Systemic sclerosis associated with pulmonary arterial hypertension: Focus on the visceral form of the disease

Introduction. Systemic sclerosis is a connective tissue disease with the development of obliterating arteriolopathy and active fibrosis formation both in internal organs and in the skin. Pulmonary arterial hypertension is a life-threatening manifestation of systemic sclerosis, leading to death if diagnosed late. The search for predictors, as well as associated disease phenotypes, can facilitate early diagnosis and improve prognosis.

Aim. To characterize the features of the visceral form in comparison with the limited variant in patients with pulmonary arterial hypertension associated with systemic sclerosis.

Materials and methods. 14 patients with visceral and 63 with a limited variant of systemic sclerosis associated with pulmonary arterial hypertension were studied. The diagnosis of systemic sclerosis was established based on the 2013 ACR-EULAR criteria; pulmonary arterial hypertension was verified by right heart catheterization. In all patients, other causes of pulmonary hypertension – heart disease, lung disease, thrombophilia were excluded.

Results. At the time of inclusion in the study, patients with visceral systemic sclerosis were younger (48 (35; 56) years) than those with limited systemic sclerosis (54 (49; 63) years, but the differences only approached significant (p = 0.057). All patients had the Raynaud’s syndrome, with limited systemic sclerosis, digital ischemic disorders were more often observed (41% compared to 14%, p = 0.11). Anticentromere antibodies caused by pulmonary arterial hypertension predominated; antibodies to topoisomerase-I were detected only in two patients with limited systemic sclerosis. The severity index was significantly higher in patients with limited systemic sclerosis (p < 0.05). The clinical manifestations of pulmonary arterial hypertension in both groups were also the same. When studying central hemodynamics, no significant differences were found. The median follow-up of patients was 68 (39; 111) months. Survival also did not differ: with visceral systemic sclerosis it was 63 (40; 99) months, with limited systemic sclerosis – 69 (36; 116) months.

Conclusion. A comparative analysis demonstrated the similarity of the two systemic sclerosis phenotypes, which suggests the universality of approaches to the early diagnosis of pulmonary arterial hypertension.

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