A newsletter destaca a revisão do índice de dano (SDI) no Lúpus Eritematoso Sistêmico, que busca maior precisão clínica ao remover itens de atividade inflamatória e incluir critérios de gravidade. Além disso, apresenta novos guidelines da EULAR para vasculites e polimialgia reumática, e discute evidências recentes sobre terapias biológicas e inibidores de fosfodiesterase em doenças autoimunes.
A newsletter analisa um estudo populacional que revela alta prevalência de edema ósseo sacroilíaco em indivíduos saudáveis, alertando para a necessidade de contexto clínico no diagnóstico de espondiloartrites. Além disso, discute as divergências entre diretrizes globais para esclerose sistêmica e apresenta avanços em novas terapias biológicas para o lúpus.
A newsletter discute a descoberta de que a assinatura do interferon tipo 1 pode preceder o diagnóstico da Síndrome de Sjögren em até 14 anos, definindo um endotipo biológico específico. Além disso, aborda o aumento da detecção de miopatias necrosantes por estatinas e a integração de mecanismos inflamatórios na perda de massa óssea e muscular em pacientes obesos.
A newsletter discute a alta prevalência de manifestações cardíacas na Síndrome de Behçet, frequentemente subestimada e associada à atividade sistêmica da doença. O conteúdo também aborda a importância do rastreio de HPV em pacientes imunossuprimidos e apresenta a LBFSS, a primeira escala específica para mensurar a disfunção cognitiva no Lúpus.
Abstract Objectives Subglottic stenosis (SGS) is a challenging manifestation of granulomatosis with polyangiitis (GPA), often relapsing and poorly responsive to immunosuppressive treatment. Current guidelines lack specific recommendations for SGS management and evidence is limited. This study aimed to identify features of SGS associated with a more aggressive course and to assess the efficacy of available treatments in preventing relapses. Methods We conducted a multicenter, retrospective cohort study including GPA patients with SGS. Patients were stratified into higher relapsers (≥2 flares) and lower relapsers (≤1 flare). Clinical and treatment features were compared across groups. Univariate and multivariate analyses were conducted to identify independent predictors of relapse and multiple flares. Kaplan–Meier curves assessed time-to-relapse across regimens. Results Eighty-nine patients were included (30% male). Forty-eight patients (54%) were higher relapsers, with a median time-to-relapse of 36 months. Systemic immunosuppressive therapy was associated with fewer relapses (82% vs 18%, p = 0.04) compared to local treatments alone. Glucocorticoids in induction regimens reduced relapse risk (86% vs 12%, p = 0.03). Cyclophosphamide (CYC) was associated with the longest relapse-free survival and reduced 5-year relapse risk (OR 0.3, p = 0.049). By contrast, glucocorticoid monotherapy in either induction or maintenance phase was associated with higher relapse rate (p = 0.006). No specific maintenance regimen was significantly protective, though rituximab and DMARDs showed a trend toward improved outcomes. Conclusions Systemic immunosuppressive therapy, particularly CYC-based induction, was associated with fewer SGS relapses and prolonged relapse-free survival, while glucocorticoid monotherapy was less effective. Prospective studies are needed to optimize induction and maintenance strategies in GPA-related SGS.