以肺外症状为主要表现的42例结节病误诊探析

Analysis of Misdiagnosis of 42 Cases of Sarcoidosis with Extrapulmonary Symptoms as the Main Manifestation

目的探讨以肺外症状为主要表现结节病的临床特点及误诊原因、防范措施。方法检索中国知网和万方医学网近5年文献,对纳入文献及我院同期收治的以肺外症状为主诉的结节病误诊病例进行汇总分析。结果共纳入42例。以皮肤症状为主诉就诊25例,误诊为结节性红斑和神经性皮炎各4例,过敏性皮炎和湿疹各3例,红皮病和结核病各2例,寻常狼疮、扁平苔藓、脂囊瘤、硬皮病、银屑病、普通感染和耳廓炎性纤维增生各1例;以神经系统症状为主诉就诊7例,误诊为Guillain-Barres综合征3例,周围神经病2例,结核病和脑转移瘤各1例;以关节症状为主诉就诊4例,误诊为反应性关节炎3例,致密性骨炎1例;以脾脏病变为主诉就诊3例,误诊为转移瘤、淋巴瘤和纤维化型先天性肝内胆管扩张症致门静脉高压、脾大及脾功能亢进各1例;以甲状腺受累症状为主诉就诊2例,均误诊为甲状腺功能亢进;以心脏症状为主诉就诊1例,误诊为冠心病。误诊时间10 d~20年。42例均经病理组织学检查确诊,予相应治疗,1例视力受损严重未恢复,1例神经受累仍有反复及进行性加重,余病情均缓解。结论以肺外症状为主要表现的结节病患者症状无特异性,及时行胸部CT及病理检查非常重要,对累及肺外重要脏器者糖皮质激素和(或)免疫抑制剂治疗要充分。

Objective To explore the clinical features,cause of misdiagnosis and preventive measures of sarcoidosis patients with extrapulmonary symptoms as the main manifestation.Methods The literatures of the past five years in the database of CNKI and Wanfang Medical Network were searched,and the misdiagnosed sarcoidosis patients with extrapulmonary symptoms as the chief complaint in the literatures and in our hospital during the same period were collected and analyzed.Results A total of 42 cases were included.There were 25 cases with skin symptoms as chief complaint,including 4 misdiagnosed as nodular erythema and neurodermatitis respectively,3 as allergic dermatitis and eczema respectively,2 as erythroderma and tuberculosis respectively,and 1 as lupus vulgaris,lichen planus,lipocystic tumor,scleroderma,psoriasis,common infection and auricle inflammatory fibroplasia,respectively.There were 7 cases with neurological symptoms as chief complaint,and 3 cases were misdiagnosed as Guillain-Barre syndrome,2 cases as peripheral nerve disease,and 1 as brain tuberculosis and metastatic neoplasm respectively.There were 4 cases with joint symptoms as chief complaint,3 cases as adjuvant arthritis and 1 case as condensing osteitis.There were 3 cases with spleen lesions as chief complaint and 1 was misdiagnosed as metastatic tumor,lymphoma and portal hypertension caused by fibrotic congenital intrahepatic biliary dilatation,splenomegaly,and hypersplenism,respectively.There were 2 cases with symptoms of thyroid involvement as chief complaint and were misdiagnosed as hyperthyroidism.There was 1 case with cardiac symptoms as chief complaint and was misdiagnosed as coronary heart disease.The duration of misdiagnosis was 10 d-20 years.All 42 patients were confirmed by histopathological examination and were treated accordingly.Except 1 case with severe visual impairment that was not recovered and 1 case with recurrent aggravation of nerve involvement,the remaining patients had achieved clinical remission.Conclusion The symptoms manifested as extrapulmonary sarcoidosis are not specific,therefore,it is very important to perform timely chest CT and pathological biopsy.Patients with sarcoidosis involving important extrapulmonary organs should be adequately treated with glucocorticoid and/or immunosuppressants.