Nejm 1999;341:1284; 1990;322:326

Cause:Vasculitis

Some combination of environmental and genetic factors; genetic ones associated (27%) with HLA B51 and, to a lesser extent, B27; may be precipitated by H. simplex, and parvovirus B19. Highest prevalence along ancient silk route w highest rates in Turkey and Turkish descendants. Male/female = 1.7:1 in eastern Mediterranean type, 1/2 in US at Mayo Clinic

Sx:Onset in 20s-30s. Blurred vision, photophobia, eye pain and tearing; painful oral and/or genital lesions

Si:Recurrent

• Oral aphthous ulcerations, deep, painful, numerous, may scar
• Genital ulcers: scrotal, prepuce, or glans; labial or cervical; usually painful but not always
• Uveitis, red eye; hypopyon, anterior as well as retinal scarring
• Erythema nodosum, acneiform lesions, superficial thrombophlebitis, and “delayed hypersensitivity reaction” to saline or any shot

Each attack lasts 1-4 wk. CNS disease has bad prognosis

• Blindness (Bull Rheum Dis 1985;35[5]:1), rx often titrated therefore to the uveitis
• Cardiovascular: cardiac (Ann IM 1983;98:639), DVT, aneurysms
• Inflammatory mono- or polyarthritis with polys >200 000
• Neurologic: aseptic meningitis, and MS-like syndromes in 5-10% (Nejm 2009;360:2341)
• Colitis

Lab:

Hem:ESR elevated

Serol:Increased acute phase reactants

Skin tests:Positive delayed hypersensitivity reaction to a saline shot is diagnostic

Rx:

Anti-inflammatory meds like ASA, indomethacin, pentoxifylline (Trental) 300 mg po bid (Ann IM 1996;124:891), colchicine, or thalidomide (Thalomid) 100 mg po qd (Ann IM 1998;128:443); cmplc: teratogenicity in pregnant women, polyneuropathy

Steroids, high dose, often w azathioprine (Nejm 1990;322:281), cyclophosphamide, chlorambucil, TNF blockers