Table 1

Summary of clinical and patient data

Case no1234567
Age, sex, nationality30 years, male Turkish31 years, female German30 years, female Greek22 years, male German36 years, male Italian38 years, male Turkish27 years, female Turkish
History, previous therapy6/83 recurrent oral aphthosis, 6/89 panuveitis R/L, 2/90 skin lesions, orogenital aphthosis, recurrent panuveitis, 11/92 AZA/CSA/pred for 18 months. Development of Kaposi’s sarcoma4/76 recurrent erosive arthrits, oral aphthosis, skin lesions, IFN γ 1986–90 effective11/91 recurrent oral aphthosis, 12/94 panuveitis R, CSA effective, stopped because of hirsutism3/85 recurrent oral aphthosis, 2/94 panuveitis R/L, 11/95 orogenital aphthosis, skin lesions, prednisolone (40 mg/day) partially effective1/90 recurrent orogenital aphthosis, gonarthritis, IFN γ effective, discontinued in 19941974 recurrent oral aphthosis, 1/84 panuveitis R/L, recurrent under prednisolone 1989 stroke 1994 panuveitis L, prednisolone ineffective
BD diagnosed2/902/8612/9411/951/901/847/96
Referral4/94 (panuveitis R/L after cessation of immunosuppressive therapy)12/93 (occlusive vasculitis L, prednisolone ineffective)2/95 (relapse of panuveitis R after cessation of CSA)12/95 (relapse of panuveitis R/L under 20 mg prednisolone)1/96 (panuveitis L, no therapy)8/96 (subacute relapse of panuveitis R/L)8/96 (panuveitis L)
Pathergy testnegativenegativenegativepositivenegativenegativenegative
HLA-B51positivenegativepositivepositivepositivepositivenegative
IFNα2a therapy start (s) maintainance (m) duration (d) complete remission (cr)s: 18×106 IU/day (for Kaposi’s sarcoma) m:3×106 IU 3 times weekly
d: 40 months
cr: for 38 months
s: 6×106 IU/day m : 3–6×106 IU every other day d: 1 year, then discontinued.
cr: for 22 months after cessation
s: 6×106 IU/day m: 3–6×106 IU every other day d: 26 months then discontinued
cr: for 4 months after cessation
s: 6×106 IU/day
m: 3–6×106 IU every other day
d: 20 months
cr: for 18 months
s: 3×106IU/day m: 3×106 IU every other day d: 20 months
cr: for 18 months
s: 3×106 IU/day
m: 3×106 IU every other day
d: 21 months
cr: for 17 months
s: 6×106 IU/day m: 3×106 IU every other day d: 14 months
cr: for 6 months after cessation