Autoimmune disorders in patients with idiopathic thrombotic thrombocytopenic purpura^[*]

Summary

76 German patients suffering from thrombotic thrombocytopenic purpura (TTP) were interrogated about the prevalence of cooccurring autoimmune disorders. In order to analyze a possible association of TTP with the questioned diseases, a comparison of prevalence rates between the patient group and the general population has been made for each disease. Results: Compared to the estimated prevalence rates, the statistical analysis revealed an unexpected high occurrence of the following disorders within the patient group: Hashimoto’s thyroiditis (23.5% within the patients compared to 0.7% within the general population, p<0.001), systemic lupus erythematosus (SLE) (6.5% in patients to 0.025% in the general population, p<0.001), immune thrombocytopenic purpura (ITP) (6.3% in patients to 0.02% in the general population, p < 0.001), psoriasis (9.4% in patients to 2.5% in the general population, p = 0.005) and celiac disease (3.1% in patients to 0.2% in the general population, p = 0.007). Conclusion: These findings confirm the mentioned tendency of autoimmune diseases to co-occur in one individual and argue once more for a genetic susceptibility in idiopathic TTP as well as in autoimmune disorders.

Zusammenfassung

Bei 76 deutschen Patienten mit thrombotisch untersucht. Als Vergleichswerte dienten die zugehörigen Prävalenzen für die jeweilige Erkrankung in der Allgemeinbevölkerung. Die statistische Auswertung erfolgte mit dem zweiseitigen Binomialtest.Ergebnisse: Im Vergleich mit den Prävalenzen in der Allgemeinbevölkerung, zeigte sich für fünf Erkrankungen ein gehäuftes Auftreten im Patientenkollektiv: Hashimoto-Thyreoiditis (23,5% in der Patientengruppe zu 0,7% in der Allgemeinbevölkerung; p < 0,001), systemischer Lupus erythematodes (SLE) (6,5% der Patienten im Gegensatz zu 0,025% in der Allgemeinbevölkerung, p < 0,001), Immunthrombozytopenie (ITP) (6,3% der Patienten zu 0,02% in der Allgemeinbevölkerung; p < 0,001), Psoriasis (9,4% der Patienten zu 2,5% in der Allgemeinbevölkerung; p = 0,005) und Zöliakie (3,1% der Patienten zu 0,2% in der Allgemeinbevölkerung; p = 0,007). Schlussfolgerung: Diese Ergebnisse sprechen für eine Assoziation verschiedener Autoimmunkrankheiten untereinander und unterstützen die Annahme einer möglichen genetischen Prädisposition zur Ausbildung mehrerer autoimmuner Störungen innerhalb eines Individuums.

Keywords

TTP - autoimmunity - genetic susceptibility - HLA

Schlüsselwörter

TTP - Autoimmunität - genetische Prädisposition - HLA

^* The content of this paper is part of the dissertation from Marie-Luise John.

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