Scleroderma (progressive systemic sclerosis, PSS); pathophysiological, clinical and pharmacological aspects of the syndrome.

Abstract

Scleroderma is an uncommon complex disease. The onset is slow and the progress is chronic. The main pathophysiological changes vary; they affect blood vessels, connective tissue, collagen fibres, cause fibrin deposition and inflammatory reactions. There may be early oedema and a wide spectrum of organic involvement. Clinically, all the fibril-containing and connective tissue organs can be attacked in various degrees. The most common organ manifestations are the Raynaud's phenomenon in the arms and hands, vascular fibrosis, stiff and hard facial skin, restriction of joint movement by pericapsular hardening, calcium deposition and capsular rigidity. In the gastrointestinal tract muscle atrophy, collagen and connective tissue damage are common, especially at the cardia. Malabsorption may occur. Progressive pulmonary fibrosis leads to cor pulmonale and respiratory insufficiency. The liver, kidneys and the endocrine glands are, however, seldom involved. Therapeutic trials have been performed using many different groups of drugs: vasodilatating agents, corticosteroids, drugs found experimentally to influence connective tissue, thyroxine and a variety of anti-rheumatic agents. In the last decade best short-term clinical results have been achieved with penicillamine, some vasodilators, chlorambucil and in recent years with cyclofenil a potent anti-oestrogen, which has marked connective tissue and collagen metabolism influencing properties. Good therapeutic effects without serious side effects have been achieved.