Vaskulitis und Nervensystem

ZusammenfassungBei der Mehrzahl der systemischen Vaskulitiden handelt es sich um immunologisch bedingte Entzündungen der Gefäßwand, wobei in der Auslösung Autoantikörper und zirkulierende Immunkomplexe eine Rolle spielen können. Eine Miteinbeziehung des Nervensystems führt typischerweise zu einer Kombination von Enzephalopathie, symptomatischer Epilepsie, multifokalen Ausfällen und Kopfschmerzen. Die Beteiligung des peripheren Nervensystems zeigt sich oftmals unter dem Bild der asymmetrischen schmerzhaften Mononeuritis multiplex. Die Diagnose stützt sich auf klinisches Verteilungsmuster, Laborbefunde sowie den Nachweis der Gefäßwandentzündung im histologischen Befund. Die Therapie erfolgt kombiniert mit Kortikosteroiden und Immunsuppressiva.

Thrombangitis obliterans: Subpopulationen der Leukozyten und zirkulierende Immunkomplexe

Background: The etiology of thrombangitis obliterans is still unclear. Although cellular infiltration of the vessel wall is known, no studies on peripheral blood mononuclear cells are reported. Therefore, we assessed leucocyte subpopulations and circulating immune complexes in patients with thrombangitis obliterans and a control group of normal people. Patients and methods: 31 patients (40 ± 2 years, 24 male, 7 female) with thrombangitis obliterans were included, based on the following criteria: age of manifestation, acral ischemia in legs and arms, previous thrombophlebitis or phlebitis saltans. Manifestation of atherosclerosis or other vasculitic manifestations were excluded. Leucocyte subpopulations, levels of C-reactive protein (CRP) and circulating immune complexes (CIC) were investigated. An age-matched control group (n = 25) was recruited from voluntary blood donors. Results: Leucocyte counts in the thrombangitis group (mean ± SD: 10839 ± 782/nl) were significantly different from the control group (6205 ± 414/nl, p < 0.0001). The same was true for absolute counts of granulocytes, monocytes and lymphocytes. The results were independent from CRP, which was elevated only in 6 patients. Relative counts of naive helper T-cells were significantly lower in the patient group. HLA-DR expression on B-cells was lower on the patients’ lymphocytes. The concentrations of IgA, IgG and IgM in CIC were higher in the thrombangitis patients compared to the control group. C1q-binding capacity and phosphatidylserine antibodies showed no differences. Conclusions: Patients suffering from thrombangitis obliterans show alterations of leucocyte counts and their subpopulations as well as alterations of the humoral (IgCIC) immune system.