UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

弥漫性肺泡出血综合征

Author
Marvin I Schwarz, MD
Section Editor
Talmadge E King, Jr, MD
Deputy Editor
Helen Hollingsworth, MD
Translators
赵燕妮, 主治医师

引言

血液流入肺泡腔是弥漫性肺泡出血(diffuse alveolar hemorrhage, DAH)综合征的特征，是由肺泡-毛细血管基底膜破裂引起的。这是由小动脉、小静脉或肺泡间隔(肺泡壁或间质)毛细血管损伤或炎症而引起。咯血是常见症状；然而它并非总是出现，即使是出血严重时[1-3]。

DAH综合征的概述见本专题。咯血的评估和治疗将单独讨论。 (参见“成人咯血的病因及评估 ”和“大咯血：初始处理”)

病因学和组织学

多种疾病与DAH综合征的发生相关(表 1)[1,2,4-20]。可见以下3种不同组织学类型之一：

肺毛细血管炎 — 这种病理学类型也被称为肺泡毛细血管炎，其特点是肺泡间隔(肺间质)出现中性粒细胞浸润(图片 1A-B)，进而导致这些结构的坏死、毛细血管结构完整性的破坏以及红细胞涌入肺泡腔和间质中[1,4-6,21-24]。

大量中性粒细胞发生破裂并最终固缩，该发现证实中性粒细胞副产物(毒性氧自由基和蛋白水解酶)在该类型肺损伤发病机制中的作用。破裂的中性粒细胞、核尘和纤维蛋白也进入肺泡腔，可见间质纤维素样坏死。

                        

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: 2017-06 . | This topic last updated: 2017-07-11.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clin Chest Med 2004; 25:583.
  2. Franks TJ, Koss MN. Pulmonary capillaritis. Curr Opin Pulm Med 2000; 6:430.
  3. Lara AR, Frankel SK, Schwarz MI. Diffuse alveolar hemorrhage. In: Interstitial Lung Disease, 5th ed, Schwarz MI, King TE Jr (Eds), People's Medical Publishing House, Shelton, CT, USA 2011. p.805.
  4. Mark EJ, Ramirez JF. Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis. Arch Pathol Lab Med 1985; 109:413.
  5. Leatherman JW. The lung in systemic vasculitis. Semin Respir Infect 1988; 3:274.
  6. Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol 1990; 14:1112.
  7. Lenclud C, De Vuyst P, Dupont E, et al. Wegener's granulomatosis presenting as acute respiratory failure with anti-neutrophil-cytoplasm antibodies. Chest 1989; 96:345.
  8. Bosch X, López-Soto A, Mirapeix E, et al. Antineutrophil cytoplasmic autoantibody-associated alveolar capillaritis in patients presenting with pulmonary hemorrhage. Arch Pathol Lab Med 1994; 118:517.
  9. Gaudin PB, Askin FB, Falk RJ, Jennette JC. The pathologic spectrum of pulmonary lesions in patients with anti-neutrophil cytoplasmic autoantibodies specific for anti-proteinase 3 and anti-myeloperoxidase. Am J Clin Pathol 1995; 104:7.
  10. Savage CO, Winearls CG, Evans DJ, et al. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med 1985; 56:467.
  11. Kelly PT, Haponik EF. Goodpasture syndrome: molecular and clinical advances. Medicine (Baltimore) 1994; 73:171.
  12. Tobler A, Schürch E, Altermatt HJ, Im Hof V. Anti-basement membrane antibody disease with severe pulmonary haemorrhage and normal renal function. Thorax 1991; 46:68.
  13. Myers JL, Katzenstein AA. Microangiitis in lupus-induced pulmonary hemorrhage. Am J Clin Pathol 1986; 85:552.
  14. Schwarz MI, Sutarik JM, Nick JA, et al. Pulmonary capillaritis and diffuse alveolar hemorrhage. A primary manifestation of polymyositis. Am J Respir Crit Care Med 1995; 151:2037.
  15. Torralbo A, Herrero JA, Portolés J, Barrientos A. Alveolar hemorrhage associated with antineutrophil cytoplasmic antibodies in rheumatoid arthritis. Chest 1994; 105:1590.
  16. Jennings CA, King TE Jr, Tuder R, et al. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med 1997; 155:1101.
  17. Gertner E, Lie JT. Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome. J Rheumatol 1993; 20:1224.
  18. Robboy SJ, Minna JD, Colman RW, et al. Pulmonary hemorrhage syndrome as a manifestation of disseminated intravascular coagulation: analysis of ten cases. Chest 1973; 63:718.
  19. Haselton PS. Adult respiratory distress syndrome. In: Spencers Pathology of the Lung, Haselton PS (Ed), McGraw Hill, New York 1996. p.375.
  20. Schwarz MI, Zamora MR, Hodges TN, et al. Isolated pulmonary capillaritis and diffuse alveolar hemorrhage in rheumatoid arthritis and mixed connective tissue disease. Chest 1998; 113:1609.
  21. Colby TV, Fukuoka J, Ewaskow SP, et al. Pathologic approach to pulmonary hemorrhage. Ann Diagn Pathol 2001; 5:309.
  22. Schwarz MI, Brown KK. Small vessel vasculitis of the lung. Thorax 2000; 55:502.
  23. Lazor R, Bigay-Gamé L, Cottin V, et al. Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. Medicine (Baltimore) 2007; 86:181.
  24. Dhillon SS, Singh D, Doe N, et al. Diffuse alveolar hemorrhage and pulmonary capillaritis due to propylthiouracil. Chest 1999; 116:1485.
  25. Bansal, S, Khan, R, Cicenia, J. et al. Alveolar hemorrhage associated with administration of glycoprotein IIA/IIIB inhibitor eptifibatide. Crit Care Med 2006; 34:A165.
  26. Kim EA, Lee KS, Primack SL, et al. Viral pneumonias in adults: radiologic and pathologic findings. Radiographics 2002; 22 Spec No:S137.
  27. Alexandrescu DT, Dutcher JP, O'Boyle K, et al. Fatal intra-alveolar hemorrhage after rituximab in a patient with non-Hodgkin lymphoma. Leuk Lymphoma 2004; 45:2321.
  28. Anantham D, Chan KP, Chuah KL, et al. Pulmonary capillaritis in IgA nephropathy. South Med J 2007; 100:605.
  29. Luks AM, Lakshminarayanan S, Hirschmann JV. Leptospirosis presenting as diffuse alveolar hemorrhage: case report and literature review. Chest 2003; 123:639.
  30. Schwarz MI, Mortenson RL, Colby TV, et al. Pulmonary capillaritis. The association with progressive irreversible airflow limitation and hyperinflation. Am Rev Respir Dis 1993; 148:507.
  31. De Lassence A, Fleury-Feith J, Escudier E, et al. Alveolar hemorrhage. Diagnostic criteria and results in 194 immunocompromised hosts. Am J Respir Crit Care Med 1995; 151:157.
  32. Vlahakis NE, Rickman OB, Morgenthaler T. Sirolimus-associated diffuse alveolar hemorrhage. Mayo Clin Proc 2004; 79:541.
  33. Zeiss CR, Wolkonsky P, Chacon R, et al. Syndromes in workers exposed to trimellitic anhydride. A longitudinal clinical and immunologic study. Ann Intern Med 1983; 98:8.
  34. Lauque D, Cadranel J, Lazor R, et al. Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore) 2000; 79:222.
  35. Mandel J, Mark EJ, Hales CA. Pulmonary veno-occlusive disease. Am J Respir Crit Care Med 2000; 162:1964.
  36. Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest 2010; 137:1164.
  37. Yeager H Jr, Powell D, Weinberg RM, et al. Idiopathic pulmonary hemosiderosis: ultrastructural studies and responses to azathioprine. Arch Intern Med 1976; 136:1145.
  38. Savage CO, Pusey CD, Bowman C, et al. Antiglomerular basement membrane antibody mediated disease in the British Isles 1980-4. Br Med J (Clin Res Ed) 1986; 292:301.
  39. Madore F, Lazarus JM, Brady HR. Therapeutic plasma exchange in renal diseases. J Am Soc Nephrol 1996; 7:367.
  40. Glassock RJ. Intensive plasma exchange in crescentic glomerulonephritis: help or no help? Am J Kidney Dis 1992; 20:270.
  41. Lewis EJ, Hunsicker LG, Lan SP, et al. A controlled trial of plasmapheresis therapy in severe lupus nephritis. The Lupus Nephritis Collaborative Study Group. N Engl J Med 1992; 326:1373.
  42. Gómez-Puerta JA, Hernández-Rodríguez J, López-Soto A, Bosch X. Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory disease. Chest 2009; 136:1101.
  43. Wanko SO, Broadwater G, Folz RJ, Chao NJ. Diffuse alveolar hemorrhage: retrospective review of clinical outcome in allogeneic transplant recipients treated with aminocaproic acid. Biol Blood Marrow Transplant 2006; 12:949.