Cryoglobulin/albumin complexes in a patient with severe autoimmune syndrome
Détails
ID Serval
serval:BIB_E1040B7526F9
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Cryoglobulin/albumin complexes in a patient with severe autoimmune syndrome
Périodique
Scandinavian Journal of Rheumatology
ISSN
0300-9742 (Print)
Statut éditorial
Publié
Date de publication
2003
Volume
32
Numéro
6
Pages
367-73
Notes
Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Journal Article
Research Support, Non-U.S. Gov't
Résumé
We describe the case of a 30-year-old man with a severe autoimmune disease characterized by cryoglobulinaemia, pulmonary hypertension, Raynaud's phenomenon, lymphadenopathy, and glomerulonephritis. Despite initial remission following autologous stem cell transplantation, his disease relapsed and he died from pulmonary hypertension. At presentation the patient had hypergammaglobulinaemia and a number of autoantibodies, including rheumatoid factor (1:10240). The most striking feature was the extremely high level of cryoglobulins. The cryoprecipitate consisted of polyclonal IgM, IgG and albumin. Interestingly, the albumin in the cryoprecipitate was exclusively present in SS-bonded oligomeric forms, and contained an abnormal acidic component as judged by 2D gel electrophoresis. Oxidized albumin was also present in serum, and represented a small but significant fraction. None of the many known albumin variants have so far been associated with a particular disease; thus our results may represent the first description of an altered albumin associated with severe disease.
Mots-clé
Adult
Albumins/*analysis
Autoantibodies/*analysis
Autoimmune Diseases/*diagnosis/*immunology/therapy
Combined Modality Therapy
Cryoglobulins/*analysis
Disease Progression
Electrophoresis, Gel, Two-Dimensional
Fatal Outcome
Humans
Immune Complex Diseases/diagnosis/immunology/therapy
Immunoblotting
Immunoglobulin G/analysis
Immunoglobulin M/analysis
Immunosuppressive Agents/therapeutic use
Male
Plasmapheresis/methods
Risk Assessment
Severity of Illness Index
Pubmed
Création de la notice
25/01/2008 15:34
Dernière modification de la notice
20/08/2019 16:05