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(HHT): a Systematic Review of the Literature UCLA UCLA Previously Published Works Title Gastrointestinal Manifestations of Hereditary Hemorrhagic Telangiectasia (HHT): A Systematic Review of the Literature. Permalink https://escholarship.org/uc/item/6p83t8dt Journal Digestive diseases and sciences, 62(10) ISSN 0163-2116 Authors Jackson, Samuel B Villano, Nicholas P Benhammou, Jihane N et al. Publication Date 2017-10-01 DOI 10.1007/s10620-017-4719-3 License https://creativecommons.org/licenses/by/4.0/ 4.0 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Dig Dis Sci (2017) 62:2623–2630 DOI 10.1007/s10620-017-4719-3 MENTORED REVIEW Gastrointestinal Manifestations of Hereditary Hemorrhagic Telangiectasia (HHT): A Systematic Review of the Literature 1,2,4 1,2,4 1,2,4 Samuel B. Jackson • Nicholas P. Villano • Jihane N. Benhammou • 3 1,2,4 1,2,4 Michael Lewis • Joseph R. Pisegna • David Padua Received: 31 August 2016 / Accepted: 9 August 2017 / Published online: 23 August 2017 Ó Springer Science+Business Media, LLC (Outside the USA) 2017 Abstract Hereditary hemorrhagic telangiectasia (HHT), Abbreviations also called Osler–Weber–Rendu syndrome, is an autosomal HHT Hereditary hemorrhagic telangiectasia dominant genetic disease that affects the vasculature of AVM Arteriovenous malformation numerous organs. The prevalence of HHT is estimated to JPHT Juvenile polyposis/hereditary hemorrhagic be between 1.5 and 2 persons per 10,000. While there is telangiectasia still much to learn about this condition, there is an GI Gastrointestinal increasing understanding its underlying pathophysiology, VCE Video capsule endoscopy genetic basis, presentations, and management. Recognizing EGD Esophagogastroduodenoscopy that the clinical manifestations of HHT can involve a number of organ systems will provide clinicians with a higher index of suspicion for the disease. This early diag- Introduction nosis and genotyping can greatly reduce mortality for a patient with HHT through appropriate screening for com- Hereditary hemorrhagic telangiectasia (HHT), also called plications. This review will focus on the gastrointestinal Osler–Weber–Rendu syndrome, is an inherited autosomal manifestations of HHT and how these can dictate treatment dominant vascular disease with varying clinical manifes- and prognosis. tations. Patients typically present with epistaxis, gastroin- testinal bleeding, and iron deficiency anemia due to Keywords Hereditary hemorrhagic telangiectasia (HHT) Á mucocutaneous telangiectasias. However, patients with Osler–Weber–Rendu syndrome Á Arteriovenous HHT are also at risk of developing arteriovenous malfor- malformation Á Juvenile polyposis syndrome mations in the cerebral, pulmonary, and hepatic circula- tions, which can lead to end-organ damage. Initial screening for HHT often utilizes the Curac¸ao criteria, consisting of epistaxis, telangiectasia, visceral vascular & David Padua [email protected] malformations, and a first-degree relative with HHT [1]. Treatment is largely based on management of symptoms 1 Division of Digestive Diseases, David Geffen School of and complications and early screening for AVMs. In this Medicine at UCLA, Los Angeles, CA, USA article, we will further review the pathophysiology, pre- 2 Department of Medicine, David Geffen School of Medicine sentation, and defining characteristics of HHT, with a at UCLA, Los Angeles, CA, USA particular focus on gastrointestinal, dermatological, and 3 Department of Pathology and Laboratory Medicine, pulmonary manifestations. Department of Human Genetics, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA Epidemiology 4 Division of Gastroenterology, Hepatology and Parenteral Nutrition, Department of Veterans Affairs, VA Greater Los Angeles Healthcare System (691/111C), 11301 Wilshire The prevalence of HHT is estimated to be between 1.5 and Blvd., Los Angeles, CA 90073, USA 2 persons per 10,000 [2], although some sources estimate 123 2624 Dig Dis Sci (2017) 62:2623–2630 that the disease is underreported due to variable penetrance present in the lung, brain, and liver. Pulmonary hyperten- and many patients having only minor symptoms until later sion, defined as a sustained elevation of mean pulmonary ages [3]. As a result, established diagnoses of the disease artery pressure above 25 mg at rest (or 30 mg during are significantly lower than the estimated global preva- exercise), is commonly seen in HHT [11]. Olivieri et al. lence. The disease has a higher prevalence in certain found ultrasound evidence of elevated right ventricular populations, including in Afro-Caribbean residents of systolic pressure on 9 of 44 HHT patients (20.5%), while Curac¸ao and Bonaire [4]. Sopen˜a et al. noted similar findings in 9 of 29 (31%) of patients hospitalized for HHT [12, 13]. This is a chronic, Genetics of HHT progressive disease which manifests shortness of breath and dyspnea on exertion and which may progress to right- HHT is an autosomal dominant disease that can result from sided heart failure. It is commonly divided into pre- a wide variety of mutations that ultimately cause a dis- capillary and post-capillary variants, though either may be ruption in transforming growth factor ß (TGF-ß)-mediated seen in HHT. Most commonly, hepatic AVMs lead to high- pathways in vascular endothelial cells. A given disruption output cardiac failure, causing increased venous return and in this pathway results in aberrant blood vessel develop- compensatory pulmonary arterial dilation, left arterial ment, leading to extreme fragility and arteriovenous mal- congestion, and finally post-capillary pulmonary hyper- formations [4]. There are over 600 such mutations that tension (these AVMs were found in 67% of patients with have been discovered and three HHT disease-causing genes evidence of pulmonary hypertension in Sopen˜a et al.) that have been identified to date [5]. HHT type 1 results [11, 13]. However, HHT may also lead to a pre-capillary from mutations in ENG, which encodes endoglin, while condition from remodeling of small pulmonary arteries and HHT type 2 results from mutations in ACVRL1 which which is clinically and histologically identical to primary encodes ALK1. Both of the resulting proteins are compo- pulmonary hypertension that is seen in individuals with nents of the cell surface receptor for the TGF-ß family of BMPRII mutations [11, 14]. It has been hypothesized that ligands. HHT caused by a mutation in MADH4 is associ- this is due to both conditions originating from mutations in ated with juvenile polyposis (JPHT); MADH4 encodes the the TGF-ß receptor superfamily, though pulmonary Smad4 co-transcriptional factor downstream of the TGF-ß hypertension seems more common in patients with pathway [6–8]. There are at least two further unidentified ACVRL1 mutations. Among patients with pulmonary mutations that can cause classical HHT: HHT3 and HHT4. hypertension and equivalent treatment, presence of this The two most common mutations, ENG and ACVRL1, mutation leads to shorter survival times and no evidence comprise about 61% and 37% of cases, respectively. exists for those with ENG mutations [15]. Treatment rec- MADH4 mutations are less common and account for nearly ommendations are currently identical to other pulmonary 2% of cases. Pulmonary AVMs are more commonly seen hypertension patients and include endothelin receptor in HHT1, while hepatic AVMs are more common in HHT2 antagonists, phosphodiesterase inhibitors, prostacyclins, [9]. There has been some debate about whether HHT1 or and symptomatic treatment with diuretics, oxygen, and HH2 represents a more severe manifestation of the disease, digoxin [14]. but a more recent study showed no significant difference in mortality over a 90-month period [9]. Physical Examination Findings Clinical Presentation The classic physical examination finding of HHT is the presence of telangiectasias, which appear in approximately Patients with HHT will most commonly report a history of 74% of patients [16]. These vascular malformations are epistaxis and/or gastrointestinal bleeding. Epistaxis is dilated blood vessels that appear as thin spider-web-like extremely common; half of patients will become symp- red and dark purple lesions that blanch with pressure. They tomatic before age 20s, and the lifetime prevalence of are found on the skin, especially mucocutaneous surfaces epistaxis is 78–96% [10]. The classic vascular malforma- including the lips, oral and nasal cavity, and buccal tions of HHT present throughout the body and are known mucosa. They can also appear on the chest, face, and hands to widely affect the gastrointestinal (GI) system. These [17]. Telangiectasias in the fingertips can be more easily malformations include telangiectasias (small dilated blood found by transillumination with a penlight [18]. In the vessels), arteriovenous malformations (AVMs, tangles of nasal cavity, these telangiectasias can promote epistaxis, abnormal arteries and veins), aneurysms, venous varicosi- which is the most common physical sign of HHT [16]. ties, and arteriovenous fistulas (abnormal direct connec- Telangiectasias develop abruptly, typically before the age tions between arteries and veins). More severe of 30. Other physical examination findings are particular to complications develop when these malformations are complications that may be present, including signs of GI 123 Dig Dis Sci (2017) 62:2623–2630 2625 hemorrhage, liver dysfunction, and pulmonary hyperten- sion. Patients may demonstrate pallor from their anemia, signs of high-output heart failure from
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