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Systemic amyloidosis in a patient with hypogammaglobulinaemia
Author(s) -
KOTILAINEN P.,
VUORI K.,
KAINULAINEN L.,
AHO H.,
SAARIO R.,
ASOLA M.,
NIKOSKELAINEN J.
Publication year - 1996
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1046/j.1365-2796.1996.497838000.x
Subject(s) - medicine , amyloidosis , polyarthritis , rheumatoid arthritis , arthritis , nephrotic syndrome , al amyloidosis , immunopathology , immunology , gastroenterology , dermatology , antibody , immunoglobulin light chain
A 49‐year‐old patient with an 18‐year history of hypogammaglobulinaemia presented with nephrotic syndrome due to systemic amyloidosis. Recurrent infections as a consequence of an inadequate gamma‐globulin substitution therapy were regarded as the main reason for the development of amyloidosis. When a high‐dose intravenous immunoglobulin therapy was started, the clinical symptoms declined and the patient felt moderately well. Later the patient developed symmetrical polyarthritis clinically suggestive of rheumatoid arthritis. Although the incidence of arthritis is increased in hypogammaglobulinaemia, arthritis has not been reported in any of the few previously described patients with hypogammaglobulinaemia‐associated amyloidosis. Moreover, this case provides further evidence that, in these patients, the amyloid fibrils may be of the AA type.