Diffuse Alveolar Hemorrhage (DAH): Current Approach and Treatment
Keywords:
diffuse alveolar heworrhage, treatmentAbstract
Diffuse alveolar hemorrhage (DAH) is a rare life-threatening condition. The presentations are hemoptysis, anemia, diffuse lung infiltration, and acute hypoxemic respiratory failure. However, one-third of patients don’t have hemoptysis and need sequential bronchoalveolar lavage to demonstrate bleeding in 3 bottles of bronchoalveolar lavage fluids. The most common cause of DAH is capillaritis from ANCA-associated vasculitis (e.g., granulomatosis with polyangiitis and microscopic polyangiitis) and connective tissue disease (systemic lupus erythematosus, SLE). The treatment for ANCA-associated vasculitis is pulse methylprednisolone plus cyclophosphamide and maintenance with immunosuppressive drugs. The study showed rituximab plus pulse methylprednisolone was not inferior to cyclophosphamide plus pulse methylprednisolone for induction of remission in severe ANCA-associated vasculitis. For connective tissue disease (e.g., SLE) with DAH responded well with high dose corticosteroid, however precaution superinfection with bacteria and fungus and suggestion empirical antibiotic at initial. Anti-glomerular basement membrane disease is a cause in bland pulmonary hemorrhage group and the treatment is plasmapheresis to remove anti-GBM antibodies plus pulse methylprednisolone. In refractory DAH cases from SLE and Anti-glomerular basement membrane disease, rituximab and recombinant-activated human factor VII may be use to stop bleeding. If DAH from diffuse alveolar damage (DAD) usually from sepsis, treatment infection together with support ventilation and oxygenation.
References
Collard HR, King TE Jr, Schwarz MI. Alveolar hemorrhage and rare infiltrative diseases. In: Broaddus VC, Masson RJ, Ernst JD, King TE Jr, Lazarus SC, Murray JF, eds. Murray & Nadel’s textbook of respiratory medicine. Philadelphia: Elsevier Saunders, 2016: 1207-20.
de Prost N, Parrot A, Picard C, Ancel PY, Mayaud C, Fartoukh M, et al. Diffuse alveolar haemorrhage: factors associated with in-hospital and long-term mortality. Eur Respir J 2010; 35: 1303–11.
Abigail R Lara and Marvin I Schwarz. Diffuse alveolar hemorrhage. Chest 2010; 137:1164–71.
Cordier JF and Cottin V. Alveolar hemorrhage in vasculitis: primary and secondary. Semin Respir Crit Care Med. 2011; 32: 310-21.
Stanzel F. Bronchoalveolar lavage. Principles and Practice of Interventional Pulmonology. 2012; 165-76.
Park MS. Diffuse alveolar hemorrhage. Tuberc Respir Dis (Seoul) 2013; 74: 151-62.
Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener's granulomatosis: review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991; 15: 315–33.
Mark EJ, Matsubara O, Tan-Liu NS, Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener’s (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol 1988; 19: 1065–71.
Rarok AA, Stegeman CA, Limburg PC, Kallenberg CG. Neutrophil membrane expression of proteinase 3 (PR3) is related to relapse in PR3-ANCA-associated vasculitis. J Am Soc Nephrol 2002; 13: 2232–8.
Tervaert JW, van der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WK, et al. Association between active Wegener’s granulomatosis and anticytoplasmic antibodies. Arch Intern Med 1989; 149: 2461-5.
Kitching AR, Anders HJ, Basu N. Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020; 6: 71.
Casian A and Jayne D. Management of alveolar hemorrhage in lung vasculitides. Semin Respir Crit Care Med 2011; 32: 335–45.
Falk RJ, Nachman PH, Hogan SL, Jennette JC. ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective. Semin Nephrol 2000; 20: 233–43.
Tang s, Li X, Zhao KY, Zhou Q, Tang XK. Clinical characteristics and prognostic analysis of microscopic polyangiitis with diffuse alveolar hemorrhage. J Rheumatol 2021; 48: 410–6.
Donaghy M and Rees AJ. Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane. Lancet 1983; 2: 1390-3.
Jones JG, Minty BD, Lawler P, Hulands G, Crawley JC, Veall N. Increased alveolar epithelial permeability in cigarette smokers. Lancet 1980; 1: 66–8.
Perl SI, Pussell BA, Charlesworth JA, Macdonald GJ, Wolnizer M. Goodpasture’s (anti-GBM) disease and HLA-DRw2. N Engl J Med 1981; 305: 463–4.
Martinez-DV Martinez MU, Oostdam DAH, Abud-Mendoza C. Diffuse alveolar hemorrhage in autoimmune diseases. Curr Rheumatol Rep 2017; 19: 27.
Kwok SK, Moon SJ, Ju JH, Park KS, Kim WU, Cho CS, et al. Diffuse alveolar hemorrhage in systemic lupus erythematosus: risk factors and clinical outcome: results from affiliated hospitals of Catholoc University of Korea. Lupus 2011; 20: 102-7.
Rajkumar T and Vilayur E. Acute alveolar haemorrhage in hepatitis C-realated cryoglobulinaemic vasculitis. BMJ Case Rep 2018 Jan 4. (doi: 10.1136/bcr-2017-222717.)
Grosso V, Boveri E, Bogliolo L, Montecucco C, Caporali R. Diffuse alveolar hemorrhage as a manifestation of Behçet disease. Reumatismo 2013; 65: 138-41.
Rabiller A, Jaïs X, Hamid A, Resten A, Parent F, Haque R, et al. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J 2006; 27: 108-13.
Saha BK. Idiopathic pulmonary hemosiderosis: a state of the art review. Respir Med 2021; 176: 106234.
Reisman S, Chung M, Bernheim A. A Review of clinical and imaging features of diffuse pulmonary hemorrhage. AJR 2021; 216: 1500-9.
Thangaraj Y, Ather I, Chataut H, Ayach T, Holtzman A, Wakefield D, Ejaz AA. Diffuse alveolar hemorrhage as a presentation of acute poststreptococcal glomerulonephritis. Am J Med 2014; 127: e15–e17.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al: Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116: 488–98.
Schnabel A, Holl-Ulrich K, Dalhoff K, Reuter M, Gross WL. Efficacy of transbronchial biopsy in pulmonary vaculitides. Eur Respir J 1997; 10: 2738–43.
Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical Pathology of the Lung in Wegenerʼs Granulomatosis. Am J Sur Pathol 1991; 15: 315–33.
Mark EJ, Matsubara O, Tan-Liu NS, Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener’s (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol 1988; 19: 1065– 71.
Park JA. Treatment of diffuse alveolar hemorrhage: controlling inflammation and obtaining rapid and effective hemostasis. Int J Mol Sci 2021; 22, 793.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American college of rheumatology/vasculitis foundation guideline for the management of antineutrophil cytoplasmic antibody–associated vasculitis. Arthritis Care & Research 2021; 73: 1088–105.
Jones RB, Hiemstra TF, Ballarin J, Blockmans DE, Brogan P, Bruchfeld A, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial. Ann Rheum Dis 2019; 78: 399-405.
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363: 221-32.
Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 2013; 369: 417-27.
Walsh M, Merkel PA, Peh CA, Szpirt WM, Puéchal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med 2020; 382: 622-31.
Al-Adhoubi NK and Bystrom J. Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies. Lupus 2020; 29: 355-63.
Ward MM, Pyun E, Studenski S. Causes of death in systemic lupus erythematosus: long-term followup of an inception cohort. Arthritis Rheum 1995; 38: 1492-9.
Wang CR, Liu MF, Weng CT, Lin WC, Li WT, Tsai HW. Systemic lupus erythematosus-associated diffuse alveolar haemorrhage: a single-centre experience in Han Chinese patients. Scand J Rheumatol 2018; 47: 392–399.
Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med 2001; 134: 1033-42,
Marques C, Carvelli J, Biard L, Faguer S, Provôt F, Matignon M, et al. Prognostic factors in anti-glomerular basement membrane disease: a multicenter study of 119 patients. Frontiers in Immunology 2019; 10: 1665.
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