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 cryoglobulinemi...

Full description

Saved in:
Bibliographic Details
Published in:Zeitschrift für Rheumatologie 2002-12, Vol.61 (6), p.733-739
Main Authors: Strunk, J, Taborski, U, Neeck, G
Format: Article
Language:ger
Subjects:
Antibodies, Antinuclear - blood
Arthritis, Rheumatoid - diagnosis
Arthritis, Rheumatoid - pathology
Arthritis, Rheumatoid - therapy
Biopsy
Cryoglobulinemia - diagnosis
Cryoglobulinemia - pathology
Cryoglobulinemia - therapy
Cryoglobulins - metabolism
Diagnosis, Differential
Follow-Up Studies
Humans
Male
Microscopy, Electron
Middle Aged
Muscle, Skeletal - pathology
Muscular Atrophy - diagnosis
Muscular Atrophy - pathology
Muscular Atrophy - therapy
Plasmapheresis
Polyneuropathies - diagnosis
Polyneuropathies - pathology
Polyneuropathies - therapy
Skin Ulcer - diagnosis
Skin Ulcer - pathology
Skin Ulcer - therapy
Sural Nerve - pathology
Vasculitis - diagnosis
Vasculitis - pathology
Vasculitis - therapy
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0340-1855