Antigen specificity in hydralazine associated ANCA positive systemic vasculitis

The anti-hypertensive agent hydralazine can cause a lupus-likesyndrome characterized by serosal inflammation, arthralgiasand rashes. The kidneys however are usually spared. The conditionis characterized by circulating immune complexes and antinuclearantibodies, whilst antibodies against doublestranded DNA arerare. Hydralazine can also cause a systemic vasculitis witha pauci-immune rapidly progressive glomerulonephritis, whichis associated with autoantibodies directed against componentsof the neutrophil cytoplasm. In this study, ten patients withhydralazine-induced vasculitis had antibodies with specificitiesfor both myeloperoxidase and lactoferrin. We suggest that thisparticular pattern of autoantibodies, together with antibodieswith reactivity against nuclear components including double-strandedDNA, are characteristic findings in hydralazine-induced vasculitis.In addition, renal involvement appears to be more common inthis group of patients with vasculitis than in those with thelupus-like syndrome.     

ANA细胞质荧光模式在ANCA相关血管炎中的临床意义

目的分析抗核抗体(ANA)细胞质荧光模式在抗中性粒细胞细胞质抗体(ANCA)相关血管炎(AAV)中的特征,探讨ANA细胞质荧光模式在AAV中的意义。方法将德阳市人民医院232例进行了间接免疫荧光法(IIF)ANA和ANCA检测的AAV确诊患者纳入研究,并根据IIF-ANA的检测结果分为ANA细胞质荧光模式组与对照组。收集纳入研究患者的临床资料和临床实验室检测数据,将上述资料/数据在ANA细胞质荧光模式组与对照组间进行比较,分析ANA荧光模式与ANCA荧光模式的关系。结果两组间AAV患者胸部受累和心血管受累情况比较,差异有统计学意义(P<0.05)。线性回归分析显示,抗髓过氧化物酶抗体是ANA细胞质荧光模式组患者血肌酐水平的影响因素(P<0.001)。pANCA组和xANCA组ANA细胞质颗粒型检出率高于cANCA组(P<0.017)。结论ANA细胞质荧光模式与AAV的部分临床表现和实验室检查结果具有一定相关性。     

ANCA相关性小血管炎性肺炎的影像学表现及预后

目的分析ANCA相关性小血管性肺炎的病理基础及影像学特点,提示其诊断与鉴别诊断要点及预后.方法选择2001年10月～2004年3月在我院住院经实验室测检ANCA均为阳性患者7例,有多次胸部X线照片,4例有CT扫描,2例行肾穿刺活检.结果 7例患者均确诊为ANCA相关性小血管炎,表现为多系统多脏器损害.经治疗2～6周后6例肺部病变完全吸收消失,1例因多器官衰竭抢救无效死亡.结论抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎属少见的自身免疫性疾病,肺脏是最常累及的脏器之一,其肺部影像表现并无特异性改变,对有肺脏受累的多系统损害的病人,特别是有咯血者,要想到本病的可能,及时确诊,临床给予甲基强的松龙和环磷酰胺双冲击治疗,病情在短期内即有显著好转,预后较好,其肺部的反应明显好于其他系统的损害.     

Concomitant drug- and infection-induced antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis with multispecific ANCA

OBJECTIVE: To report the first case of concomitant drug- and infection-induced antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) in a patient treated with propylthiouracil (PTU) and suffering from tuberculosis. PRESENTATION AND INTERVENTION: A 28-year-old woman with PTU-treated hyperthyroidism presented with fever, purpura, pulmonary cavitations and ANCA to myeloperoxidase, bactericidal/permeability-increasing protein (BPI), proteinase-3 and elastase. Skin histopathology confirmed vasculitis. However, sputum examination revealed Mycobacterium tuberculosis. Remission was achieved after PTU withdrawal and treatment with antituberculosis drugs. CONCLUSION: Our case confirmed that BPI-ANCA are elevated in active tuberculosis. Multispecific ANCA were helpful for the diagnosis of concomitant PTU- and M. tuberculosis-induced AAV.     

Hemosorption in systemic vasculitis

The present work summarises the experience gained with the use of extracorporeal hemoperfusion in 45 patients suffering from rheumatic diseases. During hemoperfusion, there was a decrease in the initially activated LDH, CPK, AST, ALT and AP. The reduction of the content of phospholipids and cholesterol in plasma and red blood cells is assessed as an adverse effect of hemoperfusion, since the content of phospholipids and cholesterol was under normal before hemoperfusion. The changes indicated point to the necessity of administering antioxidants and exogenous phospholipids.     

The eye in systemic vasculitis

The term 'vasculitis' includes a wide range of disorders characterised by inflammation of the wall of blood vessels, sometimes with necrosis, leading to ischaemia of the affected organ. The exact pathogenesis of most of these vasculitides is not fully understood and although the presence of circulating auto-antibodies seems to be a common feature among them, each vasculitis has its unique pathogenesis and a predilection for vessels of a defined size. Systemic vasculitis may be associated with ocular complications which include scleritis, keratitis, uveitis and optic neuropathy. These can precede the symptoms/signs of the systemic disease and therefore their recognition may lead to detection of the underlying disorder. The eye may also be affected by the treatment required to control the systemic disease.     

Abdominal emergencies in systemic vasculitis

Abdominal lesions are characterized in a patient with acute vascular purpura and a patient with polyarteritis. The former patient developed obturation ileus of the small intestine, the latter--necrosis and multiple intestinal and gall bladder perforation with subsequent peritonitis. In both cases the abdominal syndrome was manifestation of the basic disease. Surgical treatment saved life of the patients. Stable remission was achieved after long-term medication.     

老年ANCA相关性小血管炎肺损害11例临床分析

目的探讨老年抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏损害的临床特征,提高对其肺损害的认识。方法回顾性分析北京大学第一医院老年科60岁以上的ANCA相关性小血管炎肺损害患者的临床病理资料。结果11例ANCA相关性小血管炎肺脏损害患者平均年龄75.9(60～89)岁,均有慢性基础病。发病至确诊的中位间隔时间为4个月(2周～1年)。小血管炎临床症状多为咳嗽、咯痰(90.9%);喘憋气短或活动耐力下降(63.6%);咯血/痰中带血(36.4%)和发热(45.5%)。胸部影像学9例(91.9%)表现为肺间质病变,其中8例呈显著肺纤维化表现;2例(18.2%)呈两肺大片渗出的弥漫肺泡出血表现(其中1例在肺纤维化基础上发生);1例呈结节性病变(9.1%)。8例(72.7%)患者初诊时无肾损害。确诊主要依靠血清ANCA的检测和组织活检病理诊断。经免疫抑制治疗后,多数患者症状好转,初诊及随访期间5例(45.5%)因呼吸衰竭或严重感染死亡。结论原发性ANCA相关性小血管炎患者常有肺部受累并可以为首发表现。其症状和胸部影像表现多样,肺间质病变突出。正确及时的诊断治疗能改善预后。     

Immunologic mechanisms in systemic vasculitis.

Thirty-four patients with systemic vasculitis were studied to determine the possible type and frequency of associated immunologic abnormalities. The patients were divided into three clinical groups--those with systemic vasculitis without respiratory tract involvement, those with systemic vasculitis with respiratory tract involvement (particularly Churg-Strauss vasculitis and Wegener's granulomatosis), and those with limited vasculitis without visceral involvement. A diminished level of serum complement was found in half the patients with systemic vasculitis without respiratory tract involvement. These patients usually had diffuse skin disease that often was associated with the presence of rheumatoid factor and cryoglobulinemia and most likely represented an immune-complex induced disease. The serum IgE often was elevated in patients who had systemic vasculitis with respiratory tract involvement, particularly those with Churg-Strauss vasculitis and Wegener's granulomatosis, and may be a clue to the pathogenesis in this group of patients.     

Update on systemic necrotizing vasculitis

The systemic necrotizing vasculitides are classified into vasculitic syndromes on the basis of the pattern of clinical and pathologic involvement. The vasculitides have certain common clinical and laboratory abnormalities. Systemic necrotizing vasculitis is diagnosed on the basis of clinical features, and the vascular nature of the disease is determined by biopsy of involved tissue or angiography. The outcome is dependent on the extent of visceral involvement. Vascular inflammation influences the physiologic features of the vessel and may trigger vasoconstriction. Although glucocorticoids combat the inflammation, they may augment vasoconstriction and platelet aggregation. These effects must be considered in designing a management approach and in evaluating the cause and management of ischemic complications.     

Gastrointestinal manifestations of systemic vasculitis

Systemic vasculitis is known to affect the gastrointestinal tract but the nature of the complication is poorly characterized. Out of 65 patients with systemic vasculitis, the majority of whom had renal disease, the intestine was found to be affected in 18. These comprised four of eight patients with polyarteritis nodosa, nine of seventeen with microscopic polyarteritis, four of thirty-six with Wegener's granulomatosis and one of four with Churg-Strauss syndrome. The features included abdominal pain (85 per cent), diarrhoea (50 per cent), gut haemorrhage (44 per cent) and abnormal liver function tests (50 per cent). Manifestations of gastrointestinal disease were evident at presentation in half the patients and led to a fetal outcome in five. Ileus, mucosal abnormalities, perforation and slow transit were evident radiographically, and selective visceral angiography showed aneurysms or organ infarcts in five patients. Histological assessment of gut biopsies (chiefly rectal) revealed non-specific inflammation or ulceration in nine patients and intramucosal haemorrhage in two. Focal areas of necrosis and ulceration in colonoscopic biopsies were highly suggestive of vasculitis whereas arteritis was only found in one full thickness biopsy. Hence the diagnosis of gastrointestinal complications depends largely on clinical evidence. In patients who survived, the gastrointestinal features remitted as the systemic illness improved following treatment with steroids, cyclophosphamide or plasma exchange.