Mycophenolate mofetil as a maintenance therapy for lupus-related diffuse alveolar hemorrhage: a case report

Rashidi, Abdulrahman Al; Alajmi, M; Hegazi, Mohamed Osama

doi:10.1177/0961203311411353

Cited by 15 publications

(10 citation statements)

References 11 publications

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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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Section: Introductionmentioning

confidence: 99%

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

et al. 2015

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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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“…Given that proliferation of both T and B lymphocytes is critically dependent on de novo purine synthesis, evidence supporting the use of antimetabolites such as azathioprine and MMF has emerged as an alternative therapeutic approach in the management of DAH, although antimetabolites are mostly limited to maintenance therapy once remission has been achieved (24–26). Based on the immunomodulatory properties of both intravenous immunoglobulin (IVIG) and mesenchymal stem cells, investigators have applied these therapies to manage different autoimmune diseases and their respective complications, including DAH (27).…”

Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus–associated Diffuse Alveolar Hemorrhage: A Case Report and Review of the Literature

Morales‐Nebreda

Alakija

Ferguson

et al. 2018

Clinical Pulmonary Medicine

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Systemic lupus erythematosus is associated with numerous pleuropulmonary complications. Although uncommon, diffuse alveolar hemorrhage represents a life-threatening cause of acute respiratory failure among patients with lupus. Here, we present a 24-year-old woman with a history of lupus who developed hemoptysis and respiratory failure associated with diffuse radiographic infiltrates and anemia. Bronchoscopy confirmed diffuse alveolar hemorrhage. She was managed with supportive care, plasmapheresis, and immunosuppressive pharmacotherapy leading to sustained resolution of her pulmonary hemorrhage and respiratory failure. We then review the available literature on the pathophysiology and management of lupus-associated diffuse alveolar hemorrhage, which centers on supportive care, reversal of coagulopathy, and immunosuppressive measures. SUMMARY FOR TABLE OF CONTENTS A 24-year-old woman with a history of systemic lupus erythematosus presented with hemoptysis, diffuse radiographic infiltrates, anemia, and respiratory failure; bronchoscopy confirmed diffuse alveolar hemorrhage. Her condition was managed with supportive care, plasmapheresis, and immunosuppression with glucocorticoids, cyclophosphamide, and mycophenolate mofetil. Diffuse alveolar hemorrhage represents an uncommon but life-threatening complication of lupus with a growing evidence base to support acute and chronic management strategies centered on immunosuppressive pharmacotherapy.

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Mycophenolate mofetil as a maintenance therapy for lupus-related diffuse alveolar hemorrhage: a case report

Cited by 15 publications

References 11 publications

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

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

Acute Respiratory Events in Connective Tissue Disorders

Systemic Lupus Erythematosus–associated Diffuse Alveolar Hemorrhage: A Case Report and Review of the Literature

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