Kryoglobulinämien

Zusammenfassung

Kryoglobulinämien sind seltene Erkrankungen, die durch den Nachweis von Kryoglobulinen und Komplikationen wie Purpura, akrale Nekrosen, Glomerulonephritis und Polyneuropathie gekennzeichnet sind. Kryoglobuline können monoklonal oder gemischt sein. Monoklonale (Typ I) Kryoglobulinämien sind Folge lymphoproliferativer Erkrankungen, die Hauptkomplikation sind akrale Nekrosen. Die Therapie der lymphoproliferativen Grunderkrankung wird vom Hämatologen überwacht. Gemischte (Typ II und Typ III) Kryoglobulinämien sind mit Hepatitis C Infektion bzw. mit Kollagenosen und rheumatoider Arthritis assoziiert. Die Hepatitis C sollte vom Hepatologen mit Interferon alpha und Ribavirin behandelt werden. Bei fehlendem Therapieerfolg oder Rezidiven nach Absetzen der Behandlung können bei Komplikationen der Vaskulitis Glucocorticosteroide, Immunsuppressiva, Plasmapheresen oder auch Rituximab eingesetzt werden.

Abstract

Cryoglobulinemia is a rare disease entity characterised by the presence of cryoglobulins and complications such as purpura, acral necrosis, glomerulonephritis and polyneuropathy. Cryoglobulins may be monoclonal or mixed. Monoclonal (type I) croglobulinemia is a consequence of lymphoproliferative disorders and may be complicated by acral necrosis. The therapy of the lymphproliferative disorder requires monitoring by a haematologist. Mixed (type II and type III) cryoglobulinemia is associated with hepatitis C infection, connective tissue disease and rheumatoid arthritis. The hepatitis C should be treated with interferon-alpha and ribavirin by a hepatologist. If the treatment is not successful or relapses occur after cessation of the therapy, vasculitis complications may be improved by glucocorticosteroids, immunosuppressants, plasmapheresis or rituximab.

Schlüsselwörter

Kryoglobuline - Vaskulitis - Hepatitis C

Key words

cryoglobulins - vasculitis - hepatitis C

Literatur

• 1 Brouet J C, Clauvel J P, Danon F. et al . Biologic and clinical significance of cryoglobulins. A report of 86 cases.  Am J Med. 1974;  57 775-788
• 2 Bryce A H, Kyle R A, Dispenzieri A, Gertz M A. Natural history and therapy of 66 patients with mixed cryoglobulinemia.  Am J Hematol . 2006;  81 511-518
• 3 Cacoub P, Poynard T, Ghillani P. et al . Extrahepatic manifestations of chronic hepatitis C.  Arthritis Rheum. 1999;  42 2204-2212
• 4 Calleja J L, Albillos A, Cacho G. et al . Interferon and prednisone therapy in chronic hepatitis C with non-organ-specific antibodies.  J Hepatol. 1996;  24 308-312
• 5 Casato M, Laganà B, Antonelli G. et al . Long-term results of therapy with interferon-alpha for type II essential mixed cryoglobulinemia.  Blood. 1991;  78 3142-3147
• 6 D’Amico G. Renal involvement in hepatitis C infection: cryoglobulinemic glomerulonephritis.  Kidney Int. 1998;  54 650-671
• 7 Dimitrakopoulos A N, Kordossis T, Hatzakis A. et al . Mixed cryoglobulinemia in HIV-1 infection: the role of HIV-1.  Ann Intern Med. 1999;  130 226-230
• 8 Ferri C, Pietrogrande M, Cecchetti R. et al . Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia.  Am J Med. 1989;  87 519-524
• 9 Fong T L, Valinluck B, Govindarajan S. et al . Short-term prednisone therapy affects aminotransferase activity and hepatitis C virus RNA levels in chronic hepatitis C.  Gastroenterology. 1994;  107 196-199
• 10 Gorevic P D, Kassab H J, Levo Y, Kohn R, Meltzer M, Prose P. et al .  Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients.  Am J Med . 1980;  69 287-308
• 11 Italian Group for the Study of Cryoglobulinaemias (GISC) .Metodologie di studio e protocolli diagnostici. Ferri C Proceedings of 10th Congresso Società Italiana di Immunologia e Immunopatologia Cagliari; S. Margherita di Pula 1989: 220
• 12 Jennette J C, Falk R J, Andrassy K. et al . Nomenclature of systemic vasculitides. Proposal of an international consensus conference.  Arthritis Rheum. 1994;  37 187-192
• 13 Jennette J C, Falk R J. Small Vessel Vasculitis.  N Engl J Med. 1997;  337 1512-1523
• 14 Jennette J C, Rosen S. Vasculitis. Damjanov I, Linder J Anderson's Pathology Philadelphia; Mosby 1996 10th ed: Chapter 47B, 1397-1445
• 15 Lamprecht P, Gause A, Gross W L. Cryoglobulinemic vasculitis.  Arthritis Rheum. 1999;  42 2507-2516
• 16 Lamprecht P, Gause A, Gross W L. Cryoglobulinemic vasculitis resistant to intermittent intravenous pulse cyclophosphamide therapy.  Scand J Rheumatol. 2000;  29 201-202
• 17 Lamprecht P, Gutzeit O, Csernok E. et al . Prevalence of ANCA in mixed cryoglobulinemia and chronic hepatitis C virus infection.  Clin Exp Rheumatol. 2003;  21 (6 Suppl 32) S89-94
• 18 Magrin S, Craxi A, Fabiano C. et al . Hepatitis C viremia in chronic liver disease: relationship to interferon-alpha or corticosteroid treatment.  Hepatology. 1994;  19 273-279
• 19 Monti G, Galli M, Invernizzi F. GISC Italian Group for the Study of Cryoglobulinemias. . Cryoglobulinaemias: a multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease.  QJM. 1995;  88 115-126
• 20 Moulin B, Ronco P M, Mougenot B. et al . Glomerulonephritis in chronic lymphocytic leukemia and related B-cell lymphomas.  Kidney Int. 1992;  42 127-135
• 21 Sansonno D, De Re V, Lauletta G. et al . Monoclonal antibody treatment of mixed cryoglobulinemia resistant to interferon alpha with an anti-CD20.  Blood. 2003;  101 3818-3826
• 22 Scotto G, Cibelli D C, Saracino A. et al . Cryoglobulinemia in subjects with HCV infection alone, HIV infection and HCV/HIV coinfection.  J Infect. 2006;  52 294-299
• 23 Tavoni A, Mosca M, Ferri C. et al . Guidelines for the management of essential mixed cryoglobulinemia.  Clin Exp Rheumatol. 1995;  Suppl 13 S191-S195
• 24 Tedeschi A, Baratè C, Minola E. et al . Cryoglobulinemia.  Blood Reviews. 2003;   21 183-200
• 25 Zaja F, De Vita S, Mazzaro C. et al . Efficacy and safety of rituximab in type II mixed cryoglobulinemia.  Blood. 2003;  101 3827-3834
• 26 Zuckerman E, Keren D, Slobodin G. et al . Treatment of refractory, symptomatic, hepatitis C virus related mixed cryoglobulinemia with ribavirin and interferon-alpha.  J Rheumatol. 2000;  27 2172-2178