原发性干燥综合征合并抗肾小球基底膜新月体肾炎一例

现报道1例原发性干燥综合征伴抗肾小球基底膜型新月体肾炎。患者,女,70岁,因眼干、口干、四肢肌痛1年,咳嗽发热2周,少尿伴血肌酐升高1周入院。患者1年前于当地医院查RF 397 U／ml,ANA 1:320,CRP 5 ,ESR 34 mm／h, at-SM(-),at-SSA(-),at-SSB(-),角膜荧光( ),腮腺造影及唇黏膜活检(唇腺病理:小唾液腺组织见灶性间质细胞淋巴浆细胞浸润,50个／HPF),支持干燥综合征诊断。 2周前元明显诱因出现发热38．9℃,畏寒,伴咳嗽、咳痰。     

抗肾小球基底膜抗体伴抗中性粒细胞胞浆抗体阳性肾炎(四例报告及文献复习)

目的　了解抗肾小球基底膜抗体（ Ａｎｔｉ Ｇ Ｂ Ｍ） 伴抗中性粒细胞胞浆抗体（ Ａ Ｎ Ｃ Ａ） 阳性患者的临床病理特点。方法　对我科近４ 年来６８ 例 Ａｎｔｉ Ｇ Ｂ Ｍ 和（ 或） Ａ Ｎ Ｃ Ａ 阳性患者进行 Ａｎｔｉ Ｇ Ｂ Ｍ 及 Ａ Ｎ Ｃ Ａ 检测,对其中４ 例两者均阳性患者进行临床病理分析。结果　６８ 例患者中４ 例两者均阳性,占全部 Ａｎｔｉ Ｇ Ｂ Ｍ 阳性患者的２４ ％ ,占全部 Ａ Ｎ Ｃ Ａ 阳性患者的７ ％ 。该４ 例患者 Ａｎｔｉ Ｇ Ｂ Ｍ 的百分结合率较单纯 Ａｎｔｉ Ｇ Ｂ Ｍ 阳性患者低。４ 例中３ 例髓过氧化物酶 Ａ Ｎ Ｃ Ａ（ Ｍ Ｐ Ｏ Ａ Ｎ Ｃ Ａ） 阳性,１ 例蛋白酶３ Ａ Ｎ Ｃ Ａ（ Ｐ Ｒ３ Ａ Ｎ Ｃ Ａ） 阳性,全身系统表现较多,与单纯性 Ａ Ｎ Ｃ Ａ 阳性患者相似。所检病例肾脏病理多为新月体肾炎,免疫荧光检查多为 Ｉｇ Ｇ、 Ｃ３ 呈细颗粒样分布于 Ｇ Ｂ Ｍ。虽经积极治疗,多数患者预后较差,类似单纯 Ａｎｔｉ Ｇ Ｂ Ｍ 肾炎。结论　 Ａｎｔｉ Ｇ Ｂ Ｍ 伴 Ａ Ｎ Ｃ Ａ 阳性患者全身表现类似单纯 Ａ Ｎ Ｃ Ａ 相关小血管炎患者,但治疗效果及预后相对较差又类似于 Ａｎｔｉ Ｇ Ｂ Ｍ 肾炎患者     

丙基硫氧嘧啶致抗中性粒细胞胞浆抗体阳性新月体肾炎2例

丙基硫氧嘧啶是治疗甲状原机能亢进最常用的药物之一 ,近年来国内屡有报道该药引起血管炎性肾损害 ,现将我们收治的二例报告如下 :病　　例例 1男性 ,13岁 ,患者于 7岁时患甲状腺机能亢进即开始服用丙基硫氧嘧啶 ,每日 15 0ｍｇ ,病情稳定后减为每日10 0ｍｇ ,一直服用 6年 ,病情控制良好。 2 0 0 0年 4月初病情加重 ,遂将药量加至每日 4 5 0ｍｇ。两周后患儿足背、踝部水肿 ,双下肢小腿内外侧出现紫红色皮疹 ,给予对症治疗后好转。 1个月后出现发热 ,全程性肉眼血尿伴双下肢水肿 ,遂收治入院。查体 :急性病客 ,贫血貌 ,面色苍白 ,精神萎靡 …     

新月体肾炎患者肾组织中浸润细胞的分布特点及临床意义

目的研究肾组织各种浸润细胞在新月体肾炎的发病机制中的作用。方法分析５６例各类月体肾炎患者肾组织中ＣＤ６８＋及ＰＣＮＡ＋细胞在各类新月体肾炎患者肾组织中的分布特点,并就它们与肾组织中ＣＤ＾＋及ＣＤ８＋细胞浸润的关系进行分析探讨。结果本组新月体肾炎患者肾组织中ＣＤ６８＋、ＰＣＮＡ＾＋、ＣＤ４＋及ＣＤ８＋细胞均明显高于正常供肾组织。在Ⅰ型新月体肾炎患者肾组织中ＣＤ６８＾＋及ＰＣＮＡ＋细胞数高于Ⅱ型及Ⅲ型     

特发性新月体肾炎临床病理分析

目的探讨特发性新月体肾炎患者临床、病理特点及转归。方法回顾性分析13例经肾活检病理诊断明确的特发性新月体肾炎患者的临床表现、实验室检查和病理检查，探讨特发性新月体肾炎临床、病理特点及转归。结果13例患者给予糖皮质激素、环磷酰胺静脉冲击治疗，对无禁忌证的患者给予抗凝和抗血小板聚集治疗，7例患者给予血液净化治疗。经过治疗后，4例完全缓解，4例部分缓解，4例未治愈，1例死亡。结论早期诊断、积极治疗对特发性新月体肾炎患者至关重要。     

IgA肾病伴部分性新月体形成的临床病理分析

目的：探讨原发性IgA肾病伴部分性新月体形成的临床和病理特点。方法：选取79例经肾活检确诊伴部分性新月体形成IgA肾病患者,分析其临床和病理特点,并根据新月体形成所累及肾小球的比例分组：≥10%为A组,31例;≤10%为B组,48例。结果：（1）临床表现：79例均有血尿＋蛋白尿,蛋白尿〉1g/24h者48例（60.8%）;两组比较,A组蛋白尿〉1g/24h28例（89.3%）,B组蛋白尿〉1g/24h20例（41.7%）,A组大量蛋白尿、肉眼血尿、高血压、肾衰竭发生率均高于B组（P〈0.05）。（2）病理表现：79例新月体形成累及肾小球3.3%～29.0%,均以细胞性为主,几乎均有肾小球硬化、内皮细胞及系膜细胞增生、球囊黏连、灶性肾小管萎缩、以及炎性细胞浸润;两组比较A组中、重度系膜细胞及内皮细胞增生、炎性细胞浸润,细胞新月体所占比例均较B组明显;病理改变硬化肾小球占55例（69.6%）。结论：IgA肾病伴部分性新月体形成患者临床均有血尿＋蛋白尿,尤其大量蛋白尿;病理改变以局灶节段性肾小球硬化常见;炎性细胞浸润、内皮细胞及系膜细胞增生等活动性病变易见并影响新月体形成;新月体的多少及纤维化程度影响临床病理表现,≥10%较≤10%严重。     

ANCA-positive crescentic glomerulonephritis associated with minocycline therapy

Minocycline is an oral antibiotic widely used for the long-term treatment of acne and rheumatoid arthritis. A few patients develop antineutrophil cytoplasmic antibodies (ANCAs) during minocycline therapy. In this report, the authors describe a case of severe pauci-immune crescentic and necrotizing glomerulonephritis associated with positive cytoplasmic ANCA (C-ANCA) titers and proteinase 3 (PR3) levels after minocycline therapy. Discontinuation of minocycline and initiation of immunosuppressive treatment resulted in improvement of renal function and decline in C-ANCA titers and PR3 levels. A high degree of suspicion, testing for ANCA titers, prompt discontinuation of the drug, and initiation of immunosuppressive treatment are crucial to the diagnosis and treatment of drug-induced ANCA-associated glomerulonephritis.     

Fever of unknown origin secondary to type I crescentic glomerulonephritis and anti-SCl 70 antibodies without clinical manifestations of systemic sclerosis

A 57-year-old woman presented with fever and cellulitis of the right leg. Urinalysis and kidney function were normal on admission. Cellulitis remitted but fever persisted for six weeks. X-ray imaging, cultures, serological assays for viruses and autoimmunity did not reveal the cause of fever. Unexpectedly anti-Scl 70 (anti-topoisomerase I) antibodies were positive. A skin biopsy ruled out scleroderma. On the fifth hospitalization week kidney function declined in association with hematuria, leucocyturia, and proteinuria. Prednisone was administered due to clinical suspicion of drug-induced interstitial nephritis. Fever declined in 24 h, but renal failure became rapidly worse requiring hemodialysis. Kidney biopsy revealed extensive crescentic glomerulonephritis (CGN), with much glomerular destruction, with an IgG-positive linear pattern on immunofluorescence microscopy. No overtly active microangiopathy or vasculitis were present. There was no pulmonary involvement and anti-glomerular basement membrane antibodies were not detected in the serum. After one year anti-Scl 70 antibodies were still positive without scleroderma manifestations and 17 months later the patient received a kidney transplant with excellent results. Presentation of type 1 CGN as a fever of unknown origin (FUO) is exceptional. Anti-Scl 70 antibodies are highly specific for scleroderma and are seldom present in other diseases. As far as we are aware there are no published cases of the association of type 1 CGN with anti-Scl 70 antibodies.     

Impact of clinical and histopathological factors on outcome of egyptian patients with crescentic glomerulonephritis

This study included 128 patients with crescentic glomerulonephritis (CGN) having sufficient clinical and histopathological data and were followed up in our institute for a mean period of 34 +/- 28 months. There were 49 males and 79 females with mean age 22.7 +/- 14 years. We studied the effect of clinical, laboratory and histopathological parameters on kidney function and patient survival at the end point of the study. The multivariate analysis revealed that serum creatinine at presentation, nephrotic range proteinuria during the follow up period, percentage of glomeruli affected by crescents, percentage of fibrous crescents and absence of cellular infiltration were significant risk factors affecting the kidney function at termination of the study. The only risk factor which correlated significantly with the patient mortality was the serum creatinine at last follows up.     

Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulnes of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.     

ANCA-negative pauci-immune crescentic glomerulonephritis complicated with recurrent massive gastrointestinal hemorrhage

On April 25, 2003, a 62-year-old Japanese man had been admitted to a hospital because of heavy proteinuria and elevated serum creatinine level, and purpura on the lower extremities. On May 15, 2003, he was referred to our hospital for evaluation and treatment. Serum immunoglobulin and complements were within normal ranges. Immune serology was negative for antinuclear antibody, antiglomerular basement membrane antibody, and antineutrophil cytoplasmic antibodies. Histological examination of a percutaneous renal biopsy specimen revealed that all of the glomeruli had severe crescent formation without deposits of immunoreactants. A diagnosis of antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis was made. The patient was treated with one cycle of steroid pulse therapy (1000 mg methylprednisolone daily, given on 3 consecutive days), and subsequently with prednisolone (60 mg/day). Despite this treatment, renal failure progressed rapidly and hemodialysis was started 1 month after the acute presentation. On May 30, 2003, he suddenly developed massive hematochezia. A technetium-targeted red-blood-cell scan suggested bleeding in the small intestine. On June 11, he presented with massive melena. A bleeding ulcer was found in the third part of the duodenum, and was treated successfully with endoscopy, using a heater probe. On June 19, he presented with massive hematochezia again. Mesenteric angiography revealed active bleeding from the iliac branch of the superior mesenteric artery. He was treated with continuous intraarterial vasopressin infusion by a catheter seated in the branch artery. The majority of patients with pauci-immune crescentic glomerulonephritis, one of the most common causes of rapidly progressive glomerulonephritis, have glomerular disease as part of a systemic vasculitis. Massive gastrointestinal bleeding, although rare, should be considered one of the serious complications in these patients.     

Analysis of clinical features and prognosis of anti-glomerular basement membrane antibody positive patients with anti-neutrophil cytoplasmic antibodies

Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody. Methods The sera of 23 anti-GBM glomerulonephritis patients were collected and were tested for ANCA respectively. Characteristics and outcome of patients with coexisting anti-GBM antibody and ANCA were analyzed, and were compared with anti-GBM glomerulonephritis patients without coexisting ANCA. Results Among the 23 sera with anti-GBM antibody, 7 sera had coexisting ANCA (7 MPO-ANCA, 1 PR3-ANCA), which represented 30.4% of the anti-GBM glomerulonephritis patients. The incidence of hemoptysis and hematuria in ANCA+-anti-GBM glomerulonephritis group was significantly higher than that in ANCA--anti-GBM glomerulonephritis group (P＜0.05). No significant difference in age, sex, other clinical manifestations and pathological features were found between patients with and without coexisting serum ANCA. Conclusion The incidence of hemoptysis and hematuria in ANCA+-anti-GBM glomerulonephritis group is significantly higher than that in ANCA--anti-GBM glomerulonephritis group, but the prognoses of the two groups were poor.     

Azurocidin-specific-ANCA-related idiopathic necrotizing crescentic glomerulonephritis

An 80-year-old woman who had rapidly progressive glomerulonephritis unaccompanied by systemic vasculitis is described. On renal biopsy, she showed necrotizing crescentic glomerulonephritis by light microscopy and pauci-immune glomerular lesions by immunofluorescent study. No dense deposits were present on electronmicroscopic study. On serum examination, indirect immunofluorescent study showed perinuclear pattern antineutrophil cytoplasmic antibody (ANCA), but myeloperoxidase-ANCA and proteinase 3-ANCA were both negative. Her serum reacted only to azurocidin excluding other ANCA antigens: bactericidal permeability-increasing protein, cathepsin G, elastase, lactoferrin, or lysozyme. Serum creatinine level decreased, and C-reactive protein turned negative after steroid therapy. Azurocidin-ANCA also turned negative. It is suggested that azurocidin-ANCA might have been related to the inflammatory process of pauci-immune necrotizing crescentic glomerulonephritis in this patient.     

The clinical and pathological characteristics of Chinese patients with pauci‐immune crescentic glomerulonephritis

SUMMARY In the present study, we investigated pauci‐immune crescentic glomerulonephritis (PICGN) in Chinese patients. During 13 years (1985–98), 6400 patients underwent non‐transplanting renal biopsy in the Nanjing Jinling Hospital. Twenty‐four patients were diagnosed as having PICGN. They were 16 women and eight men with a median age of 33 (range 10–76 years). Microscopic polyarteritis (33.3%) and polyarteritis nodosa (8.3%) were the secondary diseases. The incidence of PICGN was 0.37% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. Onset gross haematuria was noted in 58.3% of the patients, hypertension 45.8%, nephrotic syndrome 41.7%, and oliguria 25.0%. However, systemic symptoms were rare except anaemia. Pathologically, we observed necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in interstitium (53.3%), in addition to glomerulosclerosis more than 50% (45.8%), severe tubular atrophy (83.3%) and interstitial fibrosis (75.0%). Antineutrophil cytoplasmic antibodies were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were subjected to long‐term follow up (median 29.8, range 8–92 months), 12 of them had life‐sustaining renal function, four had normal range of serum creatinine (< 124 μmol/L), only four patients were dialysis dependent.