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约旦的结节病:临床表型与疾病转归研究

Sarcoidosis in Jordan: A Study of the Clinical Phenotype and Disease Outcome.

作者信息

Alnaimat Fatima, Al Oweidat Khaled, Alrwashdeh Anas, Alnashrati Ahmad, Barham Saba, Hijaz Mohammad, Murad Dina, Alshelleh Sameeha, Obeidat Nathir

机构信息

Department of Internal Medicine, Division of Rheumatology, University of Jordan, Amman, Jordan.

Department of Internal Medicine, Division of Respiratory and Sleep Medicine, University of Jordan, Amman, Jordan.

出版信息

Arch Rheumatol. 2020 Feb 7;35(2):226-238. doi: 10.46497/ArchRheumatol.2020.7584. eCollection 2020 Jun.

DOI:10.46497/ArchRheumatol.2020.7584
PMID:32851372
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7406155/
Abstract

OBJECTIVES

This study aims to evaluate the clinical phenotypic features of sarcoidosis in a single-center academic hospital in Jordan.

PATIENTS AND METHODS

A retrospective file review was performed at an academic medical center in Jordan that included all patients diagnosed with sarcoidosis between January 2000 and December 2018. A total of 150 patients with sarcoidosis (38 males, 112 females; mean age 47.8±11.7 years; range, 17 to 79 years) were evaluated. Clinical data extracted from the files included the sex of the patient, the age at time of diagnosis, diagnosis date, the season during which the diagnosis was established, and smoking history. Biopsy histopathology, spirometry, nerve conduction, echocardiography, and imaging reports including plain radiographs, ultrasonographic, magnetic resonance and computed tomography reports were reviewed. Data including laboratory values, medication usage, clinical outcomes, and morbidity/mortality were collected. Pulmonary function tests including spirometry and lung volumes along with the diffusing capacity for carbon monoxide were reviewed for the presence of restriction, obstruction or reduction in the diffusion capacity of carbon monoxide. Identification of extra-thoracic organ involvement was determined in each patient in accordance with the criteria suggested by the updated World Association of Sarcoidosis and Other Granulomatous Disorders.

RESULTS

A total of 77.3% of the patients were diagnosed by biopsy. One case of Lofgren's syndrome was identified. Of the patients, 18.0% had isolated pulmonary sarcoidosis, 75.3% had pulmonary and extra-pulmonary sarcoidosis and 6.7% had isolated extra-pulmonary sarcoidosis while 81.3% had respiratory symptoms, mostly shortness of breath and cough. Extra-thoracic organ involvement mostly involved the musculoskeletal system (33%) followed by the skin (20%). Female patients had more extra-thoracic involvement but the sex difference was only statistically significant for cutaneous involvement. Of the patients, 84% received treatment while 20% had disease remission during the first two years after diagnosis and 70% required treatment beyond two years after diagnosis.

CONCLUSION

Various sarcoidosis clinical phenotypes are seen among Jordanian patients. Jordanian females are more affected by the disease and have more extra-thoracic involvement compared to male patients. A large number of the study patients received treatment.

摘要

目的

本研究旨在评估约旦一家单中心学术医院结节病的临床表型特征。

患者与方法

在约旦的一家学术医疗中心进行了一项回顾性病历审查,纳入了2000年1月至2018年12月期间所有诊断为结节病的患者。共评估了150例结节病患者(38例男性,112例女性;平均年龄47.8±11.7岁;范围17至79岁)。从病历中提取的临床数据包括患者性别、诊断时年龄、诊断日期、确诊季节以及吸烟史。审查了活检组织病理学、肺功能测定、神经传导、超声心动图以及包括X线平片、超声、磁共振和计算机断层扫描报告在内的影像学报告。收集了包括实验室检查值、用药情况、临床结局以及发病率/死亡率等数据。审查了包括肺功能测定和肺容积以及一氧化碳弥散能力在内的肺功能测试,以确定是否存在限制、阻塞或一氧化碳弥散能力降低。根据世界结节病及其他肉芽肿性疾病协会更新建议的标准,确定每位患者胸外器官受累情况。

结果

共有77.3%的患者通过活检确诊。确诊1例 Löfgren 综合征。患者中,18.0%患有单纯性肺结节病,75.3%患有肺结节病合并胸外结节病,6.7%患有单纯性胸外结节病,81.3%有呼吸道症状,主要为气短和咳嗽。胸外器官受累主要累及肌肉骨骼系统(33%),其次为皮肤(20%)。女性患者胸外受累更多,但性别差异仅在皮肤受累方面具有统计学意义。患者中,84%接受了治疗,20%在诊断后的头两年内病情缓解,70%在诊断两年后仍需治疗。

结论

约旦患者中可见多种结节病临床表型。与男性患者相比,约旦女性受该疾病影响更大,胸外受累更多。大量研究患者接受了治疗。