Introduction:
SLE is a complex chronic autoimmune disease with multisystemic involvement characterized by the production of antibodies against self-antigens, immune complex formation, and immune dysregulation. Childhood-onset SLE (cSLE) is 10– 20% of SLE cases with an incidence of 0.3–0.9 per 100,000 and a prevalence of 3.3–24 per 100,000. Approximately half of children with SLE will experience kidney involvement at some point during their disease course and 80-90% of childhood lupus nephritis cases develop within the first year after SLE diagnosis. Between 10% and 30% of children with lupus nephritis progress to kidney failure within 15 years of diagnosis. In India, we have a very sparse number of literature regarding the demographic profile, the clinical presentation the outcomes of both SLE and Lupus nephritis in the pediatric population.So, this study aims to explore different clinical presentations and outcomes of lupus nephritis in the pediatric population for a better diagnosis and prognostication of the disease.
Methods:
Prospective Hospital-based observational study conducted at SCB MEDICAL COLLEGE and SVPPGIP, Cuttack, between April 2022 to June 2024 in Children up to 14 years of age with lupus nephritisNephropathy.After suspecting the patient to be a case of SLE, by using a proforma and after obtaining consent, demographic data (Age, sex, address) was taken along with a detailed anthropometric examination.Detailed clinical examination was done and relevant lab investigations were sent. For the diagnosis of SLE and Lupus Nephritis, CBC, Serum ANA, C3, Anti ds DNA antibody, DCT, Serum LDH, RFT, Urine routine and microscopy, Urinary spot protein/creatinine, Chest Xray, USG whole abdomen and pelvis were done. Renal biposy was done after taking consent and samples were sent for Light Microscopy and Immunoflurosence.Patients were treated with induction and continuation therapy based on UNIT protocol and were advised to follow up after 3 months. At 3-month follow-up, they were classified as No response. Partial response, and complete response as per KDIGO2021 guidelines.
Results:
In our study, Lupus Nephritis was more common in the 11-14(80%) years age group with a mean age of presentation of 11.76 years and more common in females (72%).In our study, 80% of the patient presented with lupus nephritis as their first presentation of SLE. The most common renal manifestation in our study at the time of presentation was edema(92%) followed by hypertension(44%) followed by hematuria(14%). In our study Hematological manifestation was more common (68%) followed by mucocutaneous manifestation (64%). We found that 64% of the population had albuminuria of value 3+ in urinary dipstick with a mean spot protein to creatinine ratio was 2.616. The mean eGFR at the time of presentation was 60.378 with the maximum number (44%) of patients having an eGFR value of <50 at the time of presentation. In our study all the patients were ANA positive with Anti dsDNA present in 68% and low C3 present in 96%.In our study, 88% of the patients received methylprednisolone as an induction agent 36% of the patients had a complete response,40% of the patients had a partial response and 8% of the patients had no response while mortality was 12%. We found higher age groups(11-14years), male gender, mucocutaneous manifestation at the time of presentation, with high urinary spot protein to creatinine ratio, low serum albumin, and baseline eGFR are associated with mortality. In this study, 57.8% of patients had class IV lupus nephritis in their renal biopsy finding followed by 15.7% of patients who had both class IV and V lupus nephritis,10.5% of patients had class III and 10.5% of patients had class V lupus nephritis, 5% patient had both class III and IV histological pattern. In our study, complete response to treatment at 3 months follow-up was seen more in higher age groups and male patients The patients having higher serum albumin and higher eGFR at presentation had more complete response.
Conclusions:
· We have Lupus nephritis more in 11-14 years of age and has a more female preponderance with 3/4th of the patients presented with Lupus nephritis as their first presentation. Oedema was the most common form of renal manifestation followed by hypertension. Hematological manifestation is the most common extrarenal manifestation found in 2/3rd of the patients followed by Mucocutaneous manifestation. In Lab parameters 3/4th have urinary spot protein to creatinine ratio of 2 or more with almost half of the patients having eGFR of <50. In 3 months Follow-up 1/3rd have complete response In our study a greater number of complete responses to therapy at 3 months follow-up is present in the male gender, older age, and higher serum albumin, higher eGFR at presentation.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.