Background: Pulmonary arterial hypertension(PAH) is frequently associated with systemic sclerosis(SS). Nevertheless, little is known about the triggers that may exacerbate PAH in these patients.
Case report: A 71-year-old woman, previously diagnosed with Raynaud ‘s Syndrome and without any other significant medical history, was admitted to our Emergency Room due to dyspnea and fever. She was diagnosed with pulmonary infection complicated by empyema(Figure1) and underwent surgical toilet and decortication of the lower lobe with full recovery. Echocardiography during the hospital stay was normal. Few months after discharge she developed dyspnea WHO-FC III and fatigue and eventually she was hospitalized with a diagnosis of acute heart failure. The ECG showed right bundle branch block and signs of right ventricle strain(Figure2). The echocardiogram(Figure3) was characterized by normal left ventricular systolic function, enlarged right ventricle with D shape and moderate impairment of systolic radial function; pulmonary hypertension(PH) with an estimated pulmonary artery pressure of 60mmHg was also detected. Pulmonary CT angiography excluded pulmonary embolism. Aiming to better characterize PH, a right heart catheterization was performed with the following findings: mean pulmonary artery pressures 34mmHg(55 mmHg systolic-22mmHg diastolic), wedge pressure 5mmHg, right atrium pressure 2mmHg, pulmonary vascular resistance 8WU; the cardiac output and cardiac index were only mildly reduced. Elevated levels of NT-proBNP and troponin I were also found(1926pg/m [n.v.<125] and 65.9ng/L [n.v.<19] respectively), whereas blood count, liver and renal function, rheumatoid factor, and C-reactive protein levels were normal. Based on the above findings, a diagnosis of pulmonary arterial hypertension(PAH) in intermediate-high risk class was made. Therefore, dual specific therapy for PAH with sildenafil and macitentan was initiated. The rheumatologic screening findings(CENP A/B positive, typical pathological findings at capillaroscopy) associated with the patient's history PAH, Raynaud Syndrome) were consistent with a diagnosis of SS. After three months the patient showed a good response to dual therapy and progressed to a low-risk class(WHO-FC II, 6MWD=420m, normal NT-proBNP values). In conclusion, lung abscess and its surgical treatment may have played a role in our patient. Close follow-up of SS patients should be ensured when they face interventions and/or acute stresses.
