The Turkish Journal of Pediatrics 2021 , Vol 63 , Num 5
A fatal interstitial lung disease in an anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody negative patient with juvenile dermatomyositis
Osman Yeşilbaş 1 ,Mehmet Yıldız 2 ,Can Yılmaz Yozgat 3 ,Irmak Tahaoğlu 4 ,Hakan Yazan 5 ,Erkan Çakır 5 ,Amra Adrovic 2 ,Sezgin Şahin 2 ,Kenan Barut 2 ,Özgür Kasapçopur 2
1 Departments of Pediatric Critical Care Medicine, Bezmialem Vakıf University Faculty of Medicine, İstanbul
2 Department of Pediatric Rheumatology, İstanbul University Cerrahpaşa Medical School, İstanbul, Turkey
3 Departments of Medical Student, Bezmialem Vakıf University Faculty of Medicine, İstanbul
4 Departments of Pediatrics, Bezmialem Vakıf University Faculty of Medicine, İstanbul
5 Departments of Pediatric Pulmonology, Bezmialem Vakıf University Faculty of Medicine, İstanbul
DOI : 10.24953/turkjped.2021.05.018 Background. Juvenile dermatomyositis associated interstitial lung disease, rarely seen in pediatric age groups, has adverse effects on survival. Anti-melanoma differentiation associated gene 5, one of the identified autoantibodies in juvenile dermatomyositis, preferentially affects the lung tissue and may cause rapidly progressive interstitial lung disease. It is a major cause of mortality in juvenile dermatomyositis. In this case report, we present a pediatric patient diagnosed with juvenile dermatomyositis without anti-melanoma differentiation associated gene 5 antibody positivity.

Case. A six-year-old male patient admitted to the Pediatric Intensive Care Unit with symptoms of respiratory failure, 1.5 months after the diagnosis of juvenile dermatomyositis. Thorax computed tomography examination revealed pneumomediastinum, a trace of left-sided pneumothorax, atelectasis on the left posterior lung region, ground-glass opacity, minimal subpleural patchy consolidation, and subcutaneous emphysema especially on the sides of the chest wall. Broad-spectrum antibiotics were started. His nasal swab sample was positive in terms of influenza B; therefore, oseltamivir was added to the treatment. Autoimmune myositis antibodies panel was examined but all of them including anti-melanoma differentiation associated gene 5 antibody resulted as negative. There was no notable reduction in lung infiltrations with the patient`s current treatment regimen. On the 12th day of Pediatric Intensive Care Unit admission, thorax computed tomography scan revealed progressed radiological lung findings compatible with rapidly progressive interstitial lung disease secondary to juvenile dermatomyositis. Despite intensive medical and extracorporeal treatments such as pulse steroid, intravenous immunoglobulin, methotrexate, cyclophosphamide, rituximab, therapeutic plasma exchange and, extracorporeal membrane oxygenation, the patient died on the 35th day.

Conclusions. Juvenile dermatomyositis patients should be carefully monitored for the development of interstitial lung disease. Rapidly progressive interstitial lung disease with a high mortality may develop shortly after diagnosis, even if the anti-melanoma differentiation associated gene 5 antibody is negative. Keywords : anti-melanoma differentiation associated gene 5, child, juvenile dermatomyositis, interstitial lung disease, rapidly progressive interstitial lung disease

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