Lupus Nephritis in Children: Prognostic Significance of Clinicopathological Findings

Abstract

Background: We aimed to review our experience with childhood lupus nephritis (LN) in respect to the analysis of the clinical and histopathological presentation of LN and prognostic factors affecting the kidney and patient outcomes. Method: Forty-three children (39 girls, 4 boys) with biopsy-proven LN were included in the study. The mean age of the children was 12.0 ± 2.8 years. Based on the renal histopathology and clinical presentation, patients were treated with oral prednisone, intravenous pulses of methylprednisolone or intravenous cyclophosphamide. The final clinical status was classified as follows: (1) renal and extrarenal remission; (2) clinically active renal disease, or (3) adverse outcome, i.e., end-stage renal failure (ESRF) or death. Results: The mean duration of follow-up was 7.2 ± 2.8 years (1 month to 14.2 years). All 43 children had hematuria and 53.5% had proteinuria at admission. Fourteen children were in nephrotic status at the onset of disease. Class IV (diffuse proliferative) nephritis was observed in 29 patients as the most frequent histopathology (67.4%). The patients with class IV nephritis had a tendency to develop nephrotic syndrome, heavy proteinuria, increased Cr levels and persistent hypertension at initial evaluation. Thirty-two of 43 children (74.4%) were in renal remission at the last visit. Five-year kidney and patient survival rates from the time of diagnosis to the endpoints of ESRF or death were 83.7 and 90.7% respectively in the whole group while it was 75.9 and 86.2% respectively in the class IV group. Adverse outcome was significantly associated with the persistent hypertension, anemia, high serum Cr level, heavy proteinuria, nephrotic syndrome and class IV nephritis at presentation. Conclusion: We can conclude that the prognosis of LN in children is primarily dependent on the histopathological lesions. Severity of the clinical renal disease at admission and presence of persistent hypertension are the main poor prognostic factors rather than age, gender, low C3 and C4 levels, ANA positivity and the treatment modalities in Turkish children.

Juvenile Lupus Erythematosus: Fourteen Years of Experience

“…However, western studies reported a higher percentage of class III and IV LN than did our study. [20,29,30] The high proportion of patients with class V LN (35%) and low proportion of class III LN (5%) in our study in contrast to the 2-5% and 15-25% in other series respectively is not explainable. In the past, renal disease was the major cause of mortality in patients with JSLE, but with earlier diagnosis and newer methods of treatment, the outlook for patients with lupus nephritis has vastly improved.…”

“…In 90% of SLE patients, LN appeared within two years of the onset of the disease. An earlier Turkish study of children with LN [29] reported class IV (64%) of LN as the most frequent histopathological pattern. Bakr [14] stated that the biopsy distribution of the pathological classes of LN in Egypt was as follows: class I 4.9%, class II 22%, class III 36.7%, and class IV 36.7%.…”

B lymphocyte depletion therapy in children with refractory systemic lupus erythematosus

“…Although in the last decade, there has been a significant improvement in the morbidity and mortality of treated children with SLE, there remains a cohort of children who do not respond to conventional therapy with corticosteroids and cyclophosphamide and other immunosuppressive agents (28)(29)(30)(31)(32)(33). In addition, the side effects of cytotoxic immunotherapy (mainly relating to cyclophosphamide) are not inconsequential in children.…”

Європейські Доказові Рекомендації По Діагностиці Та Лікуванню Люпус-Нефриту У Дітей: Ініціатива SHARE

“…It is acknowledged that renal biopsy is not always possible, for instance due to the patient's critical clinical condition or due to lack of resources to safely perform the procedure. Nephrotic syndrome, hypertension and impaired renal function are all correlated with class III/IV LN (26)(27)(28). In cases when a renal biopsy cannot be performed, these symptoms should be considered as reflecting class III/IV LN and treated likewise.…”