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P047  An unusual case of dermatomyositis with co-existent anti-GAD and anti-NXP2 antibody positivity
Conclusion GAD antibodies are known to be associated with many disorders including diabetes and stiff person syndrome and can recognise different epitopes in various diseases. This is the first documented case of GAD occurring in a DM patient, in the presence of NXP2 antibodies. IVIG is used to treat both s tiff man syndrome and refractory DM. It is possible that the combination of GAD and NXP2 contributed to the pathogenesis and severity of the clinical presentation in this case.Disclosure K. Beharry: None.G. Barminski: None.D. De Lord: None.
Source: Rheumatology - April 26, 2021 Category: Rheumatology Source Type: research

P035  Inflammatory myopathy and metabolic disorders causing myopathies
Conclusion This lady was initially managed as inflammatory myopathy but did not respond to high dose methylprednisolone. There were atypical features including normal inflammatory markers, MRI thighs showing predominantly fatty infiltration and muscle atrophy and the muscle biopsy with abundant lipid accumu lation suggestive of a metabolic disorder. We are awaiting full results of genetic testing. This case is a reminder of the importance of tissue diagnosis and reassessing the initial diagnosis if the clinical picture changes or patients do not respond as expected to treatment.Disclosure M. Malik: None.A. Mason: None....
Source: Rheumatology - April 26, 2021 Category: Rheumatology Source Type: research

Anti-TIF1- γ autoantibodies–positive dermatomyositis: where is the malignancy?
A77-year-old man presented with progressive proximal and distal muscle weakness of upper and lower limbs, neck flexor weakness, dysphagia, dysphonia and associated cutaneous manifestations (Fig. 1) [1]. Laboratory investigation revealed increased creatine kinase levels (630 U/l, upper normal value 174 U/l), and electrophysiological evaluation showed signs of irritative myopathy (small amplitude, brief, polyphasic motor units, fibrillations and positive sharp waves). Deltoid muscle biopsy revealed typical DM perifascicular atrophy (Fig. 1). Myositis-specific antibody analysis showed high titres (75, strongly positive 51 –...
Source: Rheumatology - August 30, 2017 Category: Rheumatology Source Type: research

Magnetic resonance measurement of muscle T2, fat-corrected T2 and fat fraction in the assessment of idiopathic inflammatory myopathies
Conclusion. Muscle T2, fc-T2 and FF measurements exhibit content validity with reference to semi-quantitative scoring of STIR and T1 MRI, and also exhibit construct validity with reference to several myositis activity and damage measures. T2 was as responsive as fc-T2 and STIR scoring, although progression of muscle damage was negligible during the study.
Source: Rheumatology - February 8, 2016 Category: Rheumatology Authors: Yao, L., Yip, A. L., Shrader, J. A., Mesdaghinia, S., Volochayev, R., Jansen, A. V., Miller, F. W., Rider, L. G. Tags: Myositis and Muscle Disease, Diagnostics and Imaging Procedures CLINICAL SCIENCE Source Type: research