结节病的诊断与治疗

Diagnosis and therapyof sarcoidosis

目的 :探讨结节病的临床特点、诊断及治疗。方法 :对 2 5例结节病患者的临床、病理及疗效进行了分析。结果 :年龄 >5 0岁的结节病患者占 6 0 %。结节病主要累及器官以肺门及纵隔淋巴结最为常见 (88%) ,皮肤损害 (36 %)、浅表淋巴结肿大 (16 %)、肝脾肿大 (12 %)、关节痛 (8%)、眼部病变 (4 %)。结节病影像学异常率为10 0 %,肺功能检查异常率 70 %,血清血管紧张素Ⅰ转换酶 (SACE)升高率为 6 4%,PPD试验阴性率为 74%。病理组织学检查中 ,皮肤结节活检阳性率 83%,浅表淋巴结活检阳性率 75 %,经支气管镜肺活检 (TBLB)阳性率 6 4%,支气管粘膜活检 (BMB)阳性率 38%。误诊率为 2 8%。结论 :结节病中老年发病率较高。本病多器官受累 ,临床表现非特异性。TBLB和BMB简便易行、阳性率高、创伤性小 ,对结节病的诊断有较高的临床实用价值。Ⅱ期以上患者 ,尤其有全身症状、有肺功能损伤的患者的治疗首选口服激素。复发患者再次激素治疗仍有效。正确评价受累器官、判断疾病的活动性是严格治疗指征的关键。

Objective:To explore the clinical features, diagnosis and therapy of sarcoidosis. Methods: Clinical symptoms, pathological features and treatment of 25 patients with sarcoidosis were analysised. Results: 60% of sarcoidosis cases were over age50. The main clinical manifestations of sarcoidosis included bilateral hilar and mediastinal lymphadenopathy (88%)? skin lesions (36%)?swelling of superficial lymph nodes(16%)?hepatosplenomegaly (12%)?joint pains (8%)?ocular lesions(4%) . Chest radiographic abnormality rate is 100%, pulmonary function abnormality rate is 70%, SACEincreasing rateis 64%, negative reaction rate of PPD test is 74%.Amongpathological examination, definitive diagnosis rate of skin nodes biopsy is 83%,definitive diagnosis rate ofbiopsies of periphery lymph nodes is 75%, definitive diagnosis rate of TBLB is 64%,definitive diagnosis rate of BMBis 38%. Misdiagnosis rate is 28%. Conclusion: The morbidity of sarcoidosis increases in middle-old age patients. Sarcoidosis is a multiorgan involvement disease. Nonspecific constitutional symptoms may occur in the cases of sarcoidosis.TBLB and BMB are the convenient methods with highly definitive diagnosis rateand less invasive values in clinical practice.Oral corticosteroids is the most important method of treatment in patients of above-stageⅡor progressive loss oflung functions. Oral corticosteroids still had effect for recurrent patients. The key indication of treatment are assessing extent and severity of organ involvement, and evaluating whether the disease is likely to progress.