Table 1.

Description of the case reports.

StudiesAge/sexSite of HemorrhageClinic/time before BleedingpANCA/MPOInflamm. MarkersEo Count, /mm3Coag.Angiography/CT AngiogramAsthmaNeuropathyRhinitis/ sinusopathyPulm. InfiltratesHTNTherapyOutcome
Maloon, et al139/FSpinal, SAHYes/14 mosNRNRNRNormalNot performedYesNoYesYesYes*PRED + CYCDeath
Chang, et al247/FSAH, IVHYes/240 mosNR↑ESR, ↑CRP22,000NRNot performedYesYesYesYesNRPRED, CYCDeath
Liou, et al327/MBilateral ICHYes/36 mospANCA↑ESR, ↑CRP13,250NormalNot performedYesYesYesYesYes*MP, CYC, PREDImp.
Nishino, et al459/MICHNo
Ojeda, et al548/MICHYes/108 mos(−)↑ESR6000NRNot performedYesYesYesYesNoPRED, CYC Imp.I
Calvo-Romero, et al647/FSAHYes/72 mosMPO↑ESR, ↑CRP15,200NormalVasculitisYesYesYesNoNoPRED, CYCImp.
Tyvaert, et al747/FSAH, bilateral ICHYes/NRMPO↑ESR, ↑CRP5600NormalVasculitisYesYesYesNoNoMP + CYC, PREDNR
Sakamoto, et al836/FSAHYes/96 mos(−)↑CRP9515NRDissecting aneurysm, vasculitisYesYesYesNoNoCoil embolization, PREDImp.
Mishira, et al945/MICH, SAH, IVHYes/24 mos(−)↑ESR14,600NRNormalYesYesYesNoYes*PRED, CYCImp.
Winek, et al1055/MICHYes/6 mospANCA↑Eo countYesYesPREDImp.
Sheerin, et al1137/FSAHNo/8 mos post-SAHMPO↑ESR, ↑CRP↑Eo countNormalVasculitisYes (post)NoYes (post)Yes (post)Yes*MP, PRED, AZA (post)Imp.
Nam, et al1232/MICHNRpANCANR↑Eo countNormalYesYesYesYesNRCYC, PRED, AZAImp.
Shimizu, et al1360/FSAHYes/108 mos↑Eo countNormalYesYesNoNoImp.
Mencacci, et al1429/MICHYes/Some mos(−)↑CRP6685NormalNormalYesYesYesNoNoMP, CYC, PRED, AZAImp.
Sairanen, et al1549/MICHYes/NRMPO, low PR3↑CRP9800Mutation§, minor ASD with shuntingMinor infarctions, no aneurysm or vasculitisYesNoYesYesNoMP, CYC, PRED, AZAImp.
Halliday, et al1643/MICH, IVHYes/At least 36 mos(−)NR13,700No anticoagulant use, normalNormalYesYesYesYesYes*MP, CYC, PREDImp.
Go, et al1739/MSAH, IVHYes/7 mosMPO↑ESR, ↑CRP3572NRIntracranial vertebral artery dissectionYesYesYesNoNoMP, CYC, PREDDeath
Go, et al1746/FICHYes/120 mosMPO↑ESR, ↑CRP9411NRNormalNoNoYesYesNRMP, PRED, deflazacortImp.
Kukita, et al1840/FSpinal hematomaAt onsetpANCANR15,000NormalNot performedYesYesYesYesNoMPImp.
Menditto, et al1964/FSAHYes/72 mosMPO↑ESR, ↑CRP1170NormalPICA aneurysm, vasculitisYesYesYesNRNoCoil embolization, PRED, CYCImp.
Murthy, et al2058/MICHYes/NRMPONR↑Eo countNRNot performedYesNoYesYesYes* (170/88)MP, CYC, PREDImp.
Yeo, et al2151/FSpinal hematomaYes/NRMPONRNRNRNot performedYesYesNRNRNRCYCImp.
Ito, et al2268/MSAHYes/NRCerebral artery dissectionImp.
Diamanti, et al2331/FSAHYes/NRMPO↑ESR, ↑CRP13,230NRNot performedYesYesYesNoNRMP, PRED, RTXImp.
Taormina, et al2458/MSAHYes/84 mospANCA↑ESR, ↑CRP5700NRVasculitis, acute ischemic lesionsYesNoYesYesNoPREDImp.
Lee, et al2548/FSAH, cerebellar, IVHYes/12 mosMPONR1553NRNo aneurysm; vertebral artery vasculitis?NoYesNo (otitis, mastoiditis)NoNoMP, PRED, CYCDeath
Aly, et al2659/MICH, ischemic infarctsYes/NRMPO↑ESR, ↑CRPNormal countNRNot performedYesYesNoNoYesPREDImp.
Our case48/MICHYes/18 mosMPO↑ESR, ↑CRP8110NormalNormalYesYesYesNoYesMP + PRED + CYCImp.
  • 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.