Generic placeholder image

Reviews on Recent Clinical Trials

Editor-in-Chief

ISSN (Print): 1574-8871
ISSN (Online): 1876-1038

Review Article

Management of Gastrointestinal Bleeding in Rendu-Osler Disease

Author(s): Annalisa Tortora*, Clelia Marmo, Antonio Gasbarrini, Guido Costamagna and Maria Elena Riccioni

Volume 15, Issue 4, 2020

Page: [321 - 327] Pages: 7

DOI: 10.2174/1574887115666200603160033

Price: $65

Abstract

Background: Hereditary hemorrhagic telangiectasia (HHT, or Rendu-Osler-Weber disease) is a rare inherited syndrome, characterized by artero-venous malformations (AVMs or telangiectasia) with autosomal dominant transmission. AVMs can occur in any organ of the body but most commonly it occurs in the nose, pulmonary, hepatic and cerebral circulations. In patients with HHT, we report teleangectasia of mucosa of the gastrointestinal tract.

Methods: Research and online content related to HHT online activity is reviewed, and DOC writing excerpts are used to illustrate key themes.

Results: Patients with HHT have a high rate of complications related to bleeding; of them gastrointestinal bleeding accounts for 10.8%. Several therapies, both medical and endoscopic, were utilized to reduce the need for transfusions and hospitalization.

Conclusion: A combination of medical and endoscopic therapy is probably the best option.

Keywords: Rendu-Osler disease, bleeding, anemia, endoscopy, argon-plasma-coagulation, endothelial-growth factor.

Graphical Abstract

[1]
Tortora A, Riccioni ME, Gaetani E, Ojetti V, Holleran G, Gasbarrini A. Rendu-Osler-Weber disease: A gastroenterologist’s perspective Orphanet J Rare Dis 2019; 14: 130.
[http://dx.doi.org/10.1186/s13023-019-1107-4]
[2]
Guttmacher AE, Marchuk DA, White RI Jr. Hereditary hemorrhagic telangiectasia. N Engl J Med 1995; 333(14): 918-24.
[http://dx.doi.org/10.1056/NEJM199510053331407] [PMID: 7666879]
[3]
Garg N, Khunger M, Gupta A, Kumar N. Optimal management of hereditary hemorrhagic telangiectasia. J Blood Med 2014; 5: 191-206.
[PMID: 25342923]
[4]
Brinjikji W, Wood CP, Lanzino G, et al. High rates of bleeding complications among hospitalized patients with hereditary hemorrhagic telangiectasia in the United States. Ann Am Thorac Soc 2016; 13(9): 1505-11.
[http://dx.doi.org/10.1513/AnnalsATS.201603-200OC] [PMID: 27387823]
[5]
Plauchu H, de Chadarévian JP, Bideau A, Robert JM. Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 1989; 32(3): 291-7.
[http://dx.doi.org/10.1002/ajmg.1320320302] [PMID: 2729347]
[6]
Jackson CS, Gerson LB. Management of gastrointestinal angiodysplastic lesions (GIADs): A systematic review and meta-analysis. Am J Gastroenterol 2014; 109(4): 474-83.
[http://dx.doi.org/10.1038/ajg.2014.19] [PMID: 24642577]
[7]
Karlsson T, Cherif H. Effect of intravenous iron supplementation on iron stores in non-anemic iron-deficient patients with hereditary hemorrhagic telangiectasia Hematol Rep 2016; 8(1): 6348.
[http://dx.doi.org/10.4081/hr.2016.6348]
[8]
Proctor DD, Henderson KJ, Dziura JD, White RI Jr. Hormonal therapy for the treatment of gastrointestinal bleeding in hereditary hemorrhagic telangiectasia. J Clin Gastroenterol 2008; 42(6): 756-7.
[http://dx.doi.org/10.1097/MCG.0b013e318032391f] [PMID: 18496387]
[9]
Fodstad P, Dheyauldeen S, Rinde M, Bachmann-Harildstad G. Anti-VEGF with 3-week intervals is effective on anemia in a patient with severe hereditary hemorrhagic telangiectasia. Ann Hematol 2011; 90(5): 611-2.
[http://dx.doi.org/10.1007/s00277-010-1063-5] [PMID: 20824275]
[10]
van Cutsem E, Rutgeerts P, Vantrappen G. Treatment of bleeding gastrointestinal vascular malformations with oestrogen-progesterone. Lancet 1990; 335(8695): 953-5.
[http://dx.doi.org/10.1016/0140-6736(90)91010-8] [PMID: 1970032]
[11]
Rossini FP, Arrigoni A, Pennazio M. Octreotide in the treatment of bleeding due to angiodysplasia of the small intestine. Am J Gastroenterol 1993; 88(9): 1424-7.
[PMID: 8362842]
[12]
Buonamico P, Sabbá C, Garcia-Tsao G, et al. Octreotide blunts postprandial splanchnic hyperemia in cirrhotic patients: a double-blind randomized echo-Doppler study. Hepatology 1995; 21(1): 134-9.
[PMID: 7806146]
[13]
Brown C, Subramanian V, Wilcox CM, Peter S. Somatostatin analogues in the treatment of recurrent bleeding from gastrointestinal vascular malformations: An overview and systematic review of prospective observational studies. Dig Dis Sci 2010; 55(8): 2129-34.
[http://dx.doi.org/10.1007/s10620-010-1193-6] [PMID: 20393879]
[14]
Bon C, Aparicio T, Vincent M, et al. Long-acting somatostatin analogues decrease blood transfusion requirements in patients with refractory gastrointestinal bleeding associated with angiodysplasia. Aliment Pharmacol Ther 2012; 36(6): 587-93.
[http://dx.doi.org/10.1111/apt.12000] [PMID: 22831465]
[15]
Klímová K, Padilla-Suárez C, Giménez-Manzorro Á, Pajares-Díaz JA, Clemente-Ricote G, Hernando-Alonso A. Octreotide long-active release in the treatment of gastrointestinal bleeding due to vascular malformations: Cost-effectiveness study. Rev Esp Enferm Dig 2015; 107(2): 79-88.
[PMID: 25659389]
[16]
Grooteman KV, van Geenen EJM, Drenth JPH. High variation in treatment strategies for gastrointestinal angiodysplasias. Eur J Gastroenterol Hepatol 2016; 28(9): 1082-6.
[http://dx.doi.org/10.1097/MEG.0000000000000673] [PMID: 27227688]
[17]
Junquera F, Saperas E, Videla S, et al. Long-term efficacy of octreotide in the prevention of recurrent bleeding from gastrointestinal angiodysplasia. Am J Gastroenterol 2007; 102(2): 254-60.
[http://dx.doi.org/10.1111/j.1572-0241.2007.01053.x] [PMID: 17311647]
[18]
Franchini M, Mannucci PM. Gastrointestinal angiodysplasia and bleeding in von Willebrand disease. Thromb Haemost 2014; 112(3): 427-31.
[http://dx.doi.org/10.1160/TH13-11-0952] [PMID: 24898873]
[19]
Buscarini E, Botella LM, Geisthoff U, Kjeldsen AD, Mager HJ, Pagella F, et al. Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia Orphanet J Rare Dis 2019; 14(1): 28.
[http://dx.doi.org/10.1186/s13023-018-0982-4]
[20]
Bauditz J. Effective treatment of gastrointestinal bleeding with thalidomide-Chances and limitations. World J Gastroenterol 2016; 22(11): 3158-64.
[http://dx.doi.org/10.3748/wjg.v22.i11.3158] [PMID: 27003992]
[21]
Ge ZZ, Chen HM, Gao YJ, et al. Efficacy of thalidomide for refractory gastrointestinal bleeding from vascular malformation. Gastroenterology 2011; 141(5): 1629-37.e1, 4.
[http://dx.doi.org/10.1053/j.gastro.2011.07.018] [PMID: 21784047]
[22]
Salgueiro P, Marcos-Pinto R, Liberal R, Lago P, Magalhães R, Magalhães M, et al. Octreotide Long-Acting Release is effective in preventing gastrointestinal bleeding due to angiodysplasias. GE Port J Gastroenterol 2014; 21(5): 176-83.
[http://dx.doi.org/10.1016/j.jpg.2014.05.001]
[23]
Nardone G, Compare D, Scarpignato C, Rocco A. Long acting release-octreotide as “rescue” therapy to control angiodysplasia bleeding: A retrospective study of 98 cases. Dig Liver Dis 2014; 46(8): 688-94.
[http://dx.doi.org/10.1016/j.dld.2014.04.011] [PMID: 24893688]
[24]
Holleran G, Hall B, Breslin N, McNamara D. Long-acting somatostatin analogues provide significant beneficial effect in patients with refractory small bowel angiodysplasia: Results from a proof of concept open label mono-centre trial. United European Gastroenterol J 2016; 4(1): 70-6.
[http://dx.doi.org/10.1177/2050640614559121] [PMID: 26966525]
[25]
Ou G, Galorport C, Enns R. Bevacizumab and gastrointestinal bleeding in hereditary hemorrhagic telangiectasia. World J Gastrointest Surg 2016; 8(12): 792-5.
[http://dx.doi.org/10.4240/wjgs.v8.i12.792] [PMID: 28070235]
[26]
Bose P, Holter JL, Selby GB. Bevacizumab in hereditary hemorrhagic telangiectasia. N Engl J Med 2009; 360(20): 2143-4.
[http://dx.doi.org/10.1056/NEJMc0901421] [PMID: 19439755]
[27]
Brinkerhoff BT, Poetker DM, Choong NW. Long-term therapy with bevacizumab in hereditary hemorrhagic telangiectasia. N Engl J Med 2011; 364(7): 688-9.
[http://dx.doi.org/10.1056/NEJMc1012774] [PMID: 21323562]
[28]
Santos S, Bernardes C, Borges V, Ramos G. Gastric antral vascular ectasia (GAVE) and hereditary hemorrhagic telangiectasia (HHT): Two different conditions, one treatment. Ann Hematol 2020; 99(2): 367-9.
[http://dx.doi.org/10.1007/s00277-019-03845-1] [PMID: 31773212]
[29]
Guilhem A, Fargeton A-E, Simon A-C, et al. Intra-venous bevacizumab in hereditary hemorrhagic telangiectasia (HHT): A retrospective study of 46 patients In: de Jesus Perez VA, editor PLOS ONE. 2017; 12 11: p. e0188943.
[30]
Iyer VN, Apala DR, Pannu BS, et al. Intravenous bevacizumab for refractory hereditary hemorrhagic telangiectasia-related epistaxis and gastrointestinal bleeding. Mayo Clin Proc 2018; 93(2): 155-66.
[http://dx.doi.org/10.1016/j.mayocp.2017.11.013] [PMID: 29395350]
[31]
Foutch PG, Rex DK, Lieberman DA. Prevalence and natural history of colonic angiodysplasia among healthy asymptomatic people. Am J Gastroenterol 1995; 90(4): 564-7.
[PMID: 7717311]
[32]
Boley SJ, Sammartano R, Adams A, DiBiase A, Kleinhaus S, Sprayregen S. On the nature and etiology of vascular ectasias of the colon. Degenerative lesions of aging. Gastroenterology 1977; 72(4 Pt 1): 650-60.
[http://dx.doi.org/10.1016/S0016-5085(77)80149-2] [PMID: 300063]
[33]
Canzonieri C, Centenara L, Ornati F, et al. Endoscopic evaluation of gastrointestinal tract in patients with hereditary hemorrhagic telangiectasia and correlation with their genotypes. Genet Med 2014; 16(1): 3-10.
[http://dx.doi.org/10.1038/gim.2013.62] [PMID: 23722869]
[34]
Ingrosso M, Sabbà C, Pisani A, et al. Evidence of small-bowel involvement in hereditary hemorrhagic telangiectasia: A capsule-endoscopic study. Endoscopy 2004; 36(12): 1074-9.
[http://dx.doi.org/10.1055/s-2004-826045] [PMID: 15578297]
[35]
Pennazio M, Spada C, Eliakim R, et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline. Endoscopy 2015; 47(4): 352-76.
[http://dx.doi.org/10.1055/s-0034-1391855] [PMID: 25826168]
[36]
Chang Y-T, Wang H-P, Huang S-P, et al. Clinical application of argon plasma coagulation in endoscopic hemostasis for non-ulcer non-variceal gastrointestinal bleeding--a pilot study in Taiwan. Hepatogastroenterology 2002; 49(44): 441-3.
[PMID: 11995469]
[37]
Sato Y, Takayama T, Takahari D, et al. Successful treatment for gastro-intestinal bleeding of Osler-Weber-Rendu disease by argon plasma coagulation using double-balloon enteroscopy Endoscopy 2008; 40(S02): E228-9.
[http://dx.doi.org/10.1055/s-2007-966562]
[38]
Romagnuolo J, Brock AS, Ranney N. Is endoscopic therapy effective for angioectasia in obscure gastrointestinal bleeding?: A systematic review of the literature. J Clin Gastroenterol 2015; 49(10): 823-30.
[http://dx.doi.org/10.1097/MCG.0000000000000266] [PMID: 25518005]
[39]
Pérez-Cuadrado RE, Bebia CP, Esteban Delgado P, et al. Emergency double-balloon enteroscopy combined with real -time viewing of capsule endoscopy: A feasible combined approach in acute overt-obscure gastrointestinal bleeding? Dig Endosc 2015; 27(3): 338-44.
[http://dx.doi.org/10.1111/den.12384] [PMID: 25251991]
[40]
Sakai E, Ohata K, Nakajima A, Matsuhashi N. Diagnosis and therapeutic strategies for small bowel vascular lesions. World J Gastroenterol 2019; 25(22): 2720-33.
[http://dx.doi.org/10.3748/wjg.v25.i22.2720] [PMID: 31235995]
[41]
Jeon SR, Byeon J-S, Jang HJ, et al. Small Intestine Research Group of the Korean Association for the Study of Intestinal Disease (KASID). Clinical outcome after enteroscopy for small bowel angioectasia bleeding: A Korean Associateion for the Study of Intestinal Disease (KASID) multiceter study. J Gastroenterol Hepatol 2017; 32(2): 388-94.
[http://dx.doi.org/10.1111/jgh.13479] [PMID: 27356264]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy