Association between sickle cell disease and autoimmune diseases in Saudi population: a single center study.

IF 2.3 4区 医学 Q2 HEMATOLOGY
Rehab Y Al-Ansari, Alexander Woodman, Hana Ibrahim Al Daajani, Mohammad Ibrahim Aljamily, Tawasoul Fadoul, Elham Hamid Alfallaj, Khaled Abdullah Hassan, Nada Rajab Al Zahrani, Zechariah Jebakumar Arulanantham, Mohammed Al Ghamdi
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Abstract

Background: The association between sickle cell disease (SCD) and autoimmune diseases (AID) is not well understood. This retrospective study aims to investigate the frequency of coexistence of AIDS with SCD.

Research design and methods: This study was conducted in a tertiary care hospital. Data of n = 168 SCD patients between January 2016 and March 2023 were extracted and analyzed. Data included demographics, medications, hospitalization, phenotypes, presence of AIDs, and laboratory data.

Results: The mean age of n = 168 SCD patients was 30.66 (SD ± 11.27), with 54.2% of cases being female. 88.56% of patients had HGBSS phenotyping. The incidence of SCD cases with negative AID was 84.52%, while incidence of SCD cases with positive AID was 15.47% (p = 0.00001). Systemic lupus erythematosus (SLE, 4.2%), hypothyroidism (3.6%), and antiphospholipid syndrome (APS, 3.6%) were the most common SCD-associated AIDs. In addition, 69.2% of AIDs-positive patients used hydroxyurea. A strong correlation was found between hospitalization rate and a positive AID (p = 0.03).

Conclusions: The result of this study represents the significant rate of AID and SCD coexistence. The findings require further study to substantiate the need to develop recommendations for screening and early detection of the SCD-associated AIDs.

沙特人群镰状细胞病与自身免疫性疾病之间的关系:一项单中心研究
背景:镰状细胞病(SCD)与自身免疫性疾病(AID)之间的关系尚不清楚。本回顾性研究旨在探讨艾滋病与SCD共存的频率。研究设计与方法:本研究在某三级医院进行。提取2016年1月至2023年3月间168例SCD患者的数据并进行分析。数据包括人口统计、药物、住院、表型、艾滋病的存在和实验室数据。结果:168例SCD患者平均年龄为30.66 (SD±11.27)岁,女性占54.2%。88.56%的患者有HGBSS表型。aids阴性SCD的发病率为84.52%,aids阳性SCD的发病率为15.47% (p = 0.00001)。系统性红斑狼疮(SLE, 4.2%)、甲状腺功能减退(3.6%)和抗磷脂综合征(APS, 3.6%)是最常见的scd相关艾滋病。此外,69.2%的艾滋病阳性患者使用羟基脲。住院率与AID阳性有很强的相关性(p = 0.03)。结论:本研究结果反映了aids与SCD共存的显著率。研究结果需要进一步研究,以证实有必要制定筛查和早期发现scd相关艾滋病的建议。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
4.70
自引率
3.60%
发文量
98
审稿时长
6-12 weeks
期刊介绍: Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.
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