Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome.

Autor:	Blay G; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil.; Pediatric Pulmonology Unit, Children's Institute, FMUSP, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Rodrigues JC; Pediatric Pulmonology Unit, Children's Institute, FMUSP, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Ferreira JCO; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Leal GN; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Gormezano NW; Division of Rheumatology, FMUSP, Sao Paulo, Brazil., Novak GV; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Pereira RMR; Division of Rheumatology, FMUSP, Sao Paulo, Brazil., Terreri MT; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Sao Paulo, Brazil., Magalhães CS; São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Sao Paulo, Brazil., Molinari BC; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Sakamoto AP; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Sao Paulo, Brazil., Aikawa NE; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil.; Division of Rheumatology, FMUSP, Sao Paulo, Brazil., Campos LMA; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Fernandes TAP; São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Sao Paulo, Brazil., Clemente G; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Sao Paulo, Brazil., Peracchi OAB; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Sao Paulo, Brazil., Bugni V; Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Sao Paulo, Brazil., Marini R; São Paulo State University of Campinas (UNICAMP), Sao Paulo, Brazil., Sacchetti SB; Irmandade da Santa Casa de Misericórdia de São Paulo, Sao Paulo, Brazil., Carvalho LM; Ribeirão Preto Medical School - University of São Paulo, Sao Paulo, Brazil., Fraga MM; Hospital Darcy Vargas, Sao Paulo, Brazil., Castro TCM; Hospital Menino Jesus, Sao Paulo, Brazil., Ramos VC; Pontifical Catholic University of Sorocaba, Sao Paulo, Brazil., Bonfá E; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil., Silva CA; Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil. clovisaasilva@gmail.com.; Pediatric Pulmonology Unit, Children's Institute, FMUSP, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil. clovisaasilva@gmail.com.
Jazyk:	angličtina
Zdroj:	Advances in rheumatology (London, England) [Adv Rheumatol] 2018 Nov 23; Vol. 58 (1), pp. 39. Date of Electronic Publication: 2018 Nov 23.
DOI:	10.1186/s42358-018-0038-4
Abstrakt:	Objective: To 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. Methods: 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). Results: 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. Conclusions: 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.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu