Ovgu Kul Cinar1,2, Charalampia Papadopoulou3,4, Clarissa A Pilkington3,5. 1. Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. ovgu.kulcinar@gosh.nhs.uk. 2. Division of Infection and Immunity, University College London, London, WC1E 6BT, UK. ovgu.kulcinar@gosh.nhs.uk. 3. Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. 4. Infection, Inflammation and Rheumatology Section, University College London Great Ormond Street Institute of Child Health, London, WC1N 1EH, UK. 5. NIHR Biomedical Research Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Abstract
PURPOSE OF REVIEW: Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterised by muscle and skin involvement. Calcinosis is a debilitating complication of JDM which is difficult to treat and may cause long-term morbidity. The purpose of this review is to provide an update for the treatment of JDM-associated calcinosis based on previously published studies. RECENT FINDINGS: Evidence-based studies are lacking for the management of calcinosis, and current treatment modalities have been largely based on case reports, case series, cohort studies, limited controlled studies and anecdotal clinical experience. The use of early aggressive therapy for resistant cases is strongly suggested to halt persistent disease activity which may help in reducing steroid use and their associated complications. Recent insights into disease pathogenesis, myositis-specific antibodies and genetic associations have led to identification of novel therapeutic targets such as Janus kinase (JAK) 1/2. Different treatment regimens with variable outcomes are in use for the treatment of refractory calcinosis; nevertheless, the level of evidence is not sufficient to propose specific guidelines. Recently, JAK 1/2 inhibitors have shown to be effective as an emerging therapeutic option highlighting that translational and clinical research is crucial to develop targeted treatment for JDM-associated calcinosis.
PURPOSE OF REVIEW: Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterised by muscle and skin involvement. Calcinosis is a debilitating complication of JDM which is difficult to treat and may cause long-term morbidity. The purpose of this review is to provide an update for the treatment of JDM-associated calcinosis based on previously published studies. RECENT FINDINGS: Evidence-based studies are lacking for the management of calcinosis, and current treatment modalities have been largely based on case reports, case series, cohort studies, limited controlled studies and anecdotal clinical experience. The use of early aggressive therapy for resistant cases is strongly suggested to halt persistent disease activity which may help in reducing steroid use and their associated complications. Recent insights into disease pathogenesis, myositis-specific antibodies and genetic associations have led to identification of novel therapeutic targets such as Janus kinase (JAK) 1/2. Different treatment regimens with variable outcomes are in use for the treatment of refractory calcinosis; nevertheless, the level of evidence is not sufficient to propose specific guidelines. Recently, JAK 1/2 inhibitors have shown to be effective as an emerging therapeutic option highlighting that translational and clinical research is crucial to develop targeted treatment for JDM-associated calcinosis.
Authors: Melody P Chung; Carrie Richardson; David Kirakossian; Amir B Orandi; Lesley A Saketkoo; Lisa G Rider; Adam Schiffenbauer; Carlos A von Mühlen; Lorinda Chung Journal: Autoimmun Rev Date: 2020-03-28 Impact factor: 9.754
Authors: Sarah L Tansley; Zoe E Betteridge; Harsha Gunawardena; Thomas S Jacques; Catherine M Owens; Clarissa Pilkington; Katie Arnold; Shireena Yasin; Elena Moraitis; Lucy R Wedderburn; Neil J McHugh Journal: Arthritis Res Ther Date: 2014-07-02 Impact factor: 5.156
Authors: H Gunawardena; L R Wedderburn; H Chinoy; Z E Betteridge; J North; W E R Ollier; R G Cooper; C V Oddis; A V Ramanan; J E Davidson; N J McHugh Journal: Arthritis Rheum Date: 2009-06