Abstract
We describe the case of a 78-year-old woman with peripheral neuropathy, neurogenic muscular atrophy, skin ulcers, arthritis and immune complex glomerulonephritis. Detection of mixed cryoglobulins in combination with typical clinical symptoms, and the exclusion of hepatitis C and other underlying diseases, led to the rare diagnosis of essential cryoglobulinaemic vasculitis type III. Because initial interventions with prednisolone, plasmapheresis and cyclophosphamide pulse therapy failed to induce remission, therapy with rituximab, a chimeric monoclonal antibody that reacts specifically with the CD20 antigen, was initiated. Rituximab was administered intravenously at a dose of 375 mg/m2 body surface. After five applications, the patient showed remission of clinical symptoms and complete normalisation of laboratory values. These results suggest that rituximab is an alternative therapeutical approach with strikingly good clinical outcome in patients with cryoglobulinaemic vasculitis type III.
This is a preview of subscription content, log in via an institution to check access.
References
Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M (1974) Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med 57:775–788
Rieu V, Cohen P, Andre MH et al (2002) Characteristics and outcome of 49 patients with symptomatic cryoglobulinaemia. Rheumatology 41:290–300
Agnello V, Chung RT, Kaplan LM (1992) A role for hepatitis C virus infection in type 2 cryoglobulinaemia. N Engl J Med 327:1490–1495
Ferri C, Marzo E, longbardo G (1993) Interferon alpha in mixed cryoglobulinimia patients. A randomized cross over controlled trial. Blood 81:1123–1126
Perek J, Mittelmann M, Eisbruch A, Djaldette M (1984) Systemic lupus erythematosus preceeded by long-term cryoglobulinaemia. Ann Rheum Dis 43:339–340
Strunk J, Taborski U, Neeck G (2002) Essential cryoglobulinaemic vasculitis with severe peripheral neuropathy and neurogenic muscular atrophy––including remission by cascade filtration. Z Rheumatol 61:733–739
Lamprecht P, Gause A, Gross WL (1999) Cryoglobulinaemic vasculitis (review). Arthritis Rheum 42:2507–2516
Zaja F, de Vita S, Mazarro C, Sacco S, Damiani D, de Marchi G, Micheluti A, Baccarani M, Fanin R, Ferraccioli G (2003) Efficacy and safety of rituximab in type II mixed cryoglobulinaemia. Blood 101:3827–3834
Ghobrial IM, Uslan DZ, Call TG, Witzig TE, Gertz MA (2004) Initial increase in the cryoglobulin level after rituximab therapy for type II cryoglobulinaemia secondary to Waldenström macroglobulinemia does not indicate failure of response. Am J Hematol 77:329–30
Lamprecht P, Lerin-Lozano C, merz H, Dennin RH, Gause A, Voswinkel J, Peters SO, Gutzeit O, Arlt AC, Solbach W, Gross WL (2003) Rituximab induces remission in refractory HCV associated cryoglobulinaemic vasculitis. Ann Rheum Dis 62:1230–1233
Basse G Ribes D, Kamar N, Mehrenberger M, Sallusto F, Esposito L, Guitard J, Lavayssiere L, Oksman F, Durand D, Rostaing L (2006) Rituximab therapy for mixed cryoglobulinemia in seven renal transplant patients. Transplant Proc 38:2308–2310
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Braun, A., Neumann, T., Oelzner, P. et al. Cryoglobulinaemia type III with severe neuropathy and immune complex glomerulonephritis: remission after plasmapheresis and rituximab. Rheumatol Int 28, 503–506 (2008). https://doi.org/10.1007/s00296-007-0462-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-007-0462-y