Long-term use of somatostatin analogs for chronic gastrointestinal bleeding in hereditary hemorrhagic telangiectasia.

Background: Chronic bleeding due to gastrointestinal (GI) involvement in patients with hemorrhagic hereditary telangiectasia (HHT) can provoke severe anemia with high red blood cells (RBC) transfusion requirements. However, the evidence about how to deal with these patients is scarce. We aimed to assess the long-term efficacy and safety of somatostatin analogs (SA) for anemia management in HHT patients with GI involvement. Methods: This is a prospective observational study including patients with HHT and GI involvement attended at a referral center. SA were considered for those patients with chronic anemia. Anemia-related variables were compared in patients receiving SA before and during treatment. Patients receiving SA were divided into responders (patients with minimal hemoglobin levels improvement >10 g/L and maintaining hemoglobin levels ≥80 g/L during treatment), and non-responders. Adverse effects during follow-up were collected. Results: Among 119 HHT patients with GI involvement, 67 (56.3%) received SA. These patients showed lower minimal hemoglobin levels (73 [60-87] vs. 99 [70.2-122.5], p < 0.001), and more RBC transfusion requirements (61.2% vs. 38.5%, p = 0.014) than patients without SA therapy. Median treatment period was 20.9 ± 15.2 months. During treatment, there was a statistically significant improvement in minimum hemoglobin levels (94.7 ± 29.8 g/L vs. 74.7 ± 19.7, p < 0.001) and a reduction of patients with minimal hemoglobin levels <80 g/L (39 vs. 61%, p = 0.007) and RBC transfusions requirement (33.9% vs. 59.3%, p < 0.001). Sixteen (23.9%) patients showed mild adverse effects, mostly diarrhea or abdominal pain, leading to treatment discontinuation in 12 (17.9%) patients. Fifty-nine patients were eligible for efficacy assessment and 32 (54.2%) of them were considered responders. Age was associated with non-responder patients, OR 95% CI; 1.070 (1.014-1.130), p = 0.015. Conclusion: SA can be considered a long-term effective and safe option for anemia management in HHT patients with GI bleeding. Older age is associated with poorer response.

Increased Prevalence of Moderate to Severe Mitral and Aortic Valve Dysfunction in Systemic Sclerosis: A Case-control Study.

OBJECTIVE: To investigate the prevalence, severity, and associated clinical factors of mitral and aortic valvular involvement in patients with systemic sclerosis (SSc). METHODS: Our case-control study included 172 patients with SSc and 172 non-SSc adults without known cardiac disease matched by age, sex, and prevalence of cardiovascular (CV) risk factors. The screening of mitral and aortic valvular involvement was performed by transthoracic Doppler echocardiogram. The prevalence of aortic stenosis (AS) was also compared with that reported in a population-based study performed in our community during the same period. RESULTS: Patients with SSc showed an almost 5-fold increased prevalence of moderate to severe mitroaortic valve dysfunction compared to non-SSc controls (OR 4.60, 95% CI 1.51-13.98; P = 0.003). The most common lesion was mitral regurgitation (MR), which was observed in 5.2% of patients, followed by AS in 3.5%, and aortic regurgitation (AR) in 1.7%. Analyzing the different types of valvular lesion separately, we observed a significantly higher frequency of MR compared to controls (OR 4.69, 95% CI 1.12-22.04; P = 0.032), as well as a higher frequency of AS in the 65-75 (OR 7.51, 95% CI 1.22-46.23, P = 0.01) and 76-85 age groups (OR 3.53, 95% CI 1.03-12.22, P = 0.043) when compared to the general population in our community. CONCLUSION: We found an increased prevalence of moderate to severe MR and AS in SSc compared to age-matched non-SSc controls with similar CV comorbidities. While results from this study do not allow for establishing a direct causal relationship, they strongly support the contribution of SSc-specific factors in the development of these complications.

Gastrointestinal Bleeding in Patients with Hereditary Hemorrhagic Telangiectasia: Risk Factors and Endoscopic Findings.

BACKGROUND: We aimed to describe risk factors for gastrointestinal (GI) bleeding and endoscopic findings in patients with hereditary hemorrhagic telangiectasia (HHT). METHODS: This is a prospective study from a referral HHT unit. Endoscopic tests were performed when there was suspicion of GI bleeding, and patients were divided as follows: with, without, and with unsuspected GI involvement. RESULTS: 67 (27.9%) patients with, 28 (11.7%) patients without, and 145 (60.4%) with unsuspected GI involvement were included. Age, tobacco use, endoglin (ENG) mutation, and hemoglobin were associated with GI involvement. Telangiectases were mostly in the stomach and duodenum, but 18.5% of patients with normal esophagogastroduodenoscopy (EGD) had GI involvement in video capsule endoscopy (VCE). Telangiectases ≤ 3 mm and ≤10 per location were most common. Among patients with GI disease, those with hemoglobin < 8 g/dL or transfusion requirements (65.7%) were older and had higher epistaxis severity score (ESS) and larger telangiectases (>3 mm). After a mean follow-up of 34.2 months, patients with GI involvement required more transfusions and more emergency department and hospital admissions, with no differences in mortality. CONCLUSIONS: Risk factors for GI involvement have been identified. Patients with GI involvement and severe anemia had larger telangiectases and higher ESS. VCE should be considered in patients with suspicion of GI bleeding, even if EGD is normal.