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Granulomatosis With Polyangiitis (GPA): Isolated Nasal Bridge Involvement.

Joud Enabi¹, Waqar Sharif¹, Maneesh Mannem¹

¹Internal Medicine, Texas Tech University Health Sciences Center, Odessa, USA.

|July 23, 2024

Summary

Granulomatosis with polyangiitis (GPA), a rare autoimmune vasculitis, can present atypically. Early diagnosis and treatment are crucial for managing this systemic condition and preventing severe damage.

Keywords:

autoimmune gpa isolated nasal bridge rheumatology vasculitis

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Area of Science:

Rheumatology
Immunology
Otolaryngology

Background:

Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune vasculitis affecting small blood vessels.
GPA commonly involves the respiratory tract and kidneys, but presentations can be variable.
Diagnosis relies on clinical evaluation, serological tests (ANCA, ANA), and biopsy.

Observation:

A 68-year-old morbidly obese woman with chronic sinusitis, hypertension, and hyperlipidemia presented with a saddle nose deformity.
She lacked typical respiratory or renal symptoms of GPA.
Nasal septal biopsy confirmed GPA despite atypical clinical presentation.

Findings:

Positive antineutrophil cytoplasmic antibodies (ANCA) and antinuclear antibodies (ANA) were detected.
The patient was treated with methotrexate and folic acid.
The case highlights the diverse clinical manifestations of GPA.

Implications:

This case emphasizes the importance of considering GPA even with non-classical symptoms.
Early diagnosis and prompt treatment are vital to prevent irreversible organ damage.
Recognizing atypical presentations of GPA improves patient outcomes and disease management.