[Systemic sclerosis].
Systemic sclerosis (scleroderma) is a rare connective tissue disease characterized by vasculopathy and immune dysfunction, leading to fibrosis with damage of multiple organs. Two major clinical subtypes are the diffuse and limited forms. The combination of Raynaud's phenomenon, puffy fingers and positive antinuclear antibodies are red flag features that should alert the clinician to the presence of very early systemic sclerosis, which can be treated with vasodilator, antithrombotic, and immunosuppressive drugs. Progress has been made even in the management of the most severe manifestations, including interstitial lung disease, pulmonary artery hypertension and scleroderma renal crisis.
HUS Yleissisätautien Klininen Laitos.
Ritva
R
Peltomaa
Tom
T
Pettersson
Riitta
R
Tuompo
Riitta
R
Luosujärvi
D003937:Diagnosis, Differential; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D014665:Vasodilator Agents / Q000627:therapeutic use
D005343:Fibrinolytic Agents; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents