Clinical Characteristics and Risk Factors of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: a Systematic Review and Meta-Analysis Based on Observational Studies

Xu, Ting; Zhang, Guangfeng; Lin, Haobo; Xie, Yuanliang; Feng, Yong; Zhang, Xiao; Dong, Guang‐Hui

doi:10.1007/s12016-019-08763-8

Cited by 15 publications

(7 citation statements)

References 19 publications

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“…Lupus is an autoimmune condition characterized by dysregulated adaptive and innate immune responses in tissues and the vasculature which can have damaging and potentially lethal effects on end organs such as the kidneys and lungs (195). Elshikha et al investigated the protective effects of A1AT therapy in a series of preclinical studies.…”

Section: Alpha-1-antitrypsin: a Promiscuous Serpin With Potent Therapeutic Propertiesmentioning

confidence: 99%

Fibrinolytic Serine Proteases, Therapeutic Serpins and Inflammation: Fire Dancers and Firestorms

Yaron

Zhang

Guo

et al. 2021

Front. Cardiovasc. Med.

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The making and breaking of clots orchestrated by the thrombotic and thrombolytic serine protease cascades are critical determinants of morbidity and mortality during infection and with vascular or tissue injury. Both the clot forming (thrombotic) and the clot dissolving (thrombolytic or fibrinolytic) cascades are composed of a highly sensitive and complex relationship of sequentially activated serine proteases and their regulatory inhibitors in the circulating blood. The proteases and inhibitors interact continuously throughout all branches of the cardiovascular system in the human body, representing one of the most abundant groups of proteins in the blood. There is an intricate interaction of the coagulation cascades with endothelial cell surface receptors lining the vascular tree, circulating immune cells, platelets and connective tissue encasing the arterial layers. Beyond their role in control of bleeding and clotting, the thrombotic and thrombolytic cascades initiate immune cell responses, representing a front line, “off-the-shelf” system for inducing inflammatory responses. These hemostatic pathways are one of the first response systems after injury with the fibrinolytic cascade being one of the earliest to evolve in primordial immune responses. An equally important contributor and parallel ancient component of these thrombotic and thrombolytic serine protease cascades are the serine protease inhibitors, termed serpins. Serpins are metastable suicide inhibitors with ubiquitous roles in coagulation and fibrinolysis as well as multiple central regulatory pathways throughout the body. Serpins are now known to also modulate the immune response, either via control of thrombotic and thrombolytic cascades or via direct effects on cellular phenotypes, among many other functions. Here we review the co-evolution of the thrombolytic cascade and the immune response in disease and in treatment. We will focus on the relevance of these recent advances in the context of the ongoing COVID-19 pandemic. SARS-CoV-2 is a “respiratory” coronavirus that causes extensive cardiovascular pathogenesis, with microthrombi throughout the vascular tree, resulting in severe and potentially fatal coagulopathies.

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Section: Alpha-1-antitrypsin: a Promiscuous Serpin With Potent Therapeutic Propertiesmentioning

confidence: 99%

Fibrinolytic Serine Proteases, Therapeutic Serpins and Inflammation: Fire Dancers and Firestorms

Yaron

Zhang

Guo

et al. 2021

Front. Cardiovasc. Med.

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“…9 It is reported that inflammatory cytokines may suppress maturation of red blood cell (RBC). 10,11 Therefore, it has been suggested that RBC-related parameters such as red blood cell distribution width (RDW) and hemoglobin (Hb) contents may be used as inflammatory indicators for predicting the severity of several autoimmune diseases including systemic lupus erythematosus, 12,13 primary Sjögren's syndrome, 14 and autoimmune hepatitis. 15 However, there are few studies that have systematically assessed the association of PLT, RBC, Hb, red blood cells-platelet ratio (RPR) and hemoglobin-platelet ratio (HPR) with the disease activity of RA patients.…”

Section: Introductionmentioning

confidence: 99%

Association Between Blood PLT and RBC Related Indices and Disease Activity in Patients with Rheumatoid Arthritis

Li¹,

Tao²,

Sun³

et al. 2022

IJGM

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Background: Platelet (PLT) and red blood cell (RBC) have been demonstrated to play a critical role in inflammatory processes. This study aimed to evaluate the association of blood PLT and RBC related parameters with the disease activity in rheumatoid arthritis (RA) patients, and also to investigate the role of these indices in differentiating among RA patients with different disease activity. Methods: Clinical data from RA patients were retrospectively analyzed. RA patients were divided into inactive group and active group according to DAS28-CRP. The relationship between blood PLT and RBC counts-related indices and DAS28-CRP was detected by Spearman correlation. ROC curve was used to assess the diagnostic value of these indices in differentiating active RA from inactive RA. Results: Active RA patients exhibited higher level of PLT counts but significantly lower levels of RBC counts, hemoglobin (Hb), red blood cells-platelet ratio (RPR) and hemoglobin-platelet ratio (HPR) compared with inactive RA. PLT counts were positively but RBC counts, Hb, RPR and HPR were negatively related with DAS28-CRP. Conclusion: Blood PLT and RBC related indices were significantly associated with RA disease activity. These indices may be used to distinguish active RA from inactive RA.

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“…Overall, our patients had very severe active disease with corresponding high SLEDAI score, consistent with pooled results from various studies. 14 There are several reports investigating clinical risk factors in the development of DAH. Kazzaz 12 found that previous thrombocytopaenia was the strongest predictor of DAH; however, only a small proportion of patients had platelet count of less than <100,000/microlitre, negating the hypothesis that bleeding diathesis was caused by thrombocytopaenia per se.…”

Section: Discussionmentioning

confidence: 99%

Diffuse alveolar haemorrhage in systemic lupus erythematosus: A multicentre retrospective study in Singapore

Law

Chuah

Lee

et al. 2023

Lupus

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Objective Diffuse alveolar haemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). We describe the clinical characteristics, treatment and survival outcomes of SLE patients with DAH in Singapore. Methods We conducted a retrospective review of the medical records of SLE patients with DAH hospitalised in 3 tertiary hospitals between January 2007 and October 2017. Patient demographics, clinical characteristics, laboratory, radiologic and bronchoscopic findings, as well as the treatments, were compared between survivors and non-survivors. Survival rates were analysed between the various treatment groups. Results A total of 35 patients with DAH were included in this study. Majority of them were female (71.4%) and of Chinese ethnicity (62.9%). Median age was 40.0 years (IQR: 25–54), with a median disease duration of 8.9 months (IQR: 0.13–102.4). Haemoptysis was the most common clinical presentation, and majority had concomitant cytopaenia and lupus nephritis. All patients received high dose glucocorticoids; 27 (77.1%), 16 (45.7%) and 23 (65.7%) received cyclophosphamide (CYP), rituximab (RTX), and plasmapheresis (PLEX), respectively. Twenty-two patients required mechanical ventilation with a median duration of 12 days. Overall mortality rate was 40%, with a median survival time of 162 days. Twenty-six patients (74.3%) achieved remission, with an overall median time to remission of 12 days (IQR: 6–46) after diagnosis of DAH. Patients on triple therapy (CYP, RTX and PLEX) had a median survival of 162 days as compared to 14 days in patients on PLEX alone ( p = .0026). Conclusions The overall mortality of DAH in SLE patients remained high. There were no significant differences in patient demographics or clinical characteristics between the survivors and non-survivors. However, better survival appears to be associated with treatment with cyclophosphamide.

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Clinical Characteristics and Risk Factors of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: a Systematic Review and Meta-Analysis Based on Observational Studies

Cited by 15 publications

References 19 publications

Fibrinolytic Serine Proteases, Therapeutic Serpins and Inflammation: Fire Dancers and Firestorms

Fibrinolytic Serine Proteases, Therapeutic Serpins and Inflammation: Fire Dancers and Firestorms

Association Between Blood PLT and RBC Related Indices and Disease Activity in Patients with Rheumatoid Arthritis

Diffuse alveolar haemorrhage in systemic lupus erythematosus: A multicentre retrospective study in Singapore

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