Review Article

Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

Table 2

Recommendations for right heart catheterization for SSc and SSc-spectrum disorder.

ModalitiesParameter thresholds required for RHCSigns/ required for RHC

EchocardiographyTR velocity
 2.5–2.8 m/sYes
 >2.8 m/sNo
Cavity enlargements irrespective of TR velocityNo
 Right atrial major dimension > 53 mm or
 Right ventricular mid-cavity dimension > 35 mm

Pulmonary function testsFVC/DLCO ratio > 1.6 and/or DLCO < 60%Yes
FVC/DLCO ratio > 1.6 and/or DLCO < 60% and NT-pro BNP > 2 times upper limit of No

Composite measuresMeets DETECT algorithm in patients with DLCO < 60% and disease duration of >3 years from 1st non-Raynaud’s symptomNo

: dyspnea on rest or exercise, fatigue, presyncope/syncope, chest pain, palpitations, dizziness, and lightheadedness. Signs: loud pulmonic sound and peripheral edema. overt systolic dysfunction, greater than grade I diastolic dysfunction or greater than mild mitral or aortic valve disease, or evidence of PAH in echocardiography; DLCO: diffusing capacity of carbon monoxide; FVC: forced vital capacity; NT-proBNP: N-terminal probrain natriuretic peptide; PAH: pulmonary arterial hypertension; SSc: systemic sclerosis; TR: tricuspid regurgitation (cited and modified from “Recommendations for Screening and Detection of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension” by D. Khanna, C. H. Tseng, N. Farmani et al. Arthritis Rheum 2013; 65: 3194-201).