Development of ANCA-associated vasculitis after COVID-19 in a patient with Lynch syndrome and atypical hemolytic uremic syndrome

Year 2022, Volume: 47 Issue: 2, 898 - 900, 30.06.2022

Hakan Ozer , Yasin Öztürk , Fethi Yönet

https://doi.org/10.17826/cumj.1094334

Abstract

COVID-19 can be asymptomatic or cause very serious clinical pictures that can affect multiple systems. As the disease progresses, our clinical experience and the clinical presentations and management strategies we encounter also become more diverse and distinct. Anti Neutrophil Cytoplasmic Antibody (ANCA) positivity is particularly used as an indicator in the diagnosis of small vessel vasculitis such as granulomatous polyangitis, microscopic poliarteritis nodosa (m-PAN), and eosinophilic granulomatous polyangiitis (eGPA). ANCA-associated vasculitis can present with severe respiratory symptoms. Management of this group of patients is very difficult because COVID -19 pneumonia can very often be mistaken for these complaints and leads to serious limitations in the immunosuppressive treatment that should be used in the management of ANCA-associated vasculitis. At the beginning of the epidemic, our knowledge was limited by the increased susceptibility to infections and more severe disease in patients receiving immunosuppressive therapy for the combination of COVID -19 and vasculitic syndromes, whereas subsequently vasculitis cases were reported secondary to infections. In our case, the diagnoses of atypical hemolytic uremic syndrome (A-HUS) and Lynch syndrome were made, and the diagnosis of vasculitis due to new-onset ANCA positivity and alveolar hemorrhage after COVID -19 pneumonia was successfully treated with immunosuppressants.

Keywords

Covid-19, ANCA-ASSOCIATED VASCULITIS, LYNCH SYNDROME

Lynch sendromu ve atipik hemolitik üremik sendrom ile takipli bir hastada COVID-19 sonrası ANCA ilişkili vaskülit gelişimi

Abstract

COVID-19 asemptomatik olabileceği gibi birden fazla sistemi etkileyebilecek çok ciddi klinik tablolara da neden olabilir. Hastalık ilerledikçe, klinik deneyimimiz ve karşılaştığımız klinik sunumlar ve yönetim stratejileri de daha çeşitli ve farklı hale gelir. ANCA pozitifliği özellikle granülomatöz polianjit, mikroskobik PAN ve eozinofilik granülomatöz polianjit gibi küçük damar vaskülitlerinin tanısında bir gösterge olarak kullanılır. ANCA ile ilişkili vaskülit şiddetli solunum semptomları ile kendini gösterebilir. Bu hasta grubunun yönetimi çok zordur çünkü COVID -19 pnömonisi sıklıkla bu şikayetlerle karışabilir ve ANCA ile ilişkili vaskülit tedavisinde kullanılması gereken immünosupresif tedavide ciddi kısıtlamalara yol açar. Salgının başlangıcında, bilgimiz, COVID-19 ve vaskülitik sendromların kombinasyonu için immünosupresif tedavi alan hastalarda enfeksiyonlara karşı artan duyarlılık ve daha şiddetli hastalık nedeniyle sınırlıyken, daha sonra enfeksiyonlara sekonder vaskülit vakaları bildirilmiştir. Olgumuzda atipik hemolitik üremik sendrom (A-HUS) ve Lynch sendromu tanıları ile takip edilmekteyken, COVID -19 pnömonisi sonrası yeni başlayan ANCA pozitifliğine bağlı vaskülit ve alveoler kanama tanısı alarak immünosupresif tedavilerle başarıyla tedavi edilen zor ve ilginç bir vaka anlatılmıştır.

Keywords

Covid-19, ANCA İlişkili Vaskülit, Atipik-HUS, Lynch Sendromu

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