Unusual presentations in systemic lupus erythematosus with concurrent IgA nephropathy lesion: a rare case report from Eastern Nepal

Popular Pokhrel, Nitesh Neupane, Nitesh Kumar Karna, Gopal Pokhrel, Niraj Chapagain, Abhas Niraula, Ajay Kumar Yadav
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Abstract

Systemic lupus erythematous (SLE) is an autoimmune disease involving multiple organs. While lupus nephritis (LN) is seen in SLE, concurrent IgA nephropathy lesion is rare. Uncommon manifestations like cutaneous ulcers and orbital involvement present diagnostic challenges and this case from Nepal emphasizes careful diagnostic approach in such cases. A 42-year-old female presented with bilateral lower limb swelling, gum bleeding, and epistaxis. Initial evaluation revealed pancytopenia and suspected renal involvement. Renal biopsy showed IgA nephropathy lesions, but clinical and laboratory findings favored lupus nephritis. Treatment with immunosuppressive agents was initiated. Despite therapy, the patient developed cutaneous ulcers and orbital cellulitis. Decreasing anti-ds DNA levels were noted during course of treatment. The diagnosis of lupus nephritis in the presence of IgA nephropathy lesions emphasizes the complexity of SLE diagnosis. Treatment with immunosuppressive agents targeting the underlying autoimmune process, yet development of cutaneous ulcers and orbital cellulitis highlights the importance of timely intervention in managing SLE complications. In resource-limited settings, clinicians should initiate interventions based on clinical and lab findings while awaiting detailed biopsy results. This case highlights diagnostic challenges in SLE and emphasizes the necessity for careful monitoring and timely intervention in managing complications. The interplay between SLE and IgA Nephropathy (IgAN) suggests that SLE may trigger or exacerbate it, complicating disease management. Further exploration is needed to enhance understanding and management of complex autoimmune disorders like SLE.
并发 IgA 肾病病变的系统性红斑狼疮的异常表现:尼泊尔东部的罕见病例报告
系统性红斑狼疮(SLE)是一种累及多个器官的自身免疫性疾病。狼疮性肾炎(LN)可见于系统性红斑狼疮,但并发 IgA 肾病的病变却很少见。皮肤溃疡和眼眶受累等不常见的表现给诊断带来了挑战,这例来自尼泊尔的病例强调了此类病例的谨慎诊断方法。 一名 42 岁的女性患者出现双下肢肿胀、牙龈出血和鼻衄。初步评估显示患者为全血细胞减少,疑似肾脏受累。肾活检显示 IgA 肾病病变,但临床和实验室检查结果倾向于狼疮性肾炎。患者开始接受免疫抑制剂治疗。尽管接受了治疗,患者还是出现了皮肤溃疡和眼眶蜂窝织炎。在治疗过程中发现抗ds DNA水平下降。 在出现 IgA 肾病病变的情况下诊断出狼疮肾炎,这凸显了系统性红斑狼疮诊断的复杂性。针对潜在的自身免疫过程使用免疫抑制剂进行治疗,但还是出现了皮肤溃疡和眼眶蜂窝组织炎,这凸显了及时干预对控制系统性红斑狼疮并发症的重要性。在资源有限的情况下,临床医生应根据临床和实验室检查结果启动干预措施,同时等待详细的活检结果。 该病例凸显了系统性红斑狼疮在诊断方面的挑战,强调了仔细监测和及时干预对控制并发症的必要性。系统性红斑狼疮和 IgA 肾病(IgAN)之间的相互作用表明,系统性红斑狼疮可能会诱发或加重 IgA 肾病,从而使疾病的治疗变得更加复杂。为了加强对系统性红斑狼疮等复杂的自身免疫性疾病的了解和管理,还需要进一步的探索。
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