IgA Nephropathy and Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE): A Case Report

Juliana Damas, Catarina Marouço, Rui Barata, Tiago Assis Pereira, Nuno Moreira Fonseca, David Navarro, Mário Góis, Helena Viana, João Sousa, Heidi Gruner, Cristina Jorge
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Abstract

IgA nephropathy (IgAN) remains the most frequent glomerular disease worldwide, with a broad spectrum of clinical and histological presentations. It has been associated with many secondary causes. The remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is an autoimmune disorder characterized by swelling in the extremities and negative autoimmune serological tests. The primary treatment for this condition involves the use of immunosuppressive therapy. Although several triggers have been identified, the exact cause of this condition is still unknown. We report a case of a 53-year-old man who presented with acute exacerbation of chronic kidney disease, whose etiological study revealed advanced IgAN, associated with pleural and pericardial effusions. Even with volume optimization and dialysis intensification, the pericardial effusion worsened, despite the resolving pleural effusion. Upper arm arthralgias were developed afterward. An extensive study ruled out other causes and the hypothesis of RS3PE syndrome was considered. Glucocorticoid (GC) therapy was instituted for 6 months with clinical improvement and no recurrence at 2 years follow-up. The complexity of this case shows the importance of considering a wider diagnosis for the complaints of arthralgias and volume overload, reinforcing the importance of clinical awareness for other concurrent conditions, whose treatment may be lifesaving.

Abstract Image

IgA 肾病和点状水肿的缓解性 Seronegative 对称性滑膜炎 (RS3PE):病例报告
IgA 肾病(IgAN)是全球最常见的肾小球疾病,临床和组织学表现多种多样。它与许多继发性病因有关。缓解性血清阴性对称性滑膜炎伴点状水肿(RS3PE)综合征是一种自身免疫性疾病,其特征是四肢肿胀和自身免疫血清学检测阴性。这种疾病的主要治疗方法是使用免疫抑制剂。虽然已经发现了几种诱发因素,但这种疾病的确切病因仍不清楚。我们报告了一例 53 岁男性慢性肾脏病急性加重的病例,其病因学研究显示其为晚期 IgAN,伴有胸腔和心包积液。尽管胸腔积液已经消退,但即使优化了血容量并加强了透析,心包积液仍在恶化。之后又出现了上臂关节痛。一项广泛的研究排除了其他病因,并考虑了 RS3PE 综合征的假设。患者接受了为期 6 个月的糖皮质激素(GC)治疗,临床症状有所改善,随访 2 年未再复发。该病例的复杂性表明,对于关节痛和容量负荷过重的主诉,考虑更广泛的诊断非常重要,这也加强了临床上对其他并发症的认识,这些并发症的治疗可能会挽救患者的生命。
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