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Open Access 01-12-2018 | Research article

Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges

Authors: Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert

Published in: Pediatric Rheumatology | Issue 1/2018

Abstract

Background

Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms.

Study design

Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID.

Results

We identified 8 patients aged from 7 to 17 years diagnosed with AID; juvenile idiopathic arthritis (n = 3), systemic lupus erythematosus (n = 2), Sjögren’s syndrome (n = 1) and autoimmune hepatitis (n = 2). The diagnosis of AID was often delayed due to similarities of the symptoms with those of SCD. Patients treated with steroids experienced multiple vaso-occlusive crises and received prophylactic chronic blood transfusions when it was possible. Tolerance to other immunosuppressive and biological treatments, such as anti-TNF agents, was good. A remission of AID was achieved in 4 patients, without worsening the course of the SCD. One patient underwent a geno-identical hematopoietic stem cell transplantation that cured both diseases. Another one underwent a successful liver transplantation.

Conclusion

Coexistence of AID and SCD generates diagnostic and therapeutic challenges. Early diagnosis of AID is important to define the best treatment, which may include targeted biological therapy.

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Title: Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges
Authors: Valerie Li-Thiao-Te
Florence Uettwiller
Pierre Quartier
Florence Lacaille
Brigitte Bader-Meunier
Valentine Brousse
Mariane de Montalembert
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Pediatric Rheumatology / Issue 1/2018
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-017-0221-x

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges

Abstract

Background

Study design

Results

Conclusion

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Study design

Results

Conclusion

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