Cryoglobulin/albumin complexes in a patient with severe autoimmune syndrome.
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.
Trendelenburg
M
M
Medical Clinic B, University Hospital Basel, Switzerland. marten.trendelenburg@unibas.ch
Lutz
H U
HU
Tissot
J D
JD
Moll
S
S
Hoffmann
T
T
Schifferli
J A
JA
D000328:Adult; D000418:Albumins / Q000032:analysis*; D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D003131:Combined Modality Therapy; D003450:Cryoglobulins / Q000032:analysis*; D018450:Disease Progression; D015180:Electrophoresis, Gel, Two-Dimensional; D017809:Fatal Outcome; D006801:Humans; D007105:Immune Complex Diseases / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D010956:Plasmapheresis / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index
D000418:Albumins; D001323:Autoantibodies; D003450:Cryoglobulins; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents