累及气道的复发性多软骨炎2例

正复发性多软骨炎(relapsing polychondritis)本身为一种少见、长程、有潜在生命危险的炎症性疾病,而以"喉、气管及支气管软骨炎"为主要表现的复发性多软骨炎更是呼吸内科罕见病例[1]。因此,临床医生对此病通常认识不足,导致漏诊、误诊。该病如果早期诊断、及时诊治,可改善患者预后。因此,为加深呼吸科医生对该病的认识,本文将2014年我院呼吸科收治的两例复发性多软骨炎患者报道如下。     

气道和主动脉受累的复发性多软骨炎一例

患者,男,17岁,因"咳嗽25天,发热3周"于2019年9月17日收入我科.患者25天前无明显诱因出现咳嗽,4天后出现发热,体温最高达38. 5℃,伴畏寒,夜间咳少量白痰,伴胸骨旁疼痛,自行口服抗生素、复方甲氧那明胶囊和孟鲁司特,咳嗽及发热症状均未见明显好转,后来我院就诊.既往史、个人生活史无特殊.家族史:母亲患大动脉...     

复发性多软骨炎致气道严重狭窄一例并文献复习

目的 总结复发性多软骨炎(RP)的临床表现以及气道受累的特点和治疗策略,提高对该病的认识.方法 报道1例RP合并严重气管狭窄的临床特点、诊治经过,并对该病进行文献复习.结果 患者为33岁女性,以咳嗽、声嘶、气短起病,3个月后确诊RP,此时已经出现严重的气管狭窄.糖皮质激素和免疫抑制剂治疗有效.结论 RP出现呼吸道受累并不少见,部分患者为首发症状.临床上应高度重视RP气道受累的早期诊治.否则预后不良.     

累及气道的复发性多软骨炎2例并文献复习

目的提高对侵犯气管、支气管的复发性多软骨炎的认识,减少误诊和漏诊率。方法分析我院2例累及气道的复发性多软骨炎的临床资料。结果1例患者因就诊时较晚,最后死于呼吸衰竭,另1例患者较早得到诊断,激素治疗预后较好。结论对伴有气管、支气管受累的复发性多软骨炎及时正确诊断,在疾病早期糖皮质激素治疗能显著改善症状,延缓疾病进展。     

复发性多软骨炎五例分析

目的　探讨复发性多软骨炎 (RP)的临床特征、治疗方法 ,特别是RP合并严重气道狭窄的介入治疗。方法　结合文献资料回顾分析 5例临床病例。结果　RP是一种以全身多器官软骨结构及结缔组织反复发作性炎症为特征的自身免疫性疾病。目前以皮质类固醇激素、免疫抑制剂及氨苯砜为主的药物治疗常不能有效控制病变发展 ,其中气道软骨受累最危险 ,常需介入性对症治疗如气管造口、气管内插管、支架等。通过气道内可扩张的金属支架安置术成功缓解了一名RP患者因广泛气道软骨塌陷所致的严重呼吸困难。结论　对有特征性临床表现的病例应及早确诊并予药物治疗 ,当出现气道软骨受累时应及时给予压力支持及介入治疗以维持气道开放     

置入气道支架治疗复发性多软骨炎一例

患者女性 ,4 0岁。因反复咳嗽、喘息伴活动后气喘 ,进行性加重 8个月 ,于 2 0 0 0年 5月入院。患者 8个月前开始咳嗽 ,咳少量白粘痰 ,喘息伴活动后气喘并进行性加重。先后至多家医院就诊 ,按“支气管炎 ,哮喘”等治疗无效。入院查体 :体温 3 6 8℃ ,脉搏 112次 /ｍｉｎ ,呼吸 2 4次 /ｍｉｎ ,血压 10 5 /60ｍｍＨｇ( 1ｍｍＨｇ =0 13 3ｋＰａ) ,神清 ,精神差 ,口唇无紫绀 ,呼吸急促 ,三凹征 ,双肺呼吸音粗 ,散在哮鸣音 ,心率 112次 /ｍｉｎ ,律齐 ,各瓣膜听诊区未闻及病理性杂音 ,腹平软 ,无压痛 ,肝脾肋下未触及 ,双下肢不水肿。查白细胞…     

复发性多软骨炎六例诊治体会

复发性多软骨炎是一种少见的风湿病 ,主要累及耳、鼻、气管支气管等富含软骨部位和内耳、眼等特殊感官器官 ,部分患者因气管塌陷窒息或感染致死亡。本科自 1992年以来诊治 6例复发性多软骨炎病人 ,现将该 6例病人的临床表现和诊治体会总结如下。1　临床资料诊治 6例病人中 ,5例为女性 ,1例为男性 ,年龄范围在38～ 6 7岁之间。从出现症状到确诊所需时间最短为 2个月 ,最长为 3年。除例 4、例 5、例 6首发症状为耳廓软骨炎症外 ,余 3例分别为持续咳嗽 ,经抗感染治疗无效 ,双眼相继出现角膜溃疡 ,声嘶 (见表 1)。2　病历摘要例 1:患者在持续咳…     

复发性多软骨炎2例

复发性多软骨炎(RP)为少见病,临床表现复杂多样,易漏诊、误诊,尤以呼吸道受累为首发表现时因症状缺乏特异性而误诊率高。林淑媚等报道1例病史长达20年最终确诊的呼吸道受累的RP病例。以呼吸道受累为首发症状的病例较少,仅占18%,但病程中最终出现呼吸道受累的比例高达50%。严重的呼吸道软骨病变可导致死亡,成为该病致死的主要原因。早期诊断及有效治疗对于改善预后至关重要。     

以声嘶为首发症状的复发性多软骨炎1例报告并文献复习

1病历资料患者女,37岁,江西上饶人,汉族,家庭妇女。4个月前无明显诱因出现单声干咳,无痰,以夜间为主,咳嗽不剧,无咽痛、发热,无声嘶、胸痛。干咳持续2个月后出现持续性声音嘶哑,同时有咽部黏稠异物感,不能咳出。此后多次于杭州市各家医院就诊,诊断为"肺炎,上呼吸道感染"等,予"头孢美唑,克林霉素"等抗感染、补液治疗,病情好转后症     

Relapsing catastrophic antiphospholipid syndrome: report of three cases

BACKGROUND: The catastrophic variant of the antiphospholipid syndrome (CAPS), also now known as Asherson's syndrome, is defined as a potential life-threatening variant of the antiphospholipid syndrome, which is characterized by multiple small-vessel thrombosis that can lead to multiorgan failure. Relapses in patients with the CAPS are very uncommon. OBJECTIVE: To describe the clinical and laboratory features of patients with relapsing episodes of CAPS. METHODS: Three patients with relapsing CAPS are presented with their clinical and laboratory features. RESULTS: Seven episodes of CAPS that occurred in the 3 patients reported were analyzed. The median time between the episodes of CAPS was 12.5 months (range, 2.5-48). Precipitating factors were identified in 2 episodes only (Legionella respiratory tract infection and periodontal infection). The most significant manifestations of the episodes were renal involvement (5 episodes), central nervous system and cardiac involvement (4 episodes), and pulmonary and hepatic involvement (3 episodes each). Interestingly, laboratory features of definite microangiopathic hemolytic anemia (MHA) were present in 5 of 7 episodes of relapsing CAPS. The remaining episodes presented with thrombocytopenia, schistocytes, and anemia but data concerning hemolysis and Coombs tests were not reported. Rituximab was used in 2 episodes. CONCLUSIONS: Relapses occur very infrequently in patients with the CAPS. The presence of MHA is common in these patients, suggesting that an association between MHA and relapses of CAPS could be present and that a "continuum" between various MHAs might exist, as recently suggested.     

44例肺内结节病诊断方法分析并文献复习

目的回顾性分析肺内结节病的诊治过程,提高诊治水平。方法回顾分析2002年1月至2009年4月在我院住院,经各种检查确诊的肺内结节病患者44例,并复习关于肺内结节病诊断方法进展的文献。结果 44例患者中34例行支气管镜检查,其中20例次行EBB,阳性率35%。14例经纵隔镜、VATS或开胸肺活检,16例行外周淋巴结活检,阳性率均为100%,6例行前斜角肌淋巴结活检,阳性率67%,8例行皮肤病变活检,阳性率75%。结论随着支气管镜技术的发展及EBUS的应用,肺内结节病的诊断准确性增加,从而减少纵隔镜、VATS、开胸肺活检的应用。     

Wegener's granulomatosis involving the breast: Report of three cases and review of the literature

Three cases of necrotizing granulomatous vasculitis of the Wegener's type involving the breast are described. In each, the presumed diagnosis was localized or metastatic carcinoma. The breast lesion and other manifestations of Wegener's granulomatosis developed concomitantly in one patient, and the breast lesion appeared several months before classic pulmonary symptoms in another. In the third patient, not only were her upper and lower airway symptoms explained only in retrospect, but even the significance of the breast biopsy findings was clarified only after other aspects of Wegener's granulomatosis developed. All patients ultimately demonstrated a good response to corticosteroids and cyclophosphamide therapy. Wegener's granulomatosis is a rare lesion affecting the breast but should nonetheless be considered in the evaluation of any breast mass.     

血管淋巴样增生伴嗜酸性粒细胞浸润合并肾损害三例并文献复习

目的分析血管淋巴样增生伴嗜酸性粒细胞浸润（ALHE）合并肾损害的临床病理特点。方法分析北京大学第一医院肾内科诊断的3例ALHE合并肾损害的临床资料,并对其淋巴结及肾活检组织进行病理学观察。结果3例患者均为男性,以头颈部皮下肿块伴大量蛋白尿和肾功能损害为主要临床表现,外周血嗜酸性粒细胞增多和IgE增高,2例有血免疫球蛋白及补体异常。糖皮质激素及环磷酰胺治疗有效,其中例3患者停药后病情反复。病理学改变：淋巴结和肾组织内可见突出的小血管增生,内皮细胞增生肥大呈上皮细胞样,管腔狭窄或闭塞,伴有淋巴细胞、浆细胞及较多的嗜酸性粒细胞浸润。免疫组化染色可见CD31、CD34及F8在增生的血管内皮细胞呈阳性。肾脏病变主要累及肾小管间质,其中例1患者的肾脏病理改变为Ⅰ期膜性肾病。结论ALHE合并肾损害在我国并不罕见,其临床病理特征支持其为一种与变态反应有关的血管及淋巴组织增生性疾病。     

肺隔离症3例并文献复习

目的提高对肺隔离症临床特点的认识。方法对我院确诊的3例肺隔离症患者的临床资料进行回顾性分析。结果肺隔离症大多数缺乏特征性临床表现,表现为反复咳嗽、咳痰、胸痛、咯血等。所有病例均确诊,主要诊断手段为胸片、CT及CT三维重建技术。1例成功行肺叶切除,无手术死亡及并发症发生。结论肺隔离症术前极易误诊,诊断方法主要为X线、CT及CT三维重建技术检查,螺旋CT增强扫描三维重建可显示异常供血动脉,是诊断肺隔离症的首选检查方法。手术是本症最佳治疗方法。     

Relapsing polychondritis: a clinical review

OBJECTIVE: This study comprehensively reviews the literature related to relapsing polychondritis (RP). METHODS: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. RESULTS: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. CONCLUSIONS: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood.