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

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

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 Hydroxyurea^a	NSAIDs (from age 11 to 13 y)	Steroids Chronic exchange transfusions^a Hydroxychloroquine Aspirin	Steroids 6-mercaptopurine Chronic exchange transfusions^a
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 transfusions^a
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

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
Patient 2: relapse after 3 years, then lost in transition to adult care, patient 7: persistent periventricular hyperintensity on cerebral MRI
^ain order to prevent occurrence of vasoocclusive crises

ISSN: 1546-0096