[Essential cryoglobulinemic vasculitis with severe peripheral neuropathy and neurogenic muscular atrophy -- inducing remission by cascade filtration].
We report on a 60 year old patient with peripheral neuropathy, neurogenic muscular atrophy, skin ulcers, arthritis and weakness. Detection of cryoglobulins in association with typical clinical symptoms, exclusion of hepatitis C and any other disease led to a rare diagnosis: essential cryoglobulinemic vasculitis. The case demonstrates not only the difficult diagnostic process but also the problems of an adequate and effective therapy. Since the usual immunosuppressive treatments such as methotrexate, high dose corticosteroid and intermittent intravenous pulse cyclophosphamide therapy (Austin's scheme) failed, we performed plasmapheresis (cascade filtration), which brought about an immediate and long-term remission. Besides discussing various types of plasmapheresis procedures and potential pathophysiological mechanisms, we point out that this therapy could find an early use in severe essential cryoglobulinemic vasculitis because of its excellent risk/benefit ratio.
Strunk
J
J
Herz- und Rheumazentrum Kerckhoff-Klinik, Abt. Rheumatologie/Universität Giessen, Ludwigstr. 37-39, 61231 Bad Nauheim, Germany. johannes.strunk@kerckhoff.med. uni-giessen.de
Taborski
U
U
Neeck
G
G
D000974:Antibodies, Antinuclear / Q000097:blood; D001172:Arthritis, Rheumatoid / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D001706:Biopsy; D003449:Cryoglobulinemia / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D003450:Cryoglobulins / Q000378:metabolism; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009133:Muscular Atrophy / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D010956:Plasmapheresis; D011115:Polyneuropathies / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D012883:Skin Ulcer / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D013497:Sural Nerve / Q000473:pathology; D014657:Vasculitis / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*
D000974:Antibodies, Antinuclear; D003450:Cryoglobulins