Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Objective: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. / Methods: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty‐four clinical and serologic variables were used for clustering. / Results: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. / Conclusion: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis

A CASE OF ACQUIRED PARTIAL LIPODYSTROPHY ASSOCIATED WITH LOCALIZED SCLERODERMA AND UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE.

A CASE OF ACQUIRED PARTIAL LIPODYSTROPHY ASSOCIATED WITH LOCALIZED SCLERODERMA AND UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE

Selective intra-arterial terlipressin infusion stops acute lower gastrointestinal bleeding: a case report and review of literature

Acute gastrointestinal bleedings are considered clinical emergency events and implicate a difficult medical decision-making process, in particular in poor surgical candidates. Here, we report one case with acute lower gastrointestinal bleeding, for the first time, treated with selective intra-arterial infusion of terlipressin (triglycyl lysine-vasopressin), a long-acting vasopressin analogue. The patient was affected by lung adenocarcinoma with abdominal metastasis and presented severe lower intestinal bleeding. Using selective angiography, the middle colic artery was catheterized and terlipressin was infused as follows: 1.5 mg in bolus (21 microg/kg), then 1.5 mg (21 microg/kg) intra-arterially in 20 min, then 1.5 mg in bolus (21 microg/kg), determining the cessation of the lower gastrointestinal bleeding. Since no cases of intra-arterial selective infusion of terlipressin have been reported in the literature, terlipressin may represent a new useful tool in pharmaco-angiographic strategy. The present case should prompt its consideration for further clinical studies