Muscle ischaemia associated with NXP2 autoantibodies: a severe subtype of juvenile dermatomyositis

Rheumatology
Jessie AouizerateCyril Gitiaux

Abstract

Myositis-specific autoantibodies (MSAs) are increasingly used to delineate distinct subgroups of JDM. The aim of our study was to explore without a priori hypotheses whether MSAs are associated with distinct clinical-pathological changes and severity in a monocentric JDM cohort. Clinical, biological and histological findings from 23 JDM patients were assessed. Twenty-six histopathological parameters were subjected to multivariate analysis. Autoantibodies included anti-NXP2 (9/23), anti-TIF1γ (4/23), anti-MDA5 (2/23), no MSAs (8/23). Multivariate analysis yielded two histopathological clusters. Cluster 1 (n = 11) showed a more severe and ischaemic pattern than cluster 2 (n = 12) assessed by: total score severity ⩾ 20 (100.0% vs 25.0%); visual analogic score ⩾6 (100.0% vs 25.0%); the vascular domain score >1 (100.0% vs 41.7%); microinfarcts (100% vs 58.3%); ischaemic myofibrillary loss (focal punched-out vacuoles) (90.9 vs 25%); and obvious capillary loss (81.8% vs 16.7). Compared with cluster 2, patients in cluster 1 had strikingly more often anti-NXP2 antibodies (7/11 vs 2/12), more pronounced muscle weakness, more gastrointestinal involvement and required more aggressive treatment. Furthermore, patients with anti-NXP2 antibodi...Continue Reading

References

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Citations

Sep 21, 2018·Current Opinion in Rheumatology·Anna Ghirardello, Andrea Doria
Mar 16, 2018·Nature Reviews. Rheumatology·Sarah Onuora
Dec 27, 2019·JAMA Dermatology·Serena GiacaloneGianluca Nazzaro
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Dec 4, 2019·Journal of Pediatric Gastroenterology and Nutrition·Caroline BesnardBrigitte Bader-Meunier
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Apr 3, 2019·Immunological Medicine·Ran Nakashima
Feb 23, 2020·International Journal of Molecular Sciences·Eun Ha KangYun Jong Lee
Jul 21, 2020·Neuropathology and Applied Neurobiology·C DittmayerB Englert
Aug 2, 2019·Current Opinion in Neurology·Jantima Tanboon, Ichizo Nishino
Jan 25, 2020·Journal of Pediatric Gastroenterology and Nutrition·Caroline BesnardBrigitte Bader-Meunier
Oct 20, 2018·Brain : a Journal of Neurology·Yves AllenbachOlivier Benveniste
May 30, 2019·Frontiers in Neurology·Bruno StuhlmüllerEugen Feist
Dec 13, 2019·Current Rheumatology Reports·Danyang Li, Sarah L Tansley
Dec 6, 2020·Neurology·Jantima TanboonIchizo Nishino
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Feb 4, 2019·Joint, Bone, Spine : Revue Du Rhumatisme·Alain Meyer, Jean Sibilia
Apr 15, 2021·Journal of the American Academy of Dermatology·Kelsey B NusbaumBenjamin H Kaffenberger

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