological details and presence of myositis-specific (MSA) or myositis-associated (MAA) antibodies. Among patients with IIM, a significantly greater proportion had systemic sclerosis 32/648 (4.9%) than mixed connective tissue disease (12/648, p = 0.003), primary Sjogren’s syndrome (12/648, p = 0.003), systemic lupus erythematosus (10/648, p

Introduction,: Graft-versus-host disease (GVHD) is a recognized complication of allogeneic stem cell transplantation (allo-SCT) and may affect muscle. We investigated the incidence and subtypes of inflammatory myopathy (IM) in South Australian recipients of allo-SCT. Methods Recipients of allo-SCT from 2004 to 2014 at the Royal Adelaide Hospital were identified. Records were reviewed to identify patients with weakness, creatine kinase (CK) elevation, and muscle biopsy confirming IM. Results Weakness was present in 32 of 224 patients who received allo-SCT patients reviewed, and CK was raised in 7 of 20 patients with weakness. Six patients developed biopsy-confirmed IM; 3 patients had chronic GVHD-related myopathy, 2 had necrotizing myopathy, and 1 had dermatomyositis (DM) associated with anti-melanoma differentiation associated protein 5 (MDA5) antibodies. The incidence of IM was calculated to be 2 cases per thousand annually. Discussion Among recipients of allo-SCT, weakness is common, and the incidence of IM is increased. Histopathological diagnoses are varied, and we report findings of necrotizing myopathy and anti-MDA5-associated DM. Muscle Nerve 58:790-795, 2018.

We describe the successful management of Anncaliia algerae microsporidial myositis in a man with graft versus host disease after hemopoietic stem cell transplantation. We also summarize clinical presentation and management approaches and discuss the importance of research into the acquisition of this infection and strategies for prevention.

INTRODUCTION The aim of this study was to determine whether a single-nucleotide polymorphism (SNP; 1858CT, R620W) in the protein tyrosine phosphatase N22 (PTPN22) gene confers susceptibility to idiopathic inflammatory myopathy (IIM) in South Australian patients with IIM. METHODS Genotyping was performed on stored DNA from 199 patients with histologically confirmed polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM), and then compared with 455 matched controls. Associations with the 8.1 ancestral haplotype (AH), and myositis-specific (MSA) and myositis-associated (MAA) autoantibodies were investigated. RESULTS The PTPN22 R620W minor allele frequency was increased in IIM patients (50 of 398, 12.6%) compared with controls (75 of 910, 8.2%) (odds ratio 1.6, 95% confidence interval 1.1-2.3, P = 0.016). In IIM patients, there was no association between the R620W minor allele and detection of any MSA/MAA (P = 0.70), nor any evidence of epistasis with the 8.1 AH (P = 0.69). CONCLUSIONS The PTPN22 R620W minor allele is associated with susceptibility to IIM in SA patients, independent of the 8.1 AH. Muscle Nerve, 2016 Muscle Nerve 55: 270-273, 2017.

The prevalence of Raynaud’s phenomenon (RP) in most studies of the general population is between 3 and 5 %. Primary RP is reversible vasospasm in peripheral arteries occurring in the absence of an underlying disease and accounts for 80–90 % of cases. Secondary RP develops in association with an underlying disorder and is often characterised by structural vascular abnormalities and irreversible vascular occlusion. The prevalence of primary RP ranges from 2 to 20 % in women and 1–12 % in men depending on geographic location, the population studied, the definition of RP used and the method of case ascertainment.