表现为单纯镜下血尿的C1q肾病报道1例

<正>C_1q肾病是一种以系膜增生为主的肾小球疾病,其特点为免疫荧光染色可见系膜区高强度C_1q沉积,临床表现多为持续性蛋白尿或肾病范围内蛋白尿,伴有或不伴有血尿和肾功能损伤。本病例为以单纯血尿为表现的一例C_1q肾病,特报道如下。临床资料患者男,30岁,因体检发现尿潜血2年余于2016年6月10日     

系膜区IgG沉积为主的原发性系膜增生性肾小球肾炎的临床病理特征

肾小球系膜区免疫球蛋白沉积是原发性系膜增生性肾小球肾炎(MsPGN)常见的病理形态学特征,而最常见的是系膜区IgA为主的免疫复合物沉积引起的IgA肾病(IgAN).近年来提出了一种以系膜区IgM为主的免疫复合物沉积引起的IgM肾病.IgG作为一种最常见的血清免疫球蛋白,其常在多种肾小球肾炎中沉积,例如膜性肾病、系膜毛细血管性肾小球肾炎和急性感染性肾小球肾炎等.     

C1q肾病17例临床病理分析

目的探讨成人C1q肾病的临床表现、病理改变和治疗效果,以加强对成人C1q肾病的认识。方法选择我院明确诊断为C1q肾病的患者17例,从临床表现、病理改变、治疗效果3个方面进行分析。结果17例C1q肾病者,1例血肌酐超出正常水平。肾脏病理检查显示均为系膜增生性。免疫荧光检查以C1q沉积为主。电子显微镜下,除1例未见肾小球外,其余16例均可见电子致密物分布在系膜区和（或）内皮下。激素治疗效果不好。结论成人C1q肾病临床表现多样化,免疫荧光检查表现为不同的相伴沉积形式。     

电子致密物沉积病的临床及病理研究

目的：探讨电子致密物沉积病（DDD）的临床及病理特点。方法：通过5例DDD的临床及病理资料并结合文献复习,对其临床表现和组织形态学的多样性以及与治疗和预后的关系进行综合分析。结果：DDD占经肾活检证实的原发性肾小球疾病的0．2％,占膜增殖性肾炎的2％,5例中的3例临床表现为肾病综合征,组织学呈膜增生性肾炎（MPGN）,并伴有血C3降低,2例表现为慢性肾炎综合征,组织学1例呈MPGN伴有血C3降低;另1例呈系膜增生性病变,血C3正常,4例免疫荧光表现为以C3为主沿毛细血管壁呈颗粒状沉积,并有系膜区闭块状沉积,电镜下均可见电子致密物在肾小球毛细血管基底膜呈弥漫,均匀沉积,其中4例包曼囊及肾小管基底膜亦可见弥漫或节段电子致密物沉积,2例呈肾病综合征表现者对糖皮质激素治疗不敏感,结论：电子致密物沉积病是超微结构的诊断,其临床表现呈多样性,大量蛋白尿,严重高血压和较重的病理改变预示其预后不佳。     

IgA肾病的治疗

IgA肾病，是以IgA为主的免疫球蛋白在肾小球系膜区弥漫沉积所致的肾小球损害。不难看出，IgA肾病系免疫病理学诊断名词，即肾活检组织经免疫荧光染色，在肾小球系膜区可见IgA为主的免疫球蛋白颗粒状沉积，常伴有C3沉积，而少见补体的前期产物如C1q和C4的沉积。     

儿童C1q肾病临床病理研究

目的 :探讨儿童C1q肾病临床病理特征及诊断与治疗。方法 :分析 8例C1q肾病患儿临床病理特点及激素或免疫抑制剂治疗效应 ,并与同期 77例原发性NS患儿作比较。结果 :8例C1q肾病患儿临床上大多表现为原发性NS(6例 ) ,仅 2例表现为肾炎综合征和单纯性血尿。LM主要包括MC(3例 )、MsPGN(2例 )、FSGS(2例 )和ECPGN(1例 )。IF显示明显的系膜区C1q沉积 ,伴或不伴有Ig和补体沉积。EM检查仅 1例有系膜区和内皮下电子致密物沉积。与原发性NS相比 ,6例表现为NS的C1q肾病患儿对泼尼松初次治疗产生耐药的相对危险度为 2 1(P <0 .0 0 1) ,但对免疫抑制剂治疗均敏感。结论 :儿童C1q肾病临床上以对激素耐药的NS为常见表现 ,IF是其主要诊断依据 ,使用免疫抑制剂治疗有效     

膜性肾病

膜性肾病(Membranous nephropathy)又名膜性肾小球肾炎(Membranous glomerulonephritis),膜上性肾小球肾炎(Epimembranous glomerulonephritis),膜外性肾小球肾炎(Extramembranous glomerulonephritis);还有人称为膜周性肾小球肾炎(Perimembranous glomerulonephriris) (Bohle 1964)。膜性肾病原是一个病理形态学名词,是肾病综合征中常见的类型。按Gluck(1973)叙述,其特点系在肾小球毛细血管壁上有弥漫性,均匀的颗粒状免疫复合物沉积,但不伴有增生。典型病例此沉积物可致毛细血管壁均匀增厚,由于肾小球内不伴炎症,故通常被称为膜性肾病。     

儿童IgA肾病综合征

1968年Berger氏和Hinglais氏首次叙述了IgA肾病。随后发现过敏性紫癜、肝硬化等许多疾病亦可引起肾小球系膜的IgA沉积,故目前将IgA肾病称之谓IgA肾病综合征。近年来此征发病率显著提高,在美国、英国等某些欧美国家已引起小流行,普遍地认识到IgA肾病是肾小球肾炎最常见类型之一。临床病理特点原发性IgA肾病的特点是系膜区IgA沉积,且常伴有C_3、IgG、IgM和纤维蛋白原的沉积,系膜区肿胀伴有不同程度的急性增生性和节段性硬化病灶,系     

李平教授辨治乙肝病毒相关性肾炎经验

正乙肝病毒相关性肾炎(hepatitis B virus associated-glomerulonephritis,HBV-GN),简称乙肝肾,是乙肝病毒直接或间接诱发的继发性肾小球肾炎。常见的病理类型有:膜性肾病、膜增生性肾小球肾炎、系膜增生性肾小球肾炎(包括IgA肾病)、毛细血管内增生性肾小球肾炎、局灶节段性肾小球肾炎等。其中,最常见的病理类型为膜性肾病,为继发性膜性肾病中最常     

肾脏有免疫复合物沉积的抗肾小球基底膜病的临床病理特点

目的了解伴有免疫复合物沉积的抗肾小球基底膜(GBM)抗体相关疾病的肾脏病理表现及其临床特点.方法将我科近9年来确诊并行肾穿刺活检证实的10例伴有免疫复合物沉积的抗GBM抗体相关疾病患者与同期25例经典抗GBM肾炎患者的临床和病理资料进行比较.结果10例患者中,2例为膜性肾病,1例IgA肾病,1例膜增生性肾炎,1例过敏性紫癜性肾炎,1例膜型乙肝病毒相关性肾炎,其余4例系膜区有免疫复合物沉积,但原发疾病不清.与经典抗GBM肾炎患者比较,2组在年龄、性别、临床表现、血清学检查、肾脏病理、治疗及转归等方面无显著性差异.结论抗GBM肾炎可发生在其它肾小球疾病的基础上,其临床和病理特点与经典的抗GBM肾炎基本一致.临床上应及时检测血清抗GBM抗体,以利于早确诊而选择血浆置换和强化免疫抑制治疗.     

C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome

The presence, distribution, and intensity of glomerular C1q localization were evaluated by direct immunofluorescence microscopy in 800 renal biopsy specimens which were also studied by light and electron microscopy. Identified were 15 patients with extensive (mean: 3.6 + out of 4 +), predominantly mesangial, C1q localization along with C3 and immunoglobulins, but no evidence for systemic lupus erythematosus. Pathologically, this lesion most closely resembled lupus nephritis. Clinical and pathologic data from these 15 C1q nephropathy patients were compared to data from 30 lupus nephritis and 223 other proliferative glomerulonephritis patients, and the C1q nephropathy patients were found to be dissimilar to both groups. The 15 C1q nephropathy patients had an average age of 17.8 years, 8 males, 7 females, 9 Black, 100% had proteinuria (mean 7.5 g/d), 40% hematuria, 0% hypocomplementemia, and 0% antinuclear antibodies. By electron microscopy, 100% had mesangial dense deposits, 20% capillary wall dense deposits, and 0% endothelial tubuloreticular inclusions. Nine patients treated with steroids had no definite resolution of proteinuria. We proposed that C1q nephropathy is a distinct clinicopathologic entity, usually causing steroid-resistant nephrotic syndrome in older children and young adults.     

C1q nephropathy in a child presenting with recurrent gross hematuria

C1q nephropathy is a rare glomerular disease characterized by mesangial immune deposits with dominant or codominant staining for C1q. The exact pathogenesis leading to the mesangial immune deposits of C1q remains unknown. C1q nephropathy often presents with proteinuria in the nephrotic range, with an unpredictable or poor response to corticosteroid therapy. It is seen more commonly in older children and young adults and is more common in African Americans compared with Caucasians. We present a 4-year-old African American girl who presented with recurrent gross hematuria in the absence of proteinuria or hypertension and whose renal biopsy demonstrated dominant mesangial deposits of C1q. We conclude that C1q nephropathy should be considered in patients who present with recurrent gross hematuria.     

A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence

We present a 1-month-old girl with a congenital nephrotic syndrome and unusual histological findings. Immunofluorescence microscopy demonstrated granular mesangial deposition of C1q and electron microscopy revealed electron-dense mesangial deposits. Her heavy proteinuria gradually decreased and the steroid therapy did not have a significant effect. Her renal function was normal throughout the entire period of observation. The clinical evidence and histopathological features of this patient were compatible with C1q nephropathy.     

De novo C1q nephropathy in the renal allograft of a kidney pancreas transplant recipient: BK virus-induced nephropathy?

C1q nephropathy is a distinct entity characterized by extensive and dominant C1q mesangial deposition with associated steroid resistant proteinuria in the absence of systemic lupus erythematosus. Several morphological patterns ranging from very subtle glomerular alterations to focal/segmental glomerulosclerosis and mesangial proliferative changes have been described. Interstitial nephritis secondary to BK polyomavirus is a recently recognized complication in kidney transplant recipients. It may be associated with a tubulitis-like picture, mimicking sometimes acute tubular rejection. We report the case of a kidney pancreas transplant recipient who developed de novo C1q nephropathy, in the setting of BK polyomaviral interstitial nephritis. He presented with renal allograft dysfunction and a kidney biopsy was performed. It was interpreted as acute cellular rejection. C1q deposits were detected by immunofluorescence studies and electron microscopy. The patient did not respond clinically to appropriate anti-rejection treatment and a second renal biopsy was performed. The possibility of an interstitial nephritis secondary to BK polyomavirus mimicking rejection was suggested. Special immunohistochemical and blood/urine PCR studies for BK virus were performed, confirming the diagnosis of BK virus tubulonterstitial nephritis with a persistent, probable BK virus induced C1q nephropathy.     

C1Q nephropathy in children

C1q nephropathy (C1qNP) is a peculiar form of glomerulonephritis characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. We describe the incidence, manifestation, histopathologic findings, follow-up, treatment and outcome of C1qNP. Twelve C1qNP patients were identified among 131 children who had undergone renal biopsy, accounting for a 9.16% incidence of C1qNP. Light microscopy examination showed focal segmental glomerulosclerosis (FSGS) with or without diffuse mesangial proliferation (n=6), minimal change disease (MCD) (n=4) or focal glomerulonephritis (n=2). C1q deposits were found in all, while electron microscopy revealed visible deposits in nine cases. Eight children presented with nephrotic syndrome, while one had nephrotic proteinuria and renal insufficiency that progressed to end-stage renal failure. The remaining three patients presented with nonnephrotic proteinuria associated with microhematuria, hypertension or renal insufficiency. Only one nephrotic syndrome patient responded excellently to corticosteroids, while four became corticosteroid dependent, and three were corticosteroid resistant, showing a very poor response to other immunosuppressive therapy as well. Patients with non-nephrotic proteinuria demonstrated fixed laboratory findings. Most C1qNP patients had FSGS or MCD, the majority of them presenting with corticosteroid-dependent or corticosteroid-resistant nephrotic syndrome. The latter showed a very poor response to any immunosuppressive therapy and high risk for progressive renal insufficiency.     

原发性干燥综合征合并膜性肾病

目的分析原发性干燥综合征（pSS）患者合并膜性肾病（MN）的临床和肾脏病理特点，以提高对此类疾病的认识。方法回顾性分析北京协和医院2001年5月至2006年5月期间17例原发性干燥综合征伴膜性肾病的临床和肾脏病理特点。结果17例患者中男性6例，女性11例，平均年龄（47．3±17．3）岁（24～72岁）。主要临床表现为水肿和蛋白尿（17/17），其中肾病综合征占13／17，镜下血尿占12／17。根据光镜、免疫荧光和电镜的特点，17例患者的肾脏病理可分为单纯MN组（8／17）和不典型MN组（9／17）。单纯MN组：光镜下未见系膜细胞和基质增生；电镜下电子致密物（ED）仅在上皮下和（或）肾小球基底膜内沉积。不典型MN组：光镜下可见系膜细胞和基质节段或弥漫增生；免疫荧光除了IgG阳性外，常有早期补体成分C1q阳性（7／9）；电镜下可见ED分布于上皮下和系膜区。17例患者中有2例患者接受了重复肾活检，其中1例首次活检表现为MNI期，8年后再次活检表现为不典型MN；另1例首次活检表现为不典型MN，4年后再次活检却表现为狼疮肾炎（LN）Ⅳ-G（A／C）＋V型。结论原发性干燥综合征合并膜性肾病并非少见，是干燥综合征合并蛋白尿常见的病因。对于光镜下呈现不典型膜性肾病和免疫荧光早期补体成分阳性，电镜下有系膜区ED沉积的患者应警惕若干年后转化为隐匿性狼疮肾炎的可能性。     

C1q nephropathy in two young sisters

C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two sisters with C1qNP, who presented with steroid-resistant nephrotic syndrome. Both sisters presented before the age of 2 years, and they showed a poor response to other immunosuppressive therapy. Both girls had normal serum complement levels, negative antinuclear antibodies (ANAs) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis, with diffuse “full-house” positive immunofluorescence reaction in the mesangial area. The immunofluorescence reaction for C1q was most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too. We report on two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge, this is the first report of C1qNP in siblings.     

C1q nephropathy: a pediatric clinicopathologic study

We report on 15 children with proteinuria, at the nephrotic level in the majority of cases, who had no histologic glomerular alterations (eight cases), or focal and segmental glomerular scarring with (three cases) or without (four cases) mesangial proliferation. In all cases, immunofluorescence (IF) microscopy showed prominent mesangial C1q deposits with variable amounts of immunoglobulins. Ultrastructurally, most had conspicuous mesangial electron-dense deposits. Cases with no glomerular histologic alterations were histologically indistinguishable from minimal change disease (MCD), yet they uniformly had an unsatisfactory response to oral prednisone. Thus, the presence of immune deposits with a prominent C1q contribution identifies a group of cases that respond poorly to steroids and that, if light microscopy is considered in isolation, might otherwise be designated MCD.     

C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis

C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. Histology in C1q nephropathy is characterized by a slight to severe increase in mesangial cellularity and matrix, with or without segmental sclerosis. C1q nephropathy usually presents with nephrotic-range proteinuria in older children and young adults, and has a poor response to steroids. Patients may have decreased creatinine clearance at presentation, but progression to end-stage renal disease (ESRD) is slow. Severe crescentic glomerulonephritis has not been reported in C1q nephropathy. We describe a 3-year-old Hispanic girl who presented with renal insufficiency. Kidney biopsy showed C1q nephropathy with severe crescentic glomerulonephritis. The clinical and serological evaluation ruled out systemic lupus erythematosus or other immunological or infectious etiologies. In spite of immunosuppressive therapy, she progressed to ESRD within 14 weeks and is currently on chronic peritoneal dialysis. The atypical features of C1q nephropathy observed in our patient, which have not been described in earlier reports, are an early age of onset, severe crescentic glomerulonephritis, and rapid progression to ESRD. C1q nephropathy should be added to the differential diagnosis of glomerulonephritis in young children and in the patient with crescentic glomerulonephritis.