Akinori Uruha1,2, Hans-Hilmar Goebel3,4, Werner Stenzel3,5. 1. Department of Neuropathology, Charité - Universitätsmedizin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany. a.uruha@gmail.com. 2. Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan. a.uruha@gmail.com. 3. Department of Neuropathology, Charité - Universitätsmedizin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany. 4. Department of Neuropathology, Universitätsmedizin Mainz, Langenbeckstraße 1, 55131, Mainz, Germany. 5. Leibniz Science Campus Chronic Inflammation, Charitéplatz 1, 10117, Berlin, Germany.
Abstract
PURPOSE OF REVIEW: To review recent advances in immunopathology for idiopathic inflammatory myopathies, focusing on widely available immunohistochemical analyses. RECENT FINDINGS: Sarcoplasmic expression of myxovirus resistance protein A (MxA) is specifically observed in all types of dermatomyositis and informs that type I interferons are crucially involved in its pathogenesis. It is a more sensitive diagnostic marker than perifascicular atrophy. Diffuse tiny dots in the sarcoplasm highlighted by p62 immunostaining are characteristically seen in immune-mediated necrotizing myopathy. This feature is linked to a chaperone-assisted selective autophagy pathway. Myofiber invasion by highly differentiated T cells, a marker of which is KLRG1, is specific to inclusion body myositis and has a crucial role in its pathogenesis. The recent advances in immunopathology contribute to increased diagnostic accuracy and a better understanding of the underlying pathophysiology in different types of idiopathic inflammatory myopathies.
PURPOSE OF REVIEW: To review recent advances in immunopathology for idiopathic inflammatory myopathies, focusing on widely available immunohistochemical analyses. RECENT FINDINGS: Sarcoplasmic expression of myxovirus resistance protein A (MxA) is specifically observed in all types of dermatomyositis and informs that type I interferons are crucially involved in its pathogenesis. It is a more sensitive diagnostic marker than perifascicular atrophy. Diffuse tiny dots in the sarcoplasm highlighted by p62 immunostaining are characteristically seen in immune-mediated necrotizing myopathy. This feature is linked to a chaperone-assisted selective autophagy pathway. Myofiber invasion by highly differentiated T cells, a marker of which is KLRG1, is specific to inclusion body myositis and has a crucial role in its pathogenesis. The recent advances in immunopathology contribute to increased diagnostic accuracy and a better understanding of the underlying pathophysiology in different types of idiopathic inflammatory myopathies.
Authors: A Uruha; Y Allenbach; J-L Charuel; L Musset; A Aussy; O Boyer; K Mariampillai; O Landon-Cardinal; C Rasmussen; L Bolko; T Maisonobe; S Leonard-Louis; S Suzuki; I Nishino; W Stenzel; O Benveniste Journal: Neuropathol Appl Neurobiol Date: 2018-11-22 Impact factor: 8.090
Authors: Mohammad Salajegheh; Sek Won Kong; Jack L Pinkus; Ronan J Walsh; Anne Liao; Remedios Nazareno; Anthony A Amato; Bryan Krastins; Chris Morehouse; Brandon W Higgs; Bahija Jallal; Yihong Yao; David A Sarracino; Kenneth C Parker; Steven A Greenberg Journal: Ann Neurol Date: 2010-01 Impact factor: 10.422
Authors: Steven A Greenberg; Jack L Pinkus; Geraldine S Pinkus; Travis Burleson; Despina Sanoudou; Rabi Tawil; Richard J Barohn; David S Saperstein; Hannah R Briemberg; Maria Ericsson; Peter Park; Anthony A Amato Journal: Ann Neurol Date: 2005-05 Impact factor: 10.422