Vasculitis and rapidly progressive glomerulonephritis in the elderly.

The proportion of patients with vasculitis and rapidly progressive nephritis aged 70 years or over has risen from about 10% in the 1980s to over 30% in series reported in the 1990s. This study was undertaken to examine the presentation and outcome of such older patients. Seventeen of 56 patients (30%) who presented at two renal units were aged 70 years or over. Mean creatinine level at presentation was 530 mumol/l, and five patients received dialysis at presentation. Outcome was dependent on three factors, namely comorbid pathology, response to immunosuppressive therapy, and the occurrence in three cases of temporary spontaneous partial remission. Overall patient survival at one and two years was 62.5% and 50%, respectively, and 90% and 100% of surviving patients were independent of dialysis at one and two years, respectively. Response to chemotherapy was excellent, with full rehabilitation in many cases and no deaths directly attributable to adverse effects of immunosuppressive therapy. We conclude that diagnosis of vasculitis and rapidly progressive glomerulonephritis by renal biopsy and the subsequent administration of chemotherapy (including cyclophosphamide in many cases) resulted in a worthwhile benefit in these elderly patients.     

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

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Corticosteroid agents in renal disease

Corticosteroid agents have a major role in the treatment of several renal disorders which have an immune basis. They remain the treatment of choice for minimal-change glomerulonephritis, inducing remission in over 90% of patients. The role of corticosteroid therapy in patients with membranous glomerulonephritis remains controversial, although an extensive controlled trial indicated benefit from a two-month course of alternate-day therapy. Intravenously-administered methylprednisolone has been shown to benefit rapidly progressive crescentic glomerulonephritis; the benefit is probably comparable to that which is obtained with immunosuppression and plasma exchange. Corticosteroid therapy has improved dramatically the prognosis of glomerulonephritis that is associated with systemic lupus erythematosus and the various forms of vasculitis (excluding Wegener's granulomatosis), although the concomitant use of immunosuppressive therapy in these disorders reduces the required doses of corticosteroid drugs. For the last 20 years prednisolone and azathioprine have been standard therapy to prevent renal allograft rejection. However, corticosteroid agents are used currently in much lower doses or have been replaced by cyclosporin A.     

慢性肾功能不全患者的病理类型分析

目的分析肾活检时表现为慢性肾功能不全患者（Scr≥130μmol/L）的患者病理类型及特点。方法选择我院2006年1月～2008年12月行肾脏活检时表现为血清肌酐升高（Scr≥130μmol/L）的所有慢性肾脏病患者共104例作为研究对象,分析其临床和病理特点。同时重点分析了增生硬化型IgA肾病的特点。结果 104例慢性肾脏病患者在肾活检时表现为肾功能异常,占同期肾活检患者的11.9%。这些患者的平均血清肌酐（235.11±114.4）μmol/L。临床诊断主要为：慢性肾小球肾炎63例,肾病综合征27例,系统性血管炎4例,系统性红斑狼疮3例。其中78例（75.0%）患者有高血压。病理类型主要表现为IgA肾病（46.2%,其中有70.8%的患者主要表现为增生硬化性肾炎）,其次是间质性肾炎,系膜增生性肾小球肾炎,新月体肾炎,局灶节段肾小球硬化,IV型狼疮性肾炎。其中,增生硬化型的IgA肾病患者血压升高更为突出,但是尿蛋白量更少。结论表现为慢性肾功能不全的患者病理上主要为IgA肾病,其中增生硬化性肾炎是主要病理表现,其次是肾小管间质病变。慢性病变是主要的病理特点。     

显微镜下多血管炎69例临床分析

目的：分析显微镜下多血管炎（MPA）临床表现、病理特点、治疗及预后。方法回顾性分析北京协和医院2007年9月至2012年9月住院的69例MPA患者资料。结果①男30例,女39例,平均65岁。②临床表现：78.2%发热,46.3%体重下降;肾脏主要表现为中小量蛋白尿（76.8%）、血尿,部分呈急进性肾小球肾炎（23.2%）,肾脏病理特点为新月体形成、坏死性血管炎;肺部表现以肺间质纤维化（55.1%）和肺泡出血（8.7%）为主;其他包括周围神经病变（23.2%）、皮疹（17.4%）、消化道出血（13.0%）等。③65例（94.2%）抗中性粒细胞胞浆抗体（ANCA）阳性,63例为髓过氧化物酶（MPO）阳性。④全部接受糖皮质激素治疗（1/4冲击）,超过90%接受环磷酰胺治疗,9例血液透析,3例血浆置换。⑤经治疗,63例好转出院,6例因肺部感染、感染性休克死亡。结论 MPA多发于老年患者,肾脏受累突出,病理特点多见于新月体形成和坏死性血管炎,肺部、周围神经、皮肤病变等,以MPO-ANCA阳性为主,激素、免疫抑制剂治疗可改善预后。     

LUPUS NEPHRITIS-A CRITICAL ANALYSIS OF 17 PATIENTS

Over a 3 year period from June 94 to June 97, out of 28 patients of systemic lupus, 17 were diagnosed as renal lupus. Demographic data showed 12 females and 5 males, mean age being 32.2 years (range 12 to 54 years). Mean time gap between presentation and definitive diagnosis was 32.4 days (7 days to 5 years). 2 patients (11.76%) presented renal lupus, one (5.88%) with acute interstitial lung disease and the remaining had the usual systemic manifestations of lupus. Anti dsDNA antibodies were positive in all patients while ANA was negative in 3 cases. Renal involvement consisted of rapidly progressive glomerulonephritis in 2 patients (11.76%), nephrotic syndrome in 4 (23.52%) and non nephrotic range proteinuria in 11 (64.70%) patients. Mean serum creatinine at presentation was 2.4mg/dl (0.8mg/dl to 8.9 mg/dl). Three patients were dialysis dependent. Renal histology on light microscopy comprised of class II lesions in one (5.88%), class III in 4 (23.52%), class IV in 11 (64.70%-including one with crescents) and class V in one (5.88%) patient. All patients with advanced class III/IV lesions were treated with corticosteroids and cyclophosphamide pulses. Except one patient who died of pyopericardium all others improved and their serum creatinine stabilised around 2.3 mg/dl (0.8 to 4.6 mg/dl). The study highlights the importance of early diagnosis and aggressive management in this potentially treatable disease.KEY WORDS: Lupus nephritis, Pulse cyclophosphamide     

Prognosis of microscopic polyangiitis with renal involvement: report of 60 Chinese patients

With the widespread clinical application of renal biopsy and serum antineutrophil cytoplasmicantibody （ANCA） testing, the percentage of renal injuries caused by systemic small vessel vasculitis （ SVV ） including microscopic polyangiitis （ MPA ） and Wegener＇ s granulomatosis （WG） is on the rise in China. According to our previous report, SVV constituted 1.09% of diagnoses from all renal biopsies （5. 8% in secondary nephritis） and 14.4% of that from renal biopsies from patients with chronic renal failure,     

Systemic Vasculitis with Renal Involvement — A Review

Twenty five patients with renal vasculitis presenting over an eight year period were reviewed. Ten had microscopic polyarteritis, 6 classic polyarteritis, 5 overlap syndrome, 2 Churg-Strauss syndrome and 2 Wegener’s granulomatosis. Clinical features included hypertension, pulmonary involvement, neurological involvement and arthralgia. Serum creatinine was over 500 umol/1 in 13 patients, 10 of whom required dialysis. Visceral angiography was positive in 80% of those studied, Focal and segmental necrotising glomerulonephritis was the commonest renal lesion. Treatment consisted of corticosteroids and cytotoxic agents in most cases. Plasmapheresis was used for rapidly progressive renal failure, severe pulmonary haemorrhage or cerebral vasculitis. Improvement or stabilisation of renal function was seen in 68% of patients treated. There were 4 early deaths and one late death. The diagnosis, histology, treatment and outcome of renal vasculitis is discussed. The importance of early diagnosis and treatment is emphasised in this potentially reversible cause of acute renal failure.     

Mycophenolate mofetil in low doses stabilizes and improves antineutrophil cytoplasmic antibody-associated vasculitis and lupus nephritis

BACKGROUND: Clinical experience with mycophenolate mofetil (MMF) in glomerulonephritis still remains limited. METHODS: In order to assess the experience of one center with the efficacy and tolerability of MMF in patients with glomerulonephritis, we performed a retrospective 6-year analysis of 68 patients treated by MMF for glomerular disease, mainly anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV: n=34) and systemic lupus erythematosus and lupus nephritis (SLE: n=24). Indications were maintenance treatment in 40% of patients, induction treatment in patients not tolerating cyclophosphamide in 27%, and disease relapse in 33%. Mean treatment duration was 11.5 months. RESULTS: Efficacy endpoints were serum creatinine, urinary protein excretion, and steroid dose. In AAV patients, MMF was associated with significant improvement in 18%, partial improvement in 26%, stabilization in 29%, and disease progression in 12%; adverse event dropouts totalled 15%. In SLE, the respective figures were 30, 22, 9, and 22%, with 17% adverse event dropouts. The most frequent side effects were gastrointestinal events (n=7) and infections (n=3). None was life-threatening and there were no deaths. CONCLUSIONS: MMF, in the relatively low doses used, was safe and effective, stabilizing or improving AAV in 73% of patients and SLE in 61%. Further prospective randomized controlled trials with MMF in renal vasculitis and lupus nephritis are clearly warranted.     

CLINICAL AND PATHOLOGICAL MANIFESTATI—ONS OF PATIENTS WITH ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES DIRECTED AGAINST PROTEINASE 3 OR MYELOPEROXIDASE

INTRODUCTIONAntineutrophilcytoplasmicautoantibodies（ANCA）maybefoundinmanydiseases．Butantibodiesagainstproteinase３（anti－PR３）andantibodiesagainstmyeloper－oxidase（anti－MPO）arepredominantlyassociatedwithsystemicvasculitis．Tobetterdescribeandcompa     

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.     

以肾脏受累为主要表现的ANCA相关性小血管炎的诊治探讨

目的 探讨以肾脏受累为主要表现的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的早期诊断及适时合理治疗。方法 对21例肾脏受累为主要表现的ANCA相关性小血管炎患者的临床资料进行回顾性分析。结果 21例中pANCA阳性18例、cANCA阳性3例．4例表现为急进性肾炎综合征，其中2例伴有咯血；7例表现为慢性肾衰，其中鼻中隔穿孔2例、失明2例；5例表现为急性肾衰；5例以尿检异常、乏力、持续低熟、贫血、体重下降等表现入院。7例经甲基强的松龙、环磷酰胺冲击治疗，病情稳定，肾功能好转或正常；3例经甲基强龙、环磷酰胺冲击治疗配合血液透析，肾功能恢复正常；3例仍需维持性血透；2例死于透析后心衰、1例死于突发肺出血并消化道出血；5例经用激素治疗(未用MP、CTX冲击治疗)，临床症状消失，肾功能正常。结论 伴有肾脏受累的ANCA相关性小血管炎临床表现缺乏特异性．早期诊断、早期适当治疗是改善预后的关键。     

丙基硫氧嘧啶致抗中性粒细胞胞浆抗体阳性患者的临床特点

目的探讨丙基硫氧嘧啶(PTU)导致抗中性粒细胞胞浆抗体(ANCA)阳性病例的临床特点,以提高对PTU导致ANCA阳性血管炎(APV)的认识。方法分析北京协和医院自2000年以来9例因应用PTU导致的ANCA阳性病例临床资料,并进行相关文献复习。结果(1)9例患者发病年龄16—51岁(平均33．1岁),服用PTU时间3—84个月(平均32．4个月);(2)6例进行了ANCA靶抗原谱的测定,除抗髓过氧化物酶(MPO)外,均同时存在识别其他抗原的抗体,包括：抗蛋白酶3(PR3)、抗人白细胞弹力蛋白酶(HLE)、抗杀菌／通透性增高蛋白(BPI)和抗乳铁蛋白(LF);(3)6例核周型ANCA(pANCA)高滴度阳性(≥1：1280)的病例临床确诊为APV(均有肾脏受累,其中5例为肾穿刺病理证实),另外3例pANCA低滴度阳性(≤1：320)的病例无明确血管炎表现;(4)8例患者被发现ANCA阳性后停用PTU,其中1例APV仅停用PTU后病情缓解,另外4例APV同时予糖皮质激素和免疫抑制剂后病情逐渐缓解,而1例继续服用PTU随诊18个月pANCA滴度未升高。结论PTU可引起ANCA阳性,而高滴度pANCA阳性提示APV,其病情与抗体滴度相关;及时停用PTU,根据肾穿刺病理的病变程度确定治疗方案,对改善预后意义重大。     

A case of Henoch-Schoenlein purpura nephritis recurring after 3 years remission

Henoch-Schoenlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries with IgA immune complexes deposition, and about 7% of patients with HSP experience recurrence. Most patients with recurring of HSP nephritis show a recurrence of clinical symptoms over a period ranging from 2 to 5 months, even after the disappearance of initial symptoms. Here we report a 9-year-old girl diagnosed with recurrent HSP and severe crescentic glomerulonephritis 3 years after complete resolution of the initial symptoms of HSP. Our case is unique in respect of the recurrence at more than 3 years after the complete resolution of initial symptoms, suggesting that careful followup is required in spite of improved renal symptoms in cases of HSP.     

Systemic complications of acute glomerulonephritis in Nigerian children

This study was undertaken to: (i) determine the prevalence, pattern and outcome of systemic complications of acute glomerulonephritis (AGN), and (ii) evaluate some clinicolaboratory features of the disease in Nigerian children. Clinical and laboratory records of consecutive cases of AGN seen over a period of 3 years in our unit, were prospectively entered into nephrology record forms and later analysed. Some of the analysed data included age, sex, blood pressure, types of systemic complication, haematocrit, plasma electrolytes, urea, creatinine, protein and albumin. Fractional excretion of filtered sodium (FeNa, %) and 24-hours urinary protein concentration data were also analysed. Majority of the patients (18/29) were under 6 years of age, with peak age incidence of 3 years. The hospital incidence of AGN and prevalence of systemic complications were 10 new cases per year and 41.38%, respectively. Heart failure (HF) and acute renal failure (ARF) were sole systemic complications in 7 and 2 AGN patients, respectively. Three patients had double systemic complications: one each of hypertensive encephalopathy (HTE)+HF, HTE+ARF and ARF+HF. Ten of 29 patients (34.48%) had nephrotic range proteinuria. None of the AGN patients except those with ARF had FeNa >1%, plasma bicarbonate <15 mmol/l, urea 225 mmol/l and creatinine 2400 mmol/l. Two of the patients died: one each of ARF and ARF+HF, giving a case fatality and mortality rate of 6.90% and 0.08%, respectively. ARF is clearly the principal risk factor for mortality in our AGN patients. Its early detection and aggressive but purposive management which must include dialysis, will certainly improve outcome in AGN cases complicated by ARF.     

Monthly plasmapheresis for systemic lupus erythematosus with diffuse proliferative glomerulonephritis: a pilot study

Twelve patients with systemic lupus erythematosus and biopsy-proved diffuse proliferative glomerulonephritis were randomly allocated to a control group (to continue receiving conventional therapy only) or to a plasmapheresis group (to receive conventional therapy along with one 4-I plasma exchange a month). The six patients treated with plasmapheresis had better preservation of renal function, reduced disease activity, fewer admissions to hospital and less need for steroid and immunosuppressive therapy than the six control patients. The patients treated with plasmapheresis also showed evidence of reduced immunologic activity and had no side effects attributable to the plasma exchange. These results suggest that monthly plasma exchange should be assessed in a controlled randomized trial as a possible therapeutic adjunct in patients with systemic lupus erythematosus and diffuse proliferative glomerulonephritis.     

系统性小血管炎临床分析

目的总结系统性小血管炎的临床表现及其转归。方法对42例确诊为系统性小血管炎患者的临床资料进行回顾性分析。其中男24例,女18例;平均发病年龄（60.3±15.4）岁,60岁以上者共27例,占64.3%。分析其临床表现、实验室检查、影像学检查、治疗方法、疗效、并发症及转归等。结果发热在首诊症状以及主要临床表现中均占首位,分别占64.3%和85.7%,其它显著的症状包括乏力、消瘦等非特异性症状。最常受累的器官是肺和肾脏,主要症状分别为咳嗽、痰血、咯血和气急,占88.1%,以及蛋白尿和血尿,占81.0%;其中肾功能减退的患者占40.5%。92.9%的患者有3个以上脏器受累。35例患者血清抗中性粒细胞浆抗体（ANCA）阳性,其中核周型ANCA阳性24例,胞浆型ANCA阳性11例;4例血清ANCA阴性;3例无ANCA资料。94.6%的患者对于免疫抑制治疗敏感,完全缓解率和部分缓解率分别为37.8%和56.8%。共6例患者死亡,其中5例死于多器官功能障碍综合征合并严重感染,1例为急性消化道大出血。结论系统性小血管炎是一种以老年人群为主要发病对象的系统性疾病,大多数患者有全身症状,受累脏器多,诊断困难,借助ANCA或组织学检查可使多数患者得到确诊。绝大多数患者都对免疫抑制治疗反应良好,感染是主要死因之一。     

丙基硫氧嘧啶诱发的抗中性粒细胞胞质抗体阳性甲状腺功能亢进患者的临床研究

目的研究丙基硫氧嘧啶（PTU）诱发的抗中性粒细胞胞质抗体（ANCA）阳性的甲状腺功能亢进症（甲亢）患者的临床和预后特点。方法对45例PTU诱发的ANCA阳性的甲亢患者进行长期随访（3～60个月,中位数27个月）,观察其甲状腺功能和ANCA百分结合率的变化,对其中17例有小血管炎临床表现的患者进一步观察血管炎的病情变化。结果（1）血管炎组17例患者中13例（76．5％）肾脏受累,表现为血尿、蛋白尿及不同程度的肾功能受损,7例（41．2％）肺脏受累,多表现为肺出血。核周型ANCA（p-ANCA）阳性率为100％,其中16例（94．1％）髓过氧化物酶（MPO）-ANCA抗体阳性。所有患者均停用PTU,6例改用他巴唑治疗甲亢,13例内脏受累较重的患者应用免疫抑制剂治疗6～12个月。除2例诊治较晚的患者发展至终末期肾衰竭外,其余患者临床症状均得到缓解,随访期间（8～60个月,中位数30个月）未发现小血管炎复发。（2）无血管炎临床表现组28例患者中有27例（96．4％）为p-ANCA阳性,14例（50．0％）的MPO—ANCA阳性。24例患者停用PTU,其中15例改用他巴唑;4例患者继续应用小剂量PTU。随访期间（3～36个月,中位数20个月）均未发生小血管炎临床表现。但血管炎组及无血管炎组多数患者ANCA百分结合率下降较慢。结论PTU诱发的ANCA阳性甲亢患者应及时停用PTU,必要时改用他巴唑治疗甲亢;血管炎病情严重者应用免疫抑制治疗。停用PTU后未发现小血管炎复发,但ANCA可较长时间维持阳性。     

Gastrointestinal Cytomegalovirus infection in non-human immunodeficiency virus infected patients

This is a retrospective study of fourteen patients who had proven Cytomegalovirus (CMV) infection of the gastrointestinal tract with no Human Immunodeficiency virus infection. The median age was 60.5 (Range 28 to 81) years. Eight patients were below (Group 1) and six above sixty five years old (Group 2). Areas of gastro-intestinal involvement were: oesophagus (2), stomach (1), colon (10) and multiple sites (1). Seven patients from Group 1 had received immunosuppressive therapy at the time of presentation and one had diabetes mellitus. We found a high prevalence of co-morbidities such as chronic renal failure and diabetes mellitus in Group 2. At median follow up of 13.9 months, there was a mortality rate of 50%. Only four patients were treated with ganciclovir. Our study concludes that the gastrointestinal CMV diseases in young patients were associated with immunosuppression whereas the older patients had chronic renal failure or diabetes.