Background Juvenile dermatomyositis (JDM) is thought to involve an autoimmune myositis, yet the elements of the immune response which damage muscle tissue in JDM remain unclear. Muscle tissue from early JDM shows infiltration by predominantly macrophage/myeloid cells. Traditional histopathology would suggest that these cells have a scavenger or 'repair' function; our data suggest otherwise. We ...

In recent years, antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibodies to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, "mechanic's hands," Rayna...

Dermatomyositis (DM) is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophe...

Masticatory muscle myositis is the most common inflammatory myopathy among canines and is associated with an autoimmune process; however, the cause is unknown. A 28-year-old young woman was notices with left facial swelling and pain, particularly during mouth opening. No obvious trigger factors, including trauma, were found. Computed tomography (CT) scans of the head and neck revealed a heterog...

Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by ot...

Juvenile dermatomyositis (JDM) is a rare, potentially life-threatening systemic autoimmune disease primarily affecting muscle and skin. Recent advances in the recognition, standardised assessment and treatment of JDM have been greatly facilitated by large collaborative research networks. Through these networks, a number of immunogenetic risk factors have now been defined, as well as a number of...

Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with no...

Idiopathic inflammatory myopathy (IIM) is the umbrella term including dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), sporadic inclusion body (IBM) and necrotising autoimmune (NAM), also known as immune-mediated myopathy. There some debate to whether PM exists a discrete entity, or perhaps an overly generalising encompassing connective tissue disease associated myositis, OM, pre...

Interferon alpha (IFN-alpha) therapy is associated with a number of immunological side-effects, including autoimmune diseases and a 10% prevalence of thyroiditis. Hepatitis C virus (HCV) infection itself predisposes to autoimmune phenomena including hypothyroidism and myositis. The development of clinical hypothyroidism in the presence of positive thyroid antibodies in patients infected with HC...

OBJECTIVE Serological testing for myositis-specific or associated autoantibodies [myositis-specific antibody (MSA) and myositis-associated antibody (MAA)] is useful for the diagnosis of idiopathic inflammatory myopathies (IIMs). However, available assays are neither standardized nor validated. The objective is to evaluate the accuracy of a commercial line blot assay for myositis diagnosis. ME...