Pulmonary hypertension in patients with interstitial pneumonia with autoimmune features

Alzghoul, Bashar; Hamburger, Robert; Lewandowski, Thomas; Janssen, B.; Grey, Daniel; Xue, Wei; Ataya, Ali; Alnuaimat, Hassan; Gomez-Manjarres, Diana; Patel, Divya; Reddy, Raju

doi:10.1177/2045894020944117

Cited by 5 publications

(4 citation statements)

References 27 publications

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“…Similar findings were noted by Hervier et al who found higher prevalence of ILD, lower DLCO and higher FVC/DLCO in those with PH and no difference in serology between those with and without PH 4 . Subjects with FAC < 35% also had higher rates of RV remodeling, a finding noted in other studies examining ILD patients with and without PH 10,28 . Another significant finding was higher mortality rates or lung transplant requirements in those with FAC < 35% compared to those with FAC ≥ 35%.…”

Section: Discussionsupporting

confidence: 85%

“…10 In another study of subjects with interstitial pneumonia with autoimmune features, 15/39 (38%) of subjects had FAC < 35%. 28 The lower percentage of subjects with impaired FAC% in our study compared to the study by Prins et al could be due to variation in the study population. 10 Their study consisted of a mixed group of Group 3 PH such as chronic obstructive pulmonary disease, combined pulmonary fibrosis and emphysema, obesity related lung disease and ILD, whereas our study was limited to antisynthetase syndrome alone.…”

Section: Discussioncontrasting

confidence: 63%

“…In a study of 167 patients (89 of whom had FAC% measured) with Group 3 PH by Prins et al, impaired RV function defined as FAC < 28% was present in 58% of subjects 10 . In another study of subjects with interstitial pneumonia with autoimmune features, 15/39 (38%) of subjects had FAC < 35% 28 . The lower percentage of subjects with impaired FAC% in our study compared to the study by Prins et al could be due to variation in the study population 10 .…”

Section: Discussionmentioning

confidence: 98%

“…4 Subjects with FAC < 35% also had higher rates of RV remodeling, a finding noted in other studies examining ILD patients with and without PH. 10,28 Another significant finding was higher mortality rates or lung transplant requirements in those with FAC < 35% compared to those with FAC ≥ 35%. Similar findings were reported by Prins et al who not only found higher mortality in those with impaired FAC% but also a direct association between reduced FAC% and mortality or hospitalization.…”

Section: Discussionmentioning

confidence: 99%

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Echocardiographic characteristics of patients with antisynthetase syndrome

et al. 2022

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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.

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Section: Discussionsupporting

confidence: 85%