Sub-phenotyping of juvenile dermatomyositis: can it assist clinical decisions?
نویسندگان
چکیده
Introduction Juvenile Dermatomyositis (JDM) is a rare serious disease (affecting 2-3 million children/year) presenting with rash and proximal muscle weakness. Serious complications can include calcinosis, GI ulceration, interstitial lung disease (ILD) and even death. It is becoming clear that JDM is a heterogeneous condition. Dividing JDM into sub-phenotypes would allow better prediction of disease severity and more targeted treatments. We have identified novel auto-antibodies in subtypes of JDM that may correlate with specific phenotypes.
منابع مشابه
Non-Hodgkin lymphoma in a 13-year-old boy with provisional hypomyopathic juvenile Dermatomyositis
Provisional hypomyopathic juvenile dermatomyositis is a subgroup of clinically amyopathic juvenile dermatomyositis provisional. The diagnostic criteria include: Classic dermatomyositis skin lesions – which have to be confirmed by biopsy –, no involvement of proximal muscles, subclinical involvement of these muscles and normal level of muscle enzymes. These criteria should be present for a minim...
متن کاملModelling disease activity in juvenile dermatomyositis: A Bayesian approach.
Juvenile dermatomyositis is the most common form of the juvenile idiopathic inflammatory myopathies characterised by muscle and skin inflammation, leading to symmetric proximal muscle weakness and cutaneous symptoms. It has a fluctuating course and varying prognosis. In a Bayesian framework, we develop a joint model for four longitudinal outcomes, which accounts for within individual variabilit...
متن کاملSerological subsets of juvenile idiopathic inflammatory myopathies--an update.
In this review we explore the different characteristics of the serological phenotypes identified in juvenile-onset myositis and consider how the serological sub-classification of patients with juvenile myositis can be advantageous both in terms of reaching what can be a difficult diagnosis and informing on prognosis. Recent studies have described the autoantibody associated disease phenotypes a...
متن کاملJuvenile dermatomyositis: extramuscular manifestations and their management.
PURPOSE OF REVIEW Over the last few years, there have been several collaborative efforts focusing on how to assess the many aspects of juvenile dermatomyositis (JDM). It has become apparent that skin manifestations are an important part of the disease process that need to be evaluated, treated and controlled. Other extramuscular manifestations, such as calcinosis, are particularly difficult to ...
متن کاملJuvenile dermatomyositis in a 4‐year‐old Kenyan girl
To our knowledge, this is the first case report of juvenile dermatomyositis (JDM) in Tanzania. It demonstrates that the characteristic cutaneous findings of JDM may easily be overlooked, especially on dark skin, and the difficulty of clinical management in resource-constrained settings.
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 12 شماره
صفحات -
تاریخ انتشار 2014