Pattern and prognosis of haematological and non-haematological presentatations of juvenile SLE: five years longitudinal study

ABSTRACT

To assess the pattern of hematological disorders in juvenile systemic lupus erythematosus [SLE] and its impact on prognosis and survival. The prevalence of SLE in a Pediatric Hematology Unit was also assessed through a five years longitudinal study. A prospective follow up study of 32 SLE patients diagnosed and treated in the Children's Hospital and in the Rheumatology and Rehabilitation Department, Ain Shams University Hospitals, during a period of 5 years was done. Follow up of patients with acquired hematological diseases [n=235] diagnosed in a Pediatric Hematology Unit, in the same period was done to assess the prevalence of SLE in that population. Among the 32 SLE patients, 30 were females and two were males, the mean age at diagnosis was 13.3 +/- 3.5 years. Fifteen patients had an initial hematological presentation. Anemia and thrombocytopenia were the commonest hematological presentation [68.8% and 40.6% respectively]. The main non-hematological presenting features included nephropathy [71.9%], cutaneous manifestations [50%] and arthritis [65.6%]. At presentation, ANA antibodies and anti DNA were positive in 67.7% and 67.8% respectively, reaching values of 90.4% and 86.4% respectively during follow up. As regard fate, 25.2% were in remission, 15.6% lost follow up and 59.4% had progressive disease. Ten children out of the studied 235 patients with acquired hematological diseases [4.3%] developed four or more American College of Rheumatology criteria for the diagnosis of SLE during five years follow up. They were 5 out of 186 children with idiopathic thrombocytopenic purpura [2.7%], 2 out of 12 with autoimmune hemolytic anemia [16.7%], 2 out of 3 with Evan's Syndrome [66.6%] and 1 out of 5 with pure red cell aplasia [20.0%]. None of hypoplastic anemia [n=29] developed SLE. Hematological disorders are common features in childhood SLE with an impact on the prognosis. SLE constitutes the underlying pathology in 4.3% of children with acquired hematological disorders