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Table 2 Treatment and last follow-up in the 8 patients

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

Patient 1 2 3 4 5 6 7 8
Treatment related to SCD before the diagnosis of AID None Hydroxyurea started at 13 y Hydroxyurea started at 7 y None None Hydroxyurea started at 9 y Hydroxyurea started at 14 y Chronic exchange transfusion programme started at 3 y splenectomy
AID SLE JIA JIA JIA AIH Sjogrën’s syndrome SLE AIH
Treatment of AID First line NSAIDs Hydroxychloroquine NSAIDs NSAIDs for 3 years Etanercept (No methotrexate because of chronic hepatitis B) Steroids Chronic blood transfusions then Hydroxyureaa NSAIDs (from age 11 to 13 y) Steroids Chronic exchange transfusionsa Hydroxychloroquine Aspirin Steroids 6-mercaptopurine Chronic exchange transfusionsa
2nd line Genoidentical hematopoietic stem cell transplantation at 18 y Etanercept Steroids Intra-articular steroids   Azathioprine NSAIDs Methotrexate Hydroxychloroquine Aspirin Mycophenolate mofetil Steroids Azathioprine Steroids Chronic exchange transfusionsa
3rd line and more    Etanercept for 5 years; (Failure of abatacept, infliximab, adalimumab)
Current treatment: Tolicizumab + methotrexate
   Mycophenolate mofetil   Liver transplantation at 10 y Ciclosporin and steroids
Last follow-up CR PR Persistant activity CR CR CR PR Normal liver function Controlled blood pressure
  1. AID autoimmune disease, SLE systemic lupus erythematosus, JIA juvenile idiopathic arthritis, AIH autoimmune hepatitis, NSAIDs non steroidal antiinflammatory drugs, CR complete remission, PR partial remission
  2. Patient 2: relapse after 3 years, then lost in transition to adult care, patient 7: persistent periventricular hyperintensity on cerebral MRI
  3. ain order to prevent occurrence of vasoocclusive crises