EULAR 2018 update on management of Behcet’s syndrome

Behcet’s syndrome (BS) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and the gastrointestinal system.European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs has released its 2018 update of the EULAR recommendations for the management of Behcet’s syndrome.These Guidelines update the ones released by EULAR in 2008.They have been published in Annals of the Rheumatic Diseases.

Key recommendations -

1.Mucocutaneous involvement
• Topical measures such as steroids should be used for the treatment of oral and genital ulcers. Colchicine should be tried first for the prevention of recurrent mucocutaneous lesions especially when the dominant lesion is erythema nodosum or genital ulcer (Level of evidence: IB; strength of recommendation: A)

• Papulopustular or acne-like lesions are treated with topical or systemic measures as used in acne vulgaris. (Level of evidence: IV; strength of recommendation: D)

• Leg ulcers in BS might be caused by venous stasis or obliterative vasculitis. Treatment should be planned with the help of a dermatologist and vascular surgeon. (Level of evidence: IV; strength of recommendation: D)

• Drugs such as azathioprine, thalidomide, interferon-alpha, tumour necrosis factor-alpha inhibitors or apremilast should be considered in selected cases. (Level of evidence: IB; strength of recommendation: A)

2.Eye involvement
• Any patient with BS and inflammatory eye disease affecting the posterior segment should be on a treatment regime such as azathioprine (level of evidence: IB; strength of recommendation: A), cyclosporine-A (level of evidence: IB; strength of recommendation: A), interferon-alpha (level of evidence: IIA; strength of recommendation: B) or monoclonal anti-TNF antibodies (level of evidence: IIA; strength of recommendation: B)

• Systemic glucocorticoids should be used only in combination with azathioprine or other systemic immunosuppressives. (level of evidence: IIA; strength of recommendation: B)

• Patients presenting with an initial or recurrent episode of acute sight-threatening uveitis should be treated with high-dose glucocorticoids, infliximab or interferon-alpha. Intravitreal glucocorticoid injection is an option in patients with unilateral exacerbation as an adjunct to systemic treatment (Level of evidence: IIA; strength of recommendation: B)

3.Isolated anterior uveitis
• Systemic immunosuppressives could be considered for those with poor prognostic factors such as young age, male sex and early disease onset (Level of evidence: IV; strength of recommendation: D)

Acute deep vein thrombosis
• Glucocorticoids and immunosuppressives such as azathioprine, cyclophosphamide or cyclosporine-A are recommended. (Level of evidence: III; strength of recommendation: C)

4.Refractory venous thrombosis
• Monoclonal anti-TNF antibodies could be considered in refractory patients. Anticoagulants may be added, provided the risk of bleeding in general is low and coexistent pulmonary artery aneurysms are ruled out (Level of evidence: III; strength of recommendation: C)

5.Arterial involvement
• For the management of pulmonary artery aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended. Monoclonal anti-TNF antibodies should be considered in refractory cases. For patients who have or who are at high risk of major bleeding, embolisation should be preferred to open surgery. (Level of evidence: III; strength of recommendation: C)

• For both aortic and peripheral artery aneurysms, medical treatment with cyclophosphamide and corticosteroids is necessary before intervention to repair. Surgery or stenting should not be delayed if the patient is symptomatic. (Level of evidence: III; strength of recommendation: C)

6.Gastrointestinal involvement
• Gastrointestinal involvement of BS should be confirmed by endoscopy and/or imaging. NSAID ulcers, inflammatory bowel disease and infections such as tuberculosis should be ruled out (Level of evidence: III; strength of recommendation: C)

7.Refractory/severe gastrointestinal involvement
• Urgent surgical consultation is necessary in cases of perforation, major bleeding and obstruction. Glucocorticoids should be considered during acute exacerbations, together with disease-modifying agents such as 5-ASA or azathioprine. For severe and/or refractory patients, monoclonal anti-TNF antibodies and/or thalidomide should be considered (Level of evidence: III; strength of recommendation: C)

8.Nervous system involvement
• Acute attacks of parenchymal involvement should be treated with high-dose glucocorticoids followed by slow tapering, together with immunosuppressives such as azathioprine. Cyclosporine-A should be avoided. Monoclonal anti-TNF antibodies should be considered in severe disease as first line or in refractory patients (Level of evidence: III; strength of recommendation: C)

• The first episode of cerebral venous thrombosis should be treated with high-dose glucocorticoids followed by tapering. Anticoagulants may be added for a short duration. Screening is needed for vascular disease at an extracranial site (Level of evidence: III; strength of recommendation: C)

9.Joint involvement
• Colchicine should be the initial treatment in BS patients with acute arthritis. Acute monoarticular disease can be treated with intra-articular glucocorticoids. Azathioprine, interferon-alpha or tumour necrosis factor alpha inhibitors should be considered in recurrent and chronic cases (Level of evidence: IB; strength of recommendation: A)

For developing the Guidelines systematic search of the literature databases yielded 3927 articles. After reviewing the title and abstracts, 395 were selected for full-text evaluation and 11 additional articles were identified through hand search. Finally, 192 studies on the management of mucocutaneous, joint, eye, vascular, nervous system and gastrointestinal system involvement of BS were included.

For further reference log on to : http://dx.doi.org/10.1136/annrheumdis-2018-213225