Policondrite recidivante: revisão sistemática e relato de caso.

Abstract

Relapsing polychondritis (RP) is a rare systemic autoimmune disease, uncommon in childhood and adolescence, characterized by recurrent episodes of inflammation of cartilaginous tissues and those rich in proteoglycans. The goal of this study is to describe, through a case report and a systematic review of 24 publications between 1998 and 2018, the epidemiological profile, manifestations clinics and therapeutic management in RP. This report is a descriptive study, individual, observational and cross-sectional, based on data collected from medical records doctor of a patient with atypical epidemiology and clinical presentation refractory, with control of activity and disease progression only after use of biological agent. The review selected 24 viable articles based on the criteria of pre-defined inclusion and exclusion and descriptors equation ―Polychondritis Relapsing‖ AND ―Tocilizumab‖ in the PubMed database (Service of the United States National Library of Medicine) and, ―Polychondritis Relapsing‖ OR ―Tocilizumab‖ in LILACS (Scientific and Technical Literature of Latin America and the Caribbean), totaling 21 case reports, a series of cases and two cohorts. The sample varied according to the information available in each study. There was a predominance of females, white, with an average age of onset of the disease of 41.2 years and variable interval until the diagnosis, of less than a month to 25 years. Auricular involvement, observed in 96.7% of cases, corresponded to the most frequent set of clinical signs, followed by articular, in 86.7%, and nasal, in 56.7%. ANA-HEp2 (antinuclear factor of type II epithelial cells) and anti-collagen II were reactive in 42.8% and 20% of submitted to this analysis, respectively. Biopsy was performed in 15.8% of cases, mostly in the auricular region. Dyslipidemia was the most common comorbidity prevalent. Oral corticosteroid therapy was instituted as a first-line treatment in 90.5% of patients, alone or in association with DMARDS (antirheumatic drugs disease modifiers), with a failure rate of 57.1%. There was need for the use of biologicals in 28.5% of the reported cases. evolved with favorable clinical outcomes 80.9%. One death was recorded, attributed to poor therapeutic adherence. Of all the biological agents used in the patients of the case series and one of the cohorts, anti-TNF was the most used (78%). O tocilizumab stood out as a therapeutic alternative in refractory cases and with laryngotracheal involvement. The management of the reported case was compatible with the most studies and contributes to the collection of literature on the subject, since there are no evidence-based guidelines for the treatment of RP. Associated with systematic review confirms the importance of early diagnosis and new therapeutic alternatives.