Systemic Lupus Erythematosus in Thai Children: Clinicopathologic Findings and Outcome in 82 Patients
Anirut Pattaragarn MD*, Achra Sumboonnanonda MD*, Paisal Parichatikanond MD**, Suroj Supavekin MD*, Vibul Suntornpoch MD*, Arun Vongjirad MD*
Affiliation : *Department of Pediatrics, **Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Thailand
Objectives : To define the patterns of clinicopathologic findings and to identify the risk factors for renal failure
and mortality of childhood-onset systemic lupus erythematosus (SLE) in Thailand.
Materials and Methods : The study is a retrospective analysis of clinical manifestations, laboratory data, and
pathologic findings, treatment modalities, and outcome of 82 patients with biopsy-proven lupus nephritis
(LN) with disease onset between 1 January 1987 and 31 December 1997. All children developed these first
manifestations at the age 13 years or under.
Results : Sixty-four (78%) patients were females and eighteen (22%) were males (ratio female/male = 3.5:1).
The patients were followed for a mean period of 53.6 months (range 1 -141). The mean age at disease onset was
9.2 years (range 2-12.6). Class-IV LN, observed in 40 (48.8%) patients, was the most frequent histopathology
on initial renal biopsy. Less frequent findings were class-II (30.5%), V (14.6%), I (3.7%) and III (2.4%) LN.
Based on the renal histopathology and clinical presentations, patients were treated with corticosteroids
alone or in combination with azathioprine or with intravenous cyclophosphamide (CYC). Methylpredniso-
lone pulses were given in patients with clinically more severe disease. Follow-up biopsies, performed in 12
patients, showed no change in 4 patients, and were progressive in 8 patients. On final clinical evaluation, 20
patients died, 65% died from serious infections, 15% from cardiopulmonary complications, and 10% from end
stage renal disease. As the whole group, survival rates were 89% and 74% at 12 and 60 months, respectively.
The 5-year patient survival in class-II, class-IV and class-V LN patients were 83%, 67% and 64%, respectively.
Within the group of class-IV LN, the 5-year survival in the patients treated with intravenous CYC was signifi-
cantly better than those receiving prednisolone with or without azathioprine. Five-year kidney survival rates
from the time of diagnosis to the endpoints of terminal renal failure were 94% for the whole group, and 100%,
96%, 91% in the class-V, class-II, and class-IV group, respectively. Initial presence of hypertension, hematuria,
renal insufficiency were independent factors significantly associated with lower patient survival probabilities.
There was no association of either patient and kidney survival with gender, age, cytopenia, and autoantibody
level.
Conclusions : Infectious complications were the most common cause of morbidity and mortality in our pediat-
ric patients with SLE. The immunosuppressive agents used to treat SLE seemed to be a major contribution to
the patient survival. With judicious use of corticosteroid, intravenous CYC in severe SLE showed superior
efficacy over oral prednisolone with or without azathioprine.
Keywords : Lupus nephritis, Hypertension, Infection, Survival, Cyclophosphamide, Methylprednisolone, Azathioprine
