表现为进展性纤维化性间质性肺疾病的过敏性肺炎一例并文献复习

A case of hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease and literature review

目的分析一例表现为进展性纤维化性间质性肺疾病的过敏性肺炎患者的临床特征及诊治过程,结合文献复习,加强临床医生对本病的认识,以期早期诊断、规范治疗,改善患者预后。方法回顾性分析一例过敏性肺炎患者资料,结合检索到的282篇相关文献,总结了过敏性肺炎的诊断手段,治疗及预后不良相关因素。结果患者女,45岁,个体经营者,因"咳嗽、咳痰9年,呼吸困难6个月余"入院,无明显肺外临床表现;查体双肺底闻及湿罗音,查见杵状指(趾);高分辨率CT提示主要表现为磨玻璃、网格状影,呈间质性肺炎特征,肺内病变呈渐进加重,诊断时部分肺叶呈蜂窝肺样改变。8年前,外院行纤支镜检查,支气管肺泡灌洗液提示淋巴细胞比例增高(39%);肺功能提示极重度限制性通气功能障碍,短时间给予强的松治疗,患者自行停药,疗效不肯定;我院冷冻肺活检结果提示细支气管周围化生、间质增宽伴淋巴细胞浸润,未见肉芽肿及纤维母细胞灶。感染、免疫相关指标未见明显异常,确诊为过敏性肺炎,此时患者肺内病变部分呈蜂窝肺样改变,积极给予强的松正规抗炎3个月、吡非尼酮抗纤维化药物治疗2个月余,治疗反应不佳。文献检索报道病例87例,其中男39例(44.82%),女48例(55.18%),平均年龄(47.0±18.4)岁。70例(80.46%)有明确变应原。3.45%支气管肺泡灌洗液中淋巴细胞百分比介于20%~39%,19.54%淋巴细胞百分比≥40%。4.60%的患者通过脱离变应原获得缓解;71.27%的患者通过糖皮质激素治疗获得缓解,14.94%通过糖皮质激素治疗后病情好转,1.15%患者死亡;6.89%患者接受抗过敏治疗后获得缓解。长病程的过敏性肺炎患者预后差。结论不明原因的病变仅局限于肺部的弥漫间质性肺纤维化患者,行经纤支镜冷冻肺活检是尽早明确诊断、改善预后的可行手段。肺内已经出现纤维化改变的长病程过敏性肺炎患者对糖皮质激素等经典治疗反应欠佳,多数预后差。

Objective To strengthen the understanding to hypersensitivity pneumonitis and make early diagnosis and standard treatment by analyzing the clinical features,the diagnosis and treatment of a patient diagnosed as hypersensitivity pneumonitis presenting as progressive fibrotic interstitial lung disease in combination with literature review.Methods The diagnosis and treatment process and relevant clinical data of the patient were analyzed retrospectively and literatures were reviewed.Based on 282 relevant literatures,the diagnostic methods,treatment and prognostic factors of hypersensitivity pneumonitis were summarized.Results The patient,female,45 years old,selfemployed,was admitted to the hospital due to"cough,sputum for 9 years,dyspnea for more than 6 months"without obvious extrapulmonary clinical manifestations.Creaks at the bottom of the lungs and clubbed toes were found through physical examination.High-resolution computed tomography indicated that the main manifestations were ground glass and grid-like shadows,presenting characteristics of interstitial pneumonia.The pulmonary lesions aggravated gradually,and part of the lung lobe presented honeycomb lung at the time of diagnosis.Eight years ago,she performed fibrobronchoscopy in other hospital revealing an increased lymphocyte proportion(39%)in bronchoalveolar lavage fluid.Lung function suggested very severe restrictive ventilation dysfunction.She was given prednisone for a short time,and the drug was stopped by herself with uncertain efficacy.Later,she performed frozen lung biopsy suggesting peribronchiolar metaplasia interstitial broadening with lymphocytic infiltration,without granuloma or fibroblast lesions.No obvious abnormity was seen in makers of infections and immunology.At this point,the patient was clearly diagnosed as hypersensitivity pneumonitis.Meanwhile,the patient’s pulmonary lesions were still mainly made of ground glass and plaques,partly with changes like honeycomb.Poor response was obtained with anti-inflammatory treatment of prednisone for 3 months and anti-fibrosis treatment of pirfenidon for more than 2 months.Literature review resulted in87 patients,including 39 males(44.82%)and 48 females(55.18%),with an average age of(47.0±18.4)years.Seventy patients(80.46%)had clear allergens.3.45%patients’lymphocyte percentage in bronchoalveolar lavage fluid ranged from20%to 39%,and 19.54%patients’lymphocyte percentage was more than 40%.4.60%of the patients achieved remission through detachment from allergens;71.27%of the patients achieved remission with glucocorticoid treatment,14.94%improved with glucocorticoid treatment,and 1.15%died;6.89%of the patients achieved remission after receiving antiallergy therapy.Patients with chronic hypersensitivity pneumonia were found with poor prognosis.Conclusions In patients with diffuse interstitial pulmonary fibrosis with unknown cause,transbronchoscope freezing lung biopsy as early as possible is a feasible method for early diagnosis and improving prognosis.Patients with hypersensitivity pneumonitis with a long course of pulmonary fibrosis have a poor response to glucocorticoid and other classic treatments,and most of them have a poor prognosis.