Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

Table 1

Brief summary of the main clinical manifestations of Behçet’s disease.

Organ involvements	Clinical manifestations	Recommended treatment
Mucocutaneous	Oral aphthae, genital ulcers, pseudofolliculitis, papulopustular lesions, erythema nodosum-like lesions, and pathergy reaction	Colchicine, azathioprine, interferon-α, and TNF-α antagonist
Eye disease	Recurrent bilateral uveitis (anterior segment, posterior segment, or both), retinal vasculitis, retinal vein occlusion, and optic neuritis	Azathioprine, local or systemic corticosteroids, cyclosporine, infliximab (in combination with azathioprine and corticosteroids), and interferon-α.
Gastrointestinal tract	Anorexia, vomiting, dyspepsia, diarrhea, abdominal pain, ulcers, ischemic perforation, thrombosis in the terminal ileum, ileocecal region, and colon	Sulfasalazine, corticosteroids, azathioprine, TNF-α antagonista, and thalidomide. In emergency surgical procedures are required such as ileocolectomy or hemicolectomy
Musculoskeletal system	Nonerosive arthritis, nondeforming oligoarthritis, back pain, and sacroiliitis	Colchicine, interferon-α, azathioprine, and TNF-α antagonists
Cardiovascular system	Vasculitis, superficial thrombophlebitis, deep vein thrombosis, dural sinus thrombosis, occlusion of suprahepatic veins, pericarditis, myocarditis, endocarditis, intracardiac thrombosis, coronary vasculitis, and ventricular aneurysm	Corticosteroids, azathioprine, cyclosporine, and cyclophosphamide
Central nervous system	Severe headache and pyramidal and extrapyramidal symptom (seizures, hemiplegia, and cranial nerve palsies); Central nervous system: focal necrotic cerebral lesions, vascular thrombosis, arterial vasculitis, and aseptic meningoencephalitis; Peripheral nervous system: peripheral neuropathies and multiplex mononeuritis	Corticosteroids, interferon-α, azathioprine, cyclophosphamide, methotrexate, and TNF-α antagonists.