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Dermatomyositis in a patient undergoing nivolumab therapy for metastatic melanoma: a case report and review of the literature
We present the case of a 63-year-old man with metastatic melanoma undergoing treatment with nivolumab who developed significant motor weakness, paresthesias of both hands, swollen fingers, and a pruritic rash over the face, chest, and upper back after two cycles. Creatine kinase was elevated. Electromyography revealed a myopathic pattern, muscle biopsy of the deltoid revealed an inflammatory myopathy, and skin biopsy showed interface dermatitis. There were no detectable autoantibodies except positive antinuclear antibody. He was diagnosed with immunotherapy-induced dermatomyositis, nivolumab was held, and he was treated wi...
Source: Melanoma Research - May 2, 2020 Category: Cancer & Oncology Tags: Short Communications Source Type: research

Scurfy Mice Develop Features of Connective Tissue Disease Overlap Syndrome and Mixed Connective Tissue Disease in the Absence of Regulatory T Cells
Discussion Treg represent a lineage of T cells which play a fundamental role in maintaining humoral tolerance in the periphery. This subset of “suppressor T cells” is identified as FoxP3-expressing CD4+ T cells (16, 17). The unrestrained expression of FoxP3 is essential for the development and function of Treg (4). Accordingly, a disruption of the Foxp3 gene in scurfy mice results in an autoimmune lymphoproliferative disorder with fatal multi-organ inflammation (18). Since the causative mutation occurs in orthologous genes, the scurfy phenotype is indicated as the murine equivalent of the human IPEX sy...
Source: Frontiers in Immunology - April 23, 2019 Category: Allergy & Immunology Source Type: research

Inflammatory myopathies
We report a patient with clinical scleroderma presenting with subacute myopathy with lymphoid follicle on muscle histology. A 29-year-old female with scleroderma confirmed by clinical criteria and skin biopsy, using colchicine, presented with subacute proximal muscle weakness associated with dysphagia and dropped head without CK elevation, but EMG/NCS demonstrated myopathic findings.
Source: Neuromuscular Disorders - September 6, 2018 Category: Neurology Authors: A. Silva, M. Vianna, R. Mendon ça, E. Zanoteli Source Type: research

Dropped Head Syndrome and the Presence of Rimmed Vacuoles in a Muscle Biopsy in Scleroderma-Polymyositis Overlap Syndrome Associated with Anti-Ku Antibody.
Authors: Takeshi Y, Mai Y, Kinjo M, Manabu J, Itsuro H Abstract A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory my...
Source: Internal Medicine - November 21, 2017 Category: Internal Medicine Tags: Intern Med Source Type: research

Association of Fibrosing Myopathy in Systemic Sclerosis and Higher Mortality
ConclusionFibrosing myopathy is a unique histologic subtype of muscle disease among weak patients with SSc and is associated with significantly worse mortality compared to those with inflammation and/or necrosis on muscle biopsy.
Source: Arthritis Care and Research - October 27, 2017 Category: Rheumatology Authors: Julie J. Paik, Fredrick M. Wigley, Ami A. Shah, Andrea M. Corse, Livia Casciola ‐Rosen, Laura K. Hummers, Andrew L. Mammen Tags: Brief Report Source Type: research

Fibrosing myopathy in systemic sclerosis associates with higher mortality
Conclusion: Fibrosing myopathy is a unique histological subtype of muscle disease among weak patients with SSc and is associated with significantly worse mortality compared to those with inflammation and/or necrosis on muscle biopsy. This article is protected by copyright. All rights reserved.
Source: Arthritis Care and Research - May 23, 2017 Category: Rheumatology Authors: Julie J. Paik, Fredrick M. Wigley, Ami A. Shah, Andrea M. Corse, Livia Casciola ‐Rosen, Laura K. Hummers, Andrew L. Mammen Tags: Brief Report Source Type: research

Corticosteroids in Myositis and Scleroderma
Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line a...
Source: Rheumatic Disease Clinics of North America - October 27, 2015 Category: Rheumatology Authors: Anna Postolova, Jennifer K. Chen, Lorinda Chung Source Type: research