Demographics, characteristics, and responses of enrolled subjects
| Subject ID . | Age/sex . | HES variant . | Organ involvement . | Enrollment* . | Baseline AEC on MED, cells/µL† . | MED‡ . | Final AEC at assessment of response visit,§ cells/µL† . | MEDD‡ . | Response . |
|---|---|---|---|---|---|---|---|---|---|
| 2 | 46/F | IHES | Skin, soft tissue | GC1 | 700 | 25 | 990 | 40 | NR |
| 4 | 54/M | EGPA overlap‖ | Lung, sinus | GC1 | 660 | 12.5 | 1000 | 12.5 | NR |
| 5 | 35/F | EGPA overlap | Lung, sinus, GI (esophagus, stomach, duodenum, skin) | Direct | 550 | 13.5 | 0 | 0 | R |
| 7 | 71/M | IHES | Cardiac | Direct | 280 | 20 | 0 | 0 | R |
| 8 | 51/M | EGPA Overlap | Lung | GC1 | 850 | 25 | 1790 | 15 | NR |
| 9 | 50/F | LHES | GI (colon), skin | Direct | 2540 | 15 | 1950 | 15 | NR |
| 10 | 72/F | EGPA Overlap | Lung, sinus, cardiac, skin | Direct | 390 | 17.5 | 270 | 12.5¶ | DR |
| 12 | 22/M | IHES | Lung | Direct | 930 | 20 | 1140 | 15¶ | DR |
| 14 | 38/M | EGID overlap# | GI (stomach, duodenum, and colon) | Direct | 670 | 15 | 940 | 6 | R |
| 15 | 64/M | LHES | Skin, GI, (esophagus, stomach, duodenum, and colon), bladder | GC1 | 630 | 20 | 0 | 0 | R |
| Subject ID . | Age/sex . | HES variant . | Organ involvement . | Enrollment* . | Baseline AEC on MED, cells/µL† . | MED‡ . | Final AEC at assessment of response visit,§ cells/µL† . | MEDD‡ . | Response . |
|---|---|---|---|---|---|---|---|---|---|
| 2 | 46/F | IHES | Skin, soft tissue | GC1 | 700 | 25 | 990 | 40 | NR |
| 4 | 54/M | EGPA overlap‖ | Lung, sinus | GC1 | 660 | 12.5 | 1000 | 12.5 | NR |
| 5 | 35/F | EGPA overlap | Lung, sinus, GI (esophagus, stomach, duodenum, skin) | Direct | 550 | 13.5 | 0 | 0 | R |
| 7 | 71/M | IHES | Cardiac | Direct | 280 | 20 | 0 | 0 | R |
| 8 | 51/M | EGPA Overlap | Lung | GC1 | 850 | 25 | 1790 | 15 | NR |
| 9 | 50/F | LHES | GI (colon), skin | Direct | 2540 | 15 | 1950 | 15 | NR |
| 10 | 72/F | EGPA Overlap | Lung, sinus, cardiac, skin | Direct | 390 | 17.5 | 270 | 12.5¶ | DR |
| 12 | 22/M | IHES | Lung | Direct | 930 | 20 | 1140 | 15¶ | DR |
| 14 | 38/M | EGID overlap# | GI (stomach, duodenum, and colon) | Direct | 670 | 15 | 940 | 6 | R |
| 15 | 64/M | LHES | Skin, GI, (esophagus, stomach, duodenum, and colon), bladder | GC1 | 630 | 20 | 0 | 0 | R |
DR, delayed responder; EGID, eosinophilic gastrointestinal disorder; EGPA, eosinophilic granulomatosis with polyangiitis; F, female; GI, gastrointestinal; IHES, idiopathic HES; LHES, lymphocytic variant HES; M, male; NR, nonresponder; R, responder.
Direct enrollees had evidence of persistent HES despite prednisone ≥10 mg daily (AEC >1500 cells/µL) (subject 9) or had undergone a GC taper within the 6 months prior to enrollment demonstrating inability to taper below prednisone 10 mg daily (subjects 5, 7, 10, 12, and 14).
Normal range, 40 to 540 cells/µL.
Milligrams of prednisone equivalent.
If dexpramipexole was discontinued before the predetermined end-of-study visit, then date of drug discontinuation was used.
Defined as anti-neutrophil cytoplasmic antibody–negative HES with clinical features of eosinophilic granulomatosis with polyangiitis and no histologic evidence of vasculitis.
Even though the 2 delayed responders’ (subjects 10 and 12) MEDD was not <50% of MED at the end-of-study visit, their lowest steroid dose was 29% and 37.5% of MED at months 10 and 8.5, respectively.
Defined as single organ HES with biopsy-proven eosinophilic involvement of the gastrointestinal tract and history of AEC>1500 cells/µL.