新月体性过敏紫癜性肾炎与新月体性IgA肾炎的比较

目的:比较新月体性过敏紫癜性肾炎(HSPCN)与新月体性IgA肾炎(IgACN)临床病理特征及预后的异同。方法:对1985至2004年在解放军肾脏病研究所住院肾活检确诊(新月体形成率?50%)的HSPCN和IgACN患者进行流行病学、临床表现、病理特征及预后的回顾性分析。结果:HSPCN和IgACN患者各24例,分别占同期294例新月体性肾炎患者的8·16%。HSPCN和IgACN组肾脏病程分别为(3·84±6·40)月和(5·91±9·75)月(无统计学差异)。两组患者临床多表现为肉眼血尿(HSPCNvsIgACN:62·5%vs70·8%)和大量蛋白尿(95·8%vs100%),HSPCN与IgACN组表现为急进性肾功能减退者分别为15例(62·5%)和22例(91·7%),但HSPCN组高血压及肾功能损害程度均较IgACN组轻,HSPCN组仅4例(16·7%)出现中重度肾功能减退(血清肌酐≥265μmol/L),IgACN组11例(45·8%)出现中重度肾功能减退。肾组织病理检查:两组袢坏死(58·3%vs54·2%)、节段性新月体(53·2%vs60·0%)、球囊壁粘连(91·7%vs62·5%)等节段性病变发生率均较高;肾小球硬化(平均球性硬化率3·32%vs17·6%)、间质纤维化(半定量评分0·38±0·58vs0·83±0·89)和肾小管萎缩(半定量评分0·71±0·86vs1·58±0·97)等慢性化病变程度HSPCN组轻于IgACN组。两组患者肾小球免疫病理均以IgA在系膜区沉积为主(伴或不伴血管袢沉积),其中伴C3沉积者HSPCN组显著少于IgACN组(75%vs100%)。IgG、IgM、C4和C1q沉积两组无显著差异。HSPCN肾组织炎细胞浸润程度更严重。HSPCN组患者平均随访时间(46·7±30·5)个月(13~120月,中位时间40个月),5年肾存活率91·7%;IgACN组患者平均随访时间(54·8±30·7)个月(17~144个月,中位随访时间49个月),5年肾存活率77·8%。结论:HSPCN与IgACN具有相似之处:肉眼血尿和大量蛋白尿发生率高,中重度肾功能减退发生率低,肾组织节段性病变多见,二者存在明显的不同之处:HSPCN较IgACN肾功能损害程度及肾组织慢性化病变程度轻,预后优于IgACN。     

Fractalkine及其受体与新月体性肾炎

新月体性肾炎是一组病情进展急骤的肾小球肾炎,其共同特征是大部分肾小球伴有新月体形成.在新月体的形成过程中,炎性细胞的浸润起着关键作用,而炎性细胞的浸润和活化需要趋化因子的参与.因此,研究趋化因子及其受体超家族就显得很有必要,该领域目前研究进展迅猛,新近被发现的Fractalkine有着与其他趋化因子不同的结构与功能,本文概述了Fractalkine及其受体CX3CR1在新月体肾炎中的意义,展望了抗-CX3CR1抗体在治疗中的意义.     

霉酚酸酯与环磷酰胺治疗新月体性狼疮性肾炎的疗效比较

目的:回顾性对照研究霉酚酸酯(MMF)与环磷酰胺(CTX)治疗新月体性狼疮性肾炎(CLN)的疗效、复发率、远期预后和副作用的差异。方法:对1998年1月至2005年6月在解放军肾脏病研究所住院肾活检确诊(新月体形成率≥50%)CLN且经MMF或CTX诱导治疗的患者进行一般状况、临床表现、病理特征、治疗缓解率、复发率、副作用发生率及预后的回顾性对比分析。结果:52例CLN患者中,接受CTX治疗25例(2例失访),MMF治疗27例(1例失访)。CTX组和MMF组患者一般状况、临床表现及狼疮活动性指数(SLEDAI)、病理损害程度间无显著性差异,两组治疗12个月时缓解率分别为69.6%和73.1%,MMF组完全缓解率(53.8%)显著高于CTX组(26.1%),复发率(10.5%)则显著低于CTX组(43.8%),CTX组复发者多来自部分缓解者(占部分缓解患者的40.0%)。CTX组有部分患者出现药物副作用,包括肺部感染(3例,占13.0%)、肝损害(2例,占8.70%)和白细胞下降(1例,占4.35%);MMF组带状疱疹1例(占3.85%)、白细胞下降1例(占3.85%)。CTX组和MMF组随访时间分别为:10～80(38.5±21.2)月和12～90(41.1±27.0)月,无显著性差异。CTX组有2例(8.70%)患者分别在随访第48个月和第60个月进入终末期肾衰(ESRF),3例(13.0%)死亡,其中2例死于重症肺部感染,1例患者死于尿毒症后脑出血;MMF组有2例(7.41%)患者分别在随访第8个月和第24个月进入ESRF,无死亡病例。结论:与传统CTX方案比较,MMF治疗CLN取得更高的完全缓解率和低复发率,且感染副作用较CTX少见,安全性好。     

新月体性狼疮性肾炎患者的临床转归

目的 :了解新月体性狼疮性肾炎 (CLN)患者的临床转归。　 方法 :临床及病理检查确诊为CLN 4 9例 ,根据不同的免疫抑制治疗方法分为三组 :A组 (n =12 ) ,甲基强的松龙 (MP)冲击治疗 ;B组 (n =2 2 ) ,MP冲击合并间断环磷酰胺 (MP +CTX)静脉治疗 ;C组 (n =11) ,MP冲击合并霉酚酸酯 (MP +MMF)治疗。观察他们的近、远期疗效和临床转归。　 结果 :女性 4 7例 ,男性 2例 ,平均年龄 (30 3± 9 70 )岁 ,系统性红斑狼疮 (SLE)平均病程为(2 7 0± 2 6 1)月 ,狼疮肾炎 (LN)平均病程为 (15 8± 18 3)个月 ,平均随访时间为 (2 4 6± 19 3)个月。近期疗效 :患者血清肌酐 (SCr)从治疗前 (30 5± 2 16 ) μmol/L降至 (190± 14 6 ) μmol/L ,蛋白尿和贫血也均有不同程度的改善。临床有效者 14例 ,占 2 8 6 % ,临床缓解者 6例 ,占 12 2 %。远期疗效 :6 7 3%患者随访时间超过 6个月 ,其中B组 18例、C组 8例和A组 7例患者 ,他们平均随访时间为 (30 1± 2 6 8)个月。随访终了时临床有效者 2 1例 (42 9% ) ,其中B组14例、C组 5例和A组 2例。临床缓解者 10例 (2 0 4 % ) ,其中C组 6例、B组 3例和A组 1例。比较B组和C组患者的远期疗效无明显差异 ,而优于A组。测定B组和C组患者的 3年肾功能稳定率均在 88%以上 ,明显高于     

新月体型狼疮性肾炎1例报告

新月体型狼疮性肾炎是狼疮性肾炎的特殊类型 ,临床少见 ,且预后差。本院近期收治 1例 ,现报告如下。患者男 ,2 6岁 ,因双下肢及颜面部水肿 2 0天 ,伴恶心、少尿10天 ,于 2 0 0 1年 11月 2 8日收入院。查体 :Bp2 4/16k Pa(1k Pa=7.5 mm Hg) ,神志清 ,面部无红斑 ,双眼睑轻度水肿 ,口腔无溃疡。双肺可闻及干湿性罗音。心率 90次 /min,律齐。腹水征阳性。关节无红肿 ,双下肢中度水肿。即往健康。血常规 :WBC1.13× 10 9/L,Hb 68g/L,PL T2 78× 10 9/L,ESR5 0 .9mm/h。尿常规 :蛋白 (+ + + ) ,颗粒管型 (+ + ) ,蜡样管型 (+ ) ,离心镜检 …     

72例新月体肾炎的临床分析

本文分析经肾活检确诊的７２例新月体肾炎，占全部肾活检的１．３７％，按免疫病理分型，Ⅰ型９例（１２．５％），Ⅱ型４８例（６６．７％），Ⅲ型１５例（２０．８％），原发病因以狼疮性肾炎最多见（２５例），急进性肾炎次之（１８例），血管炎居第三（１０例），在贫血，高血压及肾功能损害程度上Ⅰ型重于Ⅱ型和Ⅲ型，而大量蛋白尿，肉眼血尿Ⅲ型多于Ⅰ和Ⅱ型，Ⅲ型新月体肾炎中ＡＮＣＡ，ＭＰＯ－ＡＮＣＡ，ＡＥＣＡ阳性率分别     

表现为新月体性肾小球肾炎的纤维性肾小球病

34岁女性患者,临床表现为水肿、高血压、大量蛋白尿、镜下血尿、低蛋白血症、血清肌酐升高。肾活检病理光镜示大量新月体形成伴袢坏死,电镜示上皮下、基膜内及系膜区杂乱分布的纤维样结构(直径12～30 nm)沉积,肾小球免疫组化染色DNAJ热休克蛋白家族成员B9(DNAJB9)阳性,诊断为DNAJB9相关纤维性肾小球病、新月体性肾小球肾炎。给予利妥昔单抗、吗替麦考酚酯、糖皮质激素等治疗,获得了较好的临床疗效。     

新月体肾炎20例临床和病理分析

目的分析新月体肾炎的临床表现、病理类型及其预后,以提高对本病的认识。方法2000年~2005年我院住院并经肾活检证实的新月体肾炎患者20例,我们对其临床、实验室检查、病理及随访资料进行回顾性分析。结果20例新月体肾炎患者,临床主要表现为蛋白尿、血尿、肾功能急剧减退、贫血、双肾大小正常或偏大。病理检查其新月体形成率为(82.83%±15.51%),袢坏死发生率为60%,球性硬化发生率为50%。其中3例为抗肾小球基底膜型(Ⅰ型),9例为免疫复合物型(Ⅱ型),8例为寡免疫复合物型(Ⅲ型)。20例患者均给予甲基强的松龙针(MP)加环磷酰胺(CTX)冲击或甲基强的松龙针加骁息治疗,10例(50%)未愈,7例(35%)好转(血肌酐有下降,但未正常),3例(15%)治愈(随访1~2年,血肌酐正常)。结论新月体肾炎临床表现及肾脏病理均损害严重,如能给予积极有效治疗,有50%的患者可避免透析治疗。     

狼疮新月体肾炎38例临床病理分析

目的　了解狼疮新月体肾炎 (CLN)患者临床和病理特征。方法　对 38例CLN的临床、血清学和病理改变进行分析归纳。并将肾功能异常和肾功能正常的CLN患者进行比较。结果　CLN患者均为女性。6 3 2 %临床表现为急进性肾炎综合症 ;5 2 %表现为慢性肾功能减退 ;31 6 %患者肾功能正常 ,临床表现为肾病综合征或慢性肾炎综合征。肾外表现主要为贫血、关节病变、皮疹和浆膜腔炎。病理上除新月体形成较多外 ,还有明显增殖性病变 (每个肾小球内细胞数 >15 0个者 ,81 6 % )、毛细血管袢坏死 (6 5 7% )、间质血管炎 (6 0 5 % )、间质大量炎细胞浸润 (92 1% )等明显活动性病变 ,平均活动指数高达 7 74± 2 6 8。结论　多数CLN患者临床表现较重为急进性肾炎综合症 ,但约有 1/ 3患者临床表现相对较轻 ,肾功能正常。两组CLN患者临床及血清学检查均具有较高的活动性 ,但肾功能正常患者病理上活动性病变更明显。从重复肾活检病例看 ,约半数CLN患者由其它类型转型所致。     

抗中性粒细胞胞浆抗体阴性的少免疫沉积型新月体性肾小球肾炎15例分析

目的　了解抗中性粒细胞胞浆抗体 (ANCA)阴性的少免疫沉积型新月体肾炎的临床病理特点。方法　对近 10年来诊断为少免疫沉积型新月体肾炎患者进行ANCA检测 ,并对其中ANCA阴性患者进行临床病理分析。结果　 33例少免疫沉积型新月体肾炎中 ,ANCA阴性患者 15例 ,占 4 5 % ,为同期所有新月体肾炎的15 %。其中 9例伴多系统受累 ,6例仅累及肾脏。 2例患者病理可见小血管炎。经积极治疗 ,1/13例临床痊愈 ,6 /13例临床缓解 ,6 /13例无效。结论　ANCA阴性少免疫沉积型新月体肾炎在临床、病理上与ANCA阳性病人相似 ,但好发年龄偏低 ,预后相对较差     

A case of anaphylactoid purpura associated with nephrosis followed by pulmonary tuberculosis

A 54-year old man was admitted to our hospital because of high fever, productive cough and purpura in both legs in June 2005. Urinalysis showed microscopic hematuria and proteinuria. Chest radiograph showed consolidation of right upper field. Because acid-fast bacilli and polymerase chain reaction test for Mycobacterium tuberculosis were positive in bronchial lavage fluid, we made a diagnosis of pulmonary tuberculosis, and prescribed antituberculosis therapy with isoniazid, rifampicin, ethambutol and pyrazinamide. In addition, anaphylactoid purpura was diagnosed by skin biopsy. In July 2005, renal function was deteriorated and nephrosis appeared. We treated with corticosteroid in addition to antituberculosis therapy. His symptoms and renal dysfunction improved. We report a rare case of an anaphylactoid purpura following occurence of pulmonary tuberculosis.     

A case of anaphylactoid purpura associated with tuberculous pleuritis

A 46-year-old man was admitted to Shin-Kokura Hospital because of fever and right chest pain. Laboratory studies showed mild leukocytosis, an increased erythrocyte sediment rate, positive C-reactive protein, and abnormal liver function. The tuberculin skin test was positive. A chest X-ray showed massive right pleural effusion, and exudative pleural effusion fluid was obtained by thoracocentesis. The pleural fluid revealed an increased adenosine deaminase concentration and cultures were negative for mycobacteria. A polymerase chain reaction test of the pleural effusion for Mycobacterium tuberculosis was positive. The patient was diagnosed with tuberculous pleuritis, and antitubercular therapy was started. The fever and chest pain was improved, but rashes appeared on the lower extremities. A biopsy of the skin lesion showed anaphylactoid purpura. Steroid ointment improved the skin lesion. Anaphylactoid purpura associated with tuberculosis is rare. The immunological response to mycobacteria may heve been related to the mechanism of the anaphylactoid purpura in this case.     

Immune thrombocytopenic purpura associated with pulmonary tuberculosis

A 22-year-old woman was admitted into our hospital because of generalized purpura and abnormalities in her chest X-ray. Isolated thrombocytopenia and elevated platelet-associated IgG levels were detected, while the bone marrow examination was normal. Mycobacterium tuberculosis was detected in the bronchoalveolar lavage fluid, and consequently she was diagnosed as having active tuberculosis. High-dose immunoglobulin therapy combined with anti-tuberculosis drugs not only rapidly and continuously corrected thrombocytopenia but also cured pulmonary tuberculosis. This case suggests a causal association between immune thrombocytopenia and tuberculosis as well as the safety and efficacy of the anti-tuberculosis drugs combined with high-dose immunoglobulin therapy.     

A case of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura

A 30-year-old Japanese woman with chest pain and nodules in the left upper lung field was diagnosed as having pulmonary tuberculosis by sputum examination. Purpura on her legs had lasted for 3 months and her platelet count was 1.9 × 10⁴/mm³ on admission. She was also diagnosed as having immune thrombocytopenic purpura because of elevation of serum PA-IgG and proliferation of megakaryocytes in the bone marrow. Anti-tubercular therapy and steroid therapy were concurrently performed, resulted in recovery of the platelet count. Steroid therapy was gradually tapered off and then withdrawn, thereafter anti-tubercular therapy was finished. She has been relapse-free.Cases of pulmonary tuberculosis accompanied with immune thrombocytopenic purpura are rare. The pathogenesis in the present case was suggested to have occurred through an immunological mechanism.     

A case report of pulmonary nocardiosis associated with bronchiectasis after pulmonary tuberculosis successfully treated with sparfloxacin

A 48-year-old female with bronchiectasis after pulmonary tuberculosis was admitted to our hospital because of bloody sputum and multiple cavity formation in the right lung. Chest X-ray & CT films revealed diffuse nodular shadows and cavity formation with a thick wall. Nocardia asteroides was isolated from the stum but no other pathogenic bacteria was isolated. Administration of sulfame-thoxazole-trimethoprim is not effective. The results of an MIC test for antimicrobial agents led to treatment with sparfloxacin and the clinical symptoms and cavity formations in the right lung improved. Nocardia asteroides may cause exacerbation of bronchiectasis.     

MPO-ANCA associated crescentic glomerulonephritis with numerous immune complexes: case report

ABSTRACT: BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) is a major cause of rapidly progressive glomerulonephritis (RPGN). ANCAassociated CGN is generally classified into pauci-immune RPGN, in which there are few or no immune complexes. Case Presentation A 78-year-old man presented with RPGN after a 7-year course of chronic proteinuria and hematuria with stable renal function. A blood examination showed a high titer of myeloperoxidase (MPO)-ANCA. A renal biopsy showed crescentic glomerulonephritis with abundant subepithelial, intramenbranous and subendothelial deposits by electron microscopy, leading to the diagnosis of ANCA-associated CGN superimposed on type 3 membranoproliferative glomerulonephritis (MPGN). CONCLUSIONS: This case is unique in that type 3 MPGN and MPO-ANCA-associated CGN coexisted, and no similar case has been reported to date. Because ANCA-associated CGN has a predilection for elderly individuals and primary type 3 MPGN is rarely seen in this age group, coincidental existence appears less likely. This case may confer valuable information regarding the link between immune complex and ANCA-associated CGN.     

A case report of anaphylactoid purpura with acute abdominal pain secondary to trauma caused by traffic accident]

A 17-year-old man was admitted to our hospital with multiple fractures resulting from traffic accident. After treatment of fractures, his general status was improved. However, one month after traffic accident, he suffered severe pain in the epigastrium. Ultrasonography and computed tomography showed thickening of the intestinal wall in the duodenum, ileum, and ascending colon. Nineteen days after the onset of abdominal pain, small hemorrhagic spots appeared on both of the lower legs. Subsequently, he developed proteinuria and hematuria. Purpura nephritis was diagnosed in biopsy specimens of the kidney. Anaphylactoid purpura associated with traffic accident is very rare and it is difficult to diagnose without skin and renal symptoms.