High Titers of Autoantibodies in Patients with Sickle-Cell Disease

Toly-Ndour, Cécile; Rouquette, Anne-Marie; Obadia, Stéphanie; M’bappe, P.; Lionnet, François; Hagège, Isabelle; Boussa-Khettab, Faiza; Tshilolo, Léon; Girot, Robert

doi:10.3899/jrheum.100667

Cited by 34 publications

(44 citation statements)

References 43 publications

(29 reference statements)

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“…ANA positive serology was present in 15% of our patients in keeping with the previously reported figures of autoimmunity [3,4,18]. Additionally, 8.3% of our patients had SMA positive serology.…”

Section: Prevalence Of Positive Serology and Connective Tissue Diseasesupporting

confidence: 91%

“…CTD is perceived to have a higher incidence in patients with SCD, supported by several studies which demonstrate high levels of autoimmunity amongst patients with SCD [3,4]. While positive autoimmune status is not always indicative of clinical disease, a prospective study of 88 patients identified high titres of autoantibodies in patients with SCD compared to controls without SCD [3]. Reports of the presence of CTDs in patients with SCD are mostly anecdotal case studies [1,[5][6][7].…”

Section: Introductionmentioning

confidence: 99%

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

Igbineweka¹,

Darbari²,

Drašar³

et al. 2016

J Gen Pract

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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, with rheumatoid 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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Section: Prevalence Of Positive Serology and Connective Tissue Diseasesupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

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

Igbineweka¹,

Darbari²,

Drašar³

et al. 2016

J Gen Pract

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“…[9] In previous repors by Cherner et alSCD was diagnosed by 13 years and SLE by 21 years but in our study we have reported early diagnosis of SLE . [10] Both the cases were treated with prednisolone and showed improvement, the swelling subsided in the first case and the fever and pain subsided in the second case.…”

Section: Introductioncontrasting

confidence: 51%

“…Cutaneous markers are not frequently mentioned similar to our 1 ST case where there was no abnormality of skin. [9] Toly-Ndour and al. reported that 50% of 88 patients with SCD have positive antinuclear antibodies and 20% with titers greather than 1/200 but only one patient developed rheumatoid arthritis 5 years later and no SLE.…”

Section: Introductionmentioning

confidence: 99%

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Systemic Lupus Erythematosus (SLE) with Sickle Cell Disease (SCD)-A Rare Case Series

Ali¹

2017

jmscr

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Partial dysfunction of Treg activation in sickle cell disease

et al. 2014

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Sickle cell disease (SCD) is a chronic inflammatory disease associated with multiple organ damage, chronic anemia, and infections. SCD patients have a high rate of alloimmunization against red blood cells (RBCs) following transfusion and may develop autoimmune diseases. Studies in mouse models have suggested that regulatory T cells (Treg) play a role in alloimmunization against RBC antigens. We characterized the phenotype and function of the Treg cell population in a homogeneous cohort of transfused SCD patients. We found that the distribution of Treg subpopulations differed significantly between SCD patients and healthy blood donors. SCD patients have a particular Treg phenotype, with strong CTLA-4 and CD39 expression and weak HLA-DR and CCR7 expression. Finally, we show that this particular phenotype is related to SCD rather than alloimmunization status. Indeed, we observed no difference in Treg phenotype or function in vitro using autologous feeder cells between strong and weak responders to alloimmunization.

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High Titers of Autoantibodies in Patients with Sickle-Cell Disease

Abstract: Patients with SCD displayed high titers of autoantibodies. This observation may be due only to immune activation and/or dysfunction in SCD, as neither pathogenic specificity of autoantibodies nor autoimmune clinical signs appeared in the majority of cases in our study.

Cited by 34 publications

References 43 publications

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

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

Systemic Lupus Erythematosus (SLE) with Sickle Cell Disease (SCD)-A Rare Case Series

Partial dysfunction of Treg activation in sickle cell disease

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