Абстрактный

Review of the literature and tasks learned by the Rheumatology residents about the primary immunodeficiency association with systemic lupus erythematosus

Robin Christensen

Primary immunodeficiency diseases (PID) represent a miscellaneous group of conditions performing from inherited blights in the development, development and normal function of vulnerable cells; therefore, turning individualities susceptible to intermittent infections, mislike, autoimmunity, and malice. In this retrospective study, autoimmune conditions (AIDs), in special systemic lupus erythematosus( SLE) which arose associated to the course of PID, are described. Classically, the literature describes three groups of PID associated with SLE( 1) insufficiency of Complement pathway factors,( 2) blights in immunoglobulin conflation, and( 3) habitual granulomatous complaint( CGD). presently, other PID have been described with clinical incarnation of SLE, similar as Wiskott – Aldrich pattern( WAS), autoimmune polyendocrinopathy candidiasis ectodermal dystrophy( APECED), autoimmune lymphoproliferative pattern( mounts) and idiopathic CD4 lymphocytopenia. Also we present findings from an adult cohort from the inpatient clinic of the Rheumatology Division of Universidade Federal de São Paulo. The PID instantiations set up by our study group were considered mild in terms of inflexibility of infections and mortality in early life. Therefore, it's possible that some immunodeficiency countries are compatible with survival regarding contagious vulnerability; still these countries might represent a strong prepping factor for the development of vulnerable diseases like those observed in SLE.