Erdheim-Chester disease in Brazil

Marques, C.

doi:10.1136/ard.62.3.270

Cited by 4 publications

(1 citation statement)

References 27 publications

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“…Many other studies have shown similar results despite being different from the current study in study design and aim (8)(9) . The current study has shown a significantly higher serum anti-nucleosome antibody concentration in SLE patients compared to control group This increase in anti-nucleosome may reflect the extent of cell damage caused by SLE which explain its flare up shown in the current study as well , these result consistent with that revealed in studies of Ahemd Ali Abid and P Cairns where anti-nucleosome concentration was significantly higher in SLE patients compared to the control group (10) (11) . In simon study, a similar result has been also obtained.…”

Section: Discussionsupporting

confidence: 91%

Assessment of Systemic Lupus Erythematosus in Term of Serum Anti-nucleosome and Interleukin 12 Markers in Relation to Epstein–Barr virus and Cytomegalovirus

Ibrahem¹,

Abdulamir²

2020

IJFMT

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Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, more dominant in female, associated with high risk of life threating complication and increase morbidity and mortality. the exact cause of SLE is not yet known with many suggestions about a possible role of viral infection in the development of the disease. Epstein -Barr virus is the most commonly studied virus in an attempt to prove its presumed association with development of SLE.The objectives of this study are the measurement of the effect of CMV and EBV seropositivity in the auto immune status and severity of disease SLE measured by estimating the level of immunological markers anti-nucleosome and IL-12 with viral marker. Materials and Method:Forty newly diagnosed female with SLE were randomly selected from patient attended rheumatology clinic at medical city in Baghdad during the period between December 2018 to May 2019. In addition, with 40 apparently healthy females, as, a control groups. Both group were investigated for IL12, anti-nucleosome, EA, VCA, EBNA and CMV by ELISA and the results were statistically evaluated. Results:The studied viral markers in the current study which included EBV markers EA, VCA, EBNA, have shown significantly higher level compared to the control group. Similarly, anti-nucleosome and IL12 have shown significantly higher level compared to the control group. Conclusions:The possible defect in controlling viral infection and increased number of latent infected cell in SLE may enhance production of auto-antibodies

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Section: Discussionsupporting

confidence: 91%

Assessment of Systemic Lupus Erythematosus in Term of Serum Anti-nucleosome and Interleukin 12 Markers in Relation to Epstein–Barr virus and Cytomegalovirus

Ibrahem¹,

Abdulamir²

2020

IJFMT

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Cardiovascular Involvement, an Overlooked Feature of Erdheim-Chester Disease

Haroche¹,

Amoura²,

Dion³

et al. 2004

Medicine

240

220

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Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by spumous ("foamy") histiocytes. As of this writing, 178 cases have been reported. ECD is characterized by heterogeneous systemic manifestations. Bone pain is the most frequent symptom. About half of all patients have extraskeletal manifestations. Cardiovascular manifestations of ECD remain underestimated. We report 6 new cases of ECD associated with periaortic fibrosis. In 4 of these cases, the whole aorta had a "coated" aspect. A literature review revealed 66 cases of ECD with cardiovascular involvement. We therefore analyzed 72 ECD patients with cardiovascular involvement: 40 (55.6%) had periaortic "fibrosis," 32 (44.4%) had pericardial involvement, and 22 (30.6%) had myocardial involvement. Six had a right atrial tumor. Symptomatic valvular heart disease (3 aortic and 3 mitral regurgitations) was found in 6 patients. Nineteen patients (26.4%) had heart failure, leading to death in 8 cases. Six patients had renovascular hypertension related to perirenal artery stenosis. Data concerning follow-up were available for 58 (80.6%) patients. Of these, 35 (60.3%) patients died, confirming the severe prognosis of ECD. Cardiovascular complications were responsible for the death of 11 of the 35 patients (31.4%).

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