Scleroderma and related disorders: therapeutic aspects

Improvements in management of systemic sclerosis have occurred through a growing understanding of pathogenic events accompanied by advances in diagnosis and assessment, as well as developments in organ-based therapeutics. Unfortunately, disease-modifying therapies of proven efficacy remain elusive and several agents in current use have been shown in well-controlled clinical trials to be of questionable benefit. More positively, there have been advances in the treatment of major visceral complications such as renal crisis, pulmonary fibrosis, pulmonary hypertension, gastrointestinal involvement and in the management of scleroderma-associated Raynaudʹs phenomenon. Current approaches are reviewed, evidence supporting or refuting the use of putative disease-modifying agents is discussed, and new strategies are described that are currently being considered for this fascinating but complex multisystem connective tissue disease.