Hyperthrophic cranial pachymeningitis in Wegener granulomatosis: 3 case report and review of liter-atures / 中华风湿病学杂志

ABSTRACT

Objective To study the clinical, laboratory, radiological and pathological findings of patients with hypertrophic cranial pachymeningitis (HCP) in Wegner's granulomatosis (WG) to improve the recognition of the disease, even when it occurs in limited form. Methods Three patients were described and English literatures of biopsy-proven pachymeningitis in WG were reviewed. Results The features of WG-associated pachymeningitis included ① Frequently occurred early in the course of active limited WG; ② Commonly presented with sever headache and cranial neuropathies in the absence of other meningeal irritative signs; ③ Variable cerebrospinal fluid findings with mild predominantly lymphocytic pleocytosis and elevated protein concentration were major laboratorg findings; ④Elevated ESR and positive serum anti-neutrophilic cytoplasmic antibody (ANCA) could be found in most patients; ⑤ Gadolinium-enhanced brain MRI is very senitive in the detection of pachymeningitis; ⑥A dural biopsy showed granulomatous necrotizing inflammation, giant cell, and evidence of vasculitis;⑦ A favorable response to standard treatment with corticosteroid, cyclophosphamide or other cytotoxic drugs could be observed. Conclusion HCP may be the initial or cardinal manifestation of the limited form of WG. Early diagnosis by ANCA, MRI and dural biopsy may facilitate diagnosis Corticosteroid and immunosupressant are the choices of treatment.