Systemic lupus erythematosus in childhood--a review of 11 patients at a single center in eastern Nepal.
We retrospectively evaluated the clinico-laboratory features of 11 children and adolescents with Systemic Lupus Erythematosus between the period of 2001 and 2006. All of them (100.0%) had renal involvement at the first visit in the hospital. Female to male ratio was 10:1. Skin and or mucosal involvement (90.9%), periorbital puffiness and or pedal edema (81.8%), fever (72.7%), hypertension (72.7%), and reticuloendothelial involvement (72.7%), were the commonest presentations. All patients had anemia (8.6 +/- 1.5 gm/dl), raised ESR (46.7 +/- 9.4 mm in first hour), proteinuria, and in disease activity as evident by raised ESR and positive anti-dsDNA antibody at the first visit. The mean duration of disease was 7.6 months and the average duration of disease activity was 63.18 days. Renal biopsy was performed in 8 patients: class IV lupus nephritis in 4 patients (50.0%), class II in 2 patients (25.0%), class III and V in patient (12.5%) each. Nephrotic range proteinuria and hypertension was observed in all patients of class IV and V of lupus nephritis. Class II and III lupus nephritis patients' were normotensive and had non-nephrotic range proteinuria. Three out of 11 patients (27.2%) expired. The commonest primary determinant of mortality was uncontrolled disease activity in 2 patients (66.6%). The third one had infection and developed disseminated intravascular coagulation. The mean duration of disease activity in patients who died (mean 30 days) was statistically lower than the survival group (75.6 days) (p < 0.01). Renal involvement during first visit and mortality could be attributed by late referrals and diagnosis at hospital.
Budhathoki
Sunil
S
Department of Pediatrics and Adolescent Medicine, BP Koirala Institute of Health Sciences, Dharan, Nepal. bb2sunil@yahoo.com
Shrestha
Pramod
P
Bhatta
Nisha
N
Singh
Rupa
R
Kalakheti
Balkrishna
B
Jha
Dhirendra Kumar
DK
Poudel
Prakash
P
Majhi
Shankar
S
Sinha
Arvind
A
D000293:Adolescent; D002648:Child; D002669:Child Welfare; D015897:Comorbidity; D005260:Female; D006801:Humans; D006973:Hypertension; D007166:Immunosuppressive Agents; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D008181:Lupus Nephritis; D008297:Male; D009390:Nepal / Q000453:epidemiology; D011507:Proteinuria; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors
D007166:Immunosuppressive Agents