Granulomatosis with Polyangiitis in otorhinolaryngology – a case report

Introduction Granulomatosis with polyangiitis (GPA), formerly Wegener's, is characterized by necrotizing granulomatous inflammation affecting the respiratory tract, with necrotizing vasculitis in small to medium vessels [1]. It is an ANCA-associated vasculitis, with upper respiratory tract involvement in 70-100% of cases [2]. Otolaryngologists, often the first consulted specialists, need a thorough understanding of GPA's clinical presentation for timely diagnosis.

Methods Case report

Results A 66-year-old female with recurrent blood-stained nasal discharge and unresolved maxillary pain despite antibiotics presented. Vulnerable nasal mucous membranes, partial crusting, a swollen septum, and elevated CRP levels were observed. Hospitalization followed, with a CT scan revealing swelling of the nasal septum and raising suspicions of lung carcinoma or metastases due to pulmonary nodules. During her stay, the patient developed fever, weakness, and increased CRP levels. Extensive investigations, including panendoscopy and imaging, failed to identify a primary tumor. Histological analysis of the nasal septum and lung biopsy revealed necrotizing inflammation with vasculitis, confirming GPA. Elevated cANCA levels supported the diagnosis, leading to the patient's transfer for rheumatology-led immunosuppressive treatment.

Discussion This case emphasizes the crucial role of early histological sampling and a profound understanding of GPA's systemic symptoms for accurate diagnosis and timely intervention.

Publikationsverlauf

Artikel online veröffentlicht:
19. April 2024

• References

• 1 Jennette JC. et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11
• 2 Greco A. et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol 2016; 29 (02) 151-9