Experience of chronic thromboembolic pulmonary hypertension (CTEPH) in two cases with scleroderma and immunopathogenesis overview: Case report

Abstract

Systemic Sclerosis (SSc) is a multi-systemic connective tissue disease of unknown etiology. Although many pathological processes play a role in the basis of pulmonary hypertension (PHT) that develops secondary to SSc, vasculopathy has an important place. Chronic thromboembolic hypertension (CTEPH) is in the group 4 PHT class. CTEPH distinguishes it from other causes of PHT by having both surgical and medical treatment options. CTEPH is a pathology that develops chronically and can be overlooked due to its nonspecific symptoms. Early diagnosis and treatment can reduce morbidity and mortality. The physiopathology of vasculopathy secondary to CTEPH and vasculopathy of SSc made us suspect that similar processes operate in both diseases. The processes that cause and follow endothelial damage are similar in both diseases. If this pathophysiological mechanism can be clarified, possible new treatment options can be discovered. We diagnosed CTEPH with the examinations we performed in two of our patients with SSc and interstitial lung disease, both of which developed PHT. We aimed to discuss the immunopathogenesis with two case reports.

Keywords

• Scleroderma
• Pulmonary Hypertension
• CTEPH

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