gms | German Medical Science

Introduction: The most severe course and extremely unfavorable prognosis are patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (PAH-SSс). SSc in a group of patients with PAH-SSс is diagnosed late, therapy is appointed untimely, which has a significant effect on life expectancy.

Methods: The study included 51 patients with PAH-SSс. All patients were evaluated for the 1980 ACR criteria and 2013 ACR-EULAR criteria for SSc.

Results: RP was detected in 51 cases. The duration of the disease from Raynaud's phenomenon (RP) to PAH was 148.5 ± 133.6 months [0; 594]. The duration of the disease from the first non-Raynaud symptom to PAH was 90.3 ± 86.4 months [0; 360]. PAH was diagnosed by right heart catheterization on the average at 24 [13; 53] months from the onset symptoms. Patients with a limited form of SSc (69%) prevailed. 10 (20%) patients had no skin involvement. Only 3 (6%) patients had a diffuse form. Digital ischemic disorders developed after 56 [16; 84] months from the onset of the SSc. Antinuclear antibodies were found in 50 (98%) pts. Anticentromere antibodies were detected in most cases (64%). Antibodies to topoisomerase-I were found only in 2 pts. In general, the diagnosis of SSc was established rather late, an average of 109 months from the beginning of the SSc [62; 231]. PAH as the first non-Raynaud symptom was detected in 1 patient. Only 22 patients met the 1980 ACR criteria. All patients (100%) met the 2013 ACR-EULAR criteria.

Conclusion: The late diagnosis of SSc in patients with PAH-SSс is associated with the scarcity of clinical symptoms, the absence of severe visceral manifestations until the moment of PAH accession, and the low sensitivity of the 1980 ACR criteria. The introduction of 2013 ACR-EULAR criteria contributes to the early diagnosis of SSc and the timely initiation of therapy.

Disclosures: None declared