Cryoglobulinaemia type III with severe neuropathy and immune complex glomerulonephritis: remission after plasmapheresis and rituximab.
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/m(2) 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.
Braun
Anke
A
University of Heidelberg, Bethanien Hospital, Rohrbacher Str. 149, 69126 Heidelberg, Germany. abraun@bethanien-heidelberg.de
Neumann
Thomas
T
Oelzner
Peter
P
Hein
Gert
G
Gröne
Hermann-Josef
HJ
Ziemer
Mirjana
M
Wolf
Gunter
G
D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D009133:Muscular Atrophy / Q000150:complications / Q000188:drug therapy; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000188:drug therapy; D010956:Plasmapheresis; D000069283:Rituximab; D012883:Skin Ulcer / Q000150:complications / Q000188:drug therapy; D014657:Vasculitis / Q000188:drug therapy* / Q000473:pathology
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab