Studies | Age/sex | Site of Hemorrhage | Clinic/time before Bleeding† | pANCA/MPO | Inflamm. Markers | Eo Count, /mm3 | Coag. | Angiography/CT Angiogram | Asthma | Neuropathy | Rhinitis/ sinusopathy | Pulm. Infiltrates | HTN | Therapy | Outcome |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Maloon, et al1 | 39/F | Spinal, SAH | Yes/14 mos | NR | NR | NR | Normal | Not performed | Yes | No | Yes | Yes | Yes* | PRED + CYC | Death |
Chang, et al2 | 47/F | SAH, IVH | Yes/240 mos | NR | ↑ESR, ↑CRP | 22,000 | NR | Not performed | Yes | Yes | Yes | Yes | NR | PRED, CYC | Death |
Liou, et al3 | 27/M | Bilateral ICH | Yes/36 mos | pANCA | ↑ESR, ↑CRP | 13,250 | Normal | Not performed | Yes | Yes | Yes | Yes | Yes* | MP, CYC, PRED | Imp. |
Nishino, et al4 | 59/M | ICH | — | — | — | — | — | — | — | — | — | — | No | — | — |
Ojeda, et al5 | 48/M | ICH | Yes/108 mos | (−) | ↑ESR | 6000 | NR | Not performed | Yes | Yes | Yes | Yes | No | PRED, CYC | Imp.I |
Calvo-Romero, et al6 | 47/F | SAH | Yes/72 mos | MPO | ↑ESR, ↑CRP | 15,200 | Normal | Vasculitis | Yes | Yes | Yes | No | No | PRED, CYC | Imp. |
Tyvaert, et al7 | 47/F | SAH, bilateral ICH | Yes/NR | MPO | ↑ESR, ↑CRP | 5600 | Normal | Vasculitis | Yes | Yes | Yes | No | No | MP + CYC, PRED | NR |
Sakamoto, et al8 | 36/F | SAH | Yes/96 mos | (−) | ↑CRP | 9515 | NR | Dissecting aneurysm, vasculitis | Yes | Yes | Yes | No | No | Coil embolization, PRED | Imp. |
Mishira, et al9 | 45/M | ICH, SAH, IVH | Yes/24 mos | (−) | ↑ESR | 14,600 | NR | Normal | Yes | Yes | Yes | No | Yes* | PRED, CYC | Imp. |
Winek, et al10 | 55/M | ICH | Yes/6 mos | pANCA | — | ↑Eo count | — | — | Yes | — | — | Yes | — | PRED | Imp. |
Sheerin, et al11 | 37/F | SAH | No/8 mos post-SAH | MPO | ↑ESR, ↑CRP | ↑Eo count | Normal | Vasculitis | Yes (post) | No | Yes (post) | Yes (post) | Yes* | MP, PRED, AZA (post) | Imp. |
Nam, et al12 | 32/M | ICH | NR | pANCA | NR | ↑Eo count | — | Normal | Yes | Yes | Yes | Yes | NR | CYC, PRED, AZA | Imp. |
Shimizu, et al13 | 60/F | SAH | Yes/108 mos | — | — | ↑Eo count | — | Normal | Yes | Yes | No | No | — | — | Imp. |
Mencacci, et al14 | 29/M | ICH | Yes/Some mos | (−) | ↑CRP | 6685 | Normal | Normal | Yes | Yes | Yes | No | No | MP, CYC, PRED, AZA | Imp. |
Sairanen, et al15 | 49/M | ICH | Yes/NR | MPO, low PR3 | ↑CRP | 9800 | Mutation§, minor ASD with shunting | Minor infarctions, no aneurysm or vasculitis | Yes | No | Yes | Yes | No | MP, CYC, PRED, AZA | Imp. |
Halliday, et al16 | 43/M | ICH, IVH | Yes/At least 36 mos | (−) | NR | 13,700 | No anticoagulant use, normal | Normal | Yes | Yes | Yes | Yes | Yes* | MP, CYC, PRED | Imp. |
Go, et al17 | 39/M | SAH, IVH | Yes/7 mos | MPO | ↑ESR, ↑CRP | 3572 | NR | Intracranial vertebral artery dissection | Yes | Yes | Yes | No | No | MP, CYC, PRED | Death |
Go, et al17 | 46/F | ICH | Yes/120 mos | MPO | ↑ESR, ↑CRP | 9411 | NR | Normal | No | No | Yes | Yes | NR | MP, PRED, deflazacort | Imp. |
Kukita, et al18 | 40/F | Spinal hematoma | At onset | pANCA | NR | 15,000 | Normal | Not performed | Yes | Yes | Yes | Yes | No | MP | Imp. |
Menditto, et al19 | 64/F | SAH | Yes/72 mos | MPO | ↑ESR, ↑CRP | 1170 | Normal | PICA aneurysm, vasculitis | Yes | Yes | Yes | NR | No | Coil embolization, PRED, CYC | Imp. |
Murthy, et al20 | 58/M | ICH | Yes/NR | MPO | NR | ↑Eo count | NR | Not performed | Yes | No | Yes | Yes | Yes* (170/88) | MP, CYC, PRED | Imp. |
Yeo, et al21 | 51/F | Spinal hematoma | Yes/NR | MPO | NR | NR | NR | Not performed | Yes | Yes | NR | NR | NR | CYC | Imp. |
Ito, et al22 | 68/M | SAH | Yes/NR | — | — | — | — | Cerebral artery dissection | — | — | — | — | — | — | Imp. |
Diamanti, et al23 | 31/F | SAH | Yes/NR | MPO | ↑ESR, ↑CRP | 13,230 | NR | Not performed | Yes | Yes | Yes | No | NR | MP, PRED, RTX | Imp. |
Taormina, et al24 | 58/M | SAH | Yes/84 mos | pANCA | ↑ESR, ↑CRP | 5700 | NR | Vasculitis, acute ischemic lesions | Yes | No | Yes | Yes | No | PRED | Imp. |
Lee, et al25 | 48/F | SAH, cerebellar, IVH | Yes/12 mos | MPO | NR | 1553 | NR | No aneurysm; vertebral artery vasculitis? | No | Yes | No (otitis, mastoiditis) | No | No | MP, PRED, CYC | Death |
Aly, et al26 | 59/M | ICH, ischemic infarcts | Yes/NR | MPO | ↑ESR, ↑CRP | Normal count | NR | Not performed | Yes | Yes | No | No | Yes | PRED | Imp. |
Our case | 48/M | ICH | Yes/18 mos | MPO | ↑ESR, ↑CRP | 8110 | Normal | Normal | Yes | Yes | Yes | No | Yes | MP + PRED + CYC | Imp. |
↵† Time before bleeding: Time since the first symptom attributed to EGPA to CNS hemorrhage presentation.
↵* Hypertension coinciding with the bleeding.
↵§ Heterozygotic clotting factor V R506Q mutation. ANCA: antineutrophil cytoplasmic antibodies; pANCA: peripheral ANCA; MPO: myeloperoxidase; Eo: eosinophils; CT: computed tomography; F: female; M: male; SAH: subarachnoid hemorrhage; IVH: intraventricular hemorrhage; ICH: intracerebral hemorrhage; NR: not reported; —: data not reported in the abstract; (−): negative; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; ↑Eo count: hypereosinophilia; ASD: atrial septal defect; PICA: posterior inferior cerebellar artery; PRED: oral prednisone; CYC: cyclophosphamide; MP: methylprednisolone; AZA: azathioprine; RTX: rituximab; Inflamm.: inflammatory; Imp.: improvement; Pulm.: pulmonary; Coag.: coagualtion.