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Full-Text Articles in Skin and Connective Tissue Diseases
Sildenafil Versus Placebo For Early Pulmonary Vascular Disease In Scleroderma (Sepvadis): Protocol For A Randomized Controlled Trial, Matthew R. Lammi, Monica Mukherjee, Lesley Ann Saketkoo, Kyle Carey, Laura Hummers, Steven Hsu, Amita Krishnan, Marie Sandi, Ami A. Shah, Stefan L. Zimmerman, Paul M. Hassoun, Steven C. Mathai
Sildenafil Versus Placebo For Early Pulmonary Vascular Disease In Scleroderma (Sepvadis): Protocol For A Randomized Controlled Trial, Matthew R. Lammi, Monica Mukherjee, Lesley Ann Saketkoo, Kyle Carey, Laura Hummers, Steven Hsu, Amita Krishnan, Marie Sandi, Ami A. Shah, Stefan L. Zimmerman, Paul M. Hassoun, Steven C. Mathai
School of Medicine Faculty Publications
BACKGROUND: Pulmonary hypertension (PH) is a leading cause of death in patients with systemic sclerosis (SSc). An important component of SSc patient management is early detection and treatment of PH. Recently the threshold for the diagnosis of PH has been lowered to a mean pulmonary artery pressure (mPAP) threshold of > 20 mmHg on right heart catheterization (RHC). However, it is unknown if PH-specific therapy is beneficial in SSc patients with mildly elevated pressure (SSc-MEP, mPAP 21-24 mmHg). METHODS: The SEPVADIS trial is a randomized, double-blind, placebo-controlled phase 2 trial of sildenafil in SSc-MEP patients with a target enrollment of 30 …
Hereditary Angioedema: Diagnosis, Clinical Implications, And Pathophysiology, Evan S. Sinnathamby, Peter P. Issa, Logan Roberts, Haley Norwood, Kevin Malone, Harshitha Vemulapalli, Shahab Ahmadzadeh, Elyse M. Cornett, Sahar Shekoohi, Alan D. Kaye
Hereditary Angioedema: Diagnosis, Clinical Implications, And Pathophysiology, Evan S. Sinnathamby, Peter P. Issa, Logan Roberts, Haley Norwood, Kevin Malone, Harshitha Vemulapalli, Shahab Ahmadzadeh, Elyse M. Cornett, Sahar Shekoohi, Alan D. Kaye
School of Medicine Faculty Publications
Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a mutation in the C1 esterase inhibitor gene. HAE affects 1/50,000 people worldwide. Three main types of HAE exist: type I, type II, and type III. Type I is characterized by a deficiency in C1-INH. C1-INH is important in the coagulation complement, contact systems, and fibrinolysis. Most HAE cases are type I. Type I and II HAE result from a mutation in the SERPING1 gene, which encodes C1-INH. Formally known as type III HAE is typically an estrogen-dependent or hereditary angioedema with normal C1-INH activity. Current guidelines now recommend subdividing …