AB1071 AUTO-IMMUNE AND INFLAMMATORY DISEASES IN CHILDREN WITH SICKLE CELL DISEASE: DIAGNOSTIC AND THERAPEUTIC ISSUES

Abstract

Background Coexistent auto-immune and inflammatory diseases (AIID) and sickle cell disease (SCD) have been recently described in adults and children, however its frequency and physiopathology remain unclear (1–6). Objectives The aim of this study is the analysis of clinical and biological characteristics at AIID diagnosis and the evolution under treatment in children with SCD Methods Between May 1991 and March 2018, 35 of 3,800 SCD children diagnosed with AID in seven hospitals in Paris and suburb were analyzed in a retrospective survey. Results Thirty-five patients reported 44 AIID: auto-immune liver disease (AILD, n=13), inflammatory bowel disease (IBD, n=7), juvenile idiopathic arthritis (JIA, n=6), systemic lupus erythematosus (n=5), autoimmune hemolytic anemia (n=3), Sjogren’s syndrome (n=2), histiocytic necrotizing lymphadenitis (n=2), vasculitis (n=2), myasthenia gravis (n=2), sarcoidosis (n=2), inflammatory uveitis (n=1), sclerodermia/juvenile dermatomyositis (n=1). Median age at diagnosis was 10 [2 – 18] years. The mean delay between first symptom and diagnosis was 15.5 (± 29) months. The time of diagnostic was significantly longer for patients with JIA compared to other AID (63 versus 10 days, p=0.004). Sixteen patients (45.7%) had hypergammaglobulinemia > 20 g/L at diagnosis. AILD had a hypergammaglobulinemia at the time of diagnosis (30.0g/L), with a statically significant decrease at the end of follow-up (18.2g/L, p=0,0048). Among 21 patients (60%) treated with systemic steroids, it triggered vaso-occlusive crisis in 14 (66.7%), one acute chest syndrome, one transient ischemic attack. Thirteen of 35 patients (37.1%) were managed with biotherapy for AIID, well tolerated. Three patients (8.6%) underwent stem cell transplantation, one died of a cortico-resistant and multipolar graft versus host reaction, two were cured of both AIID and SCD. Nine severe infections were reported, four under steroids, five under biotherapy. Conclusion Diagnosis and therapeutic care of coexistent auto-immune and inflammatory diseases are difficult and challenging in children with SCD. Annual monitoring of inflammatory markers could be recommended to detect AIID earlier and prevent diagnostic delay in case of high ascension in SCD patients.