Produção Nacional
Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome
2018; BioMed Central; Volume: 58; Issue: 1 Linguagem: Inglês
10.1186/s42358-018-0038-4
ISSN2523-3106
AutoresGabriela Blay, Joaquim Carlos Rodrigues, Juliana Caires de Oliveira Achili Ferreira, Gabriela Nunes Leal, Natali W.S. Gormezano, Gláucia V. Novak, Rosa Maria Rodrigues Pereira, Maria Teresa Terreri, Cláudia Saad Magalhães, B. Molinari, Ana Paula Sakamoto, Nádia Emi Aikawa, Lúcia Maria Arruda Campos, Taciana de Albuquerque Pedrosa Fernandes, Gleice Clemente, Octávio Augusto Bedin Peracchi, Vanessa Bugni, Roberto Marini, Silvana Sacchetti, Luciana Martins de Carvalho, Melissa Mariti Fraga, Tânia Caroline Monteiro de Castro, Valéria Cristina Santucci Ramos, Eloísa Bonfá, Clóvis A. Silva,
Tópico(s)Platelet Disorders and Treatments
ResumoTo evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity.DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022).DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1-151) vs. 4 (1-151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5-40) vs. 6 (0-44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients.This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.
Referência(s)