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3859 Antinuclear and Specific Autoantibodies Predict Autoimmune Disease in Sickle Cell Patients: A Longitudinal Cohort Study

Program: Oral and Poster Abstracts
Session: 113. Sickle Cell Disease, Sickle Cell Trait and Other Hemoglobinopathies, Excluding Thalassemias: Basic and Translational: Poster III
Hematology Disease Topics & Pathways:
adult, autoimmune disorders, Sickle Cell Disease, Clinical Practice (Health Services and Quality), Hemoglobinopathies, Immune Disorders, Diseases, Study Population, Human
Monday, December 11, 2023, 6:00 PM-8:00 PM

Frederic Truffinet1*, Caroline Compain2*, Maria Elena Manea2*, Anoosha Habibi3, Elena Fois2*, Etienne Audureau4,5*, Sophie Hue6*, Marc Michel7* and Pablo Bartolucci8*

1CHU Henri Mondor. UPEC. AP-HP, Paris 12, France
2CHU Henri Mondor. UPEC. AP-HP, Creteil, France
3sickle cell referral center, APHP, Hôpital Henri Mondor, Creteil, France, Joinville le pontCréteil, France
4Henri Mondor APHP, Creteil, France
5Unité de Recherche Clinique, Henri-Mondor University Hospital- UPEC, AP-HP, Creteil, France
6CHU Henri Mondor. UPEC. AP-HP, creteil, France
7Internal medicine department, Henri Mondor Hospital, APHP, Créteil, France
8CHU Henri Mondor. UPEC. AP-HP, Créteil, France

Introduction: Sickle cell disease (SCD), the most frequent genetic disorder, can be associated with systemic autoimmune diseases (ADs). High levels of antinuclear antibodies (ANA) are commonly described in SCD, as alloimmunization after blood transfusion. However, the specificity and the prognostic value of ANA have not been fully determined.

We aimed to evaluate the association of ANA with AD development during long-term follow-up in SCD patients. Our secondary goal was to assess mortality and serious SCD-related events in this population.

Patients and methods: We conducted a single-center longitudinal cohort study on 66 adult SCD patients with ANA titers ≥1/320 at baseline followed over a 13 year-period. Patients known to have an AD (n=9) were excluded from the risk analysis.

Results: During follow-up, 13 out of 57 patients (23%) developed AD. Patients with ANA titers ≥1/1280 had a 3-fold increased risk of developing AD (RR 3.4[1.4;8.6]) and a 9-fold increased risk in the presence of specific autoantibodies (anti-DNA, anti-ENA, RF or ACPA) (RR 9.0[3.7;23]) with a positive (PPV) and negative predictive value (NPV) of 90%. When these two immunological features were associated, specificity and PPV reached 100%, with a NPV of 84%. There was no significant difference in the occurrence of serious SCD-related events or mortality rate according to AD.

Conclusions: Sickle cell patients with high level of antinuclear antibodies and specific autoantibodies are at high risk of developing AD. The occurrence of AD does not seem to have a significant impact on the course and severity of SCD.

Disclosures: Michel: UCB: Honoraria; Sobi: Consultancy; Novartis: Consultancy; Alexion: Consultancy; argenx: Honoraria; Sanofi: Consultancy.

*signifies non-member of ASH