Background Systemic sclerosis (SSc) can virtually affect any organ system, but it is the pulmonary manifestations that account for the majority of deaths, especially interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) (60% of SSc-related deaths)1
Objectives Our aim was to ascertain ILD in patients with SSc
Methods A descriptive study was conducted, using the available data from the Registro Español de ESCLErodermia or Spanish Scleroderma Study Group (RESCLE or SSSG). Patients were classified attending the modified classification criteria proposed by LeRoy and Megdsger2
Results Fourteen referral centers for SSc participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc) and 39 (7%) with SSc sine scleroderma (ssSSc). Pulmonary functional testing (PFT) results along with high-resolution computed tomography (HRCT) findings and PAH assessment, are displayed on tables 1 and2, respectively. As to causes of death, SSc-associated mortality data were as follows: 37 (50%) for lcSSc, 39 (59%) for dcSSc and 5 (42%) for ssSSc; while those for other causes of death were: 37 (50%), 27 (41%) y 7 (58%), respectively (no statistical significance was achieved).
Conclusions ILD is more common in dcSSc and ssSSc, though its occurrence in lcSSc is far from negligible. Furthermore, even if mean systolic pulmonary arterial pressure (PAP) was higher in lcSSc, no significant differences could be found regarding PAH diagnosis among subsets. Therefore, our data suggests that ILD is not exclusive of dcSSc, nor is PAH of lcSSc.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007;66(7):940-4.
LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001;28(7):1573-6.
Disclosure of Interest None declared