NDT Advance Access originally published online on January 23, 2006
Nephrology Dialysis Transplantation 2006 21(4):1109-1112; doi:10.1093/ndt/gfk098
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© The Author [2006]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Teaching Point
(Section Editor: A. Meyrier)
A nephrotic patient with tumour necrosis factor receptor-associated periodic syndrome, IgA nephropathy and CNS involvement


1 Department of Endocrinology and Nephrology, University of Heidelberg, 2 Department of Infectious Diseases and Immunology, Children's Hospital, University of Munich, 3 Department of Neuropathology, University of Freiburg and 4 Department of Clinical Chemistry Großhadern, University of Munich, Germany
Correspondence and offprint requests to: Gottfried Rudofsky, MD, Division of Endocrinology and Nephrology, Department of Medicine, Im Neuenheimer Feld 410, D-69120 Heidelberg, Germany. Email: Gottfried_Rudofsky@med.uni-heidelberg.de
Keywords: amyloidosis; CNS manifestation; IgA nephropathy; periodic fever syndrome; TNF receptor-associated periodic syndrome
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The periodic fever syndromes are a heterogeneous group of disorders characterized by repeated attacks of fever and localized inflammation, primarily affecting serosal surfaces, the skin and the musculoskeletal system [1]. To date, five periodic fever syndromes have been characterized on the basis of their clinical features. Except for the periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome, the affected genes are known. Familial Mediterranean fever (FMF) caused by mutations in the Mediterranean fever (MEFV) gene, and the hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) due to mutations in the mevalonate kinase (MVK) gene have an autosomal recessive inheritance [1]. The others are autosomal dominant disorders: Cryopyrin-associated periodic syndrome (CAPS) as a consequence of heterozygous mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, and the tumour necrosis factor receptor-associated periodic syndrome (TRAPS) caused by dominantly inherited mutations in the
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