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Pulmonary hemorrhage and lupus

Wei Huang, Jiyuan Wu, Huiqin Yang, Yin Xiong, Rui Jiang, Tianpen Cui, Duyun Ye
Abnormal features of the systemic lupus erythematosus (SLE)-derived neutrophils, promoted aberrant immune response, have inspired new studies of the induction of autoimmunity and the development of organ damage in SLE. In this study, we explore the effect of milk fat globule-EGF factor 8 (MFG-E8) on the aberrant nitrification features in pristane-induced lupus. SLE patients and mice with pristane-induced lupus develop autoantibodies associated with MFG-E8 overproduction. However, the deletion of MFG-E8 leads to uncontrolled early pulmonary and peritoneal inflammation and tissue damage in mice with pristane-induced lupus...
October 21, 2016: Cell Death and Differentiation
Chetan Basavaraj Patil, Ramakant Dixit, Rakesh Gupta, Neeraj Gupta, Varna Indushekar
BACKGROUND: Medical thoracoscopy is a minimally invasive procedure used in diagnostic and therapeutic applications for pleural diseases. In this study, we describe our experience in the outcome and analysis of thoracoscopy in undiagnosed pleural effusion presenting to our center. MATERIALS AND METHODS: This is a prospective study conducted over last 2 years. We performed thoracoscopy in 129 cases of undiagnosed exudative pleural effusions using rigid thoracoscope...
September 2016: Lung India: Official Organ of Indian Chest Society
Saimun Singla, Debra L Canter, Timothy J Vece, Eyal Muscal, Marietta DeGuzman
BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a devastating clinical syndrome characterized by a falling hematocrit, respiratory insufficiency, and radiographic evidence of pulmonary infiltrates. Literature regarding management of DAH in childhood-onset SLE (cSLE) is limited. METHODS: We reviewed the presentation, management, and outcome of DAH in a pediatric tertiary medical center with one of the largest cSLE cohorts in North America. During a 10 year period 7 of 410 children with cSLE had DAH...
August 2016: Hospital Pediatrics
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Manabu Uematsu, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Hiromasa Ohira
Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney...
2016: Tohoku Journal of Experimental Medicine
Diana Murro, Jorge Novo, Leonidas Arvanitis
Classic cerebral toxoplasmosis typically presents with neurologic symptoms such as seizures and mental status changes and histological examination shows focal lesions with necrosis. However, in the diffuse "encephalitic" form, patients are asymptomatic with diffuse, inflammatory, non-necrotic lesions. Asymptomatic diffuse "encephalitic" toxoplasmosis has been reported only in four acquired immunodeficiency syndrome patients and one human immunodeficiency virus (HIV) negative patient with chronic lymphocytic leukemia...
July 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Takuma Isshiki, Keishi Sugino, Kyoko Gocho, Kenta Furuya, Hiroshige Shimizu, Muneyuki Sekiya, Takanori Ohata, Tomohiro Wada, Kazutoshi Isobe, Susumu Sakamoto, Yujiro Takai, Sakae Homma
Antiphospholipid syndrome (APS) is clinically characterized by arterial or venous thrombosis; however, non-thromboembolic lung manifestations, such as diffuse alveolar hemorrhage (DAH), have also been previously reported. DAH is relatively common in APS patients with systemic lupus erythematosus, although it is rare in primary APS. We encountered a 78-year-old man who presented with hemoptysis and dyspnea. Chest CT showed diffuse ground-glass opacity with pulmonary thromboembolism. He was successfully treated with corticosteroids and heparin; however, DAH recurred after the corticosteroid treatment was stopped...
2015: Internal Medicine
Srinivas Rajagopala, Baburao Kanthamani Pramod Sagar, Molly Mary Thabah, B H Srinivas, Ramanathan Venkateswaran, Sreejith Parameswaran
BACKGROUND: The etiology of patients presenting with pulmonary-renal syndrome (PRS) to Intensive Care Units (ICUs) in India is not previously reported. AIMS: The aim was to describe the prevalence, etiology, clinical manifestations, and outcomes of PRS in an Indian ICU and identify variables that differentiate immunologic causes of PRS from tropical syndromes presenting with PRS. MATERIALS AND METHODS: We conducted a prospective observational study of all patients presenting with PRS over 1-year...
June 2015: Indian Journal of Critical Care Medicine
Erika P Plata-Menchaca, V M De la Puente-Diaz de Leon, Adriana G Peña-Romero, Eduardo Rivero-Sigarroa
Introduction. Pulmonary hemorrhage secondary to disseminated strongyloidiasis is an unusual, well-recognized entity in immunocompromised patients with autoimmune disease, which is associated with the hyperinfection syndrome, sepsis, and a high mortality rate. Case Presentation. We present a case of a 44-year-old Mexican woman with systemic lupus erythematosus and acute bacterial meningitis who developed pulmonary hemorrhage with acute respiratory failure requiring mechanical ventilation, treated with broad spectrum systemic antibiotics and high dose methylprednisolone, who subsequently developed a characteristic purpuric skin eruption and septic shock and died two days later of refractory hypoxemia caused by massive pulmonary bleeding...
2015: Case Reports in Critical Care
Emily Elizabeth Bowen, Robert Hangartner, Iain Macdougall
Common causes of pulmonary-renal syndrome include anti-glomerular basement membrane (anti-GBM) disease anti-neutrophil cytoplasmic antibody (ANCA) positive vasculitis, and systemic lupus erythematosus. We describe a case of life-threatening pulmonary hemorrhage associated with Campylobacter hemolytic uremic syndrome (HUS), which we believe is a new disease entity. We hypothesize that the cause of this pulmonary-renal syndrome was an immunological reaction to Campylobacter; and that the initiation of high-dose steroids was responsible for the rapid reversal of the patient's pulmonary and renal impairment...
March 2016: Journal of General Internal Medicine
Dharmendra Bhadauria, Praveen Etta, Anupma Kaul, Narayan Prasad
BACKGROUND: Concurrent thrombotic and hemorrhagic manifestations are uncommon in patients with Antiphospholipid Syndrome. CASE CHARACTERISTICS: A 10-year-old girl with fever, edema, rash and joint pains, who later developed deep venous thrombosis (DVT), stroke, thrombotic microangiopathic hemolytic anemia and pulmonary hemorrhage. Investigations confirmed Antiphospholipid syndrome associated with systemic lupus erythematosus. OUTCOME: She went into complete remission with intravenous immunoglobulins, plasmapheresis, immunosuppression and anticoagulation...
April 2015: Indian Pediatrics
Suzhi Li, Yuliang Wang, Xuewen Huang, Jingxin Cao, Dingzhou Yang
UNLABELLED: A 26-year-old woman presented with dyspnea and dry cough soon after arriving on the Qinghai-Tibet Plateau (3650 m). Chest radiograph showed diffuse patchy infiltrates. The initial diagnosis was high altitude pulmonary edema (HAPE). However, the patient had no sputum or moist rales, and supplemental oxygen and intravenous aminophylline produced no improvement. Chest HRCT revealed symmetric and diffuse ground glass opacities. Further examination found anemia, leukopenia, urine abnormalities, and increased erythrocyte sedimentation rate...
March 2015: High Altitude Medicine & Biology
Andreas Kronbichler, Renate Frank, Michael Kirschfink, Ágnes Szilágyi, Dorottya Csuka, Zoltán Prohászka, Peter Schratzberger, Karl Lhotta, Gert Mayer
Catastrophic antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with antiphospholipid syndrome (APS) with a high morbidity and mortality.We describe a case of a 30-year old female patient with immunoglobulin A (IgA) deficiency who underwent splenectomy because of idiopathic thrombocytopenic thrombocytopenia. Subsequently, an APS and finally systemic lupus erythematosus was diagnosed. After an uncomplicated pregnancy that was terminated by cesarean section, the patient developed severe CAPS with cerebral, myocardial, renal, and pulmonary involvement...
November 2014: Medicine (Baltimore)
Pablo Finucci Curi, Matilde Pierrestegui, Alberto Ortiz, Federico Ceccato, Sergio Paira
BACKGROUND AND OBJECTIVE: Pulmonary hemorrhage (PH) in systemic lupus erythematosus (SLE) is a rare but potentially fatal complication that occurs most frequently in the context of active lupus with involvement of other organs. The objective of this study is to report the clinical features and prognosis of patients with SLE who had PH. METHODS: Patients with SLE (1982 American College of Rheumatology criteria) and PH under monitoring between June 1999 and November 2011 were studied...
November 6, 2015: Medicina Clínica
David Kurahara, Marina Morie, Maya Yamane, Sarah Lam, Wallace Matthews, Keolamau Yee, Kara Yamamoto
We describe a possible association between pulmonary hemosiderosis (PH) and a history of bronchopulmonary dysplasia (BPD). Both patients were born at 28-week gestation and presented with PH at ages 22 months and 6 years, respectively. Both initially presented with cough and tachypnea, and bronchoalveolar lavage showed evidence of hemosiderin-laden macrophages. Initial hemoglobin levels were < 4 g/dL and chest radiographs showed diffuse infiltrates that cleared dramatically within days after initiation of intravenous corticosteroids...
2014: Case Reports in Pediatrics
Yiqin Shi, Naotake Tsuboi, Kazuhiro Furuhashi, Qiuna Du, Asuka Horinouchi, Kayaho Maeda, Tomoki Kosugi, Seiichi Matsuo, Shoichi Maruyama
Diffuse pulmonary hemorrhage (DPH) is an uncommon but critical complication of systemic lupus erythematosus. Peritoneal administration of 2,6,10,14-tetramethylpentadecane (pristane) can recapitulate a lupus-like syndrome in mice, which can develop into DPH within a few weeks, especially in C57BL/6 mice. Mac-1 (CD11b/CD18), a leukocyte adhesion molecule, is known to play a role in inflammation by regulating migration of leukocytes into injured tissue. In this study, we aimed to clarify the role of Mac-1 in pristane-induced DPH, using Mac-1(-/-) and wild-type (WT) mice on a C57BL/6 background...
November 15, 2014: Journal of Immunology: Official Journal of the American Association of Immunologists
Iehab B Alabed
Diffuse alveolar hemorrhage (DAH) is a rare serious life-threatening complication in systemic lupus erythematosus (SLE) associated with a high mortality rate. The old standard treatment options include high-dose corticosteroids, cyclophosphamide, and plasmapheresis, which are unspecific, treating the underlying disease rather than the complication itself, and not effective. We report a case of DAH complicating SLE flare-up in a female patient treated with recombinant activated factor VII (rFVIIa) administered via the bronchoscope that showed clinical and radiological improvement...
September 2014: Medicine (Baltimore)
Fernando Gonzalez-Ibarra, Parag Chevli, Lindsey Schachter, Maninder Kaur, Sahar Eivaz-Mohammadi, Basheer Tashtoush, Jioty Matta, Amer K Syed, Valentin Marian
The presence of Strongyloides stercoralis infection in patients with systemic lupus erythematosus (SLE) has been described previously. Strongyloides stercoralis hyperinfection syndrome (SHS) that usually develops in patients under immunosuppressive therapy may affect a variety of organs, but the presentation with diffuse alveolar hemorrhage (DAH) is rare with only a few cases described in the literature. We present the case of a 36-year-old Hispanic female with a past medical history relevant for SLE and a recent diagnosis of lupus nephritis and hypertension...
2014: Case Reports in Medicine
L Barile-Fabris, M F Hernández-Cabrera, J A Barragan-Garfias
Systemic lupus erythematosus (SLE) is a complex heterogeneous autoimmune disease with a wide variety of clinical and serological manifestations that may affect any organ. Vasculitis prevalence in SLE is reported to be between 11% and 36%. A diverse clinical spectrum, due to inflammatory involvement of vessels of all sizes, is present. Even though cutaneous lesions, representing small vessel involvement, are the most frequent, medium and large vessel vasculitis may present with visceral affection, with life-threatening manifestations such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex, with detrimental consequences...
2014: Current Rheumatology Reports
Ji Soo Kim, Min Jae Kim, E Young Bae, Dae Chul Jeong
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids...
April 2014: Korean Journal of Pediatrics
Francisco Hugo Gomes, Luciana Carvalho, Paola Pinheiro, Tamara Resende, Virgínia Ferriani
BACKGROUND/PURPOSE: Antiphospholipid syndrome (APS) is a multisystem autoimmune disease characterized by arterial and/or venous thrombosis and persistent presence of antiphospholipid antibodies. Long term follow-up studies in children are still scarce. The aim of this study was to evaluate clinical data and long-term outcome of patients with confirmed antiphospholipid syndrome in childhood. METHODS: Between 1993 and 2013, 36 patients with confirmed pediatric antiphospholipid syndrome (22 female; aged 8 months-18 years, mean 9 years) were followed at the Pediatric Rheumatology Unit of the University Hospital of USP-RP...
March 2014: Arthritis & Rheumatology
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