Biomaterial Database

Clinically amyopathic dermatomyositis manifested after the allogeneic haematopoietic stem cell transplantation: Case presentation and literature review. 2023

Riko Miyagi, and Sawako Nakachi, and Yasutaro Tamaki, and Mototsugu Doi, and Tomo Nakajima, and Sakiko Kitamura, and Shohei Tomori, and Taeko Hanashiro, and Keita Tamaki, and Kazuho Morichika, and Yukiko Nishi, and Satoko Morishima, and Takuya Fukushima, and Hiroaki Masuzaki

Division of Endocrinology, Diabetes and Metabolism, Hematology and Rheumatology, Second Department of Internal Medicine, Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan.

Clinically amyopathic dermatomyositis (CADM) lacks muscle symptoms, associated with rapidly progressive interstitial lung disease. Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody has been identified as a disease-labelling autoantibody. We report two cases of CADM manifested after the allogeneic haematopoietic stem cell transplantation (allo-HSCT)-Case 1: a 56-year-old man with acute leukaemia received the allo-HSCT and Case 2: a 45-year-old female patient with lymphoma received the allo-HSCT. She received donor lymphocyte infusion because of a post-transplant relapse. After allo-HSCT or donor lymphocyte infusion, Gottron papules emerged, and both patients were diagnosed as CADM based on dermatological findings coupled with the positivity of anti-MDA-5 antibody, accompanied by interstitial shadows consistent with ILD on chest computed tomography. Case 2 was initially diagnosed as a kind of chronic graft versus host disease. Their symptoms were improved by the combination of immunosuppressive agents with a concomitant decrease in anti-MDA-5 antibody levels. For Case 2, rituximab was subsequently started for relapse of lymphoma, resulting in a substantial decrease in the level of anti-MDA-5 antibody and improvement in rash and ILD. Our cases raise a possibility that CADM emerges after the HSCT, highlighting the importance of early diagnosis to avoid fated progression into ILD.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D012008	Recurrence	The return of a sign, symptom, or disease after a remission.	Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D003882	Dermatomyositis	A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)	Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000072640	Interferon-Induced Helicase, IFIH1	A DEAD box RNA helicase that contains two N-terminal CASPASE ACTIVATION AND RECRUITMENT DOMAINS. It functions as a sensor of viral NUCLEIC ACIDS such as DOUBLE-STRANDED RNA and activates the INNATE IMMUNE RESPONSE by inducing the expression of INTERFERON-ALPHA and INTERFERON-BETA. It may also regulate cell growth and APOPTOSIS.	CADM-140 Autoantigen,Helicard,IFIH1 Protein,Interferon Induced with Helicase C Domain 1,Interferon-Induced Helicase C Domain-Containing Protein 1,Melanoma Differentiation-Associated Protein 5,Autoantigen, CADM-140,CADM 140 Autoantigen,Helicase, IFIH1 Interferon-Induced,IFIH1 Interferon-Induced Helicase,Interferon Induced Helicase C Domain Containing Protein 1,Interferon Induced Helicase, IFIH1,Melanoma Differentiation Associated Protein 5
D017563	Lung Diseases, Interstitial	A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.	Diffuse Parenchymal Lung Disease,Diffuse Parenchymal Lung Diseases,Interstitial Lung Disease,Interstitial Lung Diseases,Pneumonia, Interstitial,Pneumonitis, Interstitial,Interstitial Pneumonia,Interstitial Pneumonias,Interstitial Pneumonitides,Interstitial Pneumonitis,Lung Disease, Interstitial,Pneumonias, Interstitial,Pneumonitides, Interstitial