scholarly journals 291 Juvenile dermatomyositis associated with interstitial lung disease: would anti-MDA5 antibody detection improve early diagnosis and management?

Rheumatology ◽  
2018 ◽  
Vol 57 (suppl_3) ◽  
Author(s):  
Kathy Gallagher ◽  
Peter Bale ◽  
Kate Armon
2018 ◽  
Vol 56 (4) ◽  
pp. 249-254 ◽  
Author(s):  
Małgorzata Chowaniec ◽  
Marta Skoczyńska ◽  
Renata Sokolik ◽  
Piotr Wiland

2021 ◽  
pp. 004947552110020
Author(s):  
Devki Nandan ◽  
Hema Mittal ◽  
Anu Sharma ◽  
Kavita Srivastava

Children with recurrent or persistent pneumonia often have underlying chronic cardiopulmonary disease, but few reports on this subject have been published. Children with isolated common cardiac diseases, uncomplicated bronchial asthma or with incomplete records were excluded. Of 4361 children followed during a five-year period, 107 were included in our study. Underlying causes were identified in 99.0%: immunodeficiency disorders (20.2%), cardiothoracic malformations (18.3%), syndromic conditions (14.4%), infections (10.6%) bronchiectasis (10.6%), gastro-oesophageal reflux disease (6.6%), interstitial lung disease (3.8%) and other miscellaneous conditions (15.4%). Thus, children with recurrent or persistent pneumonia should be carefully evaluated for an underlying aetiology, as early diagnosis and appropriate management will decrease morbidity and mortality in most of these children.


2020 ◽  
Vol 13 (9) ◽  
pp. e236431
Author(s):  
Kelli Stager ◽  
Leanna Wise

Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.


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