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Pathological findings of hypertrophic pachymeningitis associated with eosinophilic granulomatosis with polyangiitis
Wataru Shiraishi1, Yoshitaka Tsujimoto2, Tomoko Shiraishi3
1Department of Neurology, Kokura Memorial Hospital, Kitakyushu, Japan watarus@neuro.med.kyushu-u.ac.jp.
Abstract:
The most common neurological manifestation of eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is mononeuritis multiplex caused by small-vessel vasculitis. In contrast, central nervous system involvement is rare. Among EGPA-associated central nervous system disorders, there are only a few reported cases of hypertrophic pachymeningitis (HP). Here, we report a patient with EGPA with headache and ophthalmoplegia who presented with HP and had a dural biopsy. The biopsy specimen showed lymphocytic inflammatory cell infiltration without EGPA-specific findings, that is, eosinophilic infiltration, granuloma or angiitis. To the best of our knowledge, there are no previous reports of EGPA-associated HP pathology. Here, we report the first case presentation of a patient with EGPA-associated HP with pathological findings.
Insights
Eosinophilic granulomatosis with polyangiitis (EGPA) rarely affects the central nervous system. This study presents the first pathological findings of EGPA-associated hypertrophic pachymeningitis (HP) in a patient with neurological symptoms.
Area of Science:
- Neurology
- Pathology
- Immunology
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, commonly presents with neurological issues like mononeuritis multiplex.
- Central nervous system (CNS) involvement in EGPA is infrequent, with hypertrophic pachymeningitis (HP) being exceptionally rare.
Observation:
- A patient with EGPA experienced headache and ophthalmoplegia, leading to the diagnosis of HP.
- A dural biopsy was performed, revealing lymphocytic inflammatory cell infiltration.
Findings:
- The biopsy findings lacked characteristic EGPA markers such as eosinophilic infiltration, granuloma, or angiitis.
- This represents the first reported case of EGPA-associated HP with detailed pathological examination.
Implications:
- This case expands the understanding of CNS manifestations in EGPA.
- Further research is needed to elucidate the specific pathological mechanisms of EGPA-associated HP.
