Kussmaul disease (kussmaul-maier disease; necrotizing angiitis; pan; periarteritis nodosa; polyarteritis nodosa)

General: Progressive process of vascular inflammation and necrosis, manifested by numerous nodules along the course of small- and medium-sized arteries; lesions are segmental in distribution, have a predilection for bifurcation and involve all but the pulmonary arteries; arteries in gastrointestinal tract, kidneys, and muscles are particularly affected; affects primarily males between ages 20 and 50 years.

Ocular: Retinal detachment; cotton-wool patches; polyarteritis nodosa lesion of arteries; pseudoretinitis pigmentosa; conjunctivitis; corneal ulcer; tenonitis; ptosis; exophthalmos; proptosis; uveitis; optic atrophy; cataract; scleritis; paralysis of extraocular muscles; neuroretinitis; anterior uveitis; macular star; peripheral ulcerative keratitis; retinal vasculitis; pseudotumor of the orbit; central retinal artery occlusion.

Clinical: Fever; myalgia; hypertension; gastrointestinal disorders; neuropathy; respiratory infection; weight loss; anginal pain; hemiplegia; convulsion; acute brain syndrome; skin lesions; diffuse erythema; purpura; urticaria; gangrene; tachycardia; pericarditis; aortitis; painful facial swelling; diplopia.

Akova YA, et al. Ocular presentation of polyarteritis nodosa. Clinical course and management with steroid and cytotoxic therapy. Ophthalmology 1993; 100: 1775-l781.

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

Kussmaul A, Maier R. Ueber Eine Bisher Nicht Beschriebene Eigenthumliche Artenener Krankung (Periarteritis Nodosa), die Mit Morbus Brightii und Rapid Fortschreitender Allgemeiner Muskellahumung Einhergeht. Dtsch Arch Klin Med 1866; 1:484-518.

Matsuda A, et al. A case of neuroretinitis associated with long-standing polyarteritis nodosa. Ophthalmologica 1994; 208:168-l71.

Solomon SM. Solomon JH. Bilateral central retinal artery occlusions in polyarteritis nodosa. Ann Ophthalmol 1978; 10:567-569.