guanylate cyclase c
Recently Published Documents


TOTAL DOCUMENTS

148
(FIVE YEARS 33)

H-INDEX

21
(FIVE YEARS 2)

2022 ◽  
Vol 124 (1) ◽  
pp. 151811
Author(s):  
Chengfeng Xing ◽  
Tao Zhang ◽  
Xiaolin Liu ◽  
Canmei Li ◽  
Gang Yang ◽  
...  

Author(s):  
Stuart M. Brierley ◽  
Luke Grundy ◽  
Joel Castro ◽  
Andrea M. Harrington ◽  
Gerhard Hannig ◽  
...  

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Mitchell B. Cohen ◽  
Benjamin D. Gold ◽  
Stavra A. Xanthakos ◽  
Nicholas CaJacob ◽  
Taryn Weissman ◽  
...  

2021 ◽  
Vol 206 (Supplement 3) ◽  
Author(s):  
Casey Ligon ◽  
Gehard Hannig ◽  
Carolyn Higgins ◽  
Beverley Greenwood-Van Meerveld

Author(s):  
Clayton M. Carey ◽  
Sarah E. Apple ◽  
Zoë A. Hilbert ◽  
Michael S. Kay ◽  
Nels C. Elde

2021 ◽  
Vol 62 (3) ◽  
pp. 250-263
Author(s):  
Nikola Habek ◽  
Martina Ratko ◽  
Aleksandra Dugandžić

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Eric J. Dein ◽  
Fredrick M. Wigley ◽  
Zsuzsanna H. McMahan

Abstract Background Lower gastrointestinal (GI) tract involvement can affect up to 50% of systemic sclerosis (SSc) patients, and may result in malabsorption, pseudo-obstruction, hospitalization, and death. We report our experience with linaclotide, a selective agonist of guanylate cyclase C (GC-C), for SSc patients with refractory lower GI disease. Methods We performed an analysis of patients seen at the Johns Hopkins Scleroderma Center and identified patients prescribed linaclotide for refractory lower GI manifestations. Patients had clinical data collected in our longitudinal database. Linaclotide responders were on medication for at least 12 months with documented effectiveness by the treating physician. Results Thirty-one patients with SSc were treated with linaclotide. At the time of linaclotide initiation, 23 of these patients (74%) were classified as having severe GI disease, as defined by recurrent pseudo-obstruction, malabsorption, and/or need for artificial nutrition (Medsger GI severity score ≥ 3). The majority of patients (90.3%; 28/31) had a treatment response, while only three patients (9.7%) reported ineffectiveness or intolerable side effects. Low-dose linaclotide (≤ 145 mcg daily) was used in 18 patients and was effective in 94%. High-dose therapy (> 145 mcg daily) was effective in 11 of 13 patients (85%). Common side effects were diarrhea, cramping, or bloating (11/31, 35%). Ineffectiveness, cost, and abdominal pain were complaints cited among those who discontinued therapy. Conclusion Linaclotide is a well-tolerated and efficacious pro-secretory and pro-motility agent that can be used to manage refractory lower GI manifestations in SSc. We found that low-dose linaclotide is an effective option and may be better tolerated, though a subset of patients may require high dose regimens.


Author(s):  
Eric Dein ◽  
Fredrick M. Wigley ◽  
Zsuzsanna H. McMahan

Abstract Objective: Lower gastrointestinal tract involvement can affect up to 50% of SSc patients, and may result in malabsorption, pseudo-obstruction, hospitalization, and death. We report our experience with linaclotide, a selective agonist of guanylate cyclase C, for SSc patients with refractory lower GI disease.Methods: We performed a analysis of patients seen at the Johns Hopkins Scleroderma Center and identified patients prescribed linaclotide for refractory lower GI manifestations. Patients had clinical data prospectively collected in our longitudinal database. Linaclotide responders were on medication for at least 12 months with documented effectiveness by the treating physician. Results: Thirty-one patients with SSc were treated with linaclotide. Twenty-three of these patients (74%) had severe GI disease on initiation of linaclotide (Medsger GI score ≥3), as defined by recurrent pseudo-obstruction, malabsorption, and/or need for artificial nutrition. The majority of patients (90.3%; 28/31) had a treatment response, while only three patients (9.7%) reported ineffectiveness or intolerable side effects. Low-dose linaclotide (≤ 145 mcg daily) was used in 18 patients and was effective in 94%. High-dose therapy (>145 mcg daily) was effective in 11 of 13 patients (85%). Common side effects were diarrhea, cramping, or bloating (11/31, 35%). Ineffectiveness, cost, and abdominal pain were complaints cited among those who discontinued therapy. Conclusion: Linaclotide is a well-tolerated and efficacious pro-secretory and pro-motility agent that can be used to manage refractory lower GI manifestations in SSc. We found that low-dose linaclotide is an effective option and may be better tolerated, though a subset of patients may require high dose regimens.


Sign in / Sign up

Export Citation Format

Share Document