Unmasking the nature of granulomatosis with polyangiitis – a diagnostic odyssey revealed through a compelling case report

Obrazek miniatury
Sajdak, Piotr
Krużel, Aleksandra
Stodolak, Marcel
Bednarski, Artur
Ziajor, Seweryn
Turski, Mikołaj
Żurowska, Klaudia
Kłos, Kamil
Tomasik, Justyna
Dębik, Marika
Tytuł czasopisma
Tytuł tomu
Publishing Office of the University of Rzeszow
Introduction and aim. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, presents a formidable challenge in the realm of autoimmune diseases. Granulomatosis, characterized by vasculitis and granuloma formation, exhibits diverse clinical manifestations. The rarity of GPA is evident, with an estimated incidence between 0.4 and 11.9 cases per 1 million person-years. The aim of this report is to show the complex diagnostic challenges inherent in GPA, demonstrating the diagnostic process from initial symptoms. Description of the case. This case report unfolds the diagnostic journey of a 52-year-old Caucasian male. The presented case, initially suspected as a respiratory infection, led to a comprehensive investigation owing to persistent symptoms, abnormal blood counts, and elevated inflammatory markers. This narrative aims to depict the patient’s diagnostic journey. Key diagnostic tools include ANCA testing, imaging studies, and tissue biopsy. Pulmonary nodules, lymphangitic changes, and renal involvement culminating in a GPA diagnosis confirmed by positive ANCA and anti-PR3 antibodies. The successful management of this case involved a tailored therapeutic regimen, including cyclophosphamide and methylprednisolone, addressing both vasculitic and renal components. Conclusion. This case contributes to the understanding of atypical presentations of GPA, emphasizing the importance of a holistic and dynamic diagnostic approach.
Written informed consent for publication was obtained from the patient. We complied with the policy of the journal on ethical consent.
Słowa kluczowe
autoimmune diseases , granulomatosis with polyangiitis , vasculitis
European Journal of Clinical and Experimental Medicine T. 22, z. 2 (2024), s. 462-468