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106例结节病患者的临床特征、肺功能异常及泼尼松龙治疗结果

Clinical characteristics, pulmonary function abnormalities and outcome of prednisolone treatment in 106 patients with sarcoidosis.

作者信息

Sharma S K, Mohan A, Guleria J S

机构信息

Department of Medicine, All India Institute of Medical Sciences, New Delhi.

出版信息

J Assoc Physicians India. 2001 Jul;49:697-704.

Abstract

The clinical profile of one hundred and six biopsy proved patients with sarcoidosis is reported from New Delhi. The disease occurred more frequently in males (68 of the 106; 64%) in their fifth decade. Dry cough (83%), exertional dyspnoea (61%), fever (56%) and joint pains (44%) were frequent symptoms. Hepatomegaly (45%), peripheral lymphadenopathy (45%), cutaneous involvement (42%), presence of crepitations (36%) were frequent signs. In addition, ocular symptoms (18%), parotid gland enlargement (12%), facial nerve palsy (8%) were observed. Audible wheeze was present in nine patients. Radiologically, 25 (24%), 67 (63%) and 14 (13%) of patients belonged to stage I, II and III respectively at presentation. More than one sibling was involved in six families. Pulmonary functions revealed mixed obstructive and restrictive ventilatory defect. However, obstructive ventilatory defect was predominant. Systemic steroids were used in 81 patients who regularly followed up. Frequent relapses occurred on tapering off or stopping the corticosteroids in 21 patients. History of malaise (p < 0.05), presence of crepitations (p < 0.05), wheezing (p < 0.05), peripheral blood eosinophilia (p < 0.05) and FEV1/FVC (%) < 65% of the predicted value (p < 0.05), were independent predictors of relapse. Hyperuricaemia, hitherto unreported in patients with sarcoidosis was observed in 41% of patients for whom values were available. Two patients died; one from cardiomyopathy and another from stroke. In summary, constitutional symptoms such as fever, weight loss and pulmonary infiltrates, were more frequently encountered in Indian patients with sarcoidosis as compared to western studies. A high index of clinical suspicion and histopathological confirmation early in the illness are required to ascertain the diagnosis lest these patients will get treated as tuberculosis with potentially hepatotoxic drugs.

摘要

来自新德里的报告称,对106例经活检证实的结节病患者的临床资料进行了研究。该疾病在男性中更为常见(106例中有68例;64%),发病年龄多在50岁左右。干咳(83%)、劳力性呼吸困难(61%)、发热(56%)和关节疼痛(44%)是常见症状。肝肿大(45%)、外周淋巴结病(45%)、皮肤受累(42%)、捻发音(36%)是常见体征。此外,还观察到眼部症状(18%)、腮腺肿大(12%)、面神经麻痹(8%)。9例患者有可闻及的哮鸣音。放射学检查显示,初诊时分别有25例(24%)、67例(63%)和14例(13%)患者属于I期、II期和III期。6个家庭中有不止1个兄弟姐妹患病。肺功能显示存在混合性阻塞性和限制性通气功能障碍。然而,以阻塞性通气功能障碍为主。81例接受定期随访的患者使用了全身性激素。21例患者在逐渐减量或停用糖皮质激素后频繁复发。不适病史(p<0.05)、捻发音(p<0.05)、哮鸣音(p<0.05)、外周血嗜酸性粒细胞增多(p<0.05)以及FEV1/FVC(%)<预测值的65%(p<0.05)是复发的独立预测因素。41%有数据可查的患者出现了结节病患者中迄今未报告过的高尿酸血症。2例患者死亡;1例死于心肌病,另1例死于中风。总之,与西方研究相比,印度结节病患者更常出现发热、体重减轻等全身症状以及肺部浸润。在疾病早期需要高度的临床怀疑和组织病理学确诊来明确诊断,以免这些患者被当作结核病用可能有肝毒性的药物进行治疗。

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