152例V型狼疮肾炎的临床病理研究

Clinicopathologic study in 152 patients with class V lupus nephritis

目的 研究V型狼疮肾炎(LN)两种病理亚型的临床特点及其转归。方法152例经肾穿刺活检确诊的V型LN,根据是否伴有肾小球系膜增生性病变,分为Va(61例)、Vb(91例)两种亚型,分析其临床病理特点,并与同期的488例Ⅳ型LN作比较。结果Vb型高血压、贫血、肾功能减退的发生率及低补体血症、抗dsDNA抗体阳性率明显高于Va型,与Ⅳ型接近。肾活检病理Vb型肾小球节段襻坏死、微血栓比例高于Va型,免疫复合物沉积强于Va型。在行重复肾活检的11例Va型、13例Vb型中分别有2例(18.2％)和8例(61.5％)转为Ⅳ型LN(P<0.05)。随访1年以上的47例Va型、66例Vb型中分别有3例(6.4％)和10例(15.2％)出现血肌配倍增。结论 Va型患者不仅肾组织损害轻,而且肾外表现较少。与之相比,Vb型临床特征更接近于Ⅳ型,部分患者可以转化为Ⅳ型,预后比Va型差。

Objective To investigate the clinical characteristics and outcome of the subgroups of class V lupus nephritis (LN) . Methods 152 patients with class V LN proven by renal biopsy were classified into 2 subgroups according to the 1995 WHO modified classification of LN. Class Va (61 patients) referred to pure membranous glomerulonephritis. Class Vb (91 patients) referred to diffuse membranous glomerulonephritis with superimposed mesangial widening and/or mild to moderate hypercellularity, withdeposition of immune complex in the mesangial region. 488 patients with class Ⅳ LN in the same period werechosen as control. The clinical manifestations, serology examination, transformation of histopathological pattern and prognosis were compared between patients with class Va and class Vb. Results The incidence of hypertension, anemia in patients with class Vb were higher than that in class Va significantly (38. 5% vs 21. 3% , 72. 5% vs 52. 5% respectively, P < 0. 05). The renal injury in class Vb patients was also more severe than that in class Va. The incidence of microhematuria and renal insufficiency in class Vb were 64. 8% and 15. 4% , which were higher than that in class Va (44. 2% and 3. 3% ), but were lower than thatin class Ⅳ (89. 1% and 35% ), P < 0. 05. The percentage of patients with positive anti-dsDNA antibodyand low serum C3 in class Vb were higher than that in class Va (35. 2% vs 26. 2% , 50. 6% vs 31. 2% respectively), and there was significant difference only in low serum C3 ( P < 0. 05). Besides the presence of proliferative lesion, there were more inflammatory lesions such as fibrinoid necrosis and microthrombi in class Vb. The immunohistochemical staining revealed that the percentage of patients with full house was higher in class Vb than that in class Va (79. 6% vs 49. 2% , P < 0. 05) . Repeated renal biopsies in 24 patients (11 with class Va, 13 with class Vb) revealed that 8 (61. 5% ) of patients with class Vb transformedto class ⅣLN, while only 2 (18. 2% ) of patients with class Va transformed ( P < 0. 05). A total of 113patients (47 with class Va, 66 with class Vb) were followed up for more than 1 year. Three patients in class Va (6.4% ) doubled their serum creatinine (Scr), but no patient progressed to end stage renal disease (ESRD). Ten patients in class Vb (15. 2% ) doubled their Scr, and 3 patients entered into ESRD at 6, 9 and 14 years respectively. Conclusions The renal injury and extrarenal manifestations in patients with class Vb are more severe than that in patients with class Va. Class Vb patients are more frequent to transformto class Ⅳ LN and their prognosis is poorer as compared to class Va patients.