Echocardiographic characteristics of patients with antisynthetase syndrome

Abstract

Right ventricular (RV) dysfunction and pulmonary hypertension (PH) occurs in approximately one-third of patients with interstitial lung disease (ILD) and is associated with reduced 6-minute walk distance (6MWD), and increased hospitalizations and mortality. Although the impact of RV dysfunction and PH has been well described in several types of ILD, data is scarce on antisynthetase syndrome. Therefore, we sought to examine the presence of RV dysfunction and PH in patients with antisynthetase syndrome and the impact on clinical outcomes. We conducted a retrospective study of patients with antisynthetase syndrome. Seventy-five subjects were identified. Fifty-one (68%) subjects had echocardiographic data. Patients were grouped into those with normal fractional area change (FAC) ≥ 35% and reduced FAC < 35%. Clinical, echocardiographic, and right heart catheterization data were compared between the two groups. Subjects with FAC < 35% had lower diffusion capacity of the lung for carbon monoxide (29% vs. 47%, p = 0.004), fibrotic features on computed tomography of the chest (79% vs. 33%, p = 0.005), larger RV diameter (5.4 vs. 3.9 cm, p < 0.001), higher right atrial pressures (8 vs. 5 mmHg, p = 0.02), and required supplemental oxygen more frequently (100% vs. 44%, p < 0.001) compared to those with FAC ≥ 35%. We found no difference in 6MWD and hospitalizations between the two groups.The presence of RV dysfunction in antisynthetase syndrome may identify patients at risk of poor outcomes.

Review of Pulmonary Manifestations in Antisynthetase Syndrome

“…103 Regardless, PH in ASyS is often attributed to group 3 disease owing to the extensive lung involvement in many patients and limited reports of group 1 (connective tissue disease [CTD]-related) disease exists. [104][105][106][107][108] In reports where pulmonary arterial hypertension (PAH) is diagnosed, this is often due to either limited parenchymal disease and/or hypoxemia, or when the PH is out of proportion to the degree of ILD. The prevalence of PH in ASyS can be overestimated when patients are solely screened with transthoracic echocardiography (TTE), as demonstrated by Abel et al 104 In their cohort, there was TTE evidence of PH in 23%.…”