Kashif Muhammad, Arya Divya, Niazi Masooma, Khaja Misbahuddin
Division of Pulmonary and Critical Care Medicine, Bronx Lebanon Hospital Center, Affiliated with Icahn School of Medicine at Mount Sinai, Bronx, NY, USA.
Department of Medicine, Bronx Lebanon Hospital Center, Affiliated with Icahn School of Medicine at Mount Sinai, Bronx, NY, USA.
Am J Case Rep. 2017 Apr 25;18:448-453. doi: 10.12659/ajcr.903540.
BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia. CASE REPORT Here, we present a rare case of necrotizing myopathy and fibrinous and organizing pneumonia in a 34-year-old African American man with Sjögren's syndrome and anti-EJ antibodies. The patient's presenting symptoms were cough and proximal muscle weakness of the extremities. He had elevated serum creatine kinase level, aldolase level, and erythrocyte sedimentation rate. Myositis panel was positive for anti-EJ antibodies. Chest radiography was consistent with bilateral interstitial infiltrates. CT chest showed patchy bilateral infiltrates. Quadriceps muscle biopsy revealed widespread necrotic fibers and lung biopsy showed fibrinous and organizing pneumonia. The patient responded well to immunoglobulin therapy, mycophenolate, and prednisone, which resulted in complete resolution of bilateral infiltrates and improved muscle pain and weakness. CONCLUSIONS Myopathies are characterized by myalgia and muscle weakness due to muscle fiber dysfunction and are associated with autoimmune diseases. Histopathological features may differ in idiopathic inflammatory myopathies. It is important to recognize the rare association of anti-EJ autoantibodies with necrotizing myopathy and interstitial lung disease, which responds well to methylprednisolone and intravenous immunoglobulin.
特发性炎性肌病是自身免疫性疾病，可累及皮肤、关节、肌肉和肺部。这些疾病中最常见的是皮肌炎、多发性肌炎、重叠综合征和包涵体肌炎。坏死性自身免疫性肌病是一种特发性炎性肌病，很少与干燥综合征相关。与抗EJ合成酶综合征相关的最常见肺部表现是非特异性间质性肺炎和普通间质性肺炎；这种情况很少与纤维素性和机化性肺炎相关。病例报告：在此，我们报告一例罕见的坏死性肌病合并纤维素性和机化性肺炎的病例，患者为一名34岁的非裔美国男性，患有干燥综合征和抗EJ抗体。患者的主要症状是咳嗽和四肢近端肌肉无力。他的血清肌酸激酶水平、醛缩酶水平和红细胞沉降率升高。肌炎相关抗体检测显示抗EJ抗体呈阳性。胸部X线检查显示双侧间质浸润。胸部CT显示双侧斑片状浸润。股四头肌活检显示广泛的坏死纤维，肺部活检显示纤维素性和机化性肺炎。患者对免疫球蛋白治疗、霉酚酸酯和泼尼松反应良好，双侧浸润完全消退，肌肉疼痛和无力症状改善。结论：肌病的特征是由于肌纤维功能障碍导致的肌痛和肌无力，与自身免疫性疾病相关。特发性炎性肌病的组织病理学特征可能不同。认识到抗EJ自身抗体与坏死性肌病和间质性肺病的罕见关联很重要，这种情况对甲基泼尼松龙和静脉注射免疫球蛋白反应良好。
