ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis".
ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis".
复制标题
系统性硬化症中 ANCA 相关的新月体肾小球肾炎：重新审视“正常血压硬皮病肾危象”。
作者：
Laurent Arnaud;Antoine Huart;E. Plaisier;Hélène François;B. Mougenot;K. Tiev;A. Kettaneh;Pierre Ronco;J. Rougier
硬皮病肾危象的特点是急性发作的严重高血压和迅速进行性高肾素性肾衰竭。然而，有一个非常有限的子集的患者迅速进行性肾功能衰竭谁保持血压正常，发展ANCA阳性新月体肾小球肾炎。我们报告一例弥漫性系统性硬化症患者继发于抗MPO抗体相关性新月体肾炎的血压正常的急性肾功能衰竭。她被转诊到我科，血压正常，无肾外血管炎的临床表现。她表现为快速进行性肾衰竭、镜下血尿和极轻微蛋白尿。免疫荧光法检测P-ANCA阳性，ELISA法证实P-ANCA对髓过氧化物酶具有特异性。肾活检显示典型的少免疫性肾小球肾炎特征，伴有新月体形成和纤维素样坏死。患者最初仅接受环磷酰胺静脉注射治疗。由于肾功能持续恶化，开始甲基强的松龙静脉冲击，随后口服泼尼松进行额外治疗，使肾功能改善。9个月后，血清肌酐几乎恢复到正常水平，蛋白尿极少，无血尿，ANCA检测阴性。对照肾活检仅显示瘢痕病变。ANCA阳性的新月体肾小球肾炎和系统性硬化症的相关性是非常罕见的。静脉注射环磷酰胺和皮质类固醇治疗可快速和长期改善肾功能。高剂量皮质类固醇激素不太可能引发典型的硬皮病肾危象，但需要对重叠系统性硬化症患者进行密切随访。诊断和治疗进行了讨论，以前发表的病例进行审查。
The scleroderma renal crisis is characterized by acute onset of severe hypertension and by rapidly progressive hyperreninemic renal failure. There is, however, a very limited subset of patients with rapidly progressive renal failure who remain normotensive and develop ANCA-positive crescentic glomerulonephritis. We report a case of normotensive acute renal failure secondary to anti-MPO antibody-associated crescentic glomerulonephritis in a patient with diffuse systemic sclerosis. She was referred to our department with normal blood pressure and no extrarenal clinical manifestation ofvasculitis. She presented with rapidly progressive renal failure, microscopic hematuria and minimal proteinuria. P-ANCA were positive by immunofluorescence, with ELISA-confirmed specificity for myeloperoxidase. Renal biopsy revealed typical features of pauciimmune glomerulonephritis with crescent formation and fibrinoid necrosis. The patient was initially treated with i.v. cyclophosphamide only. Because of ongoing deteriorating renal function, additional treatment with intravenous pulses of methylprednisolone followed by oral prednisone was started and allowed renal function improvement. After 9 months, serum creatinine had almost returned to normal level with minimal proteinuria, no hematuria and negative ANCA testing. Control kidney biopsy only revealed scar lesions. The association of ANCA-positive crescentic glomerulonephritis and systemic sclerosis is a very rare event. Treatment with intravenous cyclophosphamide and corticosteroids allows rapid and long-term improvement of renal function. The onset of typical scleroderma renal crisis triggered by high-dose corticosteroids is unlikely but requires a close follow-up of patients with overlapping systemic sclerosis. Diagnosis and treatment are discussed and previously published cases are reviewed.