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Wegener's Granulomatosis Combined with Pulmonary Tuberculosis: Report of 8 Cases and Literature Review
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摘要:目的 分析韦格纳肉芽肿合并肺结核的临床特征。方法 回顾性分析北京协和医院1990年5月至2010年5月收治的韦格纳肉芽肿合并肺结核8例的临床特征, 并复习相关文献。结果 8例患者中男性5例, 女性3例; 发病年龄最小19岁, 最大70岁, 平均年龄52.7岁。首先诊断韦格纳肉芽肿, 并在激素应用过程中诊断肺结核5例, 这5例患者均为痰涂片抗酸杆菌阳性患者, 其中4例否认结核病史及结核接触史; 同时诊断韦格纳肉芽肿合并肺结核2例; 首先诊断肺结核, 后诊断合并韦格纳肉芽肿1例。临床表现肺部最常见为咯血, 其次为咳嗽、咳痰, 其他器官受累包括肾脏(异型红细胞尿), 耳鼻喉(流脓涕、听力受损)。韦格纳肉芽肿合并结核感染的治疗, 在尽量不减少激素剂量的同时加强抗结核治疗。结论 韦格纳肉芽肿合并肺结核并不少见, 应引起临床医生的重视, 早期诊断, 积极治疗, 改善预后。Abstract:Objective To analyze the clinical manifestations of patients with Wegener's granulomatosis (WG) and pulmonary tuberculosis (TB).Methods We retrospectively analyzed the clinical data of 8 patients with Wegener's granulomatosis combined with pulmonary tuberculosis in Peking Union Medical College Hospital from May 1990 to May 2010.Results There were 5 men and 3 women aged 19-70 years. Five patients were initially diagnosed as Wegener's granulomatosis but were confirmed to be with pulmonary tuberculosis during corticosteroid therapy. All these five patients had positive acid-fast bacilli smear test results, and four of them denied past a history of tuberculosis or tuberculosis exposure. Two were diagnosed as Wegener's granulomatosis and pulmonary tuberculosis almost the same time. In the remaining one case, tuberculosis was diagnosed firstly, and then Wegener's granulomatosis was also identified. The most common clinical manifestation in lung was hemoptysis, followed by cough and expectoration. Other involved organs included kidney (abnormal red blood cells in urine) and ear/nose/throat (suppuration and hearing impairment). Management was mainly based on anti-TB therapy and enhanced corticosteroid therapy.Conclusions Patients with Wegener's granulomatosis combined with pulmonary tuberculosis are not uncommon. Early diagnosis and treatment can improve the prognosis.
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Keywords:
- pulmonary tuberculosis /
- Wegner's granulomatosis
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肺郎格罕细胞组织细胞增多症(pulmonary Langerhans cell histiocytosis, PLCH)是一种原因未明的少见病, 属于郎格罕细胞组织细胞增多症(Langerhans cell histiocytosis, LCH)的一个分型。为单器官肺受累, 目前无确切发病率的流行病学统计资料, 主要发生于中年人, 占肺间质性疾病肺活检的5%左右, 并与吸烟密切相关。PLCH早期常被误诊误治, 最终确诊需要肺活检病理。本文报道1例发病初表现为双肺散在囊腔样改变并最终发展至全肺弥漫性肺气肿、肺大疱的PLCH, 以提高临床医生对本病的早期认识。
临床资料
患者男性, 38岁, 因“干咳6年, 反复气胸1年余”入院。病程中无发热、皮疹、关节痛。6年前胸部X线片、CT显示双肺多发散在小囊腔, 壁厚约1 ~ 2 mm, 双上肺为著, 肺门及纵隔淋巴结不大(图 1A, 2A)。外院诊断为先天性肺囊肿, 未予特殊诊治。近1年来反复发作气胸3次, 对症治疗后好转。3月前胸部X线片、CT示全肺弥漫性肺气肿, 右侧肺大疱并气胸(图 1B, 2B)。有吸烟史20余年, 平均2包/d。入院后行胸腔镜下右肺中叶肺组织活检并上叶大疱修补。病理回报:肺组织内有多个由组织细胞、嗜酸性粒细胞及淋巴细胞构成的肉芽肿及凝固性坏死; 可见典型郎格罕细胞, 细胞核染色质细腻、均匀, 核有明显核沟, 另可见散在嗜酸性粒细胞(图 3)。免疫组化:CD68 (+); Langerhans细胞S-100阳性(+ +) (图 4); Langerhans细胞CD1a阳性(+) (图 5); 特染:粘卡(-); 六胺银(-)。肺郎格罕细胞组织细胞增多症诊断明确。
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图 2 胸部CTA.双肺弥漫磨玻璃影, 网织状间质纹理增厚, 并弥漫分布多个结节及大小不一、壁厚薄不均含气囊腔, 囊腔有融合趋势; B.6年后双肺弥漫性肺气肿, 右上肺肺大疱讨论
肺郎格罕细胞组织细胞增多症(PLCH)病变主要在肺部, 较少伴随其他脏器受累的表现。根据目前文献资料, 单肺受累的PLCH与多系统受累的LCH, 无论从病因、发病机制还是治疗方案、预后, 均截然不同。PLCH发病以成年男性为主, 与吸烟关系极为密切, 90%为吸烟者, 可能与吸烟者肺部免疫过激, 并有慢性炎症有关[1]。
PLCH一般起病隐匿, 最常见的症状为干咳, 晚期可以出现胸痛、咯血及呼吸困难。反复发生的气胸是其重要的临床特征。胸部影像学早期可见弥漫分布于两肺中、上肺野的囊腔, 囊壁厚薄不均, 常伴有多发小结节影, 肋膈角区相对较轻, 为PLCH典型改变, 但仍需与先天性肺囊肿、支气管扩张、肺转移瘤及淋巴管平滑肌瘤进行鉴别[2-3]; 晚期可发展为肺气肿、肺大疱、纤维化乃至蜂窝肺, 此时仅凭影像与其他原因引起的肺气肿和肺纤维化区分则非常困难。其早中期影像学非常有特征性, 但由于病例罕见, 如对其认识不够, 极易误诊或漏诊。本例就被误诊为先天性肺囊肿长达6年, 耽误了最佳治疗时机。
PLCH最终诊断靠病理, 可以通过支气管镜肺活检及肺泡灌洗, 但阳性率低。开胸或胸腔镜肺活检取材比较大, 可以选择相应病变部位, 诊断率高, 还可以指导治疗和判断预后[4]。郎格罕细胞在光镜下细胞核染色质细腻、均匀, 核有明显核沟, 胞质中度嗜酸。电镜下胞质内有特殊的Birbeck颗粒, 呈网球拍状或棒状。免疫组化:CD1a(特异性最高), S-100、CD68均为阳性[5]。
PLCH的郎格罕细胞表面标记与其他LCH有所不同, 其预后较好。治疗上首先主张戒烟。可采用糖皮质激素治疗(初始每天0.5 ~ 1 mg/kg, 逐渐减量), 具体疗程根据病情决定。激素治疗无效, 可考虑免疫抑制剂, 长春新碱、甲氨蝶呤、环磷酰氨等往往用于疾病进展患者。但如果没有及时诊断治疗而发展到晚期肺纤维化、蜂窝肺、肺大疱及肺气肿, 则预后很差[6]。
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[1] Cordier JF, Valeyre D, Guillevin L.Pulmonary Wegener's granulomatosis:a clinical and imaging study of 77 cases[J]. Chest, 1990, 97:906-912. DOI: 10.1378/chest.97.4.906
[2] Duna GF, Galperin C, Hoffman GS.Wegener's granulomatosis[J]. Rheum Dis Clin North Am, 1995, 21:949-986. https://pubmed.ncbi.nlm.nih.gov/14968341/
[3] Hagen EC, Daha MR, Hermans J, et al.Diagnostic value of standandized assays for anti-neutrophil cytoplasmic antibodies in idiopathicsy stemic vasculitis.EC/BCR Project for ANCA Assay Standardization[J]. Kidney Int, 1998, 53:743-753. DOI: 10.1046/j.1523-1755.1998.00807.x
[4] Pradhan VD, Badakere SS, Ghosh K, et al.Spectrum of anti-neutrophil cytoplasmic antibodies in patients with pulmonary tuberculosis overlaps with that of Wegener's granulomatosis [J]. Indian J MedSci, 2004, 58:283-288. http://www.wanfangdata.com.cn/details/detail.do?_type=perio&id=81391c72a549c8b1d5961370edf4b209
[5] Flores-Suárez LF, Cabiedes J, Villa AR, et al.Prevalence of antineutrophil cytoplasmic autoantibodies in patients with tuberculosis[J]. Rheumatology, 2003, 42:223-229. DOI: 10.1093/rheumatology/keg066
[6] Esquivel-Valerio JA, Flores-Suárez LF, Rodríguez-Amado J, et al, Antineutrophil cytoplasm autoantibodies inpatients with tuberculosis are directed against bactericidal/permeability increasing protein and are detected after treatment initiation [J]. Clin Exp Rheumatol, 2010, 28(1 Suppl57):35-39. http://www.ncbi.nlm.nih.gov/pubmed/20412700/
[7] Stone JH, Merkel PA, Spiera R, et al.Rituximab versus cyclophosphamide for ANCA-associated vasculitis[J]. N Engl J Med, 2010, 363:221-232. DOI: 10.1056/NEJMoa0909905
[8] Toyoshima M, Chida K, Suda T, et al.Wegener's granulomatosis responding to antituberculous drugs[J]. Chest, 2001, 119:643-645. DOI: 10.1378/chest.119.2.643
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