Relapsing polychondritis (jaksch wartenhost syndrome; meyenburg-altherz-vehlinger syndrome; von meyenberg ii syndrome)

General: Episodic, yet generally progressive; onset usually in middle life; possibly caused by lysosomal labilizing factor of endogenous or exogenous toxic nature or immunologic reactions; possible association with Reiter syndrome.

Ocular: Conjunctivitis; corneal ulcer; exophthalmos; panophthalmitis; phthisis bulbi; proptosis; optic neuritis; papilledema; retinal detachment; blue sclera; episcleritis; scleromalacia; vitreous opacity; cataracts; nystagmus; retinal artery thrombosis; keratoconjunctivitis sicca; secondary glaucoma; scotoma; uveitis; paresis of third or sixth nerve; conjunctival mass (salmon patch); chorioretinitis.

Clinical: Destruction of cartilage and eventual replacement with connective tissue; polyarthritis; chondritis; tracheal collapse; bronchial collapse; anemia; liver dysfunction; death; malaise; fever; dyspnea; changes in pitch of voice; hearing impairment; vertigo; deformed ears; aortic valve insufficiency.

Arkin CR, Masi AT. Relapsing polychondritis: review of current status and case report. Semin Arthritis Rheum 1975; 5:41-62.

Brink H, et al. Ocular manifestations of relapsing polychondritis. Three case histories. Doc Ophthalmol 1994; 87:159-l66.

Crovato F, et al. Exophthalmos in relapsing polychondritis. Arch Dermatol 1980; 116:383-384.

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

Isaak BL, et al. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986; 93:681-689.

McKay DAR, et al. Relapsing polychondritis and eye disease. Br J Ophthalmol 1974; 58:600-605.

Silva J, et al. Relapsing polychondritis and Reiter's syndrome. J Rheumatol 1991; 18:908-910.

Tucker SM, et al. Relapsing polychondritis, another cause for a 'salmon patch.' Ann Ophthalmol 1993; 25:389-391.