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
Auteur⸱e⸱s
Trendelenburg  M., Lutz  H. U., Tissot  J. D., Moll  S., Hoffmann  T., Schifferli  J. A.
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
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 16:34
Dernière modification de la notice
20/08/2019 17:05
Données d'usage