Authors:
Muhammad Miftahussurur
and
Joewono Soeroso
Affiliation:
Universitas Airlangga, Indonesia
Keyword(s):
Scleroderma or systemic sclerosis, pulmonary arterial hypertension, cellular immunity, humoral
Abstract:
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