Endomyocardial fibrosis in Behcet's disease

Huong, D. Lê Thi; Wechsler, B.; Papo, Thomas; Zuttere, Dominique de; Blëtry, O.; Hernigou, A.; Delcourt, A; Godeau, P; Piette, Jean‐Charles

doi:10.1136/ard.56.3.205

Cited by 98 publications

(43 citation statements)

References 11 publications

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“…It has been suggested that immunoactive cells such as T cells infiltrate into the affected regions with activation of circulating T and B lymphocytes, followed by chemotaxis of neutrophils or vice versa [16, 17, 18]. The histologic features are characterized by inflammatory processes in the arteries and veins and thrombosis as a result of vasculitis of the vasa vasorum with thickening of the media [16, 17, 19, 20]. The intermittent nature of the disease and the lack of consistent response to therapy make the underlying etiology difficult to define.…”

Section: Discussionmentioning

confidence: 99%

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Evereklioğlu

Yürekli²,

et al. 2000

Dermatology

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Background: Behçet’s disease (BD) is a chronic systemic inflammatory disorder affecting multiple organs with a generalized vasculitis of arteries and veins. Endothelial dysfunction is one of the prominent features of BD. Adrenomedullin (AM) is a peptide produced not only in normal adrenal medulla but also in the vascular smooth muscle cells and endothelial cells, and its role in the course of BD has not been previously described. Objective: To detect changes of plasma AM concentrations in patients with BD compared with age- and sex-matched healthy subjects by using high-performance liquid chromatography (HPCL). We also investigated if disease activity or the duration of BD correlates with AM levels. Methods: Forty-two consecutive patients with BD (38.5 ± 11.1 years, 19 male and 23 female) and 20 healthy age- and sex-matched control subjects (39.5 ± 10.9 years, 8 male and 12 female) were included in this study. We measured plasma AM levels by HPCL, and acute-phase reactants including α₁-antitrypsin and α₂-macroglobulin, neutrophil count and the erythrocyte sedimentation rate. Results: Mean ± SD plasma AM levels in patients with BD (73.22 ± 25.55 pmol/l) were significantly higher (p < 0.001) than in healthy control volunteers (21.35 ± 12.37 pmol/l). Patients with active BD had similar plasma AM concentrations (79.32 ± 21.89 pmol/l) with patients with inactive disease (67.44 ± 29.92 pmol/l). On the other hand, patients with longer duration of the disease (mean duration, 13.9 ± 3.8 years) had significantly higher plasma AM levels (83.99 ± 19.71 pmol/l; p = 0.005) than patients (62.45 ± 26.57 pmol/l) with shorter duration of the disease (mean duration, 5.5 ± 2.3 years). All acute-phase reaction parameters were found to be significantly increased in the active disease. Conclusion: Considering its endothelial cell implications, AM may be involved in reparatory vessel endothelium mechanisms, especially in the chronic disease.

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Section: Discussionmentioning

confidence: 99%

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Evereklioğlu

Yürekli²,

et al. 2000

Dermatology

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“…Infl ammation of small vessels is a common fi nding, and the medial layer of arteries is usually involved [24]. Involvement of arteries and arterioles owing to vasculitis with narrowing of the vascular lumen by focal fi brinoid deposition and fi broelastic proliferation is determined in BD, affecting the intramyocardial or small coronary arteries, and leading to microcirculation abnormalities and giving rise to myocardial ischemia, and endomyocardial fi brosis [12,25]. The pathologic mechanism of microvascular thrombus formation in vasculitis is believed to be endothelial cell ischemia or dysfunction that leads to coagulation [26].…”

Section: Discussionmentioning

confidence: 99%

Evaluation of myocardial perfusion and function by gated SPECT in patients with Behçet’s disease

et al. 2008

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“…It includes left ventricular dysfunction, conduction system disturbances, myocardial infarction, endocarditis, pericarditis, pericardial effusion, myocarditis, valvular regurgitation and endomyocardial fibrosis [2]. Endomyocardial fibrosis may result from endocarditis or myocarditis, and is usually associated with intracavitary thrombus formation [5]. In most cases endomyocardial fibrosis is incidentally discovered during routine echocardiogram or at surgery.…”

Section: Commentsmentioning

confidence: 98%

Misdiagnosis of Behçet’s disease with unknown protracted fever and chill after surgical excision of cardiac tumor

Zou

Liu

et al. 2010

Rheumatol Int

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Behçet's disease involving the heart is extremely rare. Sometimes it is probable to misdiagnose as infective endocarditis when protracted fever and chill occurs. We report a case of protracted pyrexia of unknown origin after surgical excision of a cardiac tumour. Clinically and pathologically a diagnosis of infective endocarditis was probable but antibiotics had no effect. After case review the diagnosis of Behçet's disease was established and the patient was treated with glucocorticoids which resulted in resolution.

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Endomyocardial fibrosis in Behcet's disease

Cited by 98 publications

References 11 publications

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Evaluation of myocardial perfusion and function by gated SPECT in patients with Behçet’s disease

Misdiagnosis of Behçet’s disease with unknown protracted fever and chill after surgical excision of cardiac tumor

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