Early-onset "sarcoidosis" and "familial granulomatous arthritis (arteritis)": the same disease.
Early-onset "sarcoidosis" and "familial granulomatous arthritis (arteritis)": the same disease.
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早发性“结节病”和“家族性肉芽肿性关节炎（动脉炎）”：同一种疾病。
DOI：
10.1016/s0022-3476(86)80411-5
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
J. Miller
中科院分区：
文献类型：
--
作者：
J. Miller
Gross等人最近的报告描述了一名患有“结节病”的儿童的血管病变，这很重要，因为它提供了两种综合征之间的联系，我认为这两种综合征代表了一种疾病。1970年，North等人2描述了6例早发性“结节性关节炎”的儿童，并回顾了6例早期类似儿童的病例报告。随后几年，零散的病例报告，38例通常是单一病例，其中一例来自该医院，证实并扩大了临床图景。典型的儿童发病年龄小于4岁，表现为多发、明显但间隔紧密的1至2毫米的橙色或棕褐色丘疹，可能广泛分布，但通常在屈肌表面最为突出，许多关节滑膜组织肿胀，活检证据显示这些器官和其他器官有非cas变性肉芽肿。葡萄膜炎很常见。肺和纵隔不受累。这个过程通常是长期渐进的。已报道一例仅涉及皮肤，但可能发生一系列严重程度，包括严重的肾脏疾病/类固醇治疗可控制但不能治愈。1982年，Rotenstein等人描述了一种类似的疾病，他们称之为家族性肉芽肿性动脉炎。它的主要区别在于它是家族性的，发展为严重的高血压，并有动脉造影证实累及中等动脉。当被问及相似性时，2他们指出他们的病人也有发烧，这在North等人最初的综述中没有描述。在这家医院，我们见过5例符合“早发性结节性关节炎”标准的病例：2例明显是散发性的，2例明显是家族性的（一对父女），1例可能是家族性的（一位住在另一个国家的阿姨，没有在这里检查过）。其中一例散发病例以发热开始，患者在青年时期出现高血压。有可能家族史的患儿也有周期性发热和一过性结节样红斑病变。因此，我们的经验提供了重叠
The recent report by Gross et al. describing vasculopathy in a child with" sarcoidosis" is important because it provides a link between two syndromes that I believe represent a single disease. In 1970 North et al. 2 described six children with the early onset of" sarcoid arthritis" and reviewed six earlier case reports of similar young children. In subsequent years, scattered case reports, 38 usually single, and one from this hospital, 9 confirmed and expanded the clinical picture. Typically the child is younger than 4 years of age at onset, has a characteristic rash of multiple, distinct but closely spaced I to 2 mm orange or tan papules, which may be widespread but are usually most prominent on flexor surfaces, has exuberantly swollen synovial tissue of many joints, and has biopsy evidence of noncaseating granulomas in these and other organs. Uveitis is common. The lungs and mediastinum are not involved. The course is usually chronically progressive. A case has been reported involving skin only,~ but a range of severity may occur, including significant renal disease/Steroid therapy provides control but not cure. In 1982 Rotenstein et al/t described a similar disease, which they called familial granulomatous arteritis. It differed primarily because it was familial, proceeded to severe hypertension, and had arteriographically demonstrable involvement of medium-sized arteries. When questioned about the similarity, 2 they pointed out that their patients had also had fever, which had not been described in the original review by North et al? At this hospital we have seen five cases that fit the criteria for" early-onset sarcoid arthritis": two apparently sporadic, two clearly familial (a father and daughter), and one probably familial (an aunt living in another country and not examined here). One of the sporadic cases began with fever, and the patient has developed hypertension as a young adult. 9 The child with the probable family history also had periodic fever and transient erythema nodosumlike lesions. Thus our experience provided overlapping