TABLE 2

Summary of Reported Patients Who Had PAI Associated With Juvenile BD

Ozen et al5 2010Cohle and Colby6 2002Vivante et al7 2009Alkaabi and Pathare8 2011Uzun et al9 2008Uzun et al9 2008Seyahi et al10 2012Bahabri et al11 1996
SexMaleMaleMaleMaleMaleMaleMaleNA
Age at BD diagnosis, y14101410171712<16
Initial symptom of PAIHemoptysisHemoptysisFever, wt loss, oral ulcers, and hemoptysisNAHemoptysis, chest pain, fever, and fatigueHemoptysis, chest pain, cough, sputum, wt loss, and abdominal painHemoptysisNA
ISG criteriaYesNoYesNANANANoYes
ICBD (revised)YesNoYesNANANAYesYes
PEDBD criteriaYesNoYesNANANAYesYes
Age at PAI, y17101415171712NA
Time frame between BD diagnosis and PAI, mo480060000NA
Type of pulmonary involvementPAAPAAPAAPAAPAAPATPAAPAA
Other vascular involvementCardiac thrombusCardiac thrombusDVTHepatic vein thrombosis- BCSNA
PathergyNANA+NANANA
Oral ulcer+++NANA++
Genital ulcer+NANANA+
Eye lesionNANANA+
Skin lesionsErythema nodosumPapulopustular lesionsNANANAErythema nodosumErythema nodosum
HLA-B5PositiveNANANANANANANA
Immunosuppressive treatmentPulse methylprednisolone (intravenous), prednisolone (po), cyclophosphamide (intravenous)Colchicine, pulse methylprednisolone (intravenous), prednisolone (po), cyclophosphamide (intravenous)cyclophosphamideCorticosteroid, colchicumCorticosteroidPrednisolone, cyclophosphamide (intravenous), infliximab (subcutaneous)NA
Anticoagulation+ (after resolution of hemoptysis)NAEnoxaparin + coumadinNA
Follow-up and outcome18-mo follow-up with no vascular relapseDead before diagnosis because of massive hemoptysis7-mo follow-up with no vascular relapseDead after 2 y of diagnosis because of massive hemoptysisDead at 16 mo because of massive hemoptysisAlive at 7 moDead at 12 mo because of hepatic failureNA
  • DVT, deep venous thrombosis; po, per oral; NA, not available.