老年患者嗜酸细胞肉芽肿性血管炎的临床特征

嗜酸细胞肉芽肿性血管炎（EGPA）是以外周血和受累脏器组织中嗜酸性粒细胞增高并伴有坏死性肉芽肿为特征的系统性血管炎,以呼吸系统受累（如哮喘）最为常见。影像学表现为肺部非固定性斑片状浸润影、磨玻璃影、实变、结节、胸腔积液等,也可出现肺间充质改变。该病在成年人中常见,无明显性别差异;年龄是预后不良的影响因素之一。临床研究分析发现老年患者有更高的患病率和死亡率,本文就老年患者EGPA的临床特征进行文献复习,并综述如下。     

Catastrophic subarachnoid hemorrhage in eosinophilic granulomatosis with polyangiitis without asthma

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic vasculitis. Patients rarely present without asthma. Cases developing subarachnoid hemorrhage from central nervous system vasculitis are rarely reported. We report a 48‐year‐old woman with rapidly evolving and progressive multi‐system eosinophilic vasculitis in the absence of asthma. Tissue eosinophilia was apparent in a breast lump biopsy. Prior otitis media and prominent lymphoid tissue in the postnasal spaces hinted at otolaryngological disease. She had rapid disease progression with mononeuritis multiplex and eventually succumbed to complications of intracranial hemorrhage secondary to central nervous system vasculitis. This case demonstrates the diagnostic dilemma and treatment considerations in EGPA without asthma. It also raises the question if a reliable biomarker can aid diagnosis in atypical presentations of disease.     

Radiotherapy of granulomatosis with polyangiitis occurring in the eyelid: A case report and literature review

Introduction:Granulomatosis with polyangiitis (GPA) is a chronic systemic vasculitis characterized by necrotizing granulomatous vasculitis. The disease mainly affects the middle and small blood vessels and mainly occurs in the upper respiratory tract (nose and paranasal sinuses), lower respiratory tract (lungs), and kidneys. Disease occurrence in the eyelid area is relatively rare. The standard GPA treatment is combination therapy with adrenocortical hormone and immunosuppressants. Radiotherapy as a treatment option for GPA has not been widely investigated.Patient concerns:A 29-year-old man presented with a 1.0 × 1.0 cm mass without exophthalmos and decreased vision in the left lower eyelid. Computed tomography revealed a mass-like high-density shadow below the left eye with a computed tomography value of 80-108 U.Diagnosis:The laboratory investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies (titer = 1:40). Biopsy of the lower left eyelid mass revealed necrosis and granulomatous reaction with a large number of inflammatory cell infiltration. After consultation with the pathology department, the diagnosis was determined as left lower eyelid GPA.Interventions:The patient received 9MeV electron beam radiation therapy in the area of the left lower eyelid lesion.Outcomes:The lesion in the patient was significantly reduced and the symptom relieved obviously. No symptom recurrence or significant toxicity occurred during or after the treatment. The patient remains under routine follow-up.Conclusion:We present a case of a male patient with GPA located exclusively in the eyelid area, who underwent successful radiotherapy and achieved a complete response. The lesson we learned from this case study is that for GPA patients, when the standard treatment model fails to achieve good results, novel treatments such as radiotherapy should be considered according to the situation.     

老年嗜酸性肉芽肿性多血管炎患者临床特点及预后

目的 年龄是影响抗中性粒细胞胞浆抗体相关性血管炎预后的重要因素,但针对老年嗜酸性肉芽肿性多血管炎(EGPA)患者的研究较为缺乏,本研究旨在探索老年EGPA患者的临床特点及预后影响因素.方法 回顾性分析2000年1月至2015年12月于北京协和医院确诊的EGPA患者,将患者分为老年组(年龄≥60岁)及非老年组(年龄<60...     

Successful Treatment with Rituximab for Granulomatosis with Polyangiitis and Multiple Cranial Neuropathies

We herein report a case of granulomatosis with polyangiitis in a 73-year-old man. He had experienced bilateral ptosis, redness of both eyes, right facial weakness, and hearing loss in the right ear for two months. Myeloperoxidase anti-neutrophil cytoplasmic antibody was positive. Corticosteroids and intravenous cyclophosphamide pulse therapy yielded a response. After the fourth pulse of cyclophosphamide, he developed headache, diplopia, restricted left eye movement, right facial palsy, and hearing loss in the right ear. A one-year remission period was achieved by increasing the steroid and rituximab doses. Rituximab was effective against the relapse of granulomatosis with polyangiitis and cranial neuropathy.     

Rituximab as a new therapeutic option in granulomatosis with polyangiitis: a report of two cases

Abstract

Findings of several reports suggest that rituximab, a chimeric monoclonal anti-CD20 antibody causing B-lymphocyte depletion, might represent a treatment option for people with granulomatosis with polyangiitis (GPA) (former Wegener’s granulomatosis). This study presents the results of rituximab treatment in two patients with treatment-refractory GPA. First patient received rituximab for a granulomatous posterior orbital mass lesion, and eye symptoms were resolved after three courses of treatment. The second patient had eye and paranasal sinus involvement and benefited from two courses of rituximab treatment, with significant clinical improvement. Rituximab may represent an effective novel treatment for remission induction in GPA.     

嗜酸细胞肉芽肿性血管炎抗中性粒细胞胞浆抗体阳性与阴性患者临床特征

嗜酸细胞肉芽肿性血管炎(EGPA)是以外周血和受累脏器组织中嗜酸性粒细胞增高并伴有坏死性肉芽肿为特征的系统性血管炎,依据血清抗中性粒细胞胞浆抗体(ANCA)检测结果可分为ANCA阳性及ANCA阴性表型,ANCA阳性患者更易出现神经系统、肾脏、皮肤紫癜和肺泡出血;心脏和肺受累在ANCA阴性患者中更常见。本文就ANCA阳性与阴性患者的临床表现及受累脏器特征进行文献复习,并综述如下。     

13例嗜酸性肉芽肿性多血管炎临床特点观察

目的 探讨嗜酸性肉芽肿性多血管炎(EGPA)的临床表现、病理及影像学特征,以提高临床诊治水平.方法 回顾性分析2006年1月-2021年4月于解放军总医院确诊的13例EGPA患者的临床资料,包括基本信息、临床表现、受累器官情况、血液学及影像学检查、病理检查和治疗、随访情况.结果 13例EGPA患者中男7例,女6例;年龄...     

Early diagnosis of granulomatosis with polyangiitis: An introduction to the newly designed Iran criteria

BackgroundsIn the absence of practical diagnostic criteria for diagnosis of granulomatosis with polyangiitis (GPA), a new diagnostic criteria for GPA is proposed based on literature review for characteristic manifestations of GPA and expert opinion. The sensitivity of the new criteria, Iran criteria for GPA, is assessed in comparison with 1990 American College of Rheumatology (ACR) criteria for Wegener's granulomatosis (WG).MethodsEvaluation of three organs (ear, nose and throat (ENT); lung; kidney) and two laboratory findings (anti-neutrophil cytoplasmic antibody; biopsy), abbreviated mnemonically as ELKAB, is suggested in our criteria. A retrospective sensitivity analysis was performed based on medical records of 35 patients. Clinical diagnosis of GPA by a single rheumatologist was used as the gold standard.ResultsRecords of a total of 15 male and 20 female patients with a mean follow-up duration of 21.26 ± 4.13 months were considered. Mean age at diagnosis and mean disease duration were 32.37 ± 2.33 years and 19.06 ± 5.41 months, respectively. The sensitivity for Iran criteria for GPA and 1990 ACR classification criteria for WG were calculated as 100% and 80%, respectively.ConclusionsIran criteria for GPA is a highly sensitive instrument for detecting GPA patients in comparison with 1990 ACR classification criteria for WG.     

Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies‐negative eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA‐related deaths. ANCA‐positive EGPA differs from ANCA‐negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA‐negative patients.     

Optical coherence tomography angiography for the diagnosis of granulomatosis with polyangiitis with serous retinal detachment: A case report

Rationale:Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune disease of unknown etiology. GPA affects multiple ocular tissues, most commonly the orbit, conjunctiva, cornea, and sclera. Retinal and choroidal manifestations are rare in GPA, but they often include choroidal neovascularization (CNV).Patient concerns:A 36-year-old man was diagnosed with GPA. He had been taking oral steroid treatment for 8 years. He experienced disease recurrence and the dose of oral prednisolone was increased after steroid pulse therapy. Fundus examination showed small retinal pigment epithelial detachment and serous retinal detachment (SRD). Optical coherence tomography (OCT) revealed a protruded lesion inside the SRD. Fluorescein angiography (FA) showed a small, dot-shaped fluorescein leakage in the SRD, and indocyanine green fluorescein fundus angiography showed choroidal vascular hyperpermeability that was consistent with the hyperfluorescence seen with FA. We had to determine whether the protruded lesion inside the SRD was CNV secondary to the inflammation due to GPA or whether it was central serous chorioretinopathy (CSC)-like condition caused by high-dose steroid treatment.Diagnoses:We confirmed that the SRD was due to CSC but not CNV because the protruded lesion examined by B-scan OCT angiography (OCTA) showed no blood flow.Interventions:We decided to reduce the dose of steroid.Outcomes:Since the reduction of steroids, no sign of worsening in the protruded lesions with SRD has been observed.Lessons:We therefore propose the effectiveness of this advanced function of OCTA for the examination of blood flow signal images to detect CNV.     

An unusual presentation of eosinophilic granulomatosis with polyangiitis with a good response to rituximab

IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that belongs to the anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. It usually presents with late onset asthma in association with ear, nose and throat, cardiac, lung and gastrointestinal involvement.Aim of the workTo present a case of EGPA man with an unusual presentation.Case reportA 24 years old patient with EGPA presented with deep vein thrombosis and nerve palsy without a history of asthma or other usual manifestations of the disease. The left leg showed a skin rash and associated deep vein thrombosis as well as a small ulcer over the pulp of the right thumb. The total leucocyte count was 14.5 × 10⁹/L (41% eosinophils), erythrocyte sedimentation rate was 30 mm/1st hour and C-reactive protein 12.2 mg/L. Hepatitis B and C serology, antinuclear antibody (ANA) and antiphospholipid antibodies were all negative. ANCA was positive: myeloperoxidase (MPO) 36.9 U/ml and proteinase-3 (PR3) 15.9 U/ml and urinalysis was normal. The dermal and subcutaneous blood vessels displayed fibrinoid deposits, necrosis, and neutrophilic infiltrates in keeping with vasculitis. The patient showed a prompt and good response to corticosteroid and rituximab therapy with resolution of his symptoms.ConclusionThere is a necessity to keep an open eye for the diagnosis of EGPA in patients with eosinophilia even in the absence of asthma or other usual manifestations, as prompt therapy may be organ and life-saving. B cell therapy should be considered in patients resistant to conventional therapy and those in whom preservation of fertility is desired.     

Spontaneously regressed granulomatosis with polyangiitis: A case report

A 71-year-old woman presented with chest pain, cough, and back pain. A chest roentgenogram showed multiple nodular shadows in both lungs. She was diagnosed with granulomatosis with polyangiitis (GPA). The multiple nodular shadows in both lungs regressed spontaneously in a few months. There are few reports of spontaneous regression of GPA, and the underlying mechanism is unclear. Neutrophil extracellular traps (NETs) have been recently shown to be involved in GPA. NETs may also be related to the natural regression of GPA.     

微型多血管炎肾损害的预后

目的 :分析微型多血管炎 (MPA)肾损害的临床、病理特点及人肾存活率 ,探讨影响存活率的因素。　 方法 :1994年 12月至 2 0 0 3年 12月明确诊断为MPA的患者 6 0例 ,其中男 34例 ,女 2 6例 ,年龄 (49± 13 5 )岁 ,病程 1～ 14 4个月 (中位 2 8个月 ) ,除肾脏损害外 ,均有一个以上肾外器官受累。所有患者行肾活检 ,对肾组织病理活动指数 (AI)和慢性指数 (CI)进行半定量积分。分析人肾存活率及影响人肾存活率的因素。　 结果 :①入院时4 0例 (6 6 7% )抗中性粒细胞胞浆抗体 (ANCA)阳性 ,SCr 4 81 2 3± 2 89 71μmol/L ,2 7例 (45 % )需透析治疗 ,肾活检病理显示新月体比例 16 7%～ 96 2 % (5 8 9± 32 0 ) % ,半数患者见间质血管炎 ,平均CI 5 36± 2 6 1。②人存活 :在随访 1～ 15 5个月 (中位 12个月 )间 ,共 13例 (2 1 7% )死亡 ,10例于确诊后一年内 [(3 6± 2 2 )个月 ) ]死亡 ,死因为肺出血 (4例 )、感染 (3例 )、尿毒症 (2例 )和心衰 (1例 )。 1年和 3年存活率分别为 76 8%、71 1% ,ANCA阳性组存活率显著低于ANCA阴性组 (1年存活率 6 7 6 %vs 10 0 % ,P =0 0 0 0 9)。年龄≥ 5 0岁、入院即需透析治疗是一年内死亡的危险因素。③肾存活 :4 5例接受强化免疫抑制治疗 (激素联合环磷酰胺 ) 71 2