Cacoub P, Sbaï A, Gatel A, Wechsler B, Godeau P, Piette J C
Service de Médecine Interne, CHU Pitié-Salpêtrière, Paris.
J Mal Vasc. 1997 Mar;22(1):29-34.
In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic.
In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.
在大多数情况下，系统性血管炎的病因不明，治疗以对症治疗为主（皮质类固醇常与免疫抑制剂联用）。我们报告了3例与感染相关的系统性血管炎病例，其中2例患者未接受皮质类固醇治疗，病情显著改善。病例报告1：一名既往超重的72岁女性因发热1年、体重减轻14千克、血管性紫癜和多发性神经病入院。实验室检查异常包括炎症综合征[红细胞沉降率（ESR）：80mm/第1小时，血小板增多症：500,000/微升，嗜酸性粒细胞增多（1200/微升）及核周抗中性粒细胞胞浆抗体（p-ANCA）阳性[抗髓过氧化物酶抗体：30 U（正常<7）]。神经肌肉活检显示中小动脉坏死性血管炎。进一步检查发现结肠有明显的憩室病，无其他严重内脏受累。随后行乙状结肠切除术。六周内，她的一般状况显著改善，未接受皮质类固醇治疗：所有全身症状消退，ESR恢复正常，p-ANCA转阴。十个月后她仍无症状。病例报告2：一名50岁男性体重在2个月内逐渐减轻15千克，四肢严重多发性神经病。他的ESR为120mm/第1小时，C反应蛋白200mg/L。神经肌肉活检显示坏死性血管炎累及神经中的小血管，无免疫沉积物。口腔检查发现多处牙齿感染灶。拔除所有这些感染灶并联合使用抗生素后，所有全身症状在8周内显著改善，同样未接受皮质类固醇治疗。八个月后患者仍无症状。病例报告3：一名30岁女性因发热、肌痛、多关节炎及四肢皮肤结节5周入院。实验室检查异常包括炎症综合征、每日蛋白尿0.7g，沙眼衣原体抗体滴度在5周内从1/64显著升至1/256。她既往有生殖器尖锐湿疣病史。入院前12天开始使用的泼尼松（0.5mg/kg/天）逐渐减量（2个月内停用），并开始使用强力霉素（200mg/天）治疗。抗菌治疗六周内，初始临床表现完全消退，实验室检查值恢复正常。十个月后她仍无症状。结论：在系统性血管炎中，寻找感染灶的检查具有双重意义：如我们的病例报告所示，可能为病因（有力证据表明与感染有关），从治疗角度看，我们必须识别血管炎综合征背后的微生物，因为如果患者患有活动性传染病，使用皮质类固醇治疗可能会产生严重后果。
