Wang F, Zhu X, He B, Zhu H, Shen N
Department of Respiratory and Critical Care Medicine, Beijing 100191, China.
Department of Pathology, Peking University Third Hospital, Beijing 100191, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Dec 18;53(6):1196-1200. doi: 10.19723/j.issn.1671-167X.2021.06.032.
A 41-year-old female patient was admitted in Department of Respiratory and Critical Care Medicine, Peking University Third Hospital because of having cough for a year. Multiple subpleural ground grass and solid nodules could be seen on her CT scan. Four months before admission, she began to experience dry mouth and eyes, blurred vision, finger joints pain, muscle pain and weakness in both lower limbs and weight loss. At the time of admission, the patient's vital signs were normal, no skin rash was seen, breath sounds in both lungs were clear, no rales or wheeze, no deformities in her hands, no redness, swelling, or tenderness in the joints. There was no edema in both lower limbs. Some lab examinations were performed. Tumor markers including squamous cell carcinoma (SCC) antigen, neuron-specific enolase (NSE), carcinoembryonic antigen (CEA), Cyfra21-1, pro-gastrin-releasing peptide (proGRP), carbohydrate antigen 125 (CA125) and carbohydrate antigen 199 (CA199) were all normal. The antinuclear antibody, rheumatoid factor, antineutrophil cytoplasmic antibody, anti-dsDNA antibody, anti-Sm antibody, anti-SSA/SSB antibody, anti-ribonucleoprotein (RNP) antibody, anti-Jo-1 antibody, anti-SCL-70 antibody and anti-ribosomal antibody were all negative. The blood IgG level was normal. The blood fungal β-1.3-D glucose, aspergillus galactomannan antigen, sputum bacterial and fungal culture, and sputum smear test for acid-fast staining were all negative. Lung function was normal. Bronchoscopy showed the airways and mucosa were normal. To clarify the diagnosis, she underwent thoracoscopic lung biopsy, the histopathology revealed follicular bronchiolitis (FB) with nonspecific interstitial pneumonia (NSIP). She did not receive any treatment and after 7 months, the lung opacities were spontaneously resolved. After 7 years of follow-up, the opacities in her lung did not relapse. To improve the understanding of FB, a literature research was performed with "follicular bronchiolitis" as the key word in Wanfang, PubMed and Ovid Database. The time interval was from January 2000 to December 2018. Relative articles were retrieved and clinical treatments and prognosis of FB were analyzed. Eighteen articles concerning FB with complete records were included in the literature review. A total of 51 adult patients with FB were reported, including 18 primary FB and 33 secondary FB, and autoimmune disease was the most common underlying cause. Forty-one (80.4%) patients were prescribed with corticosteroids and/or immunosuppressive agents, 6 (11.8%) patients were treated with anti-infective, 5 (9.8%) patients did not receive any treatment. The longest follow-up period was 107 months. Among the 5 patients without any treatment, 1 patients died of metastatic melanoma, the lung opacities were unchanged in 1 patient and getting severe in 3 patients. In conclusion, FB is a rare disease, the treatment and prognosis are controversial. Corticosteroid and immunosuppressive agents could be effective. This case report suggests the possibility of spontaneous remission of FB.
一名41岁女性患者因咳嗽1年入住北京大学第三医院呼吸与危重症医学科。胸部CT可见多个胸膜下磨玻璃影和实性结节。入院前4个月，她开始出现口干、眼干、视力模糊、手指关节疼痛、肌肉疼痛、双下肢无力及体重减轻。入院时，患者生命体征正常，未见皮疹，双肺呼吸音清，未闻及啰音或哮鸣音，双手无畸形，关节无红肿及压痛。双下肢无水肿。进行了一些实验室检查。肿瘤标志物包括鳞状细胞癌（SCC）抗原、神经元特异性烯醇化酶（NSE）、癌胚抗原（CEA）、细胞角蛋白19片段（Cyfra21-1）、胃泌素释放肽前体（proGRP）、糖类抗原125（CA125）和糖类抗原199（CA199）均正常。抗核抗体、类风湿因子、抗中性粒细胞胞浆抗体、抗双链DNA抗体、抗Sm抗体、抗SSA/SSB抗体、抗核糖核蛋白（RNP）抗体、抗Jo-1抗体、抗SCL-70抗体及抗核糖体抗体均为阴性。血IgG水平正常。血真菌β-1,3-D-葡聚糖、曲霉半乳甘露聚糖抗原、痰细菌及真菌培养、痰涂片抗酸染色检查均为阴性。肺功能正常。支气管镜检查显示气道及黏膜正常。为明确诊断，患者接受了胸腔镜肺活检，组织病理学显示为滤泡性细支气管炎（FB）合并非特异性间质性肺炎（NSIP）。患者未接受任何治疗，7个月后肺部阴影自行消退。随访7年，肺部阴影未复发。为提高对FB的认识，以“滤泡性细支气管炎”为关键词在万方、PubMed和Ovid数据库进行了文献研究。时间跨度为2000年1月至2018年12月。检索相关文章并分析FB的临床治疗及预后。文献综述纳入18篇关于FB且记录完整的文章。共报道51例成年FB患者，其中原发性FB 18例，继发性FB 33例，自身免疫性疾病是最常见的潜在病因。41例（80.4%）患者使用了糖皮质激素和/或免疫抑制剂，6例（11.8%）患者接受抗感染治疗，5例（9.8%）患者未接受任何治疗。最长随访时间为107个月。在5例未接受任何治疗的患者中，1例死于转移性黑色素瘤，1例肺部阴影无变化，3例病情加重。总之，FB是一种罕见病，治疗及预后存在争议。糖皮质激素和免疫抑制剂可能有效。本病例报告提示FB有自发缓解的可能。
