Behcet’s disease: epidemiology, clinical manifestations, and diagnosis

Davatchi, Fereydoun; Chams‐Davatchi, Cheyda; Shams, Hormoz; Shahram, Farhad; Nadji, Abdolhadi; Akhlaghi, Maassoumeh; Faezi, Tahreh; Ghodsi, Zahra; Abdollahi, Bahar Sadeghi; Ashofteh, Farimah; Mohtasham, Nooshin; Kavosi, Hoda; Masoumi, Mariam

doi:10.1080/1744666x.2016.1205486

Cited by 259 publications

(214 citation statements)

References 43 publications

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“…Gastrointestinal, neurological, vascular, and articular manifestations are less commonly seen. In addition, manifestations are somehow different among age groups affected and among countries [120][121][122].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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Section: Presentation and Diagnosismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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“…The incidence of significant macrovascular involvement varies per study and is estimated to be 2.2%–50% 4. Deep vein thromboses are the most common manifestation, occurring in over 50% of cases, and arterial disease is least common 3.…”

Section: Introductionmentioning

confidence: 99%

Coeliac artery dissection as a rare manifestation of Behcet’s disease

Ocon¹,

Mehta

Peredo-Wende

2018

BMJ Case Reports

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Behcet's disease is a multisystemic vasculitis. Arterial involvement in the form of acute dissection is rare. A 42-year-old Lebanese man with Behcet's disease presented with severe abdominal pain. On exam, blood pressure was 162/104 mm Hg, and he exhibited epigastric tenderness. CT angiogram demonstrated an acute dissection of the coeliac artery trunk, common hepatic artery and proper hepatic arteries, with asymmetric thickening of the proximal left subclavian artery and circumferential thickening of the abdominal infrarenal aorta suggestive of vasculitis. Treatment included intravenous clevidipine, nitroprusside and methylprednisolone, which transitioned to oral metoprolol, amlodipine and prednisone. He responded well. Arterial dissections have been described with Behcet's. We report a coeliac artery aneurysm in association with a flare of Behcet's disease. Arterial wall inflammation combined with the sheering forces of hypertension likely predisposes to arterial dissection.

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“…Bechet's disease, named after Turkish dermatologist Hulusi Bechet, is a rare, multisystem small-vessel vasculitis that commonly affects the oral mucosa, skin, eyes, and GI tract, and it is associated with the HLA-B51 serotype [112]. With regard to the visual system, Bechet's disease is classically associated with retinal vasculitis and uveitis, but several case reports have noted an association with orbital inflammation and myositis [112][113][114][115][116].…”

Section: Bechet's Diseasementioning

confidence: 99%

“…With regard to the visual system, Bechet's disease is classically associated with retinal vasculitis and uveitis, but several case reports have noted an association with orbital inflammation and myositis [112][113][114][115][116].…”

Section: Bechet's Diseasementioning

confidence: 99%