Abstract
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune vasculitis that primarily affects small to medium-sized blood vessels, typically involving the respiratory tract and kidneys. However, central nervous system involvement, particularly in the form of pachymeningitis, is an exceptionally rare presentation. This case report details a 55-year-old female who presented with pachymeningitis as the initial manifestation of GPA. The patient exhibited non-specific symptoms, including ear pain, voice changes such as hoarseness of voice, and dysphagia, suggestive of neurological deficits affecting lower cranial nerves IX, X, and XII. Diagnostic imaging, particularly MRI, revealed characteristic smooth dural thickening and enhancement, suggesting an inflammatory etiology. Laboratory investigations, including positive cytoplasmic-antineutrophil cytoplasmic autoantibody and anti-proteinase 3 antibodies, confirmed the diagnosis of GPA. The patient was treated with high-dose corticosteroids and mycophenolate mofetil, followed by cyclophosphamide due to a relapse, resulting in significant improvement in her condition. This case underscores the importance of considering GPA in the differential diagnosis of pachymeningitis and highlights the diagnostic challenges posed by its nonspecific symptoms. Early recognition and a multidisciplinary approach are crucial for effective management and prevention of severe neurological complications. The report also emphasizes the need for adherence to treatment regimens to avoid relapses and manage the disease effectively.