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Aktuelle Rheumatologie 2012; 37(05): 320-327
DOI: 10.1055/s-0032-1314844
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

IgG4-Related disease: the Rheumatologist’s Point of View

Die IgG4-assozierte Krankheit vom Standpunkt des Rheumatologen gesehen
N. Pipitone
1   Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera ASMN, Instituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy
,
A. Vaglio
2   Department of Internal Medicine, Nephrology and Health Sciences, Parma University, Parma, Italy
,
C. Salvarani
1   Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera ASMN, Instituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy
› Author Affiliations
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Publication History

Publication Date:
26 July 2012 (online)

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Abstract

The IgG4-related disease is a rare disorder with an estimated yearly incidence of 1 case per 100 000 population in Japan, characterized by the infiltration of IgG4-positive plasma cells into one or more organs. C-reactive protein and Serum IgG4 levels are often, but not invariably elevated. The most common clinical manifestation is lymphadenopathy, followed by autoimmune pancreatitis and sclerosing cholangitis. Imaging procedures are helpful to identify the affected organs and to monitor the disease course. However, biopsy is often required to secure the diagnosis. Glucocorticoids are the mainstay of therapy, but immunosuppressive agents are useful to prevent recurrences of disease in patients with a chronic relapsing course.

Zusammenfassung

Die IgG4-assozierte Krankheit hat eine Inzidenz von 1/100 000 Personen pro Jahr in Japan. Das histologische Kennzeichen ist ein Infiltrat von IgG4+ Plasmazellen in einem oder mehreren Organen. Im Labor sind das C-reaktive Protein und der IgG4-Spiegel in der Mehrzahl der Fälle erhöht. Die häufigsten klinischen Erscheinungen sind die Lymphadenopathie, die autoimmune Pankreatitis und die sklerosierende Cholangitis. Bildgebende Verfahren werden zum Nachweise des Organbefalls sowie zur Verlaufsbeobachtung verwendet. Eine Biopsie ist jedoch häufig erforderlich, um die Diagnose zu sichern. Steroide sind als Mittel der Wahl bei der IgG4-assozierte Krankheit anzusehen. Bei Patienten die wiederholt Rückfälle bzw. Rezidive erleiden ist es sinnvoll, steroideinsparende Medikamente einzusetzen

Key words

IgG4 - IgG4-related disease - chronic periaortitis - retroperitoneal fibrosis

Schlüsselwörter

IgG4 - IgG4-assozierte Krankheit - chronische Periaortitis - retroperitoneale Fibrose
 

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