DiFFÜZ ALVEOLAR HEMORAJi SENDROMLARI

Abstract

Diffüz alveoler hemoraji (DAH) sıklıkla akut solunum yetmezliğine yol açan klinikopatolojik bir sendromdur. DAH, genellikle bir grup immün kökenli heterojen hastalığa bağlı olarak gelişir ve alveoler septal hasardan masif hemorajiye kadar uzanan bir spektrumda seyreder. DAH tanısı klasik triadın varlığını içerir: Anemi, hemoptizi ve akciğer grafide yeni ortaya çıkan bilateral alveoler infiltratlar. Semptomlar, hemoptizi hariç siliktir. Ancak hemoptizi, olguların üçte birinde izlenmez. Radyolojik görüntüleme ve sistem tutulumları nonspesifiktir. Bronkoa-Iveoler lavaj (BAL) genellikle tanıyı doğrulamak için gereklidir: BAL örneğinde çok sayıda eritrositler ve sideofajların varlığının gösterilmesi ile tanı kesinleşir. Tedaviye başlamak için DAH’ye yol açan altta yatan patolojiye tanı koyulmalıdır. Tedavide hem alveolde meydana gelen otoimmün hasar hem de altta yatan durum hedeflenir. DAH tedavisinde kortikosteroi-dler ve immünsüpresif ajanlar uygulanır. Bu derlemede DAH’li olgularda DAH’ye yaklaşım, tanı, altta yatan sistemik hastalıklar ve tedavileri tartışıldı. Anahtar Kelimeler: Hemoraji, bronkoalveoler lavaj, tanı, hemoptizi

Keywords

• Hemoraji
• bronkoalveoler lavaj
• tanı
• hemoptizi

SYNDROMES OF DIFFUSE ALVEOLAR HEMORRHAGE

Abstract

Diffuse alveolar hemorrhage (DAH) is often a clinicopathologic syndrome causing acute respiratory failure. DAH, caused by generally a group of heterogenous disorders that origin from immüne pathologies, is characterized by the presence of widespread massive hemorrhage from damaged alveolar septa. The diagnosis of DAH involves the presence of classical triad: Anemia, hemoptysis and new bilateral alveoler infiltrates on chest radiograph. The symptoms are silent, except hemoptysis. But, hemoptysis is absent in up to one-third of the patients. Radiographic imaging and the involvement of system are nonspesific. Bronchoalveolar lavage (BAL) is generally required to confirm the diagnosis; blood in the lavage specimens (with numerous erythrocytes and siderophages) establishes the diagnosis. The underlying cause that are responsible for DAH must be diagnosed in order to initiate treatment. Both the autoimmune destruction of the alveol and the underlying conditions are targeted in the treatment. Corticosteroids and immunosuppressive agents are administered in DAH treatment. In this review, the approach to DAH, diagnosis, the underlying systemic diseases and treatment in patients with DAH are discussed.

Keywords

• Hemorrhage
• bronchoalveolar lavage
• diagnosis
• hemoptysis

References

1. 1. Lara AR, Schwarz Ml. Diffuse Alveolar Hemorrhage. Chest 2010; 137:1164-117.
2. 2. 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 :1112-1125.
3. 3. Afessa B, Tefferi A, Litzow MR, Krowka MJ, Wylam ME, Peters SG. Diffuse alveolar hemorrhage in hematopoietic stem cell transplant recipients. Am J Respir Crit Care Med. 2002; 166 : 641-645.
4. 4. Vlahakis NE, Rickman OB, Morgenthaler T. Sirolimus-associated diffuse alveolar hemorrhage. Mayo Clin Proc 2004;79:541.
5. 5. 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.
6. 6. Anantham D, Chan KP, Chuah KL, et al. Pulmonary capillaritis in IgA nephropathy. South Med J 2007; 100:605.
7. 7. 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.
8. 8. Bosch X, Lopez-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. 9. Colby TV, Fukuoka J, Ewaskow SP, et al. Pathologic approach to pulmonary hemorrhage. Ann Diagn Pathol 2001;5:309.
10. 10. Yeager H Jr, Powell D, Weinberg RM, et al. Idiopathic pulmonary hemosiderosis: ultrastructural studies and responses to azathioprine. Arch Intern Med 1976;136:1145.
11. 11. Schwarz Ml. The diffuse alveolar hemorrhage syndromes, www.uptodate. com©2013UpToDate ( Düzenlenme tarihi: Fh Oct 18 00:00:00 GMT 2013).
12. 12. Jennings CA , King TE Jr , Tuder R , Cherniack RM , Schwarz Ml. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis . Am J Respir Crit Care Med. 1997;155:1101-1109.
13. 13. loachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004; 24: 162-170.
14. 14. Green RJ, Ruoss SJ, Kraft SA, Duncan SR, Berry GJ, Raffi n TA. Pulmonary capillaritis and alveolar hemorrhage. Update on diagnosis and management. Chest. 1996;110:1305- 1316.
15. 15. Mahr A, Guillevin L, Poissonnet M, Ayme S: Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum 2004,51:92-99.
16. 16. Çalgüneri M. Romatizmal Hastalıklarda Pulmoner Sistem Bulguları. Türkiye Klinikleri J Immunol Rheumatol 2002;2:43-51.
17. 17. Franssen CF, Stegeman CA, Kallenberg CG, et al. Antiproteinase3- and antimyeloperoxidase-associated vasculitis. Kidney Int. 2000; 57: 2195-2206.
18. 18. David Semple, James Keogh, Luigi Forni and Richard Venn Critical Care 2005;9:92-97.
19. 19. Hagen EC, Daha MR, Hermans J, An-drassy K, Csernok E, Gaskin G, Le-savre P, Ludemann J, Rasmussen N, Sinico RA, et al.: Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR project for ANCA assay standardization. Kidney Int 1998,53:743-753.
20. 20. Oymak. FS, Tokgöz. B, Akgün. H, Gülmez. I, Erdoğan. N, Demir. R, Balkanlı. S, Özesmi. M. Alveoler He-morajik Sendromlar-Klinik, Patolojik ve Radyolojik özellikler: On Bir Olgu Analizi. Toraks Dergisi 2002; 3 : 052-058.
21. 21. Guillevin L, Lhote F. Polyarteritis nodosa and microscopic polyangiitis. Clin Exp Immunol. 1995; 101:22-23.
22. 22. Lazor. R. Alveolar haemorrhage syndromes. Eur Respir Mon 2011; 54:15-31.
23. 23. Conlon PJ Jr, Walshe JJ, Daly C, et al. Antiglomerular basement membrane disease: the long-term pulmonary outcome. Am J Kidney Dis 1994; 23: 794-796.
24. 24. Homma S, Matsushita H, Nakata K. Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides. Respirology 2004; 9: 190-196.
25. 25. Foulon G, Delaval P, Valeyre D, et al. ANCA-associated lung fibrosis: analysis of 17 patients. Respir Med 2008; 102: 1392-1398.
26. 26. Hervier B, Pagnoux C, Agard C, et al. Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature. Ann Rheum Dis 2009; 68: 404-407.
27. 27. Nozu T, Kondo M, Suzuki K, et al. Acorn-parison of the clinical features of ANCA-positive and ANCA-negative idiopathic pulmonary fibrosis patients. Respiration 2009; 77:407-415.
28. 28. Tzelepis GE, Kokosi M, Tzioufas A, et al. Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis. Eur Respir J 2010; 36: 116-125.
29. 29. Hicks K, Peng D, Gajewski JL. Treatment of diffuse alveolar hemorrhage after allogeneic bone marrow transplant with recombinant factor Vila. Bone Marrow Transplant 2002; 30: 975-978.
30. 30. Pastores SM, Papadopoulos E, Voigt L, et al. Diffuse alveolar hemorrhage after allogeneic hematopoietic stem-cell transplantation: treatment with recombinant factor Vila. Chest 2003; 124: 2400-2403.
31. 31. Tatopoulos A, Herbain D, Kazmierczak C, et al. Parenteral use of recombinant activated factor VII during diffuse alveolar hemorrhage secondary to leptospirosis. Intensive Care Med 2010;36:555-556.
32. 32. de Prost N, Parrot A, Picard C, et al. Diffuse alveolar haemorrhage: factors associated with in-hospital and longterm mortality. Eur Respir J 2010; 35: 1303-1311.
33. 33. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009; 68:310-317.
34. 34. Lazor R, Bigay-Game' L, Cottin V, et al. Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. Medicine 2007; 86:181-193.
35. 35. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363:221-232.
36. 36. Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007; 18: 2180-2188.