ANCA相关性血管炎肾损害合并交通性脑积水一例并文献复习

目的 探讨合并交通性脑积水的ANCA相关性血管炎（AAV）的发病机制、诊治方法,提高临床医师对AAV罕见类型的认识水平。方法 收集1例以交通性脑积水为首发症状的AAV病例的临床资料,以“ANCA相关性血管炎”“脑积水”为关键词（包括中英文）对PubMed和CNKI收录的论文进行检索,结合文献分析病例特点、实验室检查、影像学检查、肾脏活组织检查（活检）和诊治方法。结果 该例71岁男性患者,因头晕伴行走不稳半年余在门诊行CT检查提示交通性脑积水,拟行手术治疗。术前检查发现血清肌酐635 μmol/L,核周型-抗中性粒细胞胞质抗体（p-ANCA）（+）,MPO 293 U/L,尿红细胞位相示畸形红细胞240 000 /μL,经肾脏活组织检查（活检）证实存在AAV肾损害。随访30个月,经糖皮质激素（激素）联合环磷酰胺治疗后,患者血清肌酐降至335 μmol/L并脱离透析,头晕、行走不稳、智力减退症状逐渐好转,后多次复查颅脑MRI,交通性脑积水较前无明显变化。检索数据库后共收集5篇相关病例报道,患者年龄33~76岁,临床表现为脑积水和AAV,予激素联合免疫抑制剂、脑室-腹膜分流术等治疗,随访期间1例患者死亡,另外4例患者症状缓解。结论 以脑积水起病的AAV罕见且血管炎症状隐匿,易被误诊或漏诊,对不明原因伴肾衰竭的神经系统受累病变进行ANCA筛查十分必要,肾穿刺活检有助于明确诊断,及时正规治疗可缓解脑积水症状,有助改善患者预后。     

Clinical characteristics and associating risk factors of gastrointestinal perforation in children with IgA vasculitis

BackgroundIgA vasculitis (IgAV) is a common small vessel vasculitis in children. Gastrointestinal perforation (GP) rarely presents as a complication of IgAV and is not well characterized. This study is aimed to investigate the clinical features, diagnosis, and risk factors of GP in children with IgAV.MethodsWe retrospectively reviewed the clinical data of children with IgAV who attended our hospital between January 2014 and June 2018. The clinical risk factors and the corresponding treatments were analyzed for the children with IgAV complication with GP.ResultsIn total, 10,791 children with IgAV were reviewed in this study. GP was observed in 11 children with IgAV, accounted for 0.10% of the total cases. Among those GP patients, 1 case was gastric perforation, 10 cases were intestinal perforation. Five GP cases were identified by abdominal CT. Ultrasonography was failed to detect the occurrence of GP in five cases. The average duration of abdominal pain in the GP cases was 9.3 days, and 9 cases (81.8%) presented with abdominal pain for over 7 days. Gastric/intestinal perforation repair were performed for 3 IgAV GP cases under open surgery. The other eight cases were treated through enterectomy. In comparison with the patients without GP, the GP patients had significant higher rates in the aspect of the abdominal or mixed type of IgAV, abdominal pain duration more than 7 days, hematochezia, renal damage, and methylprednisolone treatment with the daily dosage more than 2 mg/kg.ConclusionGP children accounted for 0.10% of the total IgAV cases. The risk of GP is elevated in IgAV patients who has gastrointestinal symptoms and/or other symptoms such as hematochezia, renal damage, a prolonged abdominal pain (>7 days), administration of methylprednisolone (>2 mg/kg). Abdominal CT is highly recommended for the early detection of GP in IgAV patients.

Key messages

• Gastrointestinal perforation (GP) rarely presents as a complication of IgAV and is not well characterized.
• 11 out of 10,791 children with IgAV developed GP, accounting for 0.10% of the total number of cases.
• Abdominal CT is highly recommended for the early detection of GP in IgAV patients.

     

Lung adenocarcinoma and sequential antineutrophil cytoplasmic antibody-associated vasculitis: a case report

The relationship between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and lung cancer remains unclear. A 66-year-old man presented with pulmonary nodules. Histological examination of a specimen from computed tomography-guided percutaneous transthoracic biopsy revealed adenocarcinoma. The patient was treated using cryoablation and systemic chemotherapy. Sixteen months later, the patient presented with fever, nasal inflammation, recurrent lung lesions, elevated serum creatinine levels, and high levels of ANCA. Histological examination of a specimen from ultrasound-guided percutaneous renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis. The patient responded to treatment, but granulomatosis with polyangiitis recurred and he later died. This case highlights the possibility of sequential AAV with lung cancer. Although this is relatively rare, further research is needed to better understand the association or pathophysiological link between lung cancer and AAV.     

抗中性粒细胞胞质抗体相关性血管炎心脏受累影像学所见

抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)为全身坏死性小血管炎,可累及全身多个器官,肾脏和肺最常受累,心脏受累则相对少见,但多与预后不良相关.本文对AAV累及心脏的机制及其常见心电图、超声心动图及心脏M RI表现进行综述.     

Immunosuppressive Therapy for External Ocular Inflammatory Disease

Fourteen patients with progressive ocular inflammation and destructive lesions, unresponsive to conventional therapy, were treated with systemic immunosuppression. Ten patients had connective tissuelvasculitis diseases; two, cicatricial pemphigoid; and two, bilateral Mooren's corneal ulcers. Control of underlying systemic disease by immunosuppression resulted invariably in concomitant control of ocular inflammation and destruction. Encouraging results were obtained in the patients with cicatricial pemphigoid and those with bilateral Mooren's ulcers.     

Hypersensitivity reactions in neonates and infants

Although rare, several hypersensitivity reactions can occur in the infant population. Several types of hypersensitivity reactions are discussed, including urticaria, drug eruptions, erythema multiforme, allergic contact dermatitis, and vasculitis (acute hemorrhagic edema of infancy); neonatal lupus also is discussed. Although most of these conditions are benign, their presentations can be dramatic and a cause of concern for both parents and physicians. Important considerations and differences in the diagnosis and management of these conditions in children under the age of 6 months are discussed.     

Giant cell (temporal) arteritis: an overview and update

Giant cell arteritis is a systemic disease that continues to be a sight-threatening medical emergency requiring prompt recognition and treatment in order to avoid devastating ophthalmic consequences. Although there have been advances in the genetic and immunologic understanding of the underlying pathogenesis of the disease, the exact etiology of the condition, to date, remains unclear. Visual manifestations of giant cell arteritis are the common mode of presentation, making the ophthalmologist critically responsible for early diagnosis and treatment. Although temporal artery biopsy remains the only confirmatory procedure, newer laboratory investigations and blood flow studies with fundus fluorescein angiography have aided in the diagnosis of temporal giant cell arteritis. Maintenance of a high index of clinical suspicion is essential to institute prompt adequate treatment, especially in atypical cases. Corticosteroids remain the mainstay of treatment of giant cell arteritis. Recently, immunosuppressive agents as secondary steroid-sparing drugs have been used, particularly in some steroid-resistant cases. A wider recognition of the disease will minimize the prevalence of irreversible visual loss among patients with giant cell arteritis.     

Nine patients with anti-neutrophil cytoplasmic antibody-positive vasculitis successfully treated with rituximab

OBJECTIVES: Rituximab (RIT) is a monoclonal anti-CD20 antibody, which depletes B-lymphocytes but not plasma cells. RIT is used for treatment of B-cell lymphomas, but has also shown beneficial effects in autoimmune diseases. In this case series RIT was used in anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis. DESIGN: Case series with a structured follow-up of treated patients. SETTING: Departments of Nephrology and Rheumatology of a university hospital. SUBJECTS: Two women with myeloperoxidase-ANCA-positive microscopic polyangiitis and seven patients (five men and two women) with proteinase 3-ANCA-positive Wegener's granulomatosis. All patients were resistant to conventional therapy or had relapsed repeatedly after cessation of cyclophosphamide (Cyc). INTERVENTIONS: The cases were treated with intravenous infusions of RIT once a week two times (three cases) or four times (six cases). To prevent formation of antibodies to RIT, mycophenolate mofetil (five patients), azathioprine (one patient), or a short course of Cyc (two patients) were added or allowed to continue. MAIN OUTCOME MEASURES: Remission at 6 months assessed with Birmingham vasculitis activity score. The cases were followed 6-24 months and relapse rate was also noted. RESULTS: Eight of nine patients responded completely and one case responded partially. Pulmonary X-ray improved (four cases), progress of lower extremity gangrene stopped (one case), remission of neuropathy was stable (one patient), renal vasculitis went into remission (two cases), and severe musculoskeletal pain improved (one case). Minor relapse in the nose occurred in two cases. No adverse events or major infections were noted. CONCLUSION: RIT seems promising and safe in ANCA-positive vasculitis, and controlled studies should be conducted.     

Cochlear implantation in patients with bilateral deafness caused by otitis media with ANCA-associated vasculitis (OMAAV): A report of four cases

Objective

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) without systemic symptoms but with initial symptoms related to the ear, such as hearing loss, otalgia, and dizziness, has recently been reported. We have categorized this condition as otitis media with AAV (OMAAV), and have recently proposed its diagnostic criteria.