PROFILE OF CHILDREN WITH ANA NEGATIVE SLE IN A TERTIARY CARE HOSPITAL INSOUTH INDIA

Abstract

Introduction - ANA negative systemic
lupus erythematosus (SLE) is a relatively
uncommonentity in childhood but when it
occurs is usually associated with significant
multi-system involvementespecially
renal. Aim - To describe the clinical profile,
treatment and outcome of children
with ANAnegative SLE.Methodology -
160 children with SLE were being followed
up in Pediatric Rheumatologyand
Pediatric Nephrology clinic. Out of this,
16 children were diagnosed to have ANA
negative SLEwas included in the study.
Their clinical, laboratory, treatment and
outcome data were collected fromtheir
medical records and entered in the proforma
and analyzed.Results - Total of 16
children werefollowed up with ANA negative
SLE. 13 (82) were girls and 3 (18)
were boys. Mean age at diagnosiswas
10.4 years (range 7 to 14 years). ACR
classification criteria for SLE were fulfilled
by 5 (31)children. 14 (88) had renal
involvement. All underwent renal biopsy.
Among these,
WHO Class IVlupus nephritis (81) was the
most common histopathological finding. All
the children had received steroids for the
treatment of ANA negative SLE. Cyclophosphamide
was used in 3 (19), mycophenolate
mofetil was used in 4 (25) and
hydroxychloroquine was used in 9 (56) children.
8 (50) of the children had attained
complete remission, 3 (19) had attained
partial remission and 5 (31) had not attained
remission at the end of the follow
up. None of the children had become ANA
positive during the follow up period. 7(44)
patients had flares during the follow up and
the maximum number of flares in a single
patient was 3 episodes.Conclusion - ANA
negative SLE is a rare entity in childhood.
We found a prevalence of 10 in our SLE
children. Renal involvement was predominant
manifestation in our series of children.
Treatment with steroids showed adequate
response in these children.
WHO Class IVlupus nephritis (81) was the
most common histopathological