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Progression into clinical remission in systemic sclerosis

OBJECTIVE: to report four patients with systemic sclerosis who underwent complete clinical remission, with no need of current drug treatment, after using D-Penicillamine. METHODS: chart review of patients diagnosed with systemic sclerosis (ARA, 1980) who were in remission after continuous ambulatory care. Data were collected on sex, race, and patient's age at the time of diagnosis, first manifestations, systemic involvement, laboratory profile and image tests, proposed treatment, drug response and full treatment period. RESULTS: patients were white, 1 male and 3 females, with an average age of 51.2 years at the time of the first symptoms. The average period of time from symptoms beginning to the diagnosis was 8.2 months. All patients showed skin alterations, systemic involvement, and reacting antinuclear antibodies on HEp-2 cells. In none of them the most specific autoantibodies for systemic sclerosis (anti-centromere and anti-Scl-70/topo I) could be elicited. After introducing D-penicillamine the average period of time to reach remission was 60.7 ± 27.4 months. All four patients have been under no treatment for 31 to 120 months, and with no manifestations that point to disease activity, including absence of Raynaud's phenomenon. CONCLUSIONS: a) the clinical spectrum of systemic sclerosis include patients who reach clinical remission after using D-penicillamine, staying under no medication for many years; b) the absence of distinctive autoantibodies (anti-centromere and anti-Scl-70/topo I) in our patients must be explored in a larger sample population; c) the absence of specific autoantibodies for the disease might point to a better response to D-penicillamine therapy or to a higher possibility of spontaneous clinical remission.

systemic sclerosis; clinical remission; D-penicillamine


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