Cytapheresis for the treatment of myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis: report of five cases.

To minimize the adverse effects of high-dose administration of steroids and cyclophosphamide in patients with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA), granulocytapheresis (GCAP) or leukocytapheresis (LCAP) was performed to reduce inflammation. Four patients with rapidly progressive glomerulonephritis (RPGN) and one patient with pulmonary hemorrhage due to MPO-ANCA-associated vasculitis were treated by cytapheresis. The prednisolone (PSL) dose was 0.28 +/- 0.15 mg/kg/day (mean +/- SD) (range 0.18-0.50 g/kg/day). In the 4 RPGN patients, the peak serum creatinine level was 3.7 +/- 1.9 mg/dl (range 1.7 to 5.6 mg/dl). GCAP was performed in 3 RPGN patients and in 1 pulmonary hemorrhage patient. LCAP was performed in 1 RPGN patient. In the 4 RPGN patients, renal function improved after combined therapy with cytapheresis and corticosteroids. In the pulmonary hemorrhage patient, evidence of pulmonary hemorrhage on chest computed tomography scanning diminished after combined therapy with cytapheresis and corticosteroids. Cytapheresis, when combined with a low-dose or intermediate-dose PSL regimen, is effective in the treatment of ANCA-associated vasculitis.     

"Triple-positive" renal limited vasculitis presenting with rapidly progressive glomerulonephritis: A case report

Rationale: Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a "double-positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis, suggesting an ANCA-associated kidney lesion. Proteinase 3-ANCA positive diseases are also rarely reported. Patients positive for all three antibodies, i.e., triple-positive patients, are extremely rare.Patient's Concern: A 53 year-old female presented with anasarca and oliguria of 2 months' duration. Diagnosis: Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA, proteinase 3-ANCA, and anti-GBM antibody (triple-positive). Interventions: Intravenous high dose cyclophosphamide, oral azathioprine, intravenous methylprednisolone, and plasma exchange as per British Health Professionals in Rheumatology Guidelines. Outcomes: After one-month follow-up, anasarca and proteinuria were lessened, serum creatinine was normalized, titers of MPO-ANCA levels were decreased, and anti-GBM antibody levels were normalized. Lessons: Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.     

Analytical and diagnostic accuracy of the EliA automated enzyme fluoroimmunoassay for antineutrophil cytoplasmic autoantibody detection.

Anti-proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCA) and anti-myeloperoxidase antibodies (MPO-ANCA) are considered important serological markers for several forms of idiopathic systemic vasculitis. The aim of the study was to verify the analytical and clinical performance of a new automated enzyme fluoroimmunoassay, the EliA system, for PR3-ANCA and MPO-ANCA detection. For this purpose the sera of 52 consecutive well-defined patients with a clinical diagnosis of Wegener's granulomatosis (WG) (n=29) or microscopic polyangiitis (MPA) (n=23), and 70 controls suffering from connective tissue disease (25 systemic lupus erythematosus, 25 Sj?gren's syndrome and 20 rheumatoid arthritis) were tested for PR3-ANCA and MPO-ANCA with the EliA assay (Pharmacia Diagnostics, Freiburg, Germany). For comparison purposes, the same sera were also tested by indirect immunofluorescence, another direct immunometric assay (Varelisa, Pharmacia Diagnostics) and a capture PR3-ANCA (Wieslab AB, Lund, Sweden) method. Both the EliA PR3-ANCA and MPO-ANCA assays showed between- and within-assay precision of <10%. The dilution test gave straight lines (r2=0.998) for both antibody assays. The recovery ranged from 97.9% to 102.7% for PR3-ANCA and from 84.9% to 91.4% for MPO-ANCA. There was a high positive correlation between the EliA and Varelisa methods for quantitative detection of MPO-ANCA levels (r2=0.949) and a lower correlation for PR3-ANCA (r2=0.771). Conversely, poor correlation was observed between EliA PR3-ANCA and capture PR3-ANCA (r2=0.537). The overall sensitivity and specificity of EliA PR3-ANCA and MPO-ANCA for the vasculitides considered in this study were 82.7% and 97.2%, respectively, with a positive predictive value of 96.6% and a negative predictive value of 84.9%. Comparison of the results obtained with the indirect immunofluorescence, Varelisa and capture PR3-ANCA methods showed that the indirect immunofluorescence assay is the most sensitive method for the diagnosis of vasculitis (88.5%), but the least specific (94.3%); the EliA method is slightly more specific (97.2%) than the Varelisa method (95.7%), and also slightly more sensitive (82.7% vs. 80.8%). Capture PR3-ANCA proved to be the most sensitive method for detection of anti-proteinase 3 antibodies in WG (89.7% vs. 86.2% EliA and 79.3% Varelisa). In conclusion, the EliA MPO-ANCA and PR3-ANCA methods provide good diagnostic accuracy and excellent analytical accuracy, which, in association with the practicality of the automated EliA system, make this method a useful tool for the diagnosis of ANCA-associated vasculitides.     

Recent advances of clinical and basic research in rapidly progressive glomerulonephritis

Rapidly progressive glomerulonephritis(RPGN) is the most severe form of glomerulonephritis. Recently, a variety of highly specific therapeutic methods has been considered due to advances in our understandings of molecular and cellular mechanisms of glomerular crescentic formation. Although the prognosis of the patients with RPGN has improved these days, treatment related opportunistic infection and relapse are still matters of concern. Especially, in Japan, the prevalence of older patients with MPO-ANCA related RPGN is extremely high compared to other countries. To improve the prognosis of Japanese patients with RPGN, a prospective randomized trial with specific protocol for older patients with MPO-ANCA related RPGN should be conducted.     

Protective α1-antitrypsin effects in autoimmune vasculitis are compromised by methionine oxidation

BackgroundAntineutrophil cytoplasmic autoantibody–associated (ANCA-associated) vasculitidies (AAV) are life-threatening systemic autoimmune conditions. ANCAs directed against proteinase 3 (PR3) or myeloperoxidase (MPO) bind their cell surface-presented antigen, activate neutrophils, and cause vasculitis. An imbalance between PR3 and its major inhibitor α1-antitrypsin (AAT) was proposed to underlie PR3- but not MPO-AAV. We measured AAT and PR3 in healthy individuals and patients with AAV and studied protective AAT effects pertaining to PR3- and MPO-ANCA.MethodsPlasma and blood neutrophils were assessed for PR3 and AAT. WT, mutant, and oxidation-resistant AAT species were produced to characterize AAT-PR3 interactions by flow cytometry, immunoblotting, fluorescence resonance energy transfer assays, and surface plasmon resonance measurements. Neutrophil activation was measured using the ferricytochrome C assay and AAT methionine-oxidation by Parallel Reaction Monitoring.ResultsWe found significantly increased PR3 and AAT pools in patients with both PR3- and MPO-AAV; however, only in PR3-AAV did the PR3 pool correlate with the ANCA titer, inflammatory response, and disease severity. Mechanistically, AAT prevented PR3 from binding to CD177, thereby reducing neutrophil surface antigen for ligation by PR3-ANCA. Active patients with PR3-AAV showed critical methionine-oxidation in plasma AAT that was recapitulated by ANCA-activated neutrophils. The protective PR3-related AAT effects were compromised by methionine-oxidation in the AAT reactive center loop but preserved when 2 critical methionines were substituted with valine and leucine.ConclusionPathogenic differences between PR3- and MPO-AAV are related to AAT regulation of membrane-PR3, attenuating neutrophil activation by PR3-ANCA rather than MPO-ANCA. Oxidation-resistant AAT could serve as adjunctive therapy in PR3-AAV.FUNDINGThis work was supported by KE 576/10-1 from the Deutsche Forschungsgemeinschaft, SCHR 771/8-1 from the Deutsche Forschungsgemeinschaft, grant 394046635 — SFB 1365 from the Deutsche Forschungsgemeinschaft, and ECRC grants.     

Plasmapheresis for removal of myeloperoxidase antineutrophil cytoplasmic antibodies: a case report.

We report on a patient with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated glomerulonephritis who had an elevated MPO-ANCA level and necrotizing crescentic glomerulonephritis on renal biopsy. She was treated by double filtration plasmapheresis and immunoadsorption plasmapheresis combined with steroid therapy and immunosuppressive agents. After plasmapheresis, the MPO-ANCA level decreased, and the cellular crescents were reduced. We conclude that plasmapheresis combined with steroid and immunosuppressive therapy may be useful to decrease the activity of MPO-ANCA associated crescentic glomerulonephritis.     

Plasmapheresis in the treatment of rapidly progressive glomerulonephritis.

The present study was carried out to examine the efficacy of plasma exchange in patients with rapidly progressive glomerulonephritis (RPGN). Seventeen patients with RPGN were treated with plasmapheresis as adjunct to immunosuppressive therapy. Of these, 4 had antiglomerular basement membrane (GBM) antibody-mediated glomerulonephritis (GN), 8 had immune-complex GN (5 SLE, 2 HSP, 1 cryoblobulinemia), 5 had pauci-immune GN (3 peripheral antineutrophil cytoplasmic antibody [P-ANCA], 1 cytoplasmic antineutrophil cytoplasmic antibody [C-ANCA], 1 other). Treatment of 10 of these patients with plasmapheresis within the first month of disease onset resulted in a stable renal function for a period extending from 1 to 3 years, except in 2 patients who had high baseline levels of serum creatinine. In the remaining patients, 2 were treated with hemodialysis 6 years later at the end of follow-up. We conclude that plasmapheresis, when used in combination with immunosuppressive drugs, is beneficial, leading to improved renal function.     

急进性肾炎的诊治进展

急进性肾小球肾炎(rapidly progressive glomerulonephritis,RPGN)以急性肾炎综合征伴肾功能急剧恶化为临床特征,病理为新月体肾炎.早期诊断主要依靠血清免疫学检查及尽早肾活检.早期治疗应根据病理类型及患者情况采用个体化的治疗方案,包括强化治疗和免疫抑制治疗.     

Efficacy of plasma exchange in severe idiopathic rapidly progressive glomerulonephritis. A report of ten cases

The vast majority of patients with idiopathic rapidly progressive glomerulonephritis (RPGN) develop irreversible renal failure within weeks to months. We report a retrospective study of 10 patients with idiopathic RPGN who were treated with plasma exchange in addition to steroids and immunosuppression. Renal biopsies were obtained in nine patients. RPGN without immune complexes was present in four, immune complex disease in four, and anti-glomerular basement membrane disease in two. Renal function did not recover in those patients with anti-glomerular basement membrane disease. In contrast, seven of eight patients without auto-antibodies to basement membranes responded to therapy, and four did not need dialysis for two years or more. The sustained improvement in renal function in these seven patients suggests the need for evaluation of plasma exchange as an adjunct to steroids and immunosuppression in a prospective, controlled study in RPGN.     

Plasmapheresis in the treatment of rapidly progressing glomerulonephritis

The authors are of the opinion that plasmapheresis (PP) combined with immunosuppressant therapy is an effective and a relatively safe method for the treatment of rapidly progressing glomerulonephritis (RPGN). Introduction of PP in multimodality treatment of RPGN made it possible to arrest rapidly progressing renal failure in all 6 treated patients. After PP treatment was over, renal function was recovered completely in 3 patients. One patient manifested the retention of renal failure of medium degree while rare hemodialysis sessions permitted one to control water-electrolyte disorders. In two patients the discontinuation of PP treatment resulted in the progress of renal failure. The data obtained do not make it possible to relate the improvement of renal function exclusively to the action of PP, since all the patients received immunosuppressants. Nevertheless, in 2 cases, the improvement could be attributed to PP, for its discontinuation in these patients (without any changes in the remaining treatment) brought about again the progress of renal failure.     

Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge

BackgroundInfective endocarditis (IE)-associated rapidly progressive glomerulonephritis (RPGN) is rarely reported. Sporadic case reports have noted the diagnostic and therapeutic challenge in IE-associated glomerulonephritis because it may masquerade as idiopathic vasculitis.MethodsPatients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed.ResultsTwenty-four patients with IE-associated RPGN were identified. All patients presented with fever and multiorgan system involvement on top of heart and kidneys, spleen (79%, 19/24), skin (63%, 15/24), lung (33%, 8/24) and nervous system (17%, 4/24). Six of the 24 patients (25%) were initially suspected to have ANCA-associated or IgA vasculitis. Forty-five percent of patients are seropositive for ANCA. Renal histology showed mesangial and/or endocapillary hypercellularity with extensive crescents in most patients. C3-dominant deposition was the predominant pattern on immunofluorescence and pauci-immune necrotising crescentic glomerulonephritis was observed in one case. All patients received antibiotics with or without surgery. Six patients received immunosuppressive therapy before antibiotics due to misdiagnosis and seven patients received immunosuppressive therapy after antibiotics due to persistence of renal failure. Three of the 24 patients died due to severe infection. All the surviving patients had partial or complete recovery of renal function.ConclusionIE-associated RPGN is rare and the differential diagnosis from idiopathic vasculitis can be challenging due to overlaps in clinical manifestations, ANCA positivity and absence of typical presentations of IE. The prognosis is generally good if antibiotics and surgery are not delayed. The decision on introducing immunoruppressive treatment should be made carefully on a case by case basis when kidney function does not improve appropriately after proper anti-infective therapy.

Key messages

• Infective endocarditis associated RPGN is rare and differentiating it from idiopathic vasculitis can be challenging due to overlap in clinical manifestations, ANCA positivity and occasional absence of typical manifestations of infective endocarditis.
• Kidney function usually responds to antibiotic therapy alone.
• Immunosuppressive therapy may be beneficial in carefully selected patients whose kidney function does not improve with antibiotics alone.

     

The partial recovery of kidney function in chronic uremia patients during hemodialysis treatment

Out of 432 patients placed on the treatment with hemodialysis (HD) for terminal renal failure (TRF) at the All-Union Nephrologic Center from January 1, 1978 to December 31, 1987, 17 patients manifested partial recovery of renal function, which enabled dialysis treatment to be discontinued for a time. Among the 17 patients with noticeable improvement of renal function, 8 presented with lupoid rapid-progressing glomerulonephritis (RPGN), 2 with RPGN associated with hemorrhagic vasculitis, 1 with idiopathic RPGN, 4 with chronic glomerulonephritis (CGN), 1 with chronic pyelonephritis, and 1 with polycystic kidneys. In 11 patients with RPGN, the rate of renal failure progression, expressed by the regression coefficient, was much higher among those in whom HD treatment was discontinued that in the group of patients without renal function recovery. In the 4 patients with CGN, renal function was recovered after the correction of marked disorders of purine metabolism, whereas in the 1 patient with chronic pyelonephritis and in the 1 with polycystic kidneys after urinary infection elimination. According to the ultrasonography data, out of the 17 patients with partial recovery of renal function, the size of the kidneys turned out normal in 14 patients.     

Determination of anti-neutrophil cytoplasmic antibodies in small vessel vasculitis: Comparative analysis of different strategies

BACKGROUND: Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with primary small vessel vasculitis (SVV). Proteinase-3 (PR3)-ANCA are primarily associated with Wegener granulomatosis, whereas myeloperoxidase (MPO)-ANCA are primarily associated with microscopic polyangiitis (MPA) and vasculitic Churg-Strauss syndrome. We evaluated whether a strategy that is based on screening with ELISA or fluoroenzymeimmunoassay (FEIA) is an accurate alternative to screening with indirect immunofluorescence (IIF). METHODS: C-ANCA and P-ANCA were determined by IIF and PR3-ANCA and MPO-ANCA were determined by ELISA (Inova) or FEIA (Phadia) on 326 patients (38 with newly diagnosed SVV and 288 diseased controls). RESULTS: Specificity and positive likelihood ratios were higher for ELISA and FEIA than for IIF. Post-test probability for SVV of a positive test result was higher for ELISA and FEIA than for IIF. Decision tree analysis in which several testing strategies were compared revealed that a testing strategy that is based on screening with ELISA or FEIA had an expected clinical utility that was comparable to screening with IIF and confirming with ELISA or FEIA. The highest expected clinical utility was found when both IIF and ELISA or FEIA were performed on all samples. CONCLUSIONS: A strategy based on screening for ANCA with ELISA or FEIA (without prior IIF) is a valuable alternative to screening with IIF and confirming with ELISA or FEIA.     

两种方法联合检测自身免疫性疾病中ANCA的临床意义

目的评价间接免疫荧光法（IIF）和免疫印迹法检测抗中性粒细胞胞质抗体（ANCA）的临床意义。方法采用IIF和免疫印迹法联合对126例自身免疫性疾病患者的血清进行ANCA检测。结果在126例患者中类风湿关节炎（RA）、韦格纳肉芽肿瘤（WG）、皮肌炎（DM）和过敏性紫癜患者血清检测cANCA阳性与PR3-ANCA阳性是一一对应的;而在51例SLE患者中,有17例（33.3%）pANCA阳性,7例（13.7%）骨髓过氧化物酶（MPO）-ANCA阳性;在29例RA中3例（10.3%）pANCA阳性,而4例（13.7%）MPO-ANCA阳性。WG和ANCA相关性血管炎患者检测ANCA阳性率较高,分别为87.5%和93.7%。结论IIF一般情况下可以作为临床常规检测ANCA的筛选试验,免疫印迹法可以作为特异ANCA确证试验。二者联合应用可以提高ANCA的检出率,减少ANCA的误诊率。     

Urinary macrophage counts and ratio to T lymphocytes: Possible use in differential diagnosis and management of glomerular disease

The noninvasive method that can differentiate hematuria-positive patients has not yet been developed. We evaluated the clinical value of the analysis of mononuclear cells in urine in combination with urinary erythrocytes as a noninvasive differential diagnostic tool of glomerular disease. The number of macrophages (CD14⁺ cells/ml urine) and T-lymphocytes (CD3⁺ cells/ml urine) were measured by flow cytometry using samples of freshly voided urine from 203 patients with hematuria. They had various types of proliferative glomerular disease, including rapidly progressive glomerulonephritis (RPGN), IgA nephropathy (IgAN), and membranoproliferative glomerulonephritis (MPGN), or nonproliferative glomerulopathy including idiopathic renal hematuria and hereditary nephropathy. Urinary macrophage counts increased significantly with the severity of glomerulonephritis; their number consistently exceeded that of T-lymphocyte counts in patients with active proliferative glomerulonephritis. Urinary macrophage counts in patients with proliferative GN were consistently higher than those of hematuria-matched nonproliferative GN. Moreover, urinary macrophage counts in patients with RPGN were significantly higher than those of MPGN and IgAN. Most of the patients with inactive proliferative glomerulonephritis or with nonproliferative glomerulopathy showed no marked increase in urinary macrophages. Although some patients with nonproliferative glomerulopathy who exhibited gross hematuria showed a slight increase in urinary macrophage counts, such counts were consistently lower than those of T lymphocytes. These observations suggests that urinary macrophage count and its ratio to T-lymphocyte count may provide useful information for clinicians in managing patients with proliferative glomerular disease as well as deciding whether to conduct renal biopsy in patients with hematuria. © 1996 Wiley Liss, Inc.     

Computed Tomography Image Analysis Before and After Treatment of Anti-Neutrophil Cytoplasmic Antibody−Associated Pulmonary Interstitial Fibrosis in 8 Patients

PurposeThe purpose of this study was to observe the treatment response of anti-neutrophil cytoplasmic antibody (ANCA)−associated pulmonary interstitial fibrosis in 8 patients before and after glucocorticoid or immunosuppressive therapy.MethodsThe clinical features and computed tomography imaging findings of the 8 patients in our hospital from October 2011 to October 2013, were retrospectively analyzed.FindingsMean age of the 8 patients was 72.6 (range 60−80) years. Five patients exhibited cough, sputum, and chest tightness, including 2 patients with fever. One patient developed hemoptysis, 1 patient exhibited abnormal urinalysis and developed renal insufficiency, and 1 patient developed limb pain. Two patients exhibited high urine erythrocytes and 2 patients had renal dysfunction and urinary abnormalities. One of the latter patients, upon renal biopsy, had focal proliferative necrotizing glomerulonephritis (consistent with vasculitis damage) with stage II to III mild nephropathy. Seven cases were anti−myeloperoxidase-ANCA, and 1 case was anti−proteinase 3-ANCA. All 8 cases exhibited streaks and grid shadows in chest imaging; 2 cases exhibited limited ground-glass patches; 1 case displayed multiple large patches of exudative shadows, indicating diffuse alveolar hemorrhage; 2 cases exhibited obvious honeycomb manifestations; and 1 case exhibited significant traction bronchiectasis. The ground-glass opacities disappeared after corticosteroid or immunosuppressive therapy; however, for streaks and grid shadows, no significant changes in the images were observed after treatment from 2 weeks to 10 months.ImplicationsANCA-associated pulmonary interstitial fibrosis most often in elderly patients with many complications. In these patients ground-glass opacities in computed tomography images, corticosteroid or immunosuppressant therapy may be effective. Clinicians should consider the poor efficacy and side effects of these therapies in the fibrosis stage of the disease.     

Erythrocytapheresis for Plasmodium falciparum infection complicated by cerebral malaria and hyperparasitemia

In malaria due to Plasmodium falciparum, life-threatening complications are in part related to the degree of parasitemia. Whole blood exchange and red blood cell exchange (RCE) have been used for the rapid removal of parasites from the circulation of patients with a high parasite load complicated by cerebral, pulmonary, and renal dysfunction. We have treated three 5-45-year-old patients with hyperparasitemia and end-organ dysfunction with red cell exchange by automated apheresis as an adjunct to specific anti-malarial chemotherapy. Parasitemia dropped more than 80% in all three patients immediately after the exchange, and all patients had an uneventful and full recovery. In combination with effective anti-malarial chemotherapy, apheresis RCE is a safe and rapid approach to treat complicated malaria due to P. falciparum.     

Analyses of registry data of patients with anti-GBM and antineutrophil cytoplasmatic antibody-associated (ANCA) vasculitis treated with or without therapeutic apheresis

Therapeutic apheresis (TA) as a treatment for antibody-associated vasculitis (AAV) was questioned by the PEXIVAS although the MEPEX study favored TA.The aim of this study was to evaluate the efficacy of TA to improve renal function in patients consecutively included in the WAA-apheresis registry versus patients not treated with TA.Materials and methodsIncluded were 192 patients that suffered from anti-glomerular basement membrane disease (anti-GBM, n = 28) and antineutrophil cytoplasmic antibody-associated vasculitis of MPO or PR3 origin. Of these 119 had performed TA and the other 73 had not performed TA for theses diagnoses (CTRL).ResultsElderly had an increased risk to die within 12 months (p = 0.002). All 28 anti-GBM had renal involvement, 21 dialysis dependent. At 3 month nine (36 %) did not need dialysis. Baseline data regarding renal function of AAV patients, subtype MPO and PR3, were worse in the TA groups than in CTRL. Recovery out of dialysis was better for the PR3-TA group compared with 1) the controls of MEPEX (RR 0.59, CI 0.43−0.80) and 2) the MPO-TA patients (RR 0.28, CI 0.12−0.68). The MPO-TA recovered similarly as the MEPEX-CTRL. Renal function improved most for TA-patients from baseline during the first 3 months (MPO-TA and PR3-TA) and stabilized thereafter and less for MPO-CTRL and PR3-CTRL.ConclusionPR3-TA patients seem to have best chances to get out of dialysis. PR3-TA and MPO-TA improved residual renal function better than CTRL. The present study recommends reconsiderations to use TA for AAV especially those with PR3-vasculitis with severe renal vasculitis.     

调节性T细胞对肾脏疾病的影响

目的研究调节性T细胞(CD4+CD25+)在肾脏疾病中的作用。方法选择住院患者72例,其中,原发性肾病综合征26例,慢性肾小球肾炎29例,其他原因致慢性肾功能衰竭17例;患者在接受治疗前检测血常规、生化分析、C反应蛋白(CRP)、凝血象和CD4+CD25+。结果肾病综合征组(肾功能正常者),CD4+CD25+%与D-二聚体负相关(P<0.01)。慢性肾小球肾炎组(肾功能正常者),CD4+CD25+%与CRP、PT、PT-INR、TT及D-二聚体正相关(均P<0.01)。慢性肾功能衰竭组,CD4+CD25+%与PT、D-二聚体正相关(P<0.05,0.01)。结论肾病综合征患者CD4+CD25+可减轻肾病综合征的病情发展;但CD4+CD25+对慢性肾小球肾炎和慢性肾功能衰竭患者,有改善机体凝血功能的作用。     

Idiopathic membranoproliferative (mesangiocapillary) glomerulonephritis: a clinicopathologic study.

In 51 patients with type 1 and 9 patients with type 2 membranoproliferative glomerulonephritis, we found a male predominance in both types, a wide age range but with younger patients having predominantly type 2 disease, and clinical presentations that varied and included the nephrotic syndrome, an abnormal urinalysis only, acute nephritis, and recurrent hematuria. Hypertension and impaired renal function at the time of first evaluation, which were present in more than one-third of the patients, presaged a poor prognosis; in most of these patients end-stage renal failure or worsening of renal function occurred. Acute nephritis at onset was also related to a deteriorating course and was especially frequent in patients with type 2 membranoproliferative glomerulonephritis. Retrospective analysis of treatment regimens, in which patients were given an average of 1 year of therapy with prednisone alone or combined with cytotoxic agents, showed no effect in patients who had progressive forms of the glomerulopathy.