Juvenile dermatomyositis (JDM) sine myositis
نویسندگان
چکیده
Methods/case 6 y/o girl from Kazakhstan presenting with discrete heliotrope of upper eyelids, erythema of the chest and extensor surfaces of extremities, Gottron’s papules as well as scarred piecemeal necrosis on 1 finger and 2 toes. Close examination revealed nailfold tortuositas. Muscle strength was 5/5, CMAS 48/51. Inflammatory parameters were not elevated, transaminases, CK and aldolase in the normal range, ANA >1:2560. Follow-up over 12 months showed no progression or muscle involvement.
منابع مشابه
An update in the diagnosis and management of juvenile dermatomyositis
Idiopathic inflammatory myopathies (IIM) represent a group of autoimmune muscle conditions with variable organ involvement amongst the different types [1]. Juvenile dermatomyositis (JDM) is the most prevalent subgroup among children (accounting for up to 85% of cases), while polymyositis, inclusion body myositis and dermatomyositis are most common in adults [2]. JDM is primarily a capillary vas...
متن کاملPres-FINAL-2009: Pediatric rheumatology practitioners experience with biologics in juvenile dermatomyositis: survey results
Introduction Biologic therapy is increasingly prescribed in rheumatologic disorders. Juvenile dermatomyositis (JDM), the most common inflammatory myopathy in children, can be challenging to manage in a subset of patients. There are multiple reports of cytokine involvement in JDM. There is a paucity of information regarding the use of biologics for JDM among pediatric rheumatology practitioners,...
متن کاملMyeloid cells which secrete S100 proteins in juvenile dermatomyositis may contribute to disease activity
Background Juvenile dermatomyositis (JDM) is thought to involve an autoimmune myositis, yet the elements of the immune response which damage muscle tissue in JDM remain unclear. Muscle tissue from early JDM shows infiltration by predominantly macrophage/myeloid cells. Traditional histopathology would suggest that these cells have a scavenger or 'repair' function; our data suggest otherwise. We ...
متن کاملClinically inactive disease in juvenile dermatomyositis – a proposed revision to the printo criteria
Introduction Juvenile dermatomyositis (JDM) affects 3 children/million/ year with myositis and skin disease being the typical features. PRINTO have recently established criteria to classify JDM patients who are clinically inactive by meeting at least 3 out of the following 4 conditions – CK ≤150, CMAS ≥48, MMT8 ≥78 and physician global VAS (PGA) ≤0.2. CK, CMAS and MMT8 all measure muscle involv...
متن کاملMyeloid related protein induces muscle derived inflammatory mediators in juvenile dermatomyositis
INTRODUCTION The aetiopathogenesis of juvenile dermatomyositis (JDM) remains poorly understood. In particular the contribution of monocytes or macrophages, which are frequently observed to be an infiltrate within muscle tissue very early in the disease process, is unknown. We hypothesised that these cells secrete the pro-inflammatory myeloid related protein (MRP) 8/14 which may then contribute ...
متن کامل