| | Author | Year | Age/sex | Clinical features | Cerebrovascular involvement | HCP | Nose | Ear | Eye | Renal | Lung | MPO-ANCA | PR3-ANCA | Treatment | Outcome |
| | Cruz and Segal [8] | 1997 | 71/M | Headache, nausea, CN VII | SAH | − | − | + | − | + | + | + | nd | mPSL, CY | Improved |
| | Nagashima et al. [11] | 2000 | 53/F | Paraplagia, fever, CN I, II, VI, VII, VIII | ICA stenosis, loss of ophthalmic artery | + | − | + | + | + | − | + | + | PSL, AZP | Dead |
| | Thajeb and Tsai [12] | 2001 | 65/M | Headache, hyperesthesia, fever, CN III, V | ICA stenosis, cavernous sinus syndrome | + | + | + | + | − | − | − | + | PSL, CY | Improved |
| | Sivakumar and Chandrakantan [5] | 2002 | 49/M | Hemiparesis, seizure, fever, CN X | Infarction of pons and temporal lobe | − | + | + | + | + | − | nd | + | CY | Improved |
| | Fam et al. [3] | 2003 | 63/F | Headache, CN II | Narrowing of ophthalmic artery | + | + | − | + | − | − | + | − | PSL, CY, MTX | Improved |
| | Weijtens et al. [13] | 2004 | 45/F | Hearing loss, Horner’s syndrome, CN VI, X | Cavernous sinus involvement | + | + | − | + | − | − | + | − | DEX, AZP | Improved |
| | Takei et al. [9] | 2004 | 34/M | Headache, fever | SAH | − | + | − | − | + | + | − | + | mPSL, PSL, CY | Improved |
| | Peng and Wang [6] | 2012 | 58/M | Headache, ataxia, motor weakness, meningeal signs, CN III, VI, IX, X | Cerebral infarction | + | − | − | + | + | + | + | + | mPSL, PSL, CY | Improved |
| | Yajima et al. [7] | 2015 | 75/M | Headache, hemiparesis, loss of consciousness, hoarseness, hearing loss, fever | Hemorrhagic infarction | + | + | + | − | + | + | nd | + | Frontal lobectomy, mPSL, PSL, AZP | Improved |
| | Present case | 2017 | 59/M | Headache, CN VI | ICA stenosis | + | + | − | + | − | − | − | − | mPSL, PSL, CY, AZP | Improved |
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CN: cranial nerve affected, SAH: subarachnoid hemorrhage, ICA: internal carotid artery, HCP: hypertrophic cranial pachymeningitis, +: affected or positive, −: not affected or negative, nd: not detected, mPSL: methylprednisolone, CY: cyclophosphamide, PSL: prednisolone, AZP: azathioprine, MTX: methotrexate, DEX: dexamethasone.
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