Researchers have found that diffuse alveolar hemorrhage (DAH) in childhood-onset systemic lupus erythematosus (cSLE) is associated with serious disease flare, and is complicated by sepsis and high mortality rate. These findings were presented at the 2017 Annual European Congress of Rheumatology Meeting (EULAR) held June 14-17, 2017 in Madrid, Spain.
The small representation of DAH in previous case series and the focus on adult SLE has thus far prevented an accurate analysis of the associated factors and outcomes in patients with and without this serious complication. To gain a better understanding of DAH in cSLE, Dr C.A. Silva from the pediatric rheumatology divisions of the Brazilian Childhood-onset Systemic Lupus Erythematosus Group at Sao Paulo State University in Brazil and colleagues conducted a multicenter cohort study including 847 patients with cSLE with and without diffuse DAH. They evaluated the prevalence, clinical manifestations, laboratory abnormalities, and treatment of this complication, as well as accompanying severity factors.
DAH was defined as the presence of at least 3 respiratory symptoms associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography (CT), as well as a sudden drop in hemoglobin levels with no other source of bleeding.
DAH was found in 19 of the 847 patients (2.2%). In all patients with cSLE with DAH, cough, dyspnea, tachycardia, and hypoxemia occurred.
Associated factors of severity included: mechanical ventilation in 14/19 patients (74%), hemoptysis in 12/19 patients (63%), macrophage activation syndrome in 2/19 patients (10%), and death in 9/19 patients (47%).
Patients with cSLE at diagnosis of DAH compared with the 76 patients with cSLE without DAH showed that the patients with DAH had higher frequencies of constitutional involvement (74% vs 10%, P <.0001), serositis (63% vs 6%, P <.0001), and sepsis (53% vs 9%, P <.0001).
The median disease activity score (SLEDAI-2K) was also significantly higher in patients with cSLE with DAH [18 (5-40) vs 6 (0-44), P <.0001].
Rates of thrombocytopenia (53% vs 12%, P <.0001), intravenous methylprednisolone (95% vs 16%, P <.0001) and intravenous cyclophosphamide (47% vs 8%, P <.0001) were also significantly higher in patients with DAH compared with patients with cSLE without DAH.
In this largest study to date to evaluate DAH, the researchers found that “this complication, although not a disease activity score descriptor, occurs in the context of significant moderate/severe cSLE flare.”
“Importantly, we identified that this condition is associated with serious disease flare complicated by sepsis and with [a] high mortality rate,” they concluded.
This study was supported by research grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 301805/2013-0 to RMRP, 303752/2015-7 to MTT, 301479/2015-1 to CSM, 305068/2014-8 to EB and 303422/2015-7 to CAS), Federico Foundation (to EB, RMRP and CAS) and by Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS.
Silva CA, Blay G, Rodrigues JC, et al. Diffuse alveolar hemorrhage: a multicenter study in 847 childhood-onset systemic lupus erythematosus patients. Presented at: The Annual European Congress of Rheumatology Meeting (EULAR). June 14-17, 2017. doi: 10.1136/annrheumdis-2017-eular.4826