Granulomatosis With Polyangiitis Mimicking Temporal Arteritis
Ali Dehghan1, Mahya Sadat Emami Meybodi2, Shokoofeh Fooladmotlagh2
1Division of Rheumatology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran.
Abstract:
This case represents the first diagnosis of pachymeningitis due to granulomatosis with polyangiitis (GPA) in an elderly Iranian man who initially presented with persistent daily headaches. PCR tests of cerebrospinal fluid for tuberculosis, brucellosis, and fungal infections all yielded negative results. Given the pachymeningitis pattern observed on brain MRI and the absence of infectious and lymphoma diseases, along with positive anti-PR3 and proteinuria (793 mg in a 24-h urine sample), a diagnosis of GPA was established. The patient was treated with five doses of pulse methylprednisolone and one dose of pulse cyclophosphamide (1 g). Additionally, prednisolone 60 mg daily, monthly pulse cyclophosphamide, a daily calcium-D tablet, and alendronate 70 mg weekly were prescribed. Subsequently, the patient's headaches, hearing loss, and vision loss were completely resolved. GPA should be considered in older individuals with persistent daily headaches, especially when pachymeningitis is evident. The use of contrast-enhanced brain MRI is an essential diagnostic tool in such cases.
Insights
Granulomatosis with polyangiitis (GPA) can cause pachymeningitis in elderly patients, presenting as daily headaches. Early diagnosis using brain MRI and ANCA testing is crucial for effective treatment and symptom resolution.
Area of Science:
- Neurology
- Rheumatology
- Nephrology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
- Pachymeningitis, inflammation of the dura mater, is an uncommon manifestation of GPA.
- Elderly patients with persistent headaches require thorough investigation for underlying causes.
Observation:
- An elderly Iranian male presented with persistent daily headaches.
- Cerebrospinal fluid PCR for infections was negative.
- Brain MRI revealed a pachymeningitis pattern.
Findings:
- Diagnosis of GPA was confirmed by positive anti-PR3 antibodies and proteinuria.
- Treatment involved pulse methylprednisolone, cyclophosphamide, and oral prednisolone.
- Patient experienced complete resolution of headaches, hearing loss, and vision loss.
Implications:
- GPA should be considered in the differential diagnosis of pachymeningitis in elderly patients with headaches.
- Contrast-enhanced brain MRI is a vital diagnostic tool for identifying GPA-related pachymeningitis.
- Prompt diagnosis and treatment of GPA can lead to significant clinical improvement.
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