alexa Increased Prevalence of Autoimmunity and Connective Tis
ISSN: 2329-9126

Journal of General Practice
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Research Article

Increased Prevalence of Autoimmunity and Connective Tissue Diseases in Sickle Cell Disease

Norris Igbineweka1,2, Deepika S Darbari BS3,6, Emma R Drasar1,7, Sophia Steer5 and Swee Lay Thein1,3,4*
1Faculty of Life Sciences and Medicine, Molecular Hematology, Division of Cancer Studies, King’s College London, SE5 9NU, UK
2Department of Haematology, Brighton and Sussex NHS Trust, Brighton, BN2 5BE, UK
3NIH, National Heart, Lung, and Blood Institute, Sickle Cell Branch, Bethesda, MD 20892, USA
4Department of Haematological Medicine, King’s College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK
5Department of Rheumatology, King’s College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK
6Division of Hematology, Children’s National Medical Center, Washington, DC 20010, USA
7Department of Pathology, The Whittington Hospital NHS Trust, Magdala Avenue, London N19 5NF, UK
*Corresponding Author : Swee Lay Thein
National Heart
Lung, and Blood Institute
National Institute of Health
Building 10-CRC/5E-5142
10 Center Drive
Bethesda, MD 20892, USA
Tel: +1 301-435-2345
Fax: +1 301-451-7091
Received: February 16, 2016; Accepted: February 22, 2016; Published: February 29, 2016
Citation: Igbineweka N, Darbari DSBS, Drasar ER, Steer S, Thein SL (2016) Increased Prevalence of Autoimmunity and Connective Tissue Diseases in Sickle Cell Disease. J Gen Pract 4:236. doi:10.4172/2329-9126.1000236
Copyright: © 2016 Igbineweka N. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Connective tissue disease (CTD) and sickle cell disease (SCD) are diverse group of disorders; however, both of these diseases are characterized by underlying chronic inflammation. CTD is perceived to have a higher incidence and to affect the disease severity in SCD. Objectives: We seek to describe prevalence of autoimmunity and CTD and itsimpact on SCD disease severity as this is not well described in the literature. Methods: We retrospectively reviewed the medical records of 722 patients with SCD, seen over an 11 year period followed at a tertiary care hospital in London, UK. During the last 2 years of the study, number of hospitalizations and length of hospital stay were compared in patients with SCD with and without CTD. Results: In the study cohort, hemoglobin SS was the most common genotype (n = 451; 62%) and 411 (57%) were female. Twenty-three patients (3.1% had documented evidence of CTD, withrheumatoid arthritis (n = 14; 2%) and systemic lupus erythematosus (n = 2; 0.3%) being the most common. Antinuclear antibody was present in 108 (15%) of the patients and anti-smooth muscle antibody was present in 60 (8%). The mean number of hospitalizations (1.9 ± 2.7 vs. 1.5 ± 2.7) and mean length of hospital stay (6 days ± 4.1 vs. 7 days ± 11.4) were not different between those with or without autoimmunity and/or CTD. Avascular necrosis was the most common non-CTD musculoskeletal complication, affecting 72 (10%) patients. Conclusion: Our study suggests that positive autoimmune serology and CTD were more common in SCD compared to non-SCD population of similar ethnic background; however, presence of CTD does not appear to affect the SCD severity. The high frequency of CTD and overlapping of clinical symptoms suggest a need for diagnostic vigilance in diagnosing CTD in patients with SCD.


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