Background: Systemic sclerosis (SSc) is a polysystemic autoimmune disorder characterized by excessive production of extracellular matrix and vascular abnormalities, involving the skin and internal organs. There is currently no real disease-modifying therapy of SSc and there are only few reports on the use of plasmapheresis in SSc.
Objectives: Authors present 4 cases of SSc treated with plasmapheresis within one year from disease onset. All patients had diffuse cutaneous form of SSc with rapid progression and with different internal organ manifestations.
Methods: The patients were treated with plasmapheresis performed every 3 months besides symptomatic drug treatment. Clinical symptoms, Rodnan skin score, immunolaboratory markers (serum immunoglobulin, complement, several autoantibodies, leukocyte CD markers) and soluble adhesion molecules (E-selectin, intercellular adhesion molecule 1, vascular cell adhesion molecule, which were showed as the markers of disease activity in previous works) were determined before and after the plasmapheresis.
Results: The progression of the disease slowed down significantly in all patients, no more new clinical manifestation appeared, Rodnan skin score significantly decreased. There was a reduction in CD4/CD8 ratio (approaching normal ratio), in expression of CD69, as early activation marker. There was a significant suppression of soluble adhesion molecules also in all patients.
Conclusion: In patients with diffuse cutaneous form of SSc, plasmapheresis may be effective with decreasing progression of the disease and improving clinical symptoms (especially skin manifestation) in the early phase of the disease.