PubMed Health⌕ Search

Biomedical subjects

Gabriella Szúcs

Publications and source records attributed to Gabriella Szúcs.

2 recordsLinked to original sources
PubMed2003

[Plasmapheresis therapy in systemic sclerosis].

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.

Adult↗
PubMed2003

[Reactive macrophage activation syndrome].

Reactive macrophage activation syndrome or haemophagocytic lymphohistiocytosis is a life-threatening immunoregulatory disorder characterized by the activation of the mononuclear phagocytic system with prominent haemophagocytosis particularly involving the bone marrow, reticuloendothelial system and central nervous system. It frequently occurs in children as a familial autosomal recessive disorder and it has been reportedly associated with infections, underlying hematopoetic malignancies or autoimmune disorders. It has been suggested that central to the pathogenesis of this disorder is uncontrolled T-cell activation which results in the excessive secretion of cytokines. The clinical and laboratory findings are dramatic, at presentation with persistent fever, hepatosplenomegaly, profound depression of three blood cell lines, elevated serum liver enzyme values, coagulopathy and abundant haemophagocytosis can be visualized in bone marrow. Most of the described clinical findings could be explained by the oversecretion of proinflammatory cytokines and the presence of histiocytic infiltrates. In general, immunosuppressive therapy, cytotoxic chemotherapy with immunomodulatory agents will induce remissions in a substantial percentage of patients, whereas others require allogenic bone marrow transplantation.

Diagnosis, Differential↗