Khorsan Reza, Hanna Ramy M, Ameen Khalid, Arman Farid, Nobakht Niloofar, Hasnain Huma, Hyunh Ashley, Rastogi Anjay
Department of Medicine, Division of Nephrology, David Geffen School of Medicine, Los Angeles, California, USA.
Saudi J Kidney Dis Transpl. 2019 Mar-Apr;30(2):531-536. doi: 10.4103/1319-2442.256863.
Membranous nephropathy (MN), an autoimmune glomerulonephritis which can occur in primary and secondary forms, is one of the most common inflammatory glomerulopathies in elderly patients. The pathophysiology of the primary form is generally due to circulating immunoglobulin (IgG4) antibodies which often target phospholipase A2 receptors (anti-PLA2R) and Thrombospondin Type 1 Domain containing 7A (anti THSD7A). IgA nephropathy is one of the most common autoimmune glomerular diseases in the world and presents with a spectrum of disease ranging from asymptomatic mild hematuria and proteinuria to rapidly progressive crescentic glomerulonephritis. We present a rare case of concomitant IgA and primary MN in a single patient treated successfully with renin-angiotensin-aldosterone blockade, corticosteroids, and calcineurin inhibitors. The peak proteinuria was near 7.5-8 g protein/g creatinine by various measures. Serum creatinine remained normal, and anti-PLA2R was detectable and decreased with successful treatment. Clinicians should be aware of the possibility of two glomerular disorders in patients with glomerulonephritis and atypical presentations for any single disorder.
膜性肾病(MN)是一种自身免疫性肾小球肾炎,可分为原发性和继发性,是老年患者中最常见的炎症性肾小球病之一。原发性膜性肾病的病理生理学通常归因于循环免疫球蛋白(IgG4)抗体,这些抗体通常靶向磷脂酶A2受体(抗PLA2R)和含血小板反应蛋白1型结构域7A(抗THSD7A)。IgA肾病是世界上最常见的自身免疫性肾小球疾病之一,其疾病谱范围从无症状性轻度血尿和蛋白尿到快速进展的新月体性肾小球肾炎。我们报告了一例罕见的病例,一名患者同时患有IgA肾病和原发性膜性肾病,通过肾素-血管紧张素-醛固酮阻滞剂、皮质类固醇和钙调神经磷酸酶抑制剂成功治疗。通过各种测量方法,蛋白尿峰值接近7.5 - 8克蛋白质/克肌酐。血清肌酐保持正常,抗PLA2R可检测到,且随着治疗成功而下降。临床医生应意识到肾小球肾炎患者出现两种肾小球疾病的可能性以及任何单一疾病的非典型表现。