Departments of Pediatric Critical Care Medicine, Bezmialem Vakıf University Faculty of Medicine, İstanbul.
Department of Pediatric Rheumatology, İstanbul University Cerrahpaşa Medical School, İstanbul, Turkey.
Turk J Pediatr. 2021;63(5):903-908. doi: 10.24953/turkjped.2021.05.018.
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.
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 12 < sup > th < /sup > 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 35 < sup > th < /sup > day.
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.
儿童青少年皮肌炎相关性间质性肺病在儿科人群中罕见，但对生存有不良影响。抗黑色素瘤分化相关基因 5 是在儿童青少年皮肌炎中鉴定出的自身抗体之一，它优先影响肺组织，可能导致快速进展性间质性肺病。这是儿童青少年皮肌炎死亡的主要原因。在本病例报告中，我们介绍了一例未检出抗黑色素瘤分化相关基因 5 抗体阳性的皮肌炎患儿。
一名 6 岁男性患儿，在诊断为皮肌炎后 1.5 个月，因呼吸衰竭入住儿科重症监护病房。胸部计算机断层扫描检查显示纵隔积气，左侧气胸微量，左后肺区肺不张，磨玻璃影，最小程度的胸膜下斑片状实变，以及特别是在胸壁两侧皮下气肿。开始使用广谱抗生素。他的鼻拭子样本乙型流感检测阳性，因此加用奥司他韦治疗。检查自身免疫性肌炎抗体谱，但所有抗体包括抗黑色素瘤分化相关基因 5 抗体均为阴性。尽管患者目前的治疗方案，但肺部浸润未见明显减少。儿科重症监护病房入院第 12 天，胸部计算机断层扫描显示影像学肺进展符合皮肌炎相关性快速进展性间质性肺病。尽管进行了强化的内科和体外治疗，如脉冲类固醇、静脉注射免疫球蛋白、甲氨蝶呤、环磷酰胺、利妥昔单抗、治疗性血浆置换和体外膜氧合，但患者仍在第 35 天死亡。
皮肌炎患儿应密切监测间质性肺病的发生。即使抗黑色素瘤分化相关基因 5 抗体阴性，也可能在诊断后不久迅速发生高死亡率的快速进展性间质性肺病。
