scholarly journals Mimicking Behcet's disease: GM‐CSF gain of function mutation in a family suffering from a Behcet's disease‐like disorder marked by extreme pathergy

2020 ◽  
Author(s):  
Berenice Rösler ◽  
Bas Heinhuis ◽  
Xiaowen Wang ◽  
Ricardo Silvestre ◽  
Leo A.B. Joosten ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Gain Of Function ◽  
Behcet's Disease ◽  
Gm Csf

scholarly journals A case of Behcet's disease associated with myelodysplastic syndrome involving trisomy 8 and a gain-of-function mutation in SHP-2

Rheumatology ◽  
2011 ◽  
Vol 50 (7) ◽  
pp. 1342-1344 ◽  
Author(s):  
T. Shigemura ◽  
K. Agematsu ◽  
T. Yamazaki ◽  
K. Sakashita ◽  
Y. Nakayama ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Trisomy 8 ◽  
Gain Of Function ◽  
Behcet's Disease

scholarly journals Superoxide anion release by GM-CSF-primed neutrophils in response to thrombin-activated platelets: altered response of neutrophils from patients with Behcet's disease.

Ensho ◽  
1992 ◽  
Vol 12 (3) ◽  
pp. 239-243
Author(s):  
Atsushi Takahashi ◽  
Eiri Shibata ◽  
Kohkichi Yamamoto ◽  
Minoru Okuma ◽  
Takenosuke Yuasa ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Superoxide Anion ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease ◽  
Gm Csf ◽  
Activated Platelets ◽  
Altered Response

scholarly journals Alveolar proteinosis in Behçet’s disease

2010 ◽  
Vol 5 ◽  
Author(s):  
Cuneyt Tetikkurt ◽  
Seza Tetikkurt ◽  
Imran Ozdemir ◽  
Cigdem Zuhur ◽  
Nihal Bayar
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Pulmonary Alveolar Proteinosis ◽  
Behçet's Disease ◽  
Periodic Acid ◽  
Spontaneous Remission ◽  
Periodic Acid Schiff ◽  
Behcet's Disease ◽  
Gm Csf ◽  
Alveolar Proteinosis

A 51-year-old man with Behçet’s disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte- macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet’s disease with spontaneous remission.

Coagulation and Fibrinolytic Activity in Behçet's Disease

1991 ◽  
Vol 66 (03) ◽  
pp. 292-294 ◽  
Author(s):  
K K Hampton ◽  
M A Chamberlain ◽  
D K Menon ◽  
J A Davies
Keyword(s):  
Plasminogen Activator ◽  
Tissue Plasminogen Activator ◽  
Behçet’S Disease ◽  
Fibrinolytic Activity ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Plasminogen Activator Inhibitor ◽  
Behcet's Disease ◽  
Tissue Plasminogen ◽  
Von Willebrand

SummaryCoagulation and fibrinolytic activities were studied in 18 subjects with Behçet's disease and compared with results from 14 matched control patients suffering from sero-negative arthritis. Significantly higher plasma concentrations (median and range) were found in Behçet's patients for the following variables: fibrinogen 3.7 (1.7-6.9) vs 3.0 (2.0-5.1) g/1, p <0.05; von Willebrand factor antigen, 115 (72-344) vs 74 (60-119)%, p <0.002; plasminogen activator activity (106/ECLT2) 219 (94-329) vs 137 (78-197) units, p <0.002; tissue plasminogen activator inhibitor (t-PA-I) activity, 9.1 (5.5-19.3) vs 5.1 (1.8-12.0) IU/ml, p <0.002; and PAI-1 antigen, 13.9 (4.5-20.9) vs 6.4 (2.4-11.1) ng/ml, p <0.002. Protein C antigen was significantly lower: 97 (70-183) vs 126 (96-220)%, p <0.02. No differences were observed in antithrombin III activity or antigen, factor VIII coagulant activity, fibrinopeptides A and Bβ15-42, plasminogen, α-2-antiplasmin, functional and immunological tissue-plasminogen activator, thrombin-antithrombin complexes and D-dimer. Levels of tissue plasminogen activator inhibitor (activity and antigen) correlated with disease activity while fibrinogen and von Willebrand factor concentrations did not. Seven of the 18 subjects with Behçet's disease had suffered thrombotic events but it was not possible to distinguish these from the 11 patients without thrombosis using the assays performed. The results suggest the abnormal fibrinolytic activity in Behçet's disease is due to increased inhibition of tissue plasminogen activator. No abnormality of coagulation or fibrinolytic activity specific to Behçet's disease was detected.

Circulating Thrombomodulin as a Clue of Endothelial Damage in Behçet’s Disease

1996 ◽  
Vol 75 (06) ◽  
pp. 974-975 ◽  
Author(s):  
İbrahim C Haznedaroğlu ◽  
Oktay Özdemir ◽  
Osman Özcebe ◽  
Semra V Dündar ◽  
Şerafettin Kirazli
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Endothelial Damage ◽  
Behcet's Disease

Radiological evaluation of vasculo-Behcet's disease

1993 ◽  
Vol 29 (1) ◽  
pp. 62 ◽  
Author(s):  
Kyung Hwan Lee ◽  
Jae Hyung Park ◽  
Joon Ku Han ◽  
Hyun Ae Park ◽  
Jin Wook Chung ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Radiological Evaluation ◽  
Behcet's Disease

Subacute thyroiditis in association with psoriasis and behcet's disease

2018 ◽  
Author(s):  
Ferreira Paulo Carvalho ◽  
Bruno Maia ◽  
Antonio Marinho ◽  
Ana Veloso ◽  
Ivo Cunha ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Subacute Thyroiditis ◽  
Behcet's Disease
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