Poddar Sanjukta, Dasgupta Deblina, Pradhan Subal, Perungo Sangeetha, Janarthanan Mahesh, Vala Kinnari, Pais Priya, Uthup Susan, Singhal Jyoti, Guha Suparna, Raut Sumantra, Akhtar Shakil, Bathia Jigna, Balan Suma, Pal Priyankar, Sinha Rajiv
Division of Pediatric Nephrology, Institute of Child Health, Kolkata, India.
Division of Pediatric Nephrology, Sardar Vallabhbhai Patel Post Graduate Institute of Pediatrics, Cuttack, India.
Clin Rheumatol. 2025 Jul 16. doi: 10.1007/s10067-025-07576-9.
Limited prospective data exist on pediatric LN (pLN) from low- and middle-income countries (LMIC), where ethnicity, socioeconomic factors, and healthcare access are likely to differ from high-income countries.
The Indian Pediatric Lupus Nephritis registry has been running since 2020 across multiple centers in India. Children (≤ 18 years) diagnosed with lupus (as per 2012 SLICC criteria), presenting with nephritis, and confirmed by kidney biopsy are being prospectively enrolled. Clinical data, laboratory investigations, kidney biopsy results, and treatment responses have been documented prospectively. The current report documents their initial presentation.
A total of 154 children (75% female, median age 12 years-IQR 10-14 years) with biopsy-proven LN were enrolled by July 2024. Nearly two-thirds had LN at SLE diagnosis, and the rest developed within a maximum of 5 years of initial presentation. Common manifestations at presentation included edema (75%), hypertension (54%), and proteinuria (98%), of which 68% presented with nephrotic-range proteinuria. Acute kidney injury (AKI) was observed in 43%, with 20% in stage 3. Ninety-four percent of our cohort had low complements (C3, C4, or both), and 96% were ANA-positive. Class IV LN was the most common (45%) histopathological type and had significantly lower estimated glomerular filtration rate in comparison to Class V LN.
Kidneys are often involved in the initial presentation of childhood lupus, and the majority have proliferative nephropathy leading to AKI, hypertension, and significant proteinuria. Children enrolled in the registry are under active follow-up to assess the renal responses which will help optimize the management of pLN in LMICs. Key Points •It is a well-known fact that kidney involvement is more common in pediatric lupus and is among one of the most important long-term prognostic factors. •There is scarcity of data on pediatric lupus nephritis (pLN) particularly from low- and middle-income countries (LMIC), and even among them, the majority of the studies are retrospective and limited by a small cohort size. •Through this prospective registry from a LMIC, we demonstrated that 2/3rd of children with lupus have kidney involvement at presentation and almost all (90%) develop LN within 2 years of the diagnosis of lupus. •Acute kidney injury (AKI) is known to increase mortality/morbidity risks independently. Many of the previous studies have under-reported AKI in pLN, probably because the data was collected retrospectively. On the other hand, we found AKI to be very common and to be present in about half of the cases at presentation.
来自低收入和中等收入国家（LMIC）的儿童狼疮性肾炎（pLN）的前瞻性数据有限，这些国家的种族、社会经济因素和医疗保健可及性可能与高收入国家不同。
印度儿童狼疮性肾炎登记处自2020年起在印度多个中心开展工作。对诊断为狼疮（根据2012年SLICC标准）、患有肾炎且经肾活检确诊的儿童（≤18岁）进行前瞻性登记。已前瞻性记录临床数据、实验室检查、肾活检结果和治疗反应。本报告记录了他们的初始表现。
截至2024年7月，共纳入154例经活检证实为LN的儿童（75%为女性，中位年龄12岁，四分位间距10 - 14岁）。近三分之二的儿童在SLE诊断时即患有LN，其余儿童在首次出现症状后的最多5年内发病。发病时的常见表现包括水肿（75%）、高血压（54%）和蛋白尿（98%），其中68%表现为肾病范围蛋白尿。43%观察到急性肾损伤（AKI），其中20%为3期。我们队列中的94%补体水平低（C3、C4或两者均低），96%抗核抗体阳性。IV型LN是最常见的组织病理学类型（45%），与V型LN相比，其估计肾小球滤过率显著更低。
肾脏常参与儿童狼疮的初始表现，大多数患者患有增殖性肾病，导致AKI、高血压和大量蛋白尿。登记处登记的儿童正在接受积极随访，以评估肾脏反应，这将有助于优化LMICs中pLN的管理。要点 •肾脏受累在儿童狼疮中更为常见，是最重要的长期预后因素之一，这是一个众所周知的事实。 •关于儿童狼疮性肾炎（pLN）的数据稀缺，特别是来自低收入和中等收入国家（LMIC）的数据，即使在这些国家中，大多数研究也是回顾性的，且受队列规模小的限制。 •通过这个来自LMIC的前瞻性登记处，我们证明了2/3的狼疮患儿在发病时肾脏受累，几乎所有患儿（90%）在狼疮诊断后的2年内发展为LN。 •已知急性肾损伤（AKI）会独立增加死亡率/发病率风险。许多先前的研究在pLN中对AKI的报告不足，可能是因为数据是回顾性收集的。另一方面，我们发现AKI非常常见，在发病时约一半的病例中存在。
