Diffuse Alveolar Hemorrhage

Abstract

Background/Objective Diffuse alveolar hemorrhage (DAH) is an uncommon but potentially fatal complication in patients with systemic lupus erythematosus (SLE). Its prognosis and factors associated with mortality are not completely clear, although invasive mechanical ventilation (IMV), use of cyclophosphamide, a high Acute Physiology and Chronic Health Evaluation II score, and infections are associated with high mortality rates. We investigated clinical and immunologic characteristics and factors associated with mortality in a cohort of Latin American patients with SLE who developed DAH. Methods A medical records review study was conducted of patients with SLE who were admitted to the intensive care unit (ICU) with DAH between 2011 and 2018. Clinical, laboratory, and treatment variables were compared between survivors and nonsurvivors. Results A total of 17 patients with SLE presented with DAH during the study period, of whom 11 (64.70%) were women. The median age was 28 (19–38.5) years. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) on admission to the ICU was 15.94 ± 10.07. All patients received pulse methylprednisolone and therapeutic plasma exchange, and 13 (76. %) also received cyclophosphamide. During the hospital stay, 5 patients (29.41%) died. A high SLEDAI on admission, low albumin, and days of IMV and inotropic/vasoactive support were statistically significant in comparing nonsurvivors with survivors. Other scales of disease severity commonly used in the ICU, however, were not significantly associated with a fatal outcome. Conclusions Hypoalbuminemia, longer duration of IMV or inotropic/vasoactive treatment, and a high SLEDAI are potential prognostic factors for mortality in patients with SLE and DAH admitted to the ICU.

Systemic lupus erythematosus-related acute lung disease

“…Our series shares many similarities with previous cohort: very active SLE defines by high SLEDAI-2K scores, frequent renal, neuropsychiatric involvement, and use of cyclophosphamide. 6,[12][13][14][15] This cohort also brings new information on this rare manifestation. First, we show that SLE-related ALD is frequently inaugural of SLE.…”

Targeting Intra-Pulmonary P53-Dependent Long Non-Coding RNA Expression as a Therapeutic Intervention for Systemic Lupus Erythematosus-Associated Diffuse Alveolar Hemorrhage

“…Pulmonary capillaritis is the most common histopathological findings of DAH with neutrophilic infiltration of the capillaries, mediated by immune complexes (ICs) with abundant granular electron-dense materials and immunoglobulins (Igs)/C3 deposition along the alveolar and vascular walls [1,3]. Despite intensive respiratory support with mechanical ventilation or plus extra-corporeal membrane oxygenation, based on 10 recent large-scale series including our 17-case retrospective study (Table 1), SLE-associated DAH has a significant fatality with an average mortality rate around 40% [4][5][6][7][8][9][10][11][12][13]. These clinical observations have implicated a more effective control of underlying disease activity to avoid the DAH-related fatality.…”