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CC BY-NC-ND 4.0 · Klin Padiatr 2024; 236(02): 97-105
DOI: 10.1055/a-2230-1521
Review

Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature

Relapsing Poychondritis mit Beteiligung der Atemwege: Eine detaillierte Beschreibung von zwei pädiatrischen Fällen und Literaturübersicht
Greta Winter
1   Department of Paediatric Pneumology, Allergy and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
,
Tara Löffelmann
1   Department of Paediatric Pneumology, Allergy and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
,
Shaakira Chaya
2   Division of Paediatric Pulmonology, Department of Paediatrics and Child Health, University of Cape Town, Red Cross War Memorial Children’s Hospital, Cape Town, South Africa
,
Hannah Kaiser
1   Department of Paediatric Pneumology, Allergy and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
,
Nils Kristian Prenzler
3   Department of Otorhinolaryngology, Head and Neck Surgery, Hannover Medical School, Hannover, Germany
,
Athanasia Warnecke
3   Department of Otorhinolaryngology, Head and Neck Surgery, Hannover Medical School, Hannover, Germany
,
Martin Wetzke
1   Department of Paediatric Pneumology, Allergy and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
4   German Centre for Lung Research (DZL), Hannover Medical School, Hannover, Germany
,
Thorsten Derlin
5   Department of Nuclear Medicine, Hannover Medical School, Hannover, Germany
,
Diane Renz
6   Institute of Diagnostic and Interventional Radiology, Department of Paediatric Radiology, Hannover Medical School, Hannover, Germany
,
Thomas Stueber
7   Department of Anaesthesiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
,
Florian Länger
4   German Centre for Lung Research (DZL), Hannover Medical School, Hannover, Germany
8   Institute of Pathology, Hannover Medical School, Hannover, Germany
,
Katharina Schütz    *
1   Department of Paediatric Pneumology, Allergy and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
,
Nicolaus Schwerk   *
4   German Centre for Lung Research (DZL), Hannover Medical School, Hannover, Germany
9   Pediatric Pulmonology and Neonatology, Hannover Medical School Centre for Paediatrics and Adolescent Medicine, Hannover, Germany
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Abstract

Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications. Due to its rarity and variability of symptoms, the diagnosis of RP is often delayed particularly in childhood. To address this and increase awareness of this rare disease, we present a detailed case report of two adolescent females affected by RP. We aim to describe the clinical findings, consequences of a delayed diagnosis and provide a review of the current literature.

Zusammenfassung

Die rezidivierende Polychondritis (RP) ist eine seltene immunvermittelte Erkrankung, die in erster Linie den Knorpel der Ohren, der Nase und der unteren Atemwege betrifft. Das klinische Spektrum reicht von leichten Symptomen mit rezidivierenden Entzündungen an Ohren und/oder Nase bis hin zu schweren Verlaufsformen mit fortschreitender laryngotracheobronchialer Knorpeldestruktion. Letzteres kann unbehandelt zu einer lebensbedrohlichen und irreversiblen Atemwegsobstruktion führen. Eine frühzeitige Diagnose ist unerlässlich, um die Betroffenen vor schweren Komplikationen zu bewahren. Aufgrund der Seltenheit und der Variabilität der Symptome wird die Diagnose RP häufig verzögert- oder gar nicht gestellt, insbesondere im Kindesalter. Um das Bewusstsein für diese potentiell lebensbedrohliche Erkrankung zu schärfen, die typischen klinischen Befunde zu veranschaulichen und die fatalen Folgen einer verzögerten Diagnose aufzuzeigen, beschreiben wir den Krankheitsverlauf von zwei betroffenen weiblichen Jugendlichen und geben eine Übersicht über das Krankheitsbild anhand der aktuellen Literatur.

Key words

Relapsing Polychondritis - airways - childhood - adolescents - bronchial stenosis

Schlüsselwörter

Rezidivierende Polychondritis - Atemwege - Kindheit - Adoleszenz - Bronchialstenose

* These authors contributed equally




Publication History

Article published online:
15 January 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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