Extracorporeal Membrane Oxygenation in Diffuse Alveolar Hemorrhage Secondary to Systemic Lupus Erythematosus

Pacheco, Christine; Charbonney, Emmanuel; Durand, Madéleine; Kolan, Christophe; Laskine, Mikhael

doi:10.14740/jocmr1685w

Cited by 21 publications

(18 citation statements)

References 13 publications

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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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Section: Dah Syndromesmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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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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“…ECMO can also be used as a rescue therapy for DAH patients with severe hypoxemia and PaO 2 /FiO 2 of <100 mmHg despite optimal ventilator settings [11]. There is concern that the ECMO circuit will need systemic anticoagulation to prevent thrombus formation.…”

Section: Supportive Care For Treatment Of Hypoxiamentioning

confidence: 99%

Diffuse Alveolar Hemorrhage

Desai¹,

Smith²,

Williams³

2020

Evidence-Based Critical Care

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A 43-year-old Hispanic male originally from Honduras presented to the emergency room with cough producing yellow sputum for 1 month, with recent change to blood tinged over the previous days. His past medical history was pertinent for hypertension and end stage renal disease requiring thrice weekly hemodialysis. He had missed dialysis for the previous week prior to presentation due to bad weather. He also reported fever, chills, and sore throat with the onset of hemoptysis. Of note, the patient had presented to another local hospital 2 months prior with similar symptoms of hemoptysis. He was evaluated by gastroenterology and otolaryngology and an endoscopy and laryngoscopy were performed. There were no obvious signs of bleeding but endoscopy did note blood emanating from the larynx. At presentation, his physical exam was notable for bilateral inspiratory rales with normal vital signs including oxygen saturation of 98% on room air.He was initially diagnosed with end-stage renal failure 18 months prior following a motor vehicle accident. The patient was uninjured but was noted to have a serum creatinine of 21 mg/dL. A renal ultrasound showed renal parenchymal disease attributed to long-standing hypertension. He was started on hemodialysis. The patient had lived in the United States for the previous 10 years with no recent travel or sick contacts. He denied any use of tobacco, alcohol, or illicit drug use. He previously worked as a roofer, but was currently unemployed due to his dialysis dependence.At the time of presentation, his laboratory data was notable for anemia with hemoglobin of 5.9 g/dL, Potassium 6.6 mmol/L, BNP 2092 pg/mL and serum creatinine 23 mg/ dL. A chest X-ray showed multifocal bilateral alveolar opacities (Figs. 33.1 and 33.2). The patient was initially treated with hemodialysis, broad spectrum antibiotics, and placed in respiratory isolation for evaluation of tuberculosis. The patient reported that his hemoptysis improved significantly following serial sessions of dialysis, yet chest imaging remained unchanged. Sputum culture, respiratory viral antigen panel, and AFB smears were negative. A bronchoscopy was performed 4 days after admission and revealed a progressively bloody bronchoalveolar lavage ( Fig. 33.3) consistent with a diagnosis of diffuse alveolar hemorrhage. Serologic analysis confirmed the diagnosis. QuestionWhat is the diagnosis?Answer Capillaritis with diffuse alveolar hemorrhage.

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Extracorporeal Membrane Oxygenation in Diffuse Alveolar Hemorrhage Secondary to Systemic Lupus Erythematosus

Cited by 21 publications

References 13 publications

Acute Respiratory Events in Connective Tissue Disorders

Acute Respiratory Events in Connective Tissue Disorders

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

Diffuse Alveolar Hemorrhage

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