Idiopathic inflammatory myopathies are an unusual group of myopathies with annual incidence of 1 in 100 000 people in the United States. Necrotizing autoimmune myopathy comprises only 16% of this group. It usually presents with severe proximal weakness, lower extremity weakness, and severe fatigue while very rarely does it present with dysphagia and respiratory muscle weakness. Statin use, canc...

myopathy affecting proximal and distal musculature with typical onset of symptoms after age 50 years. Inclusion body myositis more often affects males and results in a classical pattern of early asymmetric weakness and wasting of forearm flexors (wrist and long finger flexors), quadriceps and ankle dorsiflexors. Inclusion body myositis is more frequently associated with a monoclonal gammopathy ...

Background: Dermatomyositis is an autoimmune inflammatory myopathy with distinct cutaneous findings. Its etiology is unknown. Objective: The purpose of this study was to evaluate demographic, clinical and paraclinical findings in patients with dermatomyositis in Ahwaz. Patients and Methods: This is descriptive, retrospective study and the records of all patients with dermatomyositis admitted to...

Inclusion body myositis is a rare condition of idiopathic inflammatory myopathy. Prior criteria for the diagnosis inclusion essentially required pathological features rimmed vacuoles, tubulofilamentous inclusions, and amyloid deposits. However, recently developed new diagnostic emphasize clinical characteristics including weakness finger flexors knee extensors. In addition, serological evaluati...

PURPOSE OF REVIEW This article reviews the results of recent therapeutic trials in dermatomyositis, polymyositis, and inclusion body myositis and suggests an approach to treating patients with inflammatory myopathy. RECENT FINDINGS We reviewed 10 double-blind, placebo-controlled therapeutic trials in patients with inflammatory myopathy. Only one, using intravenous immunoglobulin in refractory...

Statins are an extensively used class of drugs, and myopathy is an uncommon, but well-described side effect of statin therapy. Inflammatory myopathies, including polymyositis, dermatomyositis, and necrotizing autoimmune myopathy, are even more rare, but debilitating, side effects of statin therapy that are characterized by the persistence of symptoms even after discontinuation of the drug. It i...

OBJECTIVE To investigate the prevalence of anti-cyclic citrullinated peptide (anti-CCP) and anti-keratin antibodies (AKAs) in a cohort of patients with idiopathic inflammatory myopathy. METHODS In a cross-sectional study, we determined the presence of anti-CCP and AKAs by ELISA and IIF, respectively, in a cohort of 90 consecutive patients with idiopathic inflammatory myopathy. Associations be...

INTRODUCTION To the best of our knowledge, we describe for the first time the case of a woman who met the diagnostic criteria for fibromyalgia, did not respond to therapy for that disorder, and was subsequently diagnosed by biochemical and genetic studies with a mitochondrial myopathy. Treatment of the mitochondrial myopathy resulted in resolution of symptoms. This case demonstrates that mitoch...

RBCK1 deficiency is a rare congenital autoinflammatory disease that causes inflammatory disruption on the molecular level. This has three major clinical manifestations: increased sensitivity to bacterial infections, autoinflammation syndrome, and accumulation of amylopectin in skeletal muscle. The amylopectinosis myopathy cardiomyopathy. pathogenesis poorly investigated may include unnoticed re...