Zohar Daniela Noa, Seluk Lior, Yonath Hagith, Shoenfeld Yehuda, Kivity Shaye
Department of Neurology, The Chaim Sheba Medical Center, Ramat Gan, Israel.
Department of Internal Medicine A, The Chaim Sheba Medical Center, Ramat Gan, Israel.
Mediterr J Rheumatol. 2020 Mar 31;31(1):75-77. doi: 10.31138/mjr.31.1.75. eCollection 2020 Mar.
Clinically amyopathic dermatomyositis is an uncommon autoimmune disorder in the Middle East. The clinical picture of clinically amyopathic dermatomyositis is characterized mainly by pulmonary and dermatological manifestations. Occasionally muscle symptoms are observed as well. Serum anti-MDA5 autoantibody positivity is associated with rapidly progressive interstitial lung disease among clinically amyopathic dermatomyositis patients. Moreover, high serum ferritin level is correlated with poor prognosis and high mortality. Herein we describe the case of an Israeli patient with rapidly progressive interstitial lung disease and without pathognomonic dermatological features who was diagnosed with anti-MDA5 positive clinically amyopathic dermatomyositis and did not survive despite immunomodulatory therapy followed by reduction in serum ferritin levels.
临床上无肌病性皮肌炎在中东地区是一种罕见的自身免疫性疾病。临床上无肌病性皮肌炎的临床表现主要以肺部和皮肤表现为特征。偶尔也会观察到肌肉症状。血清抗MDA5自身抗体阳性与临床上无肌病性皮肌炎患者的快速进展性间质性肺病相关。此外，高血清铁蛋白水平与预后不良和高死亡率相关。在此我们描述一例以色列患者，该患者患有快速进展性间质性肺病且无特征性皮肤表现，被诊断为抗MDA5阳性的临床上无肌病性皮肌炎，尽管接受了免疫调节治疗且血清铁蛋白水平有所降低，但仍未能存活。
