Early onset sarcoidosis: not a benign disease.

Early onset sarcoidosis: not a benign disease.
复制标题

早发性结节病:不是良性疾病。

DOI:
--
复制
发表时间:
1997
影响因子:
3.9
通讯作者:
R. Cimaz
R. Cimaz
中科院分区:
医学2区
文献类型:
--
作者:
C. Fink;R. Cimaz

文献摘要

被引文献

相似文献

在儿童时期有两种不同形式的结节病。我们描述了6例结节病的早期发病类型的临床图片。所有患者均在出生后4年内发病。与成年患者中报告的黑人优势相反,我们的系列包括5名白人和1名拉丁美洲人。皮疹、关节炎和葡萄膜炎三联征似乎是这种形式的特征。我们患者的症状与文献中的症状相当。然而,长期随访(9 - 23年)使我们认识到该疾病的严重并发症,以前不太清楚,如失明(4例患者),生长迟缓(3例患者),心脏受累(2例患者),肾功能衰竭(1例患者),甚至死亡。肺部受累仅出现在病程晚期的1例患者中。这种情况可能会被忽视,有时会误诊,因为它的罕见性和它与青少年关节炎的相似性。然而,这两种疾病的临床特征不同。早期和正确的诊断是重要的,根据严重的疾病预后,和可能的治疗选择。
There are 2 distinct forms of sarcoidosis in childhood. We describe the clinical picture of 6 patients with sarcoidosis of the early onset type. All patients had their disease onset during the first 4 years of life. In contrast to the black predominance reported in adult patients, our series comprised 5 whites and one Latin-American. The triad of rash, arthritis, and uveitis seems to be characteristic for this form. The symptoms in our patients were comparable to those in the literature. However, a long followup (9-23 yrs) allowed us to recognize severe complications of the disease, previously not well known, such as blindness (4 patients), growth retardation (3 patients), heart involvement (2 patients), renal failure (1 patient), and even death in one patient. Pulmonary involvement was present only in one patient late in the disease course. This condition may be overlooked, and is sometimes misdiagnosed, due to its rarity and to its similarities with juvenile arthritis. However, the clinical characteristics of the 2 diseases are different. Early and correct diagnosis is important in light of the severe disease prognosis, and the possible therapeutic options.