Treatment of diffuse alveolar hemorrhage secondary to lupus erythematosus with recombinant activated factor VII administered with a jet nebulizer

Esper, Raúl Carrillo; Estrada, Isis Espinoza de los Monteros; León, Teresa de la Torre; Gutiérrez, Agustín Omar Rosales; López, Jorge Arturo Nava

doi:10.1186/s40560-014-0047-2

Cited by 17 publications

(15 citation statements)

References 49 publications

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“…1 Involvement of the respiratory tract occurs in up to 50-70% of the SLE patients and include pleuritis, infiltrating pneumonia, bronchiolitis obliterans, muscular and diaphragmatic involvement and vascular aberrations such as pulmonary hypertension, antiphospholipid syndrome (APS) and diffuse alveolar hemorrhage (DAH). 2,3 Defect phagocytosis, ICs, complement depletion and auto-antibodies are responsible for these respiratory tract manifestations. ICs inducing inflammation in the alveolar capillaries might be the leading cause for DAH.…”

Section: Systemic Lupus Erythematosus Impacts On the Respiratory Tractmentioning

confidence: 99%

Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies

Al-Adhoubi

Byström

2020

Lupus

113

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Diffuse alveolar hemorrhage is a severe respiratory complication of systemic lupus erythematosus. The illness develops over hours to a few days and is the systemic lupus erythematosus-associated syndrome with highest mortality. Although no specific symptoms have been identified, a number of features are associated with diffuse alveolar hemorrhage, with a drop in blood hemoglobin the most prominent. Dyspnea, blood-stained sputum, diffuse infiltrates identified by chest imaging, elevated single breath-diffusing capacity for monoxide, thrombocytopenia and C3 hypocomplementemia are other commonly reported signs of diffuse alveolar hemorrhage. The etiology is not completely understood but many patients develop diffuse alveolar hemorrhage concomitant with lupus nephritis, suggesting immune complex-driven pathology. Biopsy studies have identified both cases with capillaritis and a bland non-inflammatory phenotype. An animal model of diffuse alveolar hemorrhage has indicated requirement of B lymphocytes and complement receptor-mediated apoptotic body phagocytosis by monocytes as part of the pathogenesis. This review will discuss considerations when diagnosing the condition and available therapies. Infections and other causes of hemorrhage have to be excluded as these require different treatment strategies. Methylprednisolone and cyclophosphamide remain the most commonly used therapies. Plasmapheresis and rituximab are other beneficial treatment options. A few studies have also considered intrapulmonary Factor VII therapy, extracorporeal membrane oxygenation and mesenchymal stem cell therapy. There is an unmet need of better definition of diffuse alveolar hemorrhages etiology and pathology for development of improved treatment strategies.

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Section: Systemic Lupus Erythematosus Impacts On the Respiratory Tractmentioning

confidence: 99%

Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies

Al-Adhoubi

Byström

2020

Lupus

113

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“…Treatment consists of the administration of pulses of steroids and cyclophosphamide as early as possible [87]. In the past years, other therapeutic options have emerged, such as plasmapheresis, the administration of intravenous immunoglobulins, rituximab, mycophenolate mofetil, recombinant factor VII, and others, permitting a more individualized treatment according to the immunological status and general condition of the patient [88,89,90,91,92,93,94]. …”

Section: Dah Syndromesmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the ‘mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the ‘vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders.

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“… 13 As with most organ-threatening manifestations of SLE, cyclophosphamide has been the mainstay of therapy for DAH, 1 14 albeit in association with poor outcomes, which probably reflect its preferential use in the most critically ill patients. 1 Other reported therapies include plasmapheresis, 19 20 extracorporeal membrane oxygenation (ECMO), 21 22 rituximab, 23–30 mycophenolate mofetil, 31 recombinant factor VII 32 33 and stem cell transplantation. 34 The evidence to support any particular therapy is not strong.…”

Section: Introductionmentioning

confidence: 99%

Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival

et al. 2015

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ObjectivesWhile diffuse alveolar haemorrhage (DAH) is recognised as a life-threatening complication of systemic lupus erythematosus (SLE), little is known about its risk factors and response to treatment. We describe 22 cases of DAH in a US lupus cohort of approximately 1000 patients, and compare them to 66 controls from the same outpatient cohort.MethodsWe captured variables pertaining to diagnoses of SLE and secondary antiphospholipid syndrome (APS), and analysed them by univariate testing. Those variables with p values <0.05 were then further considered in a multivariate model. Kaplan-Meier curves were constructed for each group, and survival was analysed by Log-rank test.ResultsOf the 22 patients with DAH, 59% were diagnosed with DAH within 5 years of lupus diagnosis. By univariate testing, several manifestations of SLE and APS were more common in patients with DAH, including history of thrombocytopenia, cardiac valve disease, low C3, leucopenia, neuropsychiatric features, haemolysis, arterial thrombosis, lupus anticoagulant, secondary APS and low C4. On multivariate analysis, history of thrombocytopenia and low C3 were maintained as independent risk factors. Importantly, only two patients had platelet counts <50 000/µL at the time of the DAH episode, arguing that DAH was not simply a haemorrhagic complication of thrombocytopenia. All patients were treated with increased immunosuppression, including various combinations of corticosteroids, plasmapheresis, cyclophosphamide, rituximab and mycophenolate mofetil. Notably, all patients in the cohort survived their initial episode of DAH. While the patients with DAH did well in the short-term, their long-term survival was significantly worse than controls. Several of the deaths were attributable to thrombotic complications after recovering from DAH.ConclusionsTo the best of our knowledge, this is the largest case–control study of lupus DAH to date. History of thrombocytopenia was strongly predictive of DAH (OR ∼40). A number of APS manifestations correlated with DAH by univariate analysis, and deserve further consideration in larger studies.

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Treatment of diffuse alveolar hemorrhage secondary to lupus erythematosus with recombinant activated factor VII administered with a jet nebulizer

Cited by 17 publications

References 49 publications

Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies

Systemic lupus erythematosus and diffuse alveolar hemorrhage, etiology and novel treatment strategies

Acute Respiratory Events in Connective Tissue Disorders

Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival

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