Scleroderma and related disorders: therapeutic aspects

Improvements in management of systemic sclerosis have occurred through a gr owing understanding of pathogenic events accompanied by advances in diagnos is and assessment, as well as developments in organ-based therapeutics. Unf ortunately, disease-modifying therapies of proven efficacy remain elusive a nd several agents in current use have been shown in well-controlled clinica l trials to be of questionable benefit. More positively, there have been ad vances in the treatment of major visceral complications such as renal crisi s, pulmonary fibrosis, pulmonary hypertension, gastrointestinal involvement and in the management of scleroderma-associated Raynaud's phenomenon. Curr ent approaches are reviewed, evidence supporting or refuting the use of put ative disease-modifying agents is discussed, and new strategies are describ ed that are currently being considered for this fascinating but complex mul tisystem connective tissue disease.