Behçet's syndrome: Immune regulation, circulating immune complexes, neutrophil migration, and colchicine therapy

Jorizzo, Joseph L.; Hudson, Robert D.; Schmalstieg, Frank C.; Daniels, Jerry C.; Apisarnthanarax, Prapand; Henry, John C.; González, Emilio B.; Ichikawa, Yukinobu; Cavallo, T

doi:10.1016/s0190-9622(84)70024-7

Cited by 109 publications

(31 citation statements)

References 25 publications

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“…21 The presence of neutrophils in pathergic lesions of Behçet disease has been proposed as a reflection of altered neutrophilic function. 22 The increased activity of polymorphonuclear leukocytes has been suggested as the underlying cause of pathergy. 23 The clinical significance of this phenomenon, however, is still unknown.…”

Section: The Role Of Neutrophil Dysfunctionmentioning

confidence: 99%

Pathophysiology of pyoderma gangrenosum (PG): An updated review

Braswell

Kostopoulos

Ortega‐Loayza

2015

Journal of the American Academy of Dermatology

216

205

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Section: The Role Of Neutrophil Dysfunctionmentioning

confidence: 99%

Pathophysiology of pyoderma gangrenosum (PG): An updated review

Braswell

Kostopoulos

Ortega‐Loayza

2015

Journal of the American Academy of Dermatology

216

205

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“…BS generally presents in the third decade of life, with a slightly increased tendency to affect men more than women, 1.4:1, and with a more severe prognosis with younger age [2]. The diagnosis is classically based on the criteria set forth by the International Study Group in the year 1990, which include: oral aphthae, genital aphthae, ocular and cutaneaous lesions, and a positive pathergy test [3]. Ocular, vascular, and neurological diseases represent the major causes of morbidity and mortality in Behcet's syndrome [4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Vascular neurobehcet disease:correlation with current disease activity forum and systemic vascular involvement

et al. 2012

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Behcet's syndrome (BS) is a chronic relapsing vascular inflammatory disease of unknown etiology with high morbidity and mortality. This research aims to study the clinical patterns of CNS disease in a group of patients with BS as well as the frequency and type of the associated radiographic findings suggestive of structural cerebral vascular disease. The findings were studied in relation to disease activity and features of systemic vascular involvement. Forty patients fulfilling the diagnostic criteria of the International Study Group for Behcet's Disease, mean age of 33.56 ± 9.7 years, were enrolled. Patients were subjected to magnetic resonance imaging with conjugate survey of cerebral blood vessels' flow pattern abnormalities by transcranial Doppler study. Thirty healthy controls were included. Behcet's Disease Current Activity Form Score was used. Neuro-Behcet's syndrome (NBS) was diagnosed in 37.5% with headache being the most common (86.6% of cases), pyramidal affection (signs of upper motor neuron lesions/hemiplegia) was reported in 33.3%, attacks of disturbed conscious level in 26.6%, and cranial nerve affection in 6.5%. Of the patients, 66.6% with clinical features of NBS had statistically significant radiographic evidences of cerebrovascular disease (p = 0.01). Patients with NBS had significantly higher disease activity index score (r = 0.69, p = 0.0001). Radiographic findings and flow abnormalities were significantly less in patients on immune suppressants and antiplatelet drugs (p = 0.003, 0.04). BS patients with clinical neurologic disease were found to have radiographic findings suggestive of cerebral vascular disease with high disease activity index score. Drugs like immunosuppressants and oral antiplatelets might retard cerebral vascular disease progression and flow abnormalities, respectively.

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“…Also, histopathologically, complement mediated vasculitis or lymphocytic vasculitis is often observed [3,16,17]. Therefore, it can be presumed that a vascular damage can cause a tissue inflammation, and it may be involved in the etiological cause [1,5,6]. During an inflammation process, various inflammatory cells move to the surrounding tissues, and an attachment between vascular endothelial cells and inflammatory cells by adhesion molecules becomes the most essential.…”

Section: Discussionmentioning

confidence: 99%