Diffuse alveolar hemorrhage in inflammatory rheumatic diseases
Authors:
P.. Bradna
Authors‘ workplace:
II. interní gastroenterologická klinika LF UK a FN, Hradec Králové
Published in:
Čes. Revmatol., 31, 2023, No. 4, p. 190-196.
Category:
Review Article
Overview
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication, especially of systemic vasculitis, but also of other connective tissue diseases. The trick is the fact that for some patients, a DAH attack is the first manifestation of the disease. A typical triad of symptoms includes rapidly developing dyspnea, anemization, and the presence of diffuse alveolar hemorrhage on bronchoalveolar lavage examination. Hemoptysis is not a reliable indicator, it occurs only in a third to a half of patients. HRCT will show extensive clouding of the lung fields, usually bilateral. The acute prognosis depends on the timeliness of the diagnosis or initiation of intensive immunomodulating therapy. Urgent determination of the presence of ANCA antibodies (immediately) can be of significant help. Acute bronchoscopy with BAL is an essential part of the diagnosis.
Treatment of an acute attack of DAH consists of i.v. administration of pharmacological doses of methylprednisolone and early initiation of i.v. cyclophosphamide or rituximab therapy according to usual schemes. Concomitant rapidly progressing anuric renal failure occurs in approximately half of patients with a severe course and requires the use of elimination methods. Overcoming respiratory failure may require the temporary use of extracorporeal oxygenation (ECMO). If the acute phase of DAH can be managed, the restitution of lung and renal functions is significant, and chronic dialysis is required less often. In the further course, the prognosis of the patient probably does not differ significantly from the prognosis of the underlying disease.
Due to the significant number of patients in whom the attack of DAH is the first manifestation of a systemic disease, the possibility of DAH should be considered in any case of rapidly developing severe respiratory insufficiency without an obvious previous cause.
Keywords:
Systemic lupus erythematosus – glucocorticoids – granulomatosis with polyangiitis – diffuse alveolar hemorrhage – cyclophosphamide, rituximab – bronchoalveolar la-vage
Sources
1. Kambhatia S, Vipparthy S,Manadan AM. Rheumatic diseases associated with alveolar hemorrhage: analysis of the national in patient sample Clin Rheumatol 2023; 42: 1177–1183.
2. Thompson G, Klecka M, Roden AC, et al. Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis. Respirology 2016; 21: 734–738.
3. Mirouse A, Parrot A, Audigier V, et al. Severe diffuse alveolar hemorrhage related to autoimmune disease: a multicenter study. Crit Care 2020; 24: 231.
4. Park MS. Diffuse alveolar hemorrhage. Tuberc Respir Dis (Seoul) 2013; 74: 151–162.
5. Martínez-Martínez MU, Herrera-van Oostdam DA, Abud-Mendoza C. Diffuse alveolar hemorrhage in autoimmune diseases. Curr Rheumatol Rep 2017; 19: 27–33.
6. Xu T, Zhang G, Lin H, Xie Y, Feng Y, Zhang X, et al. Clinical characteristics and risk factors of diffuse alveolar Hemorrhage in systemic lupus erythematosus: a systematic review and meta-analysis based on observational studies. Clin Rev Allergy Immunol 2020; 59: 295–303.
7. Kao KC, Hu HC, Chang CH, et al. Diffuse alveolar damage associated mortality in selected acute respiratory distress syndrome patients with open lung biopsy. Crit Care 2015; 19: 228–338.
8. Stainer A, Rice A, Devaraj A, et al. Diffuse alveolar haemorrhage associated with subsequent development of ANCA positivity and emphysema in three young adults. BMC Pulm Med 2019; 19: 185.
9. Saha BK, Chong WH, Milman NT. Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach. Clin Rheumatol 2022; 41: 325–336.
10. Alexandre AT, Vale A, Gomes T. Diffuse alveolar hemorrhage: how relevant is etiology? Sarcoidosis Vasc Diffuse Lung Dis 2019; 36; 47–52.
11. Bradna P, Maňák J, Soukup T, et al. Difuzní alveolární hemoragie – akutní, život ohrožující stav v revmatologii. Vnitř. Lék. 2018; 54: 169–172.
12. Nasser M, Cottin V. Alveolar hemorrhage in vasculitis (primary and secondary). Semin Respir Crit Care Med 2018; 39: 169–193.
13. Frausová D, Hrušková Z, Lánská V, et al. Long-term outcome of patients with ANCA-associated vasculitis treated with plasma exchange: a retrospective, single-centre study. Arthritis Res Ther 2016; 18: 168.
14. Sattar Y, Susheela AT, Ullah W, et al. Use of plasmapheresis and immunosuppressants to treat diffuse alveolar hemorrhage in a patient with granulomatosis with polyangiitis. Medicina (Kaunas) 2019; 55: 378.
15. Hellmich B,Sanchez-Alamo B,Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis 2024; 83: 30–47.
16. Walsh M, Collister D, Zeng L, et al. The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis. BMJ 2022; 376: e064604.
17. Jayne DRW, 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.
18. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363: 221–232.
19. Cartin-Ceba R, Diaz-Caballero L, Al-Qadi MO, et al: Diffuse alveolar hemorrhage secondary to antineutrophil cytoplasmic antibody–associated vasculitis. Arthritis Rheum 2016; 68:
1467–1476.
20. Delvino P, Monti S, Balduzzi et al. The role of extra-corporeal membrane oxygenation (ECMO) in the treatment of diffuse alveolar haemorrhage secondary to ANCA associated vasculitis: report of two cases and review of the literature. Rheumatol Int 2019; 39: 367–375.
21. Vanoli J, Riva M, Vergnano B, et al. Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse. A case report. Medicine (Baltimore) 2017; 96: e6024.
22. Park JA. Treatment of diffuse alveolar hemorrhage: controlling inflammation and obtaining rapid and effective hemostasis. Int J Mol Sci 2021; 22: 793.
23. Baker MS, Diab KJ, Carlos WG, et al. Intrapulmonary recombinant factor VII as an effective treatment for diffuse alveolar hemorrhage: a case series. J Bronchology Interv Pulmonol 2016; 23(3): 255–258.
24. von Ranke FM, Zanetti G, Hochhegger B, Marchiori E. Infectious diseases causing diffuse alveolar hemorrhage in immunocompetent patients: a state-of-the-art review. Lung 2013; 191: 9–18.
25. Kostelníková P, Skácelová M, Špíšek M, Šimíček M, Horák P. Diffuse alveolar hemorrhage as a life threatening manifestation of newly diagnosed granulomatosis with polyangiitis following COVID-19 infection - a case report. Vnitř. Lék. 2022; 68: 290–294.
26. Ito Y, Nakahara H, Nakajima A. Development of pulmonary alveolar proteinosis in a patient with adult-onset Still disease treated with tocilizumab. J Rheumatol 2023; 50: 288–289.
27. Tashiro H, Takahashi K, Sadamatsu H, et al. Chronic and asymptomatic diffuse alveolar haemorrhage with microscopic polyangiitis: A case report and review of the literature. Case Rep Rheumatol 2016; 1658126.
28. Samson M, Puéchal X, Mouthon L, et al. Microscopic polyangiitis and non-HBV polyarteritis nodosa with poor-prognosis factors: 10-year results of the prospective CHUSPAN trial. Clin Exp Rheumatol 2017; 35(Suppl 103): 176–184.
29. Marques C, Carvelli J, Biard L, et al. Prognostic factors in anti-glomerular basement membrane disease: a multicenter study of 119 Patients. Front Immunol 2019; 10: 1665–1675.
30. Quadrelli S, Dubinsky D, Solis M, et al. Immune diffuse alveolar hemorrhage: Clinical presentation and outcome. Respir Med 2017; 129: 59–62.
31. Kim D, Choi J, Cho SK, et al. Clinical characteristics and outcomes of doffuse alveolar hemorrhage in patients with systemic lupus erythematosus. Semin Arthritis Rheum 2017; 46: 782–787.
32. Jiang M, Chen R, Zhao L, et al. Risk factors for mortality of diffuse alveolar hemorrhage in systemic lupus erythematosus: a systematic review and meta-analysis. Arthritis Res Ther 2021; 23: 57–67.
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