Systemic sclerosis and pulmonary arterial hypertension: A case report

Pulmonary arterial hypertension (PAH), which occurs in about 15% of patients with systemic sclerosis (SSc), is a progressive vasculopathy and despite modern therapeutic options is still one of the leading causes of death in these patients. We presented a patient diagnosed with the overlap syndrome (systemic sclerosis and rheumatoid arthritis) with a predominance of the clinical picture of systemic sclerosis (SSc), established in November 2018. She was initially treated by a rheumatologist with an antimalarial, which was soon discontinued by an ophthalmologist, followed by azathioprine which was excluded due to an allergic reaction. She has been continuously on corticosteroid therapy, and since January 2020, mycophenolate mofetil has been added to treatment. The patient was diagnosed with primary biliary cirrhosis by a gastroenterologist after clinical findings and additional examination methods; also, pulmonary fibrosis was diagnosed by a pulmonologist. In January 2020, deterioration of echocardiographic findings was registered (dilated right heart cavity, right ventricular systolic pressure (RVSP) 72 mmHg, tricuspid regurgitation 3+). Sildenafil was proposed by a responsible cardiologist for therapy that was not approved by gastroenterologist. Due to worsening of her symptoms in the form of pronounced fatigue, shortness of breath, in August 2020, a cardiologist of the Institute "Niška Banja" started bosentan therapy in a dose of 2 x 62.5 mg per day. After the applied therapy, the patient had a subjective improvement and reduction of symptoms. In November 2020, a control echocardiographic examination registered a decrease in RVSP to 55 mmHg. In addition to the therapy proposed by the responsible rheumatologist (mycophenolate mofetil 2 g daily, prednisolone 15 - 20 mg daily), the therapy prescribed by her cardiologist was also continued (bosentan 62.5 mg 2 x 1), with regular controls and monitoring of laboratory analyses. PAH in patients with SSc has a worse prognosis than idiopathic PAH, and additionally depends on RVSP and functional class. The process of treating PAH in patients with SSc requires a complex strategy that includes initial assessment of disease severity and subsequent responses to the therapy.