Hypertrophic Pachymeningitis: An Unusual Cause of Headache

Hypertrophic pachymeningitis (HP) is a rare condition characterized by inflammation and thickening of the dura mater. It can be idiopathic or secondary to various causes, including infections, tumors, or systemic inflammatory diseases. Diagnosis is challenging due to its rarity and the overlap of symptoms with other conditions. We present the case of a 42-year-old Hispanic woman with diabetes mellitus type 2 and end-stage kidney disease who presented with chest pain, dry cough, mild dyspnea, and chronic occipital headaches. Physical examination revealed cranial VI nerve palsy. Imaging showed pulmonary cavitary lesions and mediastinal lymphadenopathy. Elevated inflammatory markers and positive autoimmune tests, including rheumatoid factor and antineutrophil cytoplasmic antibody (ANCA), led to further investigation. Brain imaging revealed dural thickening, confirming HP. The patient's medical history revealed double ANCA positivity and a lung biopsy confirmed granulomatous pneumonitis. A diagnosis of ANCA-associated vasculitis (granulomatosis with polyangiitis (GPA)) was established, and treatment with rituximab and high-dose corticosteroids led to symptom improvement. GPA rarely involves meningeal inflammation, but severe and persistent headaches are common early symptoms. Inflammatory markers are often elevated, and around two-thirds of HP cases related to GPA have positive serum ANCA. MRI is the primary diagnostic tool, with characteristic findings of dural thickening and contrast enhancement. This case highlights HP as a rare cause of chronic headaches and the importance of a comprehensive medical history in diagnosis. Early recognition and treatment are crucial for improving outcomes in GPA-related HP.