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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

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

Pulmonary Pathologic Manifestations of Anti-Alanyl-tRNA Synthetase (Anti-PL-12)-Related Inflammatory Myopathy.
CONCLUSIONS: - Lung disease is often the first manifestation of anti-PL-12 ARS. There are no pathognomonic histopathologic features to distinguish anti-PL-12 ARS-related lung disease from idiopathic variants of diffuse interstitial lung disease. Increased inflammation, lymphoid aggregates, and nonspecific interstitial pneumonia-like areas in a biopsy, as well as clinical features of mechanic's hands, Raynaud phenomenon, arthritis, and fever, should prompt pathologists to suggest involvement by ARS. PMID: 28967806 [PubMed - as supplied by publisher]
Source: Archives of Pathology and Laboratory Medicine - October 2, 2017 Category: Laboratory Medicine Authors: Schneider F, Yousem SA, Oddis CV, Aggarwal R Tags: Arch Pathol Lab Med Source Type: research

An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries
Anil Kumar B Patil, AT Prabhakar, Ajith Sivadasan, Mathew Alexander, Geeta ChackoNeurology India 2015 63(1):72-76Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with ...
Source: Neurology India - March 4, 2015 Category: Neurology Authors: Anil Kumar B PatilAT PrabhakarAjith SivadasanMathew AlexanderGeeta Chacko Source Type: research

Pulmonary pathologic manifestations of anti-glycyl-tRNA synthetase (anti-EJ)-related inflammatory myopathy
Conclusions Identifying ARS-associated autoantibodies in ILD patients with or without myopathy is desirable because patients may respond well to immunosuppressive therapy, and their prognosis is better than that of patients with idiopathic forms of DAD or UIP.
Source: Journal of Clinical Pathology - July 16, 2014 Category: Pathology Authors: Schneider, F., Yousem, S. A., Bi, D., Gibson, K. F., Oddis, C. V., Aggarwal, R. Tags: Immunology (including allergy), Muscle disease, Inflammation, Interstitial lung disease, Degenerative joint disease, Musculoskeletal syndromes, Clinical diagnostic tests Original article Source Type: research