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

Abstract EN

Objective: 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.

Methods: A prospective follow up study of 32 SLE patients diagnosed and treated in the Children's Hospital and in the Rheumatology & 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.

Results: 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.

Conclusions: 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.