Steroid-responsive retinal vasculitis with a frosted branch appearance in Crohns disease.
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22 patients suffering from Birdshot retinochoroidopathy (10), idiopathic vasculitis (6) and Behcet disease (6) and treated by cyclosporine were tested at regular intervals during 11 to 38 months by the lymphocyte stimulation test (LST) and basophil degranulation test (HBDT) with S retinal antigen. These two tests were found frequently positive especially during acute inflammation for the LST. However HBDT was positive in all the patients before relapse of acute inflammation which is in favor of its predictive value and its involvement in the triggering of inflammation at the basophiles or at the local mast cell level.
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We found distinct alterations of the clotting and fibrinolytic mechanisms in a 27-year-old man who suffered from typical bilateral vasculitis, clinically and fluoroangiographically manifest as obstruction of the central retinal veins. The partial thromboplastin time was slightly increased, a partial clot lysis occurred after one hour, the plasminogen level was markedly low, and alpha2-macroglobulin was decreased, as were factor XII and IgG. Kallikrein was absent. Similar changes, though not so pronounced, were also found in the patient's son and in his brother.
A 23-year-old single female patient developed constitutional manifestations in the form of fever, weight loss, anorexia, malaise, fatigue, and generalized aches in January 1995, 2 weeks after an attack of German measles. This was followed by painful, reddish, macular skin lesions over both legs which healed by dark pigmentation (leucocytoclastic vasculitis), mononeuritis multiplex, and Raynaud's phenomena of both hands and feet. Angiography of lower limbs was done to visualize the arterial tree of both lower limbs and revealed typical beading of distal arterial branches, a diagnosis compatible with polyarteritis nodosa (PAN). At that time, the patient received prednisone (45 mg/day) and azatioprin (100 mg/day) and responded well to treatment. In a second presentation in June 2005, the patient developed sudden attack of loss of vision in her left eye. Ophthalmological examination of the patient revealed evidence of left central retinal artery occlusion, ischemic optic neuropathy. The patient received methyl prednisolone, 1 g IV infusion, daily infusion for three consecutive days followed by oral prednisolone, 30 mg/day. The patient received pulse cyclophosphamide IV infusion (0.6 g/m2) on the fourth day. One week after receiving therapy, the patient progressed from having light perception to counting of fingers from a distance of 1 m.
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