Oral granulomatosis with polyangiitis a systematic review

Area of Science:

• Rheumatology and Immunology
• Oral Medicine
• Nephrology

Background:

• Granulomatosis with polyangiitis (GPA) is a rare multisystemic inflammatory disease characterized by vasculitis affecting small- and medium-sized vessels.
• GPA typically involves the upper and lower airways, as well as the kidneys, with an unknown etiology potentially linked to genetic predisposition and environmental stimuli.

Approach:

• A comprehensive database search was conducted to gather data on GPA cases.
• Variables analyzed included demographics, disease localization, histopathological findings, antineutrophil cytoplasmic autoantibody (c-ANCA) test results, treatment protocols, and patient follow-up.

Key Points:

• Fifty-two cases were analyzed, predominantly affecting females (57.7%) and white individuals (59.6%), with a mean age of 49.6 years.
• The most frequent oral manifestation was maxillary gingival involvement (28.8%), presenting as "strawberry gingivitis" (61.5%) with pain as a common symptom (50%).
• C-ANCA positivity was observed in 71.2% of cases, and the standard treatment involved prednisone and cyclophosphamide (51.9%).

Conclusions:

• Establishing an early diagnosis and initiating prompt treatment for GPA are essential due to its potential for significant morbidity and mortality.
• Oral clinicians should consider GPA in the differential diagnosis of oral lesions, given its frequent gingival manifestations.
• The study reported an 88.5% survival rate at an average follow-up of 23.6 months, highlighting the importance of timely intervention.