Delia Reina1, Daniel Roig Vilaseca2, Vicenç Torrente-Segarra2, Dacia Cerdà2, Ivan Castellví3, Cèsar Díaz Torné3, Mireia Moreno4, Javier Narváez5, Vera Ortiz6, Rosana Blavia7, Montserrat Martín-Baranera8, Hèctor Corominas2. 1. Servicio de Reumatología, Hospital Sant Joan Despí Moisès Broggi, Spain. Electronic address: deliareinasanz@gmail.com. 2. Servicio de Reumatología, Hospital Sant Joan Despí Moisès Broggi, Spain. 3. Servicio de Reumatología, Hospital de Sant Pau, Spain. 4. Servicio de Reumatología, Hospital Parc Taulí, Spain. 5. Servicio de Reumatología, Hospital de Bellvitge, Spain. 6. Servicio de Reumatología, Hospital de Granollers, Spain. 7. Servicio de Neumología, Hospital Sant Joan Despí Moisès Broggi, Spain. 8. Clinical Epidemiology Department, Consorci Sanitari Integral, Spain.
Abstract
UNLABELLED: Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication. OBJECTIVE: To evaluate the clinical characteristics of patients diagnosed with PSS with ILD. METHODS: Multicentre cohort study with 25 patients diagnosed with PSS and ILD. Data of PSS, prognostic factors, pulmonary involvement variables, complementary tests that suggest a worse diagnosis and treatment given were collected. EULAR index was measured for Sjögren's syndrome. RESULTS: We identified 25 patients. In 15/25 the diagnosis of ILD was done before the diagnosis of PSS. The histopathological patterns found were: 12 NSIP, 5 UIP, 4 OP, 2 LIP. PFRs showed restrictive pattern. The majority of the patients received glucocorticoid therapy, antimalarial or immunosuppressive treatment. CONCLUSIONS: Patients affected with PSS must be screened to catch a precocious diagnosis of ILD. The majority of the patients were diagnosed of ILD before being diagnosed of PSS. Multicenter cohorts are increasingly demanded and a multidisciplinary management is needed.
UNLABELLED: Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication. OBJECTIVE: To evaluate the clinical characteristics of patients diagnosed with PSS with ILD. METHODS: Multicentre cohort study with 25 patients diagnosed with PSS and ILD. Data of PSS, prognostic factors, pulmonary involvement variables, complementary tests that suggest a worse diagnosis and treatment given were collected. EULAR index was measured for Sjögren's syndrome. RESULTS: We identified 25 patients. In 15/25 the diagnosis of ILD was done before the diagnosis of PSS. The histopathological patterns found were: 12 NSIP, 5 UIP, 4 OP, 2 LIP. PFRs showed restrictive pattern. The majority of the patients received glucocorticoid therapy, antimalarial or immunosuppressive treatment. CONCLUSIONS:Patients affected with PSS must be screened to catch a precocious diagnosis of ILD. The majority of the patients were diagnosed of ILD before being diagnosed of PSS. Multicenter cohorts are increasingly demanded and a multidisciplinary management is needed.