Fibrous mediastinitis: a case report and review of the literature_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

 WANG Yamin , JIANG Guoqiang

Department of Respiratory Medicine, Hospital of Sichuan Provincial Armed Police Force, Leshan, Sichuan 614000, P. R. China;

Corresponding?author：

WANG Yamin, Email: 67517267@qq.com

Keywords：

Mediastinitis; Tomography, X-Ray Computed; Pathology; Diagnosis

DOI：

10.7507/1671-6205.201709037

Video：

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ObjectiveTo explore the etiology, clinical manifestation, computed tomography (CT) manifestations, pathological character, diagnosis, differential diagnosis, and treatment of fibrous mediastinitis in China.MethodsThe clinical data of a patient with fibrous mediastinitis admitted to Hospital of Sichuan Provincial Armed Police Force were retrospectively analyzed and the related literature was reviewed. A literature research was performed with " fibrous mediastinitis, mediastinal fibrosis, sclerosing mediastinitis, chronic mediastinitis ”as the Chinese key word in WanFang database and China national knowledge internet, and " fibrous mediastinitis, mediastinal fibrosis, fibrosis of mediastinum, fibrosing mediastinitis, sclerosing mediastinitis, chronic mediastinitis” as English key words in PubMed database. The time interval was from January 1980 to December 2016.ResultsThe patient was a 59 year old male, whose chest CT scan showed soft tissue density in pulmonary hilar and mediastina, with bronchus truncation and obvious stenosis of pulmonary artery in hilar. Literature review found 12 related articles reporting 37 cases of fibrous mediastinitis in China. Fifteen cases were caused by tuberculosis infection presumably, and 9 cases were idiopathic mediastinal fibrosis. The common clinical manifestations were cough, dyspnea, chest tightness and pain, fever, and edema. CT manifested soft tissue density in pulmonary hilar and mediastina, which were diffuse in 32 cases. Trachea and bronchus were involved in 26 cases, while pulmonary artery in 29 cases, pulmonary vein in 13 cases, plural effusion in 16 cases, and vena cava and its branch in 8 cases. Other involved regions were described in 18 cases, including aorta and its branch in 4 cases, esophagus in 1 case, and there were pericardial effusion in 6 cases. Fourteen cases had pathology results.ConclusionsThe most common etiological factor of fibrous mediastinitis in China is tuberculosis infection, secondly idiopathic mediastinal fibrosis. Clinical manifestation is nonspecific. mostly diffuse, surrounding bronchus, esophagus and blood vessels in hilar and mediastina, and sometimes infiltrating into pericardium and pleurae. Its pathological character is proliferation of fibrous tissue, with proliferation of lymphocytes, and without envelope. The diagnosis depends on CT and pathology. Corticosteroid is considered to treat idiopathic mediastinal fibrosis, while surgery and vascular interventional therapy may improve symptoms of vascular compression in mediastina.

Citation： WANG Yamin, JIANG Guoqiang. Fibrous mediastinitis: a case report and review of the literature. Chinese Journal of Respiratory and Critical Care Medicine, 2018, 17(6): 593-598. doi: 10.7507/1671-6205.201709037 Copy

1.	鐘南山, 劉又寧. 呼吸病學. 第 2 版. 北京: 人民衛生出版社, 2012, 824-825.
2.	Hu Y, Qiu JX, Liao JP, et al. Clinical manifestations of fibrosing mediastinitis in Chinese patients. Chin Med J (Engl), 2016, 129(22): 2697-2702.
3.	胡艷, 廖紀萍, 王廣發. 縱隔纖維化引起肺動脈高壓一例. 中華結核和呼吸雜志, 2016, 39(3): 230-231.
4.	許凡勇, 夏進東, 姚軍, 等. 特發性縱隔纖維化 CT 與病理表現. 臨床放射學雜志, 2016, 35(2): 202-206.
5.	潘晶晶, 程波, 王憲雯, 等. 纖維性縱隔炎 CT 誤診 1 例. 中國臨床醫學影像雜志, 2014, 25(10): 755-756.
6.	余燕武, 唐永華, 魏鼎泰, 等. 特發性縱隔纖維化影像學診斷. 中國中西醫結合影像學雜志, 2011, 9(6): 510-512.
7.	王墨揚, 熊長明, 倪新海, 等. 慢性纖維性縱隔炎致肺動脈高壓一例. 中國循環雜志, 2009, 24(3): 193.
8.	張燕, 張竹花, 金征宇, 等. 多層螺旋 CT 在肺動脈高壓診斷中的應用. 中國醫學科學院學報, 2006, 28(1): 44-48.
9.	柳善剛. 纖維性慢性縱隔炎一例. 臨床放射學雜志, 2004, 23(10): 907.
10.	朱力, 郭玉林, 龔瑞, 等. 縱隔非腫瘤性病變的 CT 診斷. 中國醫學影像技術, 2004, 20(S1): 24-26.
11.	嚴四軍, 劉燕, 羅貴清, 等. 化膿性感染致慢性縱隔炎 1 例. 中國誤診學雜志, 2002, 2(11): 1755-1756.
12.	郭本樹, 成官迅, 林曰增, 等. 特發性腹膜后、縱隔、眼球后廣泛纖維化一例. 臨床放射學雜志, 2000, 19(6): 395.
13.	趙明杰, 于風, 孔祥甲. 慢性后縱隔炎引起食道外壓性狹窄 1 例報告. 黑龍江醫學, 1991, 4: 60.
14.	Peikert T, Colby TV, Midthun DE, et al. Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. Medicine (Baltimore), 2011, 90(6): 412-423.
15.	Sherrick AD, Brown LR, Harms GF, et al. The radiographic findings of fibrosing mediastinitis. Chest, 1994, 106(2): 484-489.
16.	Rossi SE, McAdams HP, Rosado-de-Christenson ML, et al. Fibrosing mediastinitis. Radiographics, 2001, 21(3): 737-757.
17.	Rossi GM, Emmi G, Corradi D, et al. Idiopathic Mediastinal fibrosis: a systemic immune-mediated disorder. A case series and a review of the literature. Clin Rev Allergy Immunol, 2017, 52(3): 446-459.
18.	Santo H, Nishiyama O, Sano H, et al. Mediastinal fibrosis and positive antineutrophil cytoplasmic antibodies: coincidence or common etiology?. Intern Med, 2014, 53(3): 275-277.
19.	Gorospe L, Ayala-Carbonero AM, Fernández-Méndez Má, et al. Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. Clin Imaging, 2015, 39(6): 993-999.
20.	Valentin LI, Kuban JD, Ramanathan R, et al. Endovascular treatment of bilateral pulmonary artery stenoses and superior vena cava syndrome in a patient with advanced mediastinal fibrosis. Tex Heart Inst J, 2016, 43(3): 249-251.
21.	Murphy JC, Johnston N, Spence MS. A pressing matter--mediastinal fibrosis with near obliteration of the pulmonary arteries. Catheter Cardiovasc Interv, 2013, 81(6): 1079-1083.
22.	Doucet KM, Labinaz M, Chandy G, et al. Pulmonary hypertension due to fibrosing mediastinitis treated successfully with stenting of pulmonary vein stenoses. Can J Cardiol, 2015, 31(4): 548. e5-7.
23.	林瑋, 陳華, 張文, 等. 以 IgG4 相關性硬化性縱隔炎為突出表現的淋巴瘤一例. 中華風濕病學雜志, 2012, 16(12): 855-857.
24.	Taki M, Inada S, Arivasu R, et al. Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. Intern Med, 2013, 52: 2645-2651.

1. 鐘南山, 劉又寧. 呼吸病學. 第 2 版. 北京: 人民衛生出版社, 2012, 824-825.
2. Hu Y, Qiu JX, Liao JP, et al. Clinical manifestations of fibrosing mediastinitis in Chinese patients. Chin Med J (Engl), 2016, 129(22): 2697-2702.
3. 胡艷, 廖紀萍, 王廣發. 縱隔纖維化引起肺動脈高壓一例. 中華結核和呼吸雜志, 2016, 39(3): 230-231.
4. 許凡勇, 夏進東, 姚軍, 等. 特發性縱隔纖維化 CT 與病理表現. 臨床放射學雜志, 2016, 35(2): 202-206.
5. 潘晶晶, 程波, 王憲雯, 等. 纖維性縱隔炎 CT 誤診 1 例. 中國臨床醫學影像雜志, 2014, 25(10): 755-756.
6. 余燕武, 唐永華, 魏鼎泰, 等. 特發性縱隔纖維化影像學診斷. 中國中西醫結合影像學雜志, 2011, 9(6): 510-512.
7. 王墨揚, 熊長明, 倪新海, 等. 慢性纖維性縱隔炎致肺動脈高壓一例. 中國循環雜志, 2009, 24(3): 193.
8. 張燕, 張竹花, 金征宇, 等. 多層螺旋 CT 在肺動脈高壓診斷中的應用. 中國醫學科學院學報, 2006, 28(1): 44-48.
9. 柳善剛. 纖維性慢性縱隔炎一例. 臨床放射學雜志, 2004, 23(10): 907.
10. 朱力, 郭玉林, 龔瑞, 等. 縱隔非腫瘤性病變的 CT 診斷. 中國醫學影像技術, 2004, 20(S1): 24-26.
11. 嚴四軍, 劉燕, 羅貴清, 等. 化膿性感染致慢性縱隔炎 1 例. 中國誤診學雜志, 2002, 2(11): 1755-1756.
12. 郭本樹, 成官迅, 林曰增, 等. 特發性腹膜后、縱隔、眼球后廣泛纖維化一例. 臨床放射學雜志, 2000, 19(6): 395.
13. 趙明杰, 于風, 孔祥甲. 慢性后縱隔炎引起食道外壓性狹窄 1 例報告. 黑龍江醫學, 1991, 4: 60.
14. Peikert T, Colby TV, Midthun DE, et al. Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. Medicine (Baltimore), 2011, 90(6): 412-423.
15. Sherrick AD, Brown LR, Harms GF, et al. The radiographic findings of fibrosing mediastinitis. Chest, 1994, 106(2): 484-489.
16. Rossi SE, McAdams HP, Rosado-de-Christenson ML, et al. Fibrosing mediastinitis. Radiographics, 2001, 21(3): 737-757.
17. Rossi GM, Emmi G, Corradi D, et al. Idiopathic Mediastinal fibrosis: a systemic immune-mediated disorder. A case series and a review of the literature. Clin Rev Allergy Immunol, 2017, 52(3): 446-459.
18. Santo H, Nishiyama O, Sano H, et al. Mediastinal fibrosis and positive antineutrophil cytoplasmic antibodies: coincidence or common etiology?. Intern Med, 2014, 53(3): 275-277.
19. Gorospe L, Ayala-Carbonero AM, Fernández-Méndez Má, et al. Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. Clin Imaging, 2015, 39(6): 993-999.
20. Valentin LI, Kuban JD, Ramanathan R, et al. Endovascular treatment of bilateral pulmonary artery stenoses and superior vena cava syndrome in a patient with advanced mediastinal fibrosis. Tex Heart Inst J, 2016, 43(3): 249-251.
21. Murphy JC, Johnston N, Spence MS. A pressing matter--mediastinal fibrosis with near obliteration of the pulmonary arteries. Catheter Cardiovasc Interv, 2013, 81(6): 1079-1083.
22. Doucet KM, Labinaz M, Chandy G, et al. Pulmonary hypertension due to fibrosing mediastinitis treated successfully with stenting of pulmonary vein stenoses. Can J Cardiol, 2015, 31(4): 548. e5-7.
23. 林瑋, 陳華, 張文, 等. 以 IgG4 相關性硬化性縱隔炎為突出表現的淋巴瘤一例. 中華風濕病學雜志, 2012, 16(12): 855-857.
24. Taki M, Inada S, Arivasu R, et al. Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. Intern Med, 2013, 52: 2645-2651.

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