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腎臟間質區IgM沈積於腎病症候羣年青患者之臨床意義
Significance of IgM Deposition in the Mesangial Region in Young Adult Nephrotic Syndrome
摘要
原因不明性腎病症候畫患者對於類固醇治療的反應，以及治療後長期預後的情形，需靠臨床的徵候，甚至病理鏡檢積腎臟組織的分類來做評估。在腎間質組織IgM沈積與輕微變化而無IgM沈準型腎病間差別和臨床的意義，各家學說莫衷一是。於22位資料完整其致病原因不明的腎病症候羣年青男性患者，其中10位的腎組織，在免疫螢光鏡檢查下，顯示IgM沈積在腎間質區域；另外的12位，其病理顯微檢查結果，證實是典型的輕微變化型腎病。二組的年齡相當（23.20±2.04與22.75±2.60歲），發病時每日尿液中蛋白漏失量、血漿中白蛋白、膽固醇、尿素氮、肌酸酐，以及肌酸酐清除率，皆無統計上差異，血清免疫球蛋白IgM、IgG、IgA及補體C3的平均值，除IgG偏低外，均在正常範圍內。這些病人給予類固醇1-2mg／kg／day治療4週後，IgM沈積組中有5位治療反應良好，3位只有部份療效，2位則沒有治療效應；而典型輕微變化型組中，有6位治療反應良好，3位只有部份療效，3位完全沒有效應。對於只有部份反應和沒有類固醇效應的病患施予endoxan 2mg／kg／day繼續治療。兩組追踩24個月至76個月不等，唯平均月數相近，此較兩組的血中尿素氮、肌酸酐，和肌酸酐清除率，皆無統計學上之差異。根據上述結果，吾等結論：對於IgM沈積與否，在腎病症候羣年青男性的類固醇治療反應及治療的預後，似乎並無多大之臨床意義，因此欲建立單獨一個所謂的「IgM腎病」類別，仍是值得多方探究與討論。
並列摘要
Clinical and laboratory studies of the idiopathic nephrotic syndrome are unable to accurately identify the result entities of steroid treatment and long-term prognosis. Most idiopathic nephrotic patients would benefit from renal biosy which is the best guide for therapy. A subgroup of nephrotic patients with messangial lgM deposits or minimal change disease has been individualized, but clinical significance has not been well established. The clinical manifestations, course, steroid response and progrnosis of mesangial IgM deposits and minimal change nephrotic syndrome were studied in 22 recently diagnosed young males, ranging in age from 20 to 27 years, divided into two groups. Glomeruli were normal by light microscopy. Ten patients demonstrated diffuse mesangial deposition of IgM by immunofluorescence while 12 patients were negative. There were no significant differences between the two groups in initial daily urine protein loss, serum levels of a1bumin, cholesterol, BUN, and creatinine (even in creatine clearance, serum content of immunoglobumin as well as complement C3 (P＞ 0.05).In the twenty-two patients receiving prednisolone treatment (1-2mgjkgj day) for 4 weeks, five showed a good response, three a partial and the remaining two did not respond in the IgM deposits group; while six patients had a good response, three had only a partial and two hand no effect in the pure minimal change nephrotic patients group. Endoxan 2mgjkgjday was given to the patients with partial or resistant steroid response for 8-12 weeks. Three patients revealed good response, one had persistent proteinuria (＞ 500mg/day), another went into chronic renal insufficiency with creatinine clearnance at 15.62rnljmin in IgM deposit group; while two patients had a good response, the other three had persistent proteinuria ranging from 500-1000ng/day. None revea1ed impaired renal function in those without the IgM deposits group. The days of follow-up-were from 24 to 76 months. There were no significant differences between the two groups for the last serum BUN, creatinine and creatinine clearance (P ＞ 0.05). We conclude that there is no significant difference in response or outcome between the patients with mesangial IgM deposits and those without this immunoglobulin deposition. The establishment of a clinicopathologic entity of IgM nephropathy needs further prospective study with more extended follow-up.