Clinical profile, laboratory parameters and management outcomes in an eight-year-old female with childhood lupus nephritis

Dr. Kruti Shah, Dr. Divya Dave
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Abstract

Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disorder characterized by widespread inflammation of connective tissues affecting the skin, joints, kidneys, heart, lungs and nervous system. SLE is most often diagnosed in women during the second to fourth decades of life, but 15-20% cases are found in childhood. Renal involvement occurs in the majority of childhood onset SLE (cSLE) patients and is often fatal and hence constitutes an important determinant of prognosis. Multidisciplinary care is necessary for children with lupus nephritis in terms of immunosuppressive regimes with a common goal of achieving and maintaining renal remission. Despite a low prevalence rate of cSLE, it manifests with fatal complications like lupus nephritis in majority of cases. Renal biopsy is gold standard for staging of lupus nephritis, thus, deciding the modality of management and prognostication of cSLE.
一名八岁女性儿童狼疮性肾炎患者的临床概况、实验室指标和治疗结果
系统性红斑狼疮(SLE)是一种慢性、多系统、自身免疫性疾病,其特点是结缔组织广泛发炎,影响皮肤、关节、肾脏、心脏、肺部和神经系统。系统性红斑狼疮最常在女性的第二至第四个十年中被诊断出来,但也有 15-20% 的病例在儿童时期就已发现。大多数儿童期发病的系统性红斑狼疮(cSLE)患者都会出现肾脏受累,而且往往是致命的,因此肾脏受累是决定预后的一个重要因素。狼疮肾炎患儿必须接受多学科治疗,采用免疫抑制方案,以达到并维持肾功能缓解为共同目标。尽管狼疮性肾炎的发病率较低,但大多数病例都会出现狼疮性肾炎等致命并发症。肾活检是狼疮肾炎分期的金标准,从而决定了狼疮的治疗方式和预后。
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