Autoimmunity

Objective: To determine the clinical and immunological characteristics and short-term outcome of children with systemic lupus erythematosus (cSLE) presented at a tertiary care center in Karachi, Pakistan.

 

Design: A descriptive observational study conducted at the Paediatric Rheumatology Clinic of Aga Khan University Hospital(AKUH), Karachi, from January 2011 to April 2015.

 

Methodology: Data of children <16 years of age, admitted to the Paediatric ward, diagnosed with cSLE, was studied.

 

Results: 32 children satisfying the criteria of American College of Rheumatology (ACR) for cSLE were enrolled. A female predominance was observed, with 87.5% of the patients being female. Mean age at symptom onset was 10.5+2.7 years and 8.8+2.1 years in females and males respectively. Mean age at diagnosis was 11.3+2.8 years in females and 9.4+1.9 years in males. Fever was the most common non-specifi c symptom found in 84% patients. 69% children were found to be anemic and 56% had signs of arthritis at presentation. Renal involvement was observed in 47% patients. Th e most common immunological markers were found to be serum Anti-neutrophil antibodies (ANA), positive in 88% patients, followed by Anti-double-stranded DNA antibodies (anti ds-DNA), raised in 81% cases. Overall response rate to therapy was 50% in 20 children who were followed for four years.

 

Conclusion: We found that cSLE encompasses a wide variety of manifestations with a female preponderance. Fever and Arthralgia are the most frequent clinical fi ndings. Hemolytic anemia is the most common laboratory abnormality, with ANA and Anti ds-DNA positivity in a majority of patients.