Diffuse alveolar hemorrhage in systemic lupus erythematosus: risk factors and clinical outcome: results from affiliated hospitals of Catholic University of Korea

Abstract

This study was undertaken to investigate clinical characteristics of diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus (SLE) and to determine risk factors and clinical outcomes of DAH in SLE patients. Among the 1521 patients with SLE admitted between January 1993 and June 2009 to affiliated hospitals of Catholic University of Korea, 21 SLE were admitted for DAH. The inclusion criteria for DAH was defined as new infiltrates on chest radiographs, an acute hemoglobin drop of at least 1.5 g/dl in the absence of an obvious source of bleeding, and one or more of the following signs: hemoptysis, hypoxemia, bronchoscopic or biopsy evidence of DAH. Included as disease controls were 83 SLE patients, matched for age and sex, who were admitted for other manifestations. Data based on medical records were analyzed retrospectively. There were no significantly differing demographic characteristics between SLE patients with DAH and those with other manifestations. Multivariate analysis demonstrated coexisting neuropsychiatric lupus (p = 0.002) and high SLE disease activity index scores (SLEDAI > 10) as independent risk factors in the development of DAH (p = 0.029). Among the 21 SLE patients with DAH, 13 died during the admission period (in-hospital mortality rate: 61.9%). Mortality was associated with infection and requirements of mechanical ventilation. Collectively, SLE patients who have neuropsychiatric manifestations or are in the active stage of the disease have an increased risk for developing DAH. Due to the high mortality of SLE patients with DAH, early recognition of risk factors and appropriate intervention is essential.

Allogeneic transplantation of umbilical cord-derived mesenchymal stem cells for diffuse alveolar hemorrhage in systemic lupus erythematosus

“…Diffuse alveolar hemorrhage (DAH) is a rare pulmonary complication of systemic lupus erythematosus (SLE) with a frequency ranging from 0.52% to 5.7% [1][2][3][4]. Once the patients are diagnosed with DAH, it is usually life-threatening.…”

“…Once the patients are diagnosed with DAH, it is usually life-threatening. The mortality rate is over 50% [1,4,5]. Though the pathogenesis of DAH is not very clear yet, Hughson MD et al [6] suggested that it might be related to the immune complex deposition in alveolar wall and the induction of apoptosis in alveolar macrophages and other smaller cells.…”

Successful Early Rituximab Treatment in a Case of Systemic Lupus Erythematosus with Potentially Fatal Diffuse Alveolar Hemorrhage

“…The most common pulmonary manifestation is pleuritis, but the most devastating pulmonary complication is pulmonary hemorrhage with a mortality rate reported to be as high as 70-90% [4,5]. In most cases DAH develops in patients with established SLE, often in the setting of active other organ involvement, including nephritis or neuropsychiatric lupus [1,4,5]. In the current case, the patient presented with DAH as the first clinical manifestation of SLE without lupus nephritis or other organ involvement.…”

“…Diffuse alveolar hemorrhage (DAH) occurs in only 5.4% of systemic lupus erythematosus (SLE) patients, although its mortality rate reaches 61.9% [1]. In response, aggressive immunosuppressive therapies such as cyclophosphamide are commonly used.…”

Investigation of Lung Involvement in Connective Tissue Disorders

“…DAH is an uncommon pulmonary manifestation in SLE but bears an ominous prognosis [106] . DAH occurs mainly in the setting of a pulmonaryrenal syndrome and requires high-dose corticosteroids plus immunosuppressants to obtain remission [107] .…”