Scleroderma Patients with Pulmonary Artery Hypertension: A Case Report

• Main Authors: Muhammad Miftahussurur, Joewono Soeroso
• Format: Book Section PeerReviewed
• Language: English; English; English
• Published: SCITEPREES-Science and Technology Publications, Lda. 2017
• Subjects: R Medicine (General); RC Internal medicine
• Online Access: http://repository.unair.ac.id/91963/1/3p.%20Scleroderma%20Patients%20with%20Pulmonary%20Artery%20Hypertension_%20A%20Case%20Report.pdf; http://repository.unair.ac.id/91963/2/3.%20scleroderma%20patient.pdf; http://repository.unair.ac.id/91963/5/scleroderma.pdf; http://repository.unair.ac.id/91963/; https://www.scitepress.org/PublicationsDetail.aspx?ID=0NSueRAnMDE=&t=1
• Institution: Universitas Airlangga

Scleroderma or systemic sclerosis (SSc) is a multiorgan connective tissue disorder involving the skin, lungs, heart, digestive tract, musculoskeletal system, and peripheral circulation. Pulmonary arterial hypertension (PAH) is an increase in mean pulmonary artery pressure > 25 mmHg as measured by echocardiography or catheterization. The incidence of PAHs with SSc is so rare that reports, in this case, are necessary. Objective: to describe the management of PAH patients with SSc. Case: a 32-year-old patient experiencing PAHs with SSc has stiffness of the mouth and is difficult to open. The ECG shows a sinus tachycardia with a Right axis destination and a cardiomegaly thorax photo with a 64% cardio-thorax ratio. Some clinical data show autoimmunity. Treatment is oxygen, diuretic, thrombolytic, and immunosuppressive drugs. Conclusions: Reports of PAH treatment with SSC are essential for the improvement of therapy