Systemic sclerosis is a rare multisystem connective tissue disease characterized by a vasculopathy, diffuse fibrosis and immune abnormalities. This disease has a high mortality rate, especially if it presents with concomitant disorders, such as interstitial lung disease or pulmonary arterial hypertension. Although pulmonary arterial hypertension is a rare disease, with an estimated frequency of 15-50 cases per million, the prevalence of this condition in patients with systemic sclerosis has been reported to be 7-12%. It is important to proceed with the screening programmes for patients with systemic sclerosis, since early diagnosis and treatment therapies may offer an improved prognosis.
Retrospective study was performed in Pulmonary hypertension coordination center and Rheumatology center of Vilnius University Hospital Santariskiu Klinikos. Patients did not receive any treatment or intervention. Indicators that describe heart and lung functional condition, inflammatory markers and treatment were analyzed in a study.
From 70 patients with systemic sclerosis in Rheumatology center database 14 (20%) were diagnosed with pulmonary arterial hypertension. In Pulmonary hypertension coordination center 16 patients were studied: 14 (87.5%) of them had systemic sclerosis, 1 (6.25%) - mixed connective tissue disease and 1 (6.25%) - systemic lupus erythematosus. Because of noncompliance, 3 (18.75%) patients of 16 did not receive specific treatment for pulmonary arterial hypertension. Monotherapy with Sildenafil was given to 3 (23.1%) patients and 8 (61.6%) patients received combination therapy.
Comparing assessed test’s (6-minute walk test (m), tricuspid annular plane systolic excursion (TAPSE, cm), diffusion capacity (DLCO%) and brain natriuretic peptide (BNP, pg/mL)) averages before receiving specific treatment and their dynamics during the treatment, statistically significant changes were not observed.
Based on literature and subjective condition of patients, specific treatment of pulmonary arterial hypertension is effective, but due to the small amount of patients in a study, statistically significant improvement in functional condition was not observed
