Granulomatose mit Polyangiitis – Manifestationen im Kopf-Hals-Bereich

Kim Vanessa Steinke; Hans-Jürgen Welkoborsky

doi:10.1055/a-1580-7037

Laryngo-Rhino-Otologie, Table of Contents

Laryngorhinootologie 2022; 101(02): 112-119
DOI: 10.1055/a-1580-7037

Übersicht

Granulomatose mit Polyangiitis – Manifestationen im Kopf-Hals-Bereich

Granulomatosis with polyangiitis – manifestations in the head and neck area

Kim Vanessa Steinke

HNO, KRH Klinikum Nordstadt, Hannover, Germany

,

Hans-Jürgen Welkoborsky

HNO, KRH Klinikum Nordstadt, Hannover, Germany

› Author Affiliations

Abstract

Zusammenfassung

Die Granulomatose mit Polyangiitis ist eine seltene chronische rheumatologische Systemerkrankung, die mit einer Vaskulitis der kleinen und mittleren Gefäße einhergeht. Am häufigsten betrifft sie die oberen Atemwege, die Lunge und die Nieren. Die Beschwerden sind unspezifisch, häufig beklagen die Patienten anfangs eine Nasenatmungsbehinderung, Borkenbildung in der Nase, Ulzera der Mundschleimhäute oder Epistaxis. Nicht selten wird deshalb der Hals-Nasen-Ohren-Arzt zu Beginn der Krankheit hinzugezogen. Langfristig können schwerwiegende kardiale, renale oder pulmonale Komplikationen auftreten. Die Ätiologie ist bis heute nicht komplett geklärt. Als Therapie wird eine Immunsuppression eingeleitet. Klinische und laborchemische Kontrollen sind lebenslang obligat.

Abstract

Granulomatosis with polyangiitis is a rare chronic rheumatologic systemic disease with a vasculitis of small- and medium-size vessels. Mostly the upper airways, lung and kidneys are affected. Symptoms are unspecific. Patients complain about stuffy nose, crustiness of nasal secretions, ulcera of the oral mucosa or epistaxis. The otorhinolaryngologist may be the first one to evaluate the patient’s health condition. Long term complications may be cardial, renal or pulmonal failure. To this day the aetiology is still unknown. Severe disease is treated with a combination of immunosuppressive medications. Clinic examinations and laboratory tests should be carried out for life-time.

Schlüsselwörter

Granulomatose mit Polyangiitis - ANCA-assoziierte Vaskulitis - Autoimmunerkrankung - Immunsuppression - seltene Erkrankungen

Key words

granulomatosis with polyangiitis - ANCA-associated vasculitis - autoimmune disease - immunosuppression - rare disease

Full Text

References

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