儿童结节病临床概述：来自土耳其的多中心经验。

A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

作者信息

Guliyeva Vafa, Demirkan Fatma Gul, Yiğit Ramazan Emre, Esen Esra, Bayındır Yagmur, Torun Ruya, Kılbas Gulsah, Gezgin Yıldırım Deniz, Otar Yener Gulcin, Cakan Mustafa, Demir Ferhat, Özturk Kübra, Baglan Esra, Yuksel Selcuk, Bakkaloglu Sevcan A, Bora Makay Balahan, Paç Kısaarslan Ayşenur, Oray Merih, Bilginer Yelda, Eker Ömeroğlu Rukiye, Ozen Seza, Sozeri Betul, Aktay Ayaz Nuray

机构信息

Department of Pediatric Rheumatology, Istanbul School of Medicine, Istanbul University, Istanbul, Türkiye.

Department of Pediatric Rheumatology, Ümraniye Research and Training Hospital, University of Health Science, Istanbul, Türkiye.

出版信息

Mod Rheumatol. 2024 Mar 28;34(3):639-645. doi: 10.1093/mr/road050.

DOI:10.1093/mr/road050

PMID:37243724

Abstract

OBJECTIVES

We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS).

METHODS

The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis.

RESULTS

Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7).

CONCLUSIONS

Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications.

摘要

目的

我们旨在概述一大群结节病患者的人口统计学数据、临床谱和治疗方法，并试图找出早发型（EOS）和晚发型儿童结节病（LOS）的差异。

方法

本研究采用回顾性描述性设计，分析诊断为儿童结节病的病例的病历。

结果

52例患者纳入研究。疾病发病的中位年龄和随访时间分别为83（28.2 - 119）个月和24（6 - 48）个月。10例（19.2%）为EOS（5岁生日前发病），42例（80.7%）为LOS。疾病发作时最常见的临床表现为眼部症状（40.4%），其次是关节表现（25%）、皮肤症状（13.5%）和多器官受累相关特征（11.5%）。前葡萄膜炎是眼部表现中最常见的（55%）。EOS患者比LOS患者更常出现关节、眼睛和皮肤表现。EOS患者（5.7%）和LOS患者（21.1%）的疾病复发率无统计学差异（P = 0.7）。