Table 1: Demographics, Clinical response and follow-up to IVIg treatment.

Age/Gender Associated Findings Duration of IVIG Therapy Therapy Prior to IVIG Therapy After IVIG Response#
45
Female [7]
ILD 5 day course
(400 mg/kg/day) total 2gm/kg
Two Courses, one month apart
PSL 60/day
CSA 250/day
PSL taper
(60 to 20 - 4 months)
CSA 250/day
Survived
(Condition not otherwise described)
72
Male [7]
ILD 5 day course
(400 mg/kg/day) total 2gm/kg
One Course
MPD*
PSL 60/day
CSA*
CYC*
PSL*
CSA*
Polymyxin B Hemoperfusion
Died
(46 days after onset of illness)
75
Female [7]
ILD 5 day course
(400 mg/kg/day) total 2gm/kg
One Course
MPD*
PSL 50/day
CSA*
PSL*
CSA*
CYC*
Died
(47 days after onset of illness)
55
Female [7]
ILD 5 day course (400 mg/kg/day) total 2gm/kg
One Course
MPD*
PSL 50/day
CSA*
PSL*
CSA*
CYC*
Died
(29 days after onset of illness)
24
Male [7]¶
ILD 5 day course
(400 mg/kg/day) total 2gm/kg
Two Courses, one month apart
MPD 3g
PSL 60/day
CSA 150 mg/day
PSL taper (60 to 10 mg)
Survival, with resolution of pulmonary and cutaneous findings
55
Female [8]
Calcinosis Cutis 5 day course (400 mg/kg/day)
total 2gm/kg
Five Courses one month apart, then one course yearly for 5 years
Pred 60/day
HCQ 400/day
CLQ 250/day
MTX 7.5/week
AZA 100/day
CSA 200/day
MMF 1500/day
No immunomodulatory therapy Complete resolution
54
Male [9]
ILD 5 day course (400 mg/kg/day)
total 2gm/kg
One Course
PRED taper
(30 mg over 12 days)
MPD 3 g
PRED 75/day
PRED 75/day Died
61
Female [10]§
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
7 courses, one month apart
HCQ*
PRED 40/day
No immunomodulatory therapy Complete resolution
29
Female [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
28 courses, one month apart
HCQ*
MMF*
MTX*
HCQ* Complete Resolution
36
Female [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
22 courses, one month apart
HCQ*
MTX*
No immunomodulatory therapy Complete Resolution
22
Female [10]§
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
6 courses, one month apart
HCQ* HCQ* Complete Resolution
53
Female [10]§
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
4 courses, one month apart
HCQ*
MTX*
No immunomodulatory Therapy Complete Resolution
93
Female [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
4 courses, one month apart
HCQ*
PRED 10/daily
No immunomodulatory Therapy >75% resolution
56
Female [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
11 courses, one month apart
HCQ*
MMF*
MTX*
HCQ*
(summer months only)
>75% resolution
87
Female [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
9 courses, one month apart
HCQ* None <75% resolution
58
Male [10]
None Noted 2 day course (1gm/kg/day)
Total 2gm/kg
7 courses, one month apart
HCQ*
THAL*
MTX*
PRED 40/day
PRED 20/day <75% resolution