Sarcoidosis

La sarcoidosis es una enfermedad sistémica de etiología desconocida que se caracteriza por el desarrollo de granulomas epiteloides no caseificantes. Los pulmones son los órganos más afectados (>90 % de los casos), seguidos de los ganglios linfáticos, la piel y los ojos. Esta revisión resume las principales manifestaciones clínicas y las opciones actuales de farmacoterapia. Los glucocorticoides son la primera línea de tratamiento para la sarcoidosis. Para los pacientes con las formas más severas de sarcoidosis (que necesitarán glucocorticoides durante largos períodos de tiempo) y para aquellos que son intolerantes o resistentes al tratamiento, se utilizan medicamentos inmunosupresores como agentes ahorradores de glucocorticoides. El manejo de la sarcoidosis extratorácica debe adaptarse al órgano u órgano específico involucrado;sin embargo, hay datos limitados de ensayos controlados para guiar el tratamiento de estos pacientes. La aparición de terapias biológicas ha aumentado el arsenal terapéutico disponible para tratar la sarcoidosis, siendo los agentes anti-TNF monoclonales los más prometedores, pero su uso todavía está limitado por la falta de licencias y costos.Sarcoidosis is a systemic disease of unknown etiology characterized by the development of non-caseating epitheloid granulomas. The lungs are the most commonly involved organ (>90% of cases), followed by the lymph nodes, the skin, and the eyes. Areas covered: This review summarizes current pharmacotherapy options and future directions for the development of new therapies. Glucocorticoids are the first-line therapy for sarcoidosis. For patients with the most severe forms of sarcoidosis (who will need glucocorticoids for long periods) and for those intolerant or refractory, immunosuppressive drugs are used as sparing agents. The management of extrathoracic sarcoidosis must be tailored to the specific organ or organs involved;however, there is limited data from controlled trials to guide the treatment of these patients. The emergence of biological therapies has increased the therapeutic armamentarium available to treat sarcoidosis, with monoclonal anti-TNF agents being the most promising, but their use is still limited by a lack of licensing and costsFil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Instituto Universitario de Ciencias Biomédicas de Córdoba; ArgentinaFil: Brito Zeron, Pilar. Hospital Cima-sanitas; EspañaFil: Pérez Álvarez, Roberto. Sociedad Española de Medicina Interna; EspañaFil: Achad, Mario Oscar. Universidad Nacional de Córdoba; ArgentinaFil: Pallarés, Lucio. Sociedad Española de Medicina Interna; EspañaFil: Cuestas, Eduardo. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; ArgentinaFil: Ramos Casals, Manuel. Sociedad Española de Medicina Interna; Españ

SARS-CoV-2 infection in patients with primary Sjögren syndrome: characterization and outcomes of 51 patients

OBJECTIVE: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. METHODS: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. RESULTS: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. CONCLUSION: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities

Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjogren's syndrome

Objective: To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Phenotyping Sjögren's syndrome: towards a personalised management of the disease

Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases.Fil: Brito Zeron, Pilar. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Hospital Cima-sanitas, Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); EspañaFil: Retamozo, Maria Soledad. Department Of Autoimmune Diseases, Hospital Clinic; España. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Sjögren Syndrome Research Group (agaur); EspañaFil: Ramos-Casals Manuel. Universidad de Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; Españ