Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose
Studies on lupus nephritis in young children below 12 years of age from developing countries are limited. This study looks at long-term outcome in North-Indian children.
Methods
This was a single-center retrospective study from a University referral hospital that provides subsidized treatment. Children seen in the center during the last 25 years were included if they had been diagnosed with systemic lupus erythematosus (as per the ACR 1997 criteria) and lupus nephritis (proteinuria >500mg/day or hematuria (>5RBC/HPF) or any cellular cast) before their 12thbirthday. Initial presentation, laboratory data and treatment received was obtained from the file. Renal biopsy data was classified as per the WHO classification. The primary endpoint was survival with functioning kidneys (absence of death or ESRD). Patients were followed up till January 2012. Kaplan-Meier analysis was used for survival and log-rank test was used to compare different classes.
Definitions used:Chronic kidney disease = elevated serum creatinine (>1.5mg/dl) for atleast 3 months. End-stage renal disease = need for renal replacement therapy > 3 months.
Results
This study included 72 children (F: M 3.2:1). The mean± SD age at onset of lupus was 9.3±2.4 years and the duration of disease before presentation was 9.2±12.6 months. Majority of the children (76%) had nephritis at presentation. Renal biopsy was done in 54 children. The histological class was class II in 9, class III in 1, class IV in 35 and class V in 7. Biopsy was not done in the remaining 18 patients due to poor general condition in 7, ongoing anticoagulation in 5, thrombocytopenia, uncontrolled hypertension and refusal by caregivers in 2 children each. The most common induction treatment was monthy pulses of cyclophosphamide (6-12 pulses) followed by maintenance with azathioprine or quarterly pulses of cyclophosphamide. At 1 year of follow up, 11 (15%) children had died (all in 1st admission), 11 (15%) were lost to follow up, 36 (50%) were in complete remission and 5 each (6.9%) were in partial remission and active disease. Data for 4 children at 1 year could not be retrieved. Another 11 (15%) children died after the 1st year. The common causes of mortality was infection, disease activity and renal failure. (Table 1). The mean duration of follow up was 4 ± 4.4 years (0.2-20 years) with a total follow up of 287 patient years. Survival with functioning kidneys was seen in 71% at 3 years, 68% at 5 years and 60% at 10 years. There was no difference in survival among various histological classes.
Conclusion We found a majority of young children presented with lupus nephritis. There was a high mortality at initial presentation. The long term outcome is still much lower as compared to those from the developed nations. Majority of deaths occur in the initial presentation due to infections, severe disease activity and renal failure.
Table 1: Causes of death
|
|
Total |
Initial visit |
Later |
|
Septicemia |
7 |
7 |
0 |
|
Severe disease activity |
3 |
3 |
– |
|
End stage renal disease |
4 |
– |
4 |
|
Disseminated kochs with shunt malfunction |
2 |
– |
2 |
|
Pulmonary thromboembolism |
2 |
– |
2 |
|
Hypertensive Intracranial bleed |
1 |
– |
1 |
|
Myocarditis with left ventricular failure |
1 |
1 |
– |
|
Unknown cause (died at home) |
2 |
– |
2 |
|
Total |
22(30) |
11 |
11 |
Disclosure:
A. Gupta,
None;
B. Abujam,
None;
D. Suri,
None;
A. Rawat,
None;
S. Singh,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcome-of-lupus-nephritis-in-children-less-than-12-years-old-from-north-india/