Singhai A M, Vanikar A V, Goplani K R, Kanodia K V, Patel R D, Suthar K S, Patel H V, Gumber M R, Shah P R, Trivedi H L
Department of Pathology, Laboratory Medicine, Civil Hospital Campus, Asarwa, Ahmedabad, India.
Indian J Pathol Microbiol. 2011 Jan-Mar;54(1):3-6. doi: 10.4103/0377-4929.77315.
Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis (GN) usually presenting clinically as steroid resistant/dependent nephrotic syndrome (NS) with pathology of mesangial proliferative GN or focal and segmental glomerulosclerosis with diffuse predominant mesangial IgM deposits. Not much information is available about its natural history. This is the first Indian study to our knowledge on IgMN in adults and adolescents.
We evaluated renal biopsies performed at our center between January,'04 to September,'09. Biopsies of all adolescents and adults were evaluated for IgMN and we studied their age, gender distribution, blood pressure (BP), disease duration, steroid/immunosuppressive management and serial serum creatinine (SCr), urinary proteins, and BP values. Patients with other systemic diseases/infections and children were excluded.
IgMN constituted 4.3% of 2702 adult renal biopsies. No significant gender predilection was noted. Males presented at average age of 23.1 years, females at 30 years. Steroid-dependent NS was the commonest presentation noted in 75% followed by steroid-resistant NS. Hypertension was noted in 10% patients. Mesangial proliferative GN (MePGN) was commonest histopathological finding noted in 74.4%, followed by focal segmental glomerulosclerosis (FSGS) in 16.2%, and minimal change disease (MCD) in 9.4% biopsies. Sole IgM deposits were noted in 88.5%. All MCD, 35.6% MePGN reached remission, FSGS progressed to renal failure by 1 year. Hypertension, proteinuria, interstitial fibrosis, and FSGS were bad prognosticators.
This is the first Indian study of IgMN in adults and adolescents carried out over a period of 5.8 years, which has shown that hypertension, proteinuria, and interstitial fibrosis at presentation have bad prognosis.
免疫球蛋白M肾病（IgMN）是一种特发性肾小球肾炎（GN），临床上通常表现为激素抵抗/依赖型肾病综合征（NS），病理表现为系膜增生性GN或局灶节段性肾小球硬化，伴有弥漫性为主的系膜IgM沉积。关于其自然病史的信息不多。据我们所知，这是印度第一项针对成人和青少年IgMN的研究。
我们评估了2004年1月至2009年9月在我们中心进行的肾活检。对所有青少年和成人的活检组织进行IgMN评估，并研究他们的年龄、性别分布、血压（BP）、病程、激素/免疫抑制治疗情况以及系列血清肌酐（SCr）、尿蛋白和BP值。排除患有其他系统性疾病/感染的患者和儿童。
IgMN占2702例成人肾活检的4.3%。未发现明显的性别倾向。男性发病平均年龄为23.1岁，女性为30岁。激素依赖型NS是最常见的表现，占75%，其次是激素抵抗型NS。10%的患者有高血压。系膜增生性GN（MePGN）是最常见的组织病理学表现，占74.4%，其次是局灶节段性肾小球硬化（FSGS），占16.2%，微小病变病（MCD）占活检组织的9.4%。88.5%的病例可见单纯IgM沉积。所有MCD、35.6%的MePGN达到缓解，FSGS在1年内进展为肾衰竭。高血压、蛋白尿、间质纤维化和FSGS是不良预后因素。
这是印度第一项针对成人和青少年IgMN进行的为期5.8年的研究，该研究表明，就诊时的高血压、蛋白尿和间质纤维化预后不良。
