Abstract

Pulmonary Hypertension in Early Systemic Sclerosis and Sine Scleroderma Systemic Sclerosis

Author(s): Luis Javier Cajas1*, Julia Recalde, Reyes2 and Javier AleFebdro Correa2

Objectives: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in patients with systemic sclerosis (SSc), presenting various etiologies. SSc manifests even before cutaneous involvement (early SSc) or, in some cases, does not develop SSc without scleroderma (ssSSc). Cases of PH have been reported even in early stages of the disease. This study aims to determine the prevalence of PH in these patients and to identify its causes and risk factors.

Methods: Data from a cohort of SSc patients in Bogotá, Colombia, were analyzed, excluding those with cutaneous sclerosis. Those meeting the ACR/EULAR criteria were considered as ssSSc, while those not meeting these criteria but meeting the VEDOSS criteria were considered as early SSc (eSSc). High probability of PH was considered when the pulmonary systolic pressure was greater than 39 mmHg or the peak tricuspid regurgitation velocity (PTRV) was >3.4 m/s, with a PASP between 33 and 39 mmHg or PTRV between 2.9 and 3.4 m/s, combined with two other suggestive findings of PH. PH was classified as type 2 if left ventricle ejection fraction <50%, coronary disease, or moderate to severe diastolic dysfunction; type 3 if extensive interstitial disease on tomography >20%, forced vital capacity (FVC) <70%, or DLCO<60%; type 4 if embolism-related abnormalities were detected on scintigraphy or tomography. Patients not meeting these criteria were classified as type 1 PH. We search for the proportion of patients with PH and risk factors associated.

Results: Out of a total of 353 patients, 71 (20%) had early SSc, and 51 (14.4%) had SSc without scleroderma. The prevalence of PH was 26%, with 25% in early SSc with intersticial lung disease (eSSc- ILD) and 27% in ssSSc. Seventy two percent and 50% were classified as group 1 in each category. Two patients were classified as group 2 and 3, but no patients with type 4 PH were found. There was no relationship found between gender, years of RP, digital ulcers, telangiectasias, or other clinical or antibody variables with the presence of PH, especially the presence of anti-centromere antibodies (p=0.5) and abnormalities in capillaroscopy (p=0.96), both in all PH groups and exclusively in type 1 group.

Conclusion: PH can occur or is highly probable in approximately a quarter of patients, in early stages of the disease or in the absence of cutaneous involvement, showing a variety of etiologies. No associated risk factors were identified.