Request pdf hemorragia alveolar difusa en pacientes con lupus eritematoso sistemico. Manifestations include pleuritis, pulmonary hypertension, and interstitial lung disease 1. It mostly occurs in patients with established diagnosis of sle but can be the initial presentation of lupus in approximately 20%. It can occur in a vast number of clinical situations and can be lifethreatening. Systemic lupus erythematosus complicated by diffuse. Diffuse alveolar hemorrhage in lupus nephritis complicated. The case of a 16yearold africanamerican girl with systemic lupus erythematosus, who developed diffuse alveolar hemorrhage with fatal consequences, is described. Recognition of dah often requires bal as symptoms are nonspecific, hemoptysis is absent in up to onethird of patients, and radiographic imaging is also nonspecific and similar to other acute alveolar filling processes.
We describe a case of a 74yearold woman who presented with dah as an initial presentation of sle. Alveolar hemorrhage in systemic lupus erythematosus. Successful rituximab therapy in a lupus patient with diffuse alveolar haemorrhage. Diffuse alveolar hemorrhage dah is a subset of diffuse pulmonary hemorrhage when bleeding is diffuse and directly into the alveolar spaces. Diffuse alveolar hemorrhage is a rare but serious complication of systemic lupus. Pulmonary alveolar hemorrhage in systemic lupus erythematosus. Gonzalezechavarri c, pernas b, ugarte a, ruizirastorza g. Diffuse alveolar hemorrhage dah is often a catastrophic clinical syndrome causing respiratory failure. Treatment of diffuse alveolar hemorrhage secondary to. Hemorragia alveolar associada a nefrite lupica alveolar.
The role of cell death, complement, immunoglobulin, tolllike receptors, and myeloid cells was examined in pris. Severe multiorganic flare of systemic lupus erythematosus successfully treated with rituximab and cyclophosphamide avoiding high doses of. Pathogenesis of diffuse alveolar hemorrhage in murine lupus. Diffuse alveolar hemorrhage in the systemic lupus erythematosus. It can be acute, developing over hours, or subacute, emerging over several days.
Hemorragia alveolar difusa en pacientes con lupus eritematoso. The use of recombinant factor viia rfviia to treat the acute phase of dah by endobronchial bronchoscopy has been shown to have a significant clinical impact on the survival and evolution of these. Dah originates from the pulmonary microcirculation, including the alveolar capillaries, arterioles, and venules and is usually diffuse, but may also be. Examination showed pericardial effusions on the echocardiogram and bilateral alveolar condensations on the thoracic radiograph. Manifestaciones clinicas, tratamiento y pronostico diffuse alveolar hemorrhage dah in patients with. Although frequently unrecognized, lung disease occurs in half of patients with systemic lupus erythematosus sle. This disruption is caused by injury or inflammation of the arterioles, venules, or alveolar septal alveolar wall or interstitial capillaries.
Compromiso pulmonar del lupus eritematoso sistemico. Lupus eritematoso sistemico julia garciaconsuegra molina 9 manifestaciones clinicas sindrome constitucional. It occurs in three distinct but overlapping phenotypes, acute capillaritis, bland pulmonary hemorrhage, and diffuse alveolar damage, each of which is. Diffuse alveolar hemorrhage dah in patients with systemic lupus erythematosus is a rare but potentially fatal condition. Alveolar hemorrhage leading to respiratory failure is uncommon. Hemorragia alveolar difusa disturbios pulmonares manuais. A hypothetical diagnosis of systemic lupus erythematosus was made.
Apr 30, 20 diffuse alveolar hemorrhage dah is a lifethreatening condition caused by a variety of disorders associated with hemoptysis, anemia, diffuse lung infiltration, and acute respiratory failure. Diffuse alveolar hemorrhage dah is a serious pulmonary complication in patients with autoimmune diseases who are undergoing chemotherapy or have had hematopoietic stem cell transplantation. Treatment of diffuse alveolar hemorrhage secondary to lupus. Dah is more common in women, and mean age of onset is around 30 years.
Pleuropulmonary complication of sle that ranges from pleuritic pain to catastrophic alveolar hemorrhage can occur during the course of illness in 50% to 70% of affected individuals. Diffuse alveolar hemorrhage dah is a rare but fatal complication in patients with systemic lupus erythematosus sle. C57bl6 mice with pristaneinduced lupus develop dah closely resembling the human disease. Various etiologies have been reported, including systemic lupus erythematosus, which generally presents as pulmonaryrenal syndrome. Pdf pulmonary alveolar hemorrhage in systemic lupus. Pulmonary hemorrhage as a manifestation of systemic lupus. It can affect individuals with systemic lupus erythematosus, and early. Protocolo clinico e diretrizes terapeuticas lupus eritematoso.
Diffuse alveolar haemorrhage dah is considered a catastrophic complication of systemic lupus erythematosus sle. Diffuse alveolar hemorrhage is a rare but serious pulmonary manifestation of systemic lupus erythematosus that may occur at some point during the course of disease. Alveolar hemorrhage secondary to lupus erythematosus treated with recombinant activated factor vii. She also had microscopic polyangiitis clinically manifesting as crescentic glomerulonephritis and purpura with positive myeloperoxidase mpoantineutrophil. Bleeding into the alveolar spaces of the lungs characterizes the syndrome of diffuse alveolar hemorrhage dah and is due to disruption of the alveolar capillary basement membrane. A hemorragia alveolar associada ao les e bastante rara, acometendo apenas 2% dos pacientes lupicos, e indica mau prognostico, com mortalidade variando entre 70 e 90%. Diffuse alveolar hemorrhage dah is a lifethreatening condition caused by a variety of disorders associated with hemoptysis, anemia, diffuse lung infiltration, and acute respiratory failure.
Hemorragia alveolar secundaria a lupus eritematoso generalizado, tratada con factor vii recombinante activado. Aug 27, 2014 hemorragia alveolar secundaria a lupus eritematoso generalizado, tratada con factor vii recombinante activado. Manifestaciones mucocutaneas exantema malar lupus pernio fotosensibilidad livedo reticularis lupus discoide purpura lupus subagudo alopecia eritema palmar paniculitis eritema generalizado vasculitis. Hemorragia alveolar associada a nefrite lupica scielo. Diffuse alveolar hemorrhage neumonia lupus eritematoso. Hemorragia alveolar, lupus eritematoso sistemico, tiroiditis autoinmune, enfermedad autoinmune. It is believed that the pathogenesis of microangiopathy is related to deposits of immune complexes that lead to activation of cellular apoptosis. Among the rheumatologic diseases, ah most frequently occurs in patients with sle and the systemic vasculitides. Diffuse alveolar hemorrhage dah is a rare lifethreatening complication in systemic lupus erythematosus sle associated with high mortality rates. The use of recombinant factor viia rfviia to treat the acute phase of dah by endobronchial bronchoscopy has been shown to have a significant clinical impact on the survival and. Hemoptisis masiva y hemorragia alveolar linkedin slideshare. Alveolar hemorrhage is a severe clinical disease with high rate of mortality. Disfuncion respiratoria cronica por hemorragia alveolar.
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