Characteristics and Outcome of Connective Tissue Diseases in Patients with Sickle-Cell Disease: Report of 30 Cases

Michel, Marc; Habibi, Anoosha; Godeau, Bertrand; Bachir, Dora; Lahary, A.; Galactéros, Frédéric; Fifi‐Mah, Aurore; Arfi, S.

doi:10.1016/j.semarthrit.2007.10.003

Cited by 49 publications

(61 citation statements)

References 34 publications

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“…SCD could potentially have an increased risk of developing CTD similar to that speculated for patients with beta-thalassemia trait and its association with higher rates of RA [1,16]. However, larger longitudinal population studies would be needed to characterize these associations between these hemoglobinopathies and the development of CTD.…”

Section: Prevalence Of Positive Serology and Connective Tissue Diseasementioning

confidence: 86%

“…Involvement of musculoskeletal system is common in patients with SCD. While painful vaso-occlusive crises (VOC) are considered the hallmark musculoskeletal manifestation of SCD, other complications involving musculoskeletal system such as connective tissue disorders (CTD) also exist and require a high index of suspicion for timely diagnosis as manifestations may be similar and overlap in both diseases [1,2].…”

Section: Introductionmentioning

confidence: 99%

“…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]. Further, diagnostic uncertainty remains over defining the onset of CTD, for example, differentiating a flare-up of RA from an acute VOC in patients with SCD.…”

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 Diseasementioning

confidence: 86%

Section: Introductionmentioning

confidence: 99%

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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“…Hydroxyurea was stopped and erythrocytapheresis was initiated to prevent severe SCD clinical manifestations [3,4]. Although glucocorticoids are the most commonly used therapeutic agent for sarcoidosis, SCD patients incur the risk of developing severe sickle cell related clinical manifestation during treatment with steroids [5]. In sarcoidosis, methotrexate (MTX) has been proposed as a standard second-line agent in patients that cannot use or do not respond to steroids [6].…”

mentioning

confidence: 99%

“…Limited data on MTX and SCD are present in the literature. In a group of SCD patients with rheumatic disorders treated with MTX, increased vaso-occlusive crisis has been reported [5]. Therefore, the patient was treated with a low dose of prednisone (25 mg/day) associated with MTX (7.5 mg weekly and folic acid 5 mg/week).…”

mentioning

confidence: 99%

An unusual case of sarcoidosis in an adult patient with sickle cell disease: Management with methotrexate and low dose of steroid

Delmonte

Zamò

Cantini³

et al. 2013

American J Hematol

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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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Characteristics and Outcome of Connective Tissue Diseases in Patients with Sickle-Cell Disease: Report of 30 Cases

Cited by 49 publications

References 34 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

An unusual case of sarcoidosis in an adult patient with sickle cell disease: Management with methotrexate and low dose of steroid

Partial dysfunction of Treg activation in sickle cell disease

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