Samuel Leal Rodriguez1, Francisco Miguel Ortiz Sanjuan2, José Ivorra Cortés3, Laura Mas Sanchez3, Pablo Muñoz Martinez4, Carmen Riesco Bárcena5, Anderson Huaylla Quispe6, Cristobal Pavez Perales3, Inés Cánovas Olmos3, Luis Gonzalez Puig7, Elena Grau García3, Isabel Martínez-Cordellat3, Carmen Nájera Herranz3, Rosa Negueroles Albuixech3, JOSE ELOY OLLER RODRIGUEZ8, Elvira Vicens Bernabeu2, Alba Torrat Novés5, Daniel Ramos Castro3 and José andrés Román ivorra1, 1Hospital Universitari i Politécnic La Fe, València, Spain, 2Hospital Universitario de La Fe, València, 3Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, Spain, 4Rheumatology Department. Hospital Universitario y Politécnico La Fe, Sagunto, Spain, 5Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, 6Medicina, València, Spain, 7Hospital La Fe, Torrente, Valencia, Spain, 8Hospital Universitario de La Fe, València, Spain
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension. Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.
Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.
Methods: Retrospective observational study of a wide and unselected series of patients diagnosed of Early-SSc from a single university hospital from 2012 to 2022. Patients were classified as Early-SSc following Le Roy criteria. However, patients did not meet the 2013 ACR/EULAR classification criteria for SSc. We performed pulmonary function tests through conventional spirometry and measurement of the diffusing capacity for carbon monoxide (DLCO).
Results: We included 61 patients with a mean age of 52.9±6.2 years (90.9% women; 9.1% men).
At the diagnosis of Early-SSc, none of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 21 patients (34.4%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 26 patients (42.6%).
After a mean follow-up period of 42.4±3.2 months, 29 (47.5%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieving SSc classification was 24.4±1.8 months. The remaining 32 patients continued classified as Early-SSc.
An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. When compared with the subgroup of patients which did not progress to SSc we found significant differences (Decreased DLCO: 51.7% vs 10.4%; p=0.01 and decreased MMEF 25-75: 42.8% vs 24.6%; p=0.05).
The analysis of pulmonary function of the subgroup of patients that remained classified as Early-SSc after did not show significative changes after follow-up.
Conclusion: In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more common in patients who progressed to definitive SSc. Patients that remained classified as Early-SSc did not experience significative changes.
Our results support the concept that pulmonary function was impaired in Early-SSc and that it should probably be considered for future Early-SSc classification criteria.
Disclosures: S. Leal Rodriguez, None; F. Ortiz Sanjuan, None; J. Ivorra Cortés, None; L. Mas Sanchez, None; P. Muñoz Martinez, None; C. Riesco Bárcena, None; A. Huaylla Quispe, None; C. Pavez Perales, None; I. Cánovas Olmos, None; L. Gonzalez Puig, None; E. Grau García, None; I. Martínez-Cordellat, None; C. Nájera Herranz, None; R. Negueroles Albuixech, None; J. OLLER RODRIGUEZ, None; E. Vicens Bernabeu, None; A. Torrat Novés, None; D. Ramos Castro, None; J. Román ivorra, None.
