PMID- 27708934 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2147-9720 (Print) IS - 2148-4279 (Electronic) IS - 2147-9720 (Linking) VI - 2 IP - 2 DP - 2015 Jun TI - High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis. PG - 83-85 AB - Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive amyopathic dermatomyositis (ADM) associated with rapidly progressive interstitial pneumonitis (RPIP) frequently has a poor prognosis and optimal treatment is not well defined. Here, we report a 62-year-old Japanese man with anti-MDA5 antibody-positive ADM associated with RPIP presented with progressive shortness of breath, Heliotrope rash, Gottron's papules, arthralgia, and fatigue but no sign of muscle weakness. Laboratory investigation revealed serum levels of the following biomarkers: ferritin, 1393 ng/mL; Krebs von der Lungen-6, 1880 U/mL; and creatine kinase, 85 U/L. Computed tomography (CT) images showed diffuse ground-glass opacity in both lung fields. Because anti-MDA5 was positive, we made a diagnosis of ADM associated with RPIP and initiated treatment. Following five courses of combination therapy with prednisolone, cyclosporine A, and intravenous cyclophosphamide (IVCY), IVCY treatment was switched to high-dose intravenous immunoglobulin therapy (IVIg) because of the reactivation of interstitial pneumonia with an increased serum ferritin level. Additional treatment with IVIg improved RPIP, with normalization of anti-ADM antibody levels. Therefore, IVIg mayt be a new candidate treatment for anti-MDA5 antibody-positive ADM associated with RPIP. FAU - Hamada-Ode, Kazu AU - Hamada-Ode K AD - Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan. FAU - Taniguchi, Yoshinori AU - Taniguchi Y AD - Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan. FAU - Kimata, Takahito AU - Kimata T AD - Department of Rheumatology, Misato Bay Side Medical Center, Kochi, Japan. FAU - Kawaguchi, Yasushi AU - Kawaguchi Y AD - Department of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan. FAU - Shimamura, Yoshiko AU - Shimamura Y AD - Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan. FAU - Kuwana, Masataka AU - Kuwana M AD - Department of Rheumatology, Nippon Medical School, Tokyo, Japan. FAU - Fujimoto, Shimpei AU - Fujimoto S AD - Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan. FAU - Terada, Yoshio AU - Terada Y AD - Department of Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi University Medical School, Kochi, Japan. LA - eng PT - Journal Article DEP - 20150331 PL - Turkey TA - Eur J Rheumatol JT - European journal of rheumatology JID - 101656068 PMC - PMC5047270 OTO - NOTNLM OT - Amyopathic OT - anti-MDA5 antibody OT - dermatomyositis OT - interstitial pneumonitis EDAT- 2015/06/01 00:00 MHDA- 2015/06/01 00:01 PMCR- 2015/06/01 CRDT- 2016/10/07 06:00 PHST- 2014/08/09 00:00 [received] PHST- 2014/10/10 00:00 [accepted] PHST- 2016/10/07 06:00 [entrez] PHST- 2015/06/01 00:00 [pubmed] PHST- 2015/06/01 00:01 [medline] PHST- 2015/06/01 00:00 [pmc-release] AID - ejr-2-2-83 [pii] AID - 10.5152/eurjrheum.2015.0076 [doi] PST - ppublish SO - Eur J Rheumatol. 2015 Jun;2(2):83-85. doi: 10.5152/eurjrheum.2015.0076. Epub 2015 Mar 31.