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Medline ® Abstract for Reference 25

of 'Hyperimmunoglobulin D syndrome: Pathophysiology'

Hereditary periodic fever with systemic amyloidosis: is hyper-IgD syndrome really a benign disease?
Siewert R, Ferber J, Horstmann RD, Specker C, Heering PJ, Timmann C
Am J Kidney Dis. 2006;48(3):e41.
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.
Department of Nephrology, Solingen General Hospital, University of Cologne, Solingen, Germany. siewert@klinikumsolingen.de