Von bekhterev-strumpell syndrome (marie-strumpell spondylitis; ankylosing spondylitis; pierre-marie syndrome; bekhterev disease; rheumatoid spondylitis) 1

General: Variant of rheumatoid arthritis; etiology unknown; autosomal dominant; male preponderance; onset at age 20 to 40 years; although genetic background determines susceptibility to uveitis, the disease pattern suggests the possibility of random environmental triggers unrelated to the course of the underlying rheumatologic disorder.

Ocular: Nongranulomatous anterior uveitis; optic nerve atrophy (occasionally); hypopyon; band keratopathy; spontaneous hyphema.

Clinical: Spondylitis of vertebra and sacroiliac joints; ankylosis; general arthralgia; kyphosis; scoliosis; displaced head and total rigidity of spine.

Amor B. Isolated uveitis-relationship with ankylosing spondylitis and Reiter's syndrome. Mod Probl Ophthalmol 1976; 16:225-227.

Edmunds L, et al. New light on uveitis on ankylosing spondylitis. J Rheumatic 1991; 18:50-52.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Klemperer I, et al. Spontaneous hyphema: an unusual complication of uveitis associated with ankylosing spondylitis. Ann Ophthalmol 1992; 24:177-l79.

Strumpell A. Bemerkung uber die Chronische Ankolysierende Ertzundung der Wirbelsaule und der Huftgelenke. Dtsch Z Nervenh 1897; 11:338.

Zervas J, et al. HLA-B27 frequency in Greek patients with acute anterior uveitis. Br J Ophthalmol 1977; 61:699-701.