Premium
Management of scleroderma
Author(s) -
Krieg Thomas
Publication year - 2002
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1046/j.1365-2230.2002.10435.x
Subject(s) - medicine , scleroderma (fungus) , disease , intensive care medicine , fibrosis , pathophysiology , systemic scleroderma , pulmonary hypertension , systemic disease , treatment modality , dermatology , pathology , surgery , inoculation
Systemic sclerosis is characterized by excessive deposition of extracellular matrix components into various internal organs and the skin. This is due to the development of fibrosis following an initial Raynaud's phenomenon and an inflammatory reaction. Although some of the basic principles in the pathophysiology of the disease are partly understood, we still lack a detailed knowledge of the initial processes. Therefore, therapy of systemic scleroderma is symptomatic and needs to be closely adapted to the subsets of the disease and to the specific involvement of affected organs. This includes the treatment of vascular lesions and the inflammatory reactions, whereas management of the fibrotic processes is still very limited and restricted to experimental protocols. Early detection of kidney and cardiac involvement is required, and the early diagnosis of pulmonary hypertension has a critical impact on the life expectancy of the individual patient. Although no causative treatment of the disease is available, organ‐specific management of complications together with general symptomatic treatment modalities and detailed counselling of the patient are of tremendous help and can considerably in modifying the development of the disease.