Hereditary periodic fever with systemic amyloidosis: is hyper-IgD syndrome really a benign disease?
We report a case of amyloidosis in association with hyperimmunoglobulinemia D syndrome (HIDS). The patient showed typical clinical features of HIDS. He had crescentic glomerulonephritis progressing to end-stage renal disease at age 13 years. Eight years later, he developed an AA-type amyloidosis with extensive involvement of the intestine, respiratory tract, and thyroid gland. These unusual complications of HIDS seriously challenge the assumption that the disease is associated with a good prognosis.
Siewert
Rainer
R
Department of Nephrology, Solingen General Hospital, University of Cologne, Solingen, Germany. siewert@klinikumsolingen.de
Ferber
Jörg
J
Horstmann
Rolf Dieter
RD
Specker
Christof
C
Heering
Peter Julius
PJ
Timmann
Christian
C
D000328:Adult; D000686:Amyloidosis / Q000209:etiology* / Q000276:immunology*; D010505:Familial Mediterranean Fever / Q000209:etiology* / Q000276:immunology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000235:genetics; D007072:Immunoglobulin D / Q000276:immunology*; D008297:Male; D013577:Syndrome
D007072:Immunoglobulin D