Kitridou R C, Akmal M, Turkel S B, Ehresmann G R, Quismorio F P, Massry S G
Semin Arthritis Rheum. 1986 Nov;16(2):135-45. doi: 10.1016/0049-0172(86)90047-8.
Eleven of 30 patients with MCTD, followed for a mean of 10 years, developed immune complex nephropathy (five membranous, two mesangial, one mixed, and one sclerosing) with NS in nine of 11. Another patient had membranous nephropathy at autopsy. Patients with renal disease tended to have more systemic manifestations than those without. NS was at times of abrupt onset, recurrent, and/or persistent. Anti-RNP and serum complement were not helpful in predicting nephritis. Seventy-two percent of nephropathy and 62% of NS episodes resolved or improved after corticosteroid therapy. Five patients became hypertensive, two developed chronic renal failure and required chronic dialysis, and one needed acute dialysis twice. One patient progressed to focal proliferative crescentic nephritis with necrotizing arteritis. Three patients with nephropathy died, two of pulmonary hypertension with acute cor pulmonale and one of overwhelming sepsis. Nephropathy is relatively common in MCTD, is associated with substantial morbidity, and with the risk of hypertension and chronic renal failure.
30例混合性结缔组织病（MCTD）患者平均随访10年，其中11例发生免疫复合物性肾病（5例膜性肾病、2例系膜增生性肾炎、1例混合性肾病和1例硬化性肾病），11例中有9例出现肾病综合征（NS）。另有1例患者尸检时发现膜性肾病。有肾脏疾病的患者往往比没有肾脏疾病的患者有更多的全身表现。NS有时起病急骤、反复发作和/或持续存在。抗RNP抗体和血清补体对预测肾炎并无帮助。72%的肾病患者和62%的NS发作在接受糖皮质激素治疗后缓解或改善。5例患者出现高血压，2例发展为慢性肾衰竭并需要长期透析，1例需要进行两次急性透析。1例患者进展为伴有坏死性动脉炎的局灶增生性新月体性肾炎。3例肾病患者死亡，2例死于肺动脉高压合并急性肺心病，1例死于严重败血症。肾病在MCTD中相对常见，与较高的发病率以及高血压和慢性肾衰竭风险相关。
