The clinical and pathological characteristics of Chinese patients with pauci-immune crescent glomerulonephritis

目的 对寡免疫复合物型新月体肾炎进行临床病理分析。方法 采用病理检查包括 :光镜检查、免疫病理分析、血清学检查、肾功能检查以及统计学分析。结果 6 4 0 0例非移植肾活检中发现 2 4例寡免疫复合物型新月体肾炎 (PICGN)患者 ,占肾活检总数的 0 37% ,占新月体肾炎总数的 2 3 9% ,其中女性 16例 ,男性 8例 ,平均年龄 33岁。血管炎中微型多动脉炎 (33 3% )和结节性多动脉炎 (8 3% )为常见继发病因。抗中性粒细胞胞浆抗体 (ANCA)阳性率为 52 2 %。临床上多表现为急进性肾功能减退 (75 0 % ) ,发作性肉眼血尿发生率高 (58 3% ) ,高血压发生率为 4 5 8% ,肾病综合症发生率为 4 1 7% ,2 5%患者出现少尿无尿症状。除贫血外 ,肾外表现较少。病理上血管襻坏死 (6 2 5% )、单核及中性粒细胞浸润(6 6 7% )及小血管炎等为常见的急性病变 ;然而 ,小球硬化 (4 5 8% )、小管萎缩 (83 3% )、间质纤维化 (75 0 % )等慢性化病变也较严重。 2 2例患者行免疫抑制治疗 ,16例随访 8— 92个月 ,有 4例肾功能在正常范围 (<12 4 μmol/L) ,8例行非透析保守治疗 ,仅 4例进入终末期肾功能衰竭需进行维持性血透析治疗。结论 免疫抑制冲击治疗是治疗PICGN的有效方法。

Antineutrophil cytoplasmic antibodies crescentic allograft glomerulonephritis after sofosbuvir therapy

Antineutrophil cytoplasmic antibodies(ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis(RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine(adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.

C3 glomerulonephritis

A case of rapidly progressive glomerulonephritis with pathological changes to the crescent glomerulonephritis“C3 glomerulonephritis”in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and its diagnosis,clinical manifestation,and pathological characteristics were explored.The experts analyzed the disease from the perspective of its causes,diagnosis,complications and treatment.Timely renal biopsy is required to clarify pathological diagnosis as the primary glomerular disease was newly recognized with a lower incidence.So misdiagnosis and delayed healing is more frequent.The paper aims to enhance the clinician’s understanding of“C3 glomerulonephritis”.

The distribution and significance of renal infiltrating cells in patients with diffuse crescentic glomerulonephritis

Objective To study the significance and distribution of renal infiltrating cells, including monocytes (CD68+ cells), proliferative cell nuclear antigen positive cells (PCNA+ cells), CD4+ and CD8+ cells in diffuse crescent glomerulonephritis (DCGN). Methods Fifty-six patients with DCGN were studied, including 10 cases of anti-glomerular basement membrane (GBM)-type Ⅰ DCGN, 26 immune complex-type Ⅱ DCGN, and 20 cases of pauci-immune-type Ⅲ DCGN. Glomerular and interstitial infiltrates of CD68+ and PCNA+ cells, and interstitial infiltrates of CD4+ and CD8+ cells were detected by using four-layer PAP methods. Results There was a significant increase of renal infiltrating CD68+, PCNA+, CD4+ and CD8+ cells in patients with DCGN compared with that in normal controls. In patients with type Ⅰ DCGN, there was a higher number of renal infiltrating CD68+ and PCNA+ cells than that in patients with type Ⅱ and Ⅲ DCGN. A glomerular infiltrates of CD68+ and PCNA+ cells correlated with the interstitial infiltrates of CD4+ cells in type Ⅰ or Ⅲ DCGN patients. In lupus DCGN patients, the numbers of renal infiltrating CD68+ and PCNA+ cells were similar to vasculitis or type Ⅲ DCGN patients.Conclusion These findings demonstrate that the renal infiltrates of CD68+ and PCNA+ cells play an important role in patients with DCGN, that the infiltrates of CD4+ cells correlate with the infiltrates of CD68+ and PCNA+ cells may be an active marker of DCGN, and that cell-mediated immunity may contribute to crescent formation in lupus DCGN patients.

Infective Endocarditis-induced Crescentic Glomerulonephritis Dramatically Improved after Removal of Vegetations and Valve Replacement

Infective endocarditis （IE） is associated with poor prognosis and higher mortality. IE-induced glomerulonephritis, especially crescentic glomerulonephritis （IE-CGN） with renal dysfunction, is an independent risk factor for mortality For the treatment of IE-CGN, many therapeutic strategies including antibiotics alone, antibiotics combined with corticosteroid, immunosuppressive agents, plasmapheresis, or cardiac surgery have been applied and obtained various results.C21 Here, we reported a case of CGN with progressive renal failure secondary to IE in which the renal function was dramatically improved by the treatment of surgical valve replacement and antibiotics.

Primary glomerular diseases in the elderly

Primary glomerular diseases in the elderly population are a frustrating topic due to difficulties in both the diagnosis and decision making about treatment. The most frequent type of primary glomerular disease in elderly is membranous nephropathy; while its counterpart in younger population is Ig A nephropathy. The most frequent cause of nephrotic syndrome in the elderly is also membranous nephropathy. Pauci-immune crescentic glomerulonephritis(GN) rate increases both in elderly and very elderly population. Pauci-immune crescentic GNs should be regarded as urgencies in elderly patients as in their younger counterparts due to potential for causing end-stage renal disease in case of delayed diagnosis and treatment, and also causing mortality due to alveolar hemorrhage in patients with pulmonary involvement. Renal biopsy is the inevitable diagnostic method in the elderly as in all other age groups. Renal biopsy prevents unnecessary treatments and provides prognostic data. So advanced age should not be the sole contraindication for renal biopsy. The course of primary glomerular diseases may differ in the elderly population. Acute kidney injury is more frequent in the course and renal functions may be worse at presentation. These patients are more prone to be hypertensive. The decision about adding immune suppressive therapies to conservative methods should be made considering many factors like co-morbidities, drug side effects and potential drug interactions, risk of infection, patient preference, life expectancy and renal functions at the time of diagnosis.

Clinical spectrum of diffuse crescentic glomerulonephritis in Chinese patients

Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.Results The distribution of patients among the three classifications was 8. 7% type Ⅰ , 68. 6% type Ⅱ and 22. 7% type Ⅲ. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30. 2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61. 6% had hypertension, 50. 6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type Ⅲ DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.Conclusion DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30. 2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.

Significance of anti neutrophil cytoplasmic antibodies in patients with pauci immune crescentic glomerulonephritis

Objective To evaluate the significance of serum anti neutrophil cytoplasmic antibodies (ANCAs) and the effects of immune suppressive treatments on its activity in patients with pauci immune crescentic glomerulonephritis (PICGN) Methods Serum ANCAs and myeloperoxidase (MPO) ANCA were detected by indirect immunofluorescence and enzyme linked immuno^sorbent assay (ELISA) methods respectively, and the renal tissues infiltrating cells including CD4 +, CD8 +, CD68 + and PCNA + cells were determined by four layer peroxidase antiperoxidase (PAP) method The clinical manifestations, pathologic features and immune pathologic changes in patients with positive ANCAs were compared with that in patients with negative ANCAs The effects of immune suppressive therapy on clinic and pathologic changes as well as ANCAs activity were also investigated in ANCAs positive and ANCAs negative patients Results Both of clinic active manifestations such as the degree of hematuria, rapidly progressive renal failure and pathologic active features including segmental capillary necrosis and vasculitis were much common in patients with positive ANCAs as compared with that in patients with negative ANCAs The number of infiltrating cells in renal tissue, especially CD4 + cells, was markedly higher in ANCAs positive patients than that in ANCAs negative patients The effects of immune suppressive therapy were also much better in patients with positive ANCAs than that in patients with negative ANCAs Conclusion Serum ANCAs is not only a marker for diagnosis of systemic vasculitis, but also a sensitive predictor for evaluation of diseases' activity and treatment in patients with PICGN The good effect of immune suppressive treatments on patients with PICGN is partially associated with the degree of ANCAs activity