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

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. Overall, 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.

Keywords: Sickle cell disease; autoimmune disease; connective tissue disease; cytokines; hydroxyurea.