British Society Guidelines for Managing Behçet’s Disease Save

An exhaustive, full read guideline from the British Association of Dermatologists (BAD) and the British Society for Rheumatology (BSR) on the management and treatment of Behçet’s disease (BD) in 2024 has been published in Rheumatology.

This guideline provides up-to-date, evidence-based recommendations for the management of Behçets disease/syndrome, affecting adults, children and young people. This paper updates therapy with recent therapeutic advances in disease management, including management of extraocular and extraarticular manifestaions of BD.

When possible, multiple medical specialties are needed to best manage BD, a rare disorder that may be difficult to diagnose and manage.

Corticosteroids (CS) are important in the control of flares or to determine if longer-term immunosuppression may be beneficial. CS may be used as initial adjunctive therapy with the initiation of an immunosuppressant (e.g. azathioprine) until therapeutic efficiency is reached. Yet there should be appropriate concern about the safety of long-term CS, thus, it is advisable to use the lowest dose of CS required, for the shortest time.

The treatment of gastrointestinal (GI) Behçet’s should be based on the reduction of GI symptoms by healing the ulcers, both macroscopically and microscopically, to reduce their associated complications.

Ocular disease in Behçet’s may be sight threatening and, if untreated, may result in complete loss of vision within 4 years in up to 90% of patients. While systemic or intravitreal CS are effective in managing sight-threatening eye disease, longer-term disease-modifying antirheumatic drugs and biologics should be considered.

Serological markers for inflammation are often not raised during BD flares, yet to endothelial vascular inflammation may increase the risk of arterial aneurysm and venous thrombosis. 

Women of childbearing age with Behçet’s should be advised to seek pre-conception counselling from a multidisciplinary team. BD may improve symptoms during pregnancy, while only 15–30% will experience a flare, usually, oral or genital ulceration, that can be treated symptomatically.

Behçet’s is very rare in children and young people up to the age of 16 years, with mucocutaneous disease as the most common finding. Other organ involvement is less common than in adults (ocular 17.9%, neurological 3.6% and vascular 5.4%). Over 83% of children and young people had three or more specialists.

This guideline has over 80 concensus recommendations. The following are some highlights from these two societies:

• R1 If Behçet’s is suspected, convene a multidisciplinary team (MDT) involving core specialties and any other relevant specialties (i.e. dermatology, oral medicine, ophthalmology, gastroenterology, rheumatology and clinical psychology, with neurology and vascular surgery input, where appropriate) 
• R2 Consider the criteria of the 1990 International Study Group (ISG) or the International Criteria for Behcet’s Disease (ICBD) when diagnosing suspected Behçets.
• R4 Assess at each visit, the extent of organ involvement and target therapy accordingly.
• R6 Assess the disease impact, by employing a quality-of-life assessment tool.
• R7 If using an anti-tumour necrosis factor therapy, consider an antimetabolite (e.g. methotrexate or azathioprine) to prevent or delay production of antidrug antibodies.
• R8 Do not offer* thalidomide to people with Behçets
• R9 BD with severe oral aphthous-like ulcers - advise simple oral hygiene measures, having regular dental appointments and the use of topical antiseptics (e.g. chlorhexidine mouthwash) and the avoidance of irritants such as sodium lauryl sulfate in toothpaste
• R11 Be aware that paradoxical Behçets has been reported in association with interleukin-17 inhibitors.
• R14  Offer potent topical corticosteroids to treat oral and genital ulcers in people with Behçets if the symptoms are mild, or as adjunctive therapy with systemic immunosuppression for more severe disease. 
• R15 Consider the triple mouthwash^§ for oral ulceration in people with Behçets.
  • ^§The triple mouthwash: betamethasone 500 micrograms soluble tablets + doxycycline 100 mg + 1 mL nystatin oral suspension.
• R17 Offer colchicine as a second-line option to treat mucocutaneous lesions in people with Behçets
• R18, R19: For Mucocutaneous lesions: 3rd line therapies include azathioprine or mycophenolate mofetil or anti-tumour necrosis factor therapy 
  • R20  Consider apremilast as a fourth-line option to treat mucocutaneous lesions in adults with Behçets 
  • R21 Consider secukinumab as a fourth-line option to treat mucocutaneous lesions 
  • R22 Consider dapsone as a treatment option in people with Behçets.
• Arthritis/arthralgia Treatment options
  • R23 colchicine as a first-line option to treat arthritis/arthralgia 
  • R25 nonsteroidal anti-inflammatory drugs (NSAIDs) as an alternative 
  • R27 azathioprine or anti-tumour necrosis factor therapy as options to treat persistent and refractory arthritis/arthralgia 
  • R28 consider apremilast, methotrexate, mycophenolate mofetil or azathioprine or secukinumab as options to treat arthritis/arthralgia 
• Gastrointestinal Treatment options
  • R32 oral/intravenous corticosteroids as a first-line for acute BD flares 
  • R33 Taper systemic corticosteroids on commencing 5-aminosalicylic acid, azathioprine or 6-mercaptopurine 
  • R34 Offer* 5-aminosalicylic acid as a first-line option to treat flares 
  • R35 Offer azathioprine or 6-mercaptopurine as a first- or second-line option to people with gastrointestinal Behçets 
  • R36 Consider anti-tumour necrosis factor therapy early in the management approach
  • R42 Consider thalidomide as a last resort in people with gastrointestinal Behçets refractory to other treatments
• Ocular Treatment options
  • R43 oral/intravenous corticosteroids as a first- line option to treat sight-threatening eye disease in people with ocular Behçets. Topical corticosteroids may be coprescribed. 
    • Sight-threatening eye disease may be defined as one or more of the following features:
      • clinically significant vitritis
      • retinal infiltrate
      • retinal vascular occlusion
      • optic disc swelling
      • macular oedema.
  • R44 Offer a steroid-sparing agent (e.g. azathioprine, methotrexate or a calcineurin inhibitor) along with oral/intravenous corticosteroids for sight-threatening eye disease or
    • R45 Offer anti-tumour necrosis factor therapy as a second-line option to treat sight-threatening eye disease 
    • R46 Offer interferon-α-2a (if available) to treat sight-threatening eye disease in people with ocular Behçets.
    • R47 Offer tocilizumab to people with ocular Behçets who have not responded to the above
    • R48 Only consider chlorambucil and cyclophosphamide as options to treat sight-threatening eye disease in people with ocular Behçets when biologic therapy has failed.
• Investigations 
  • R53 serological markers for inflammation (i.e. erythrocyte sedimentation rate and C-reactive protein) might be normal in people with suspected neuro-Behçets.
  • R54 Perform magnetic resonance imaging including contrast, and magnetic resonance venography in people with suspected neuro-Behçets to investigate parenchymal disease and cerebral venous thrombosis
  • R55 Offer cerebrospinal fluid (CSF) examination in people with suspected neuro-Behçets to establish the diagnosis and exclusion of other pathologies. Note neuro-Behçets is usually associated with CSF pleocytosis, raised CSF protein and absence of oligoclonal bands; nonparenchymal neuro-Behçets is associated with elevated CSF pressure. However, a completely normal CSF does not exclude parenchymal neuro-Behçets.
  • R56 Consider monitoring interleukin (IL)-6 cytokine serum and cerebrospinal fluid (CSF) levels in people with suspected neuro-Behçets. Elevated CSF IL-6 levels are an indicator of ongoing disease activity.

The remainder of this lengthy article reviews treatment of BD complications, such as: neurological, obstetric, vascular, Pulmonary arterial aneurysm, deep vein thrombosis and psychological complications of BD.