Respir Case Rep. 2024; 13(3): 110-114 | DOI: 10.5505/respircase.2024.93195

Granülomatöz Polianjit ve Kartagener Sendromunun Nadir Birlikteliði: Ýlginç Bir Olgu Sunumu

Gülce Cansu Serin, Fatma Arslan, Elif Selçuk, Miraç Öz, Aslýhan Gürün Kaya, Serhat Erol, Aydýn Çiledað, Akýn Kaya
Ankara Üniversitesi Týp Fakültesi Göðüs Hastalýklarý Anabilim Dalý, Ankara

Primer siliyer diskinezi (PSD), yetersiz siliyer motilite kaynaklý tekrarlayan solunum yolu enfeksiyonlarý ile karakterize çoðunlukla otozomal resesif geçiþli bir bozukluktur. Granülamatöz polianjit, sýklýkla akciðerlerde kaviter nodüller tutulumlar, kitleler ve konsolidasyonlar yapan damar duvarýnda granülamatöz inflamasyon ile seyreden nekrotizan bir vaskülittir. Yirmi dört yaþýnda kadýn hasta, kliniðimize dispne, prodüktif öksürük ve plöretik göðüs aðrýsý ile baþvurdu. Hastanýn situs inversus, bronþektazi ve sinüzit bulgularý göz önüne alýnarak, genetik testler ile Kartagener Sendromu tanýsý konuldu. Toraks tomografisinde kaviteleþen nodüllerinin olmasý, PR3-ANCA pozitifliði ve cilt biyopisi sonucunda lökositik vaskülit saptanmasý üzerine PSD ve granülamatöz polianjit tanýsý doðrulandý.

Anahtar Kelimeler: Granülomatöz Polianjit, Kartagener Sendromu, Primer Siliyer Diskinezi

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Rare Coexistence of Kartagener Syndrome and Granulomatous Polyangiitis: A Compelling Case Report

Gülce Cansu Serin, Fatma Arslan, Elif Selçuk, Miraç Öz, Aslýhan Gürün Kaya, Serhat Erol, Aydýn Çiledað, Akýn Kaya
Department of Chest Diseases, Ankara University Faculty of Medicine, Ankara, Türkiye

Primary ciliary dyskinesia (PCD) is predominantly an autosomal recessive disorder that is characterized by recurrent respiratory infections stemming from impaired ciliary motility. Granulomatous polyangiitis is a necrotizing vasculitic disease marked by granulomatous inflammation in the vascular wall that often manifests in the lungs with cavitating nodules, masses and consolidations. A 24-year-old female patient presented to our clinic complaining of dyspnea, productive cough and pleuritic chest pain, and was diagnosed with Kartagener Syndrome based on her situs inversus, bronchiectasis and sinusitis. Subsequent genetic tests and further clinical evaluations, including thoracic CT, revealed findings of cavitating nodules, PR3-ANCA positivity and leukocytic vasculitis from a skin biopsy pathology, confirming the coexistence of PCD and granulomatous polyangiitis.

Keywords: Granulomatosis with polyangiitis, Kartagener’s Syndrome, Primary ciliary

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