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

Piotr Sajdak
ID
Email src
Aleksandra Krużel
ID
Marcel Stodolak
ID
Artur Bednarski
ID
Seweryn Ziajor
ID
Mikołaj Turski
ID
Klaudia Żurowska
ID
Kamil Kłos
ID
Justyna Tomasik
ID
Marika Dębik
ID
Łukasz Szydłowski
ID
Morphological Sciences Department, Institute of Medical Sciences, Medical College of University of Rzeszów, Rzeszów, Poland
Medical University of Silesia, Katowice, Poland
Specialist Hospital, E. Szczeklik, Tarnów, Poland
University Teaching Hospital, F. Chopin in Rzeszów, Rzeszów, Poland
Medical Center in Łańcut, Łańcut, Poland
Specialist Hospital, E. Szczeklik, Tarnów, Poland
Lower Silesian Specialist Hospital, T. Marciniak- Emergency Medicine Center, Wrocław, Poland
Medical University of Silesia, Katowice, Poland
Medical University of Lublin, Lublin, Poland
Provincial Specialist Hospital in Wrocław, Wrocław, Poland
Polish Red Cross Maritime Hospital, Gdynia, Poland
Received: 5 December 2023 / Revised: 31 January 2024 / Accepted: 17 January 2024 / Published: 30 June 2024

Abstract

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.

 

Cite

Sajdak P, Krużel A, Stodolak M et al. Unmasking the nature of granulomatosis with polyangiitis – a diagnostic odyssey revealed through a compelling case report. Eur J Clin Exp Med. 2024;22(2):462–468. doi: 10.15584/ejcem.2024.2.27.

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited