Clinical characteristics and evaluation of the efficacy of infliximab in 12 patients with Erdheim-Chester disease
| Case | Age*, sex | Previous treatments | Metabolic assessment | Cardiac evaluation | CNS evaluation | AE | Duration under infliximab (months) | Reason for treatment interruption |
| #1 | F, 80 | IFN, anakinra | Progression | – | – | Urinary tract infection | 40 | Progression |
| #2 | F, 23 | IFN, anakinra | Stable | – | – | Bronchitis | 30 | Personal reason (mild symptoms) |
| #3 | M, 66 | IFN | PMR | Improved | – | None | 60 | No interruption |
| #4 | M, 72 | IFN | Progression | – | Stable | None | 18 | Personal reason (metabolic progression but no symptoms) |
| #5 | M, 54 | IFN, cladribine | PMR | – | Improved | Myelodysplasia | 35 | AE |
| #6 | M, 76 | Anakinra | PMR | Stable | Lung infection | 14 | Death (ischaemic stroke) | |
| #7 | M, 73 | IFN | Stable | New | – | Pneumocystis infection | 13 | Progression |
| #8 | F, 62 | IFN | Progression | Worsened | None | 12 | Progression | |
| #9 | F, 55 | Cladribine | PMR | Stable | Improved | None | 11 | Switch to vemurafenib† |
| #10 | M, 67 | – | Progression | Stable | – | Lung infection | 22 | AE and progression |
| #11 | M, 57 | IFN | Stable | – | – | Hypogammaglobulinaemia | 43 | No interruption |
| #12 | M, 69 | – | PMR | Stable | New | None | 12 | Progression |
*Age at the initiation of infliximab treatment.
†The patient had neurological involvement, and despite improvement with infliximab, she was given vemurafenib due to severe CNS involvement.
–, no data; AE, adverse event; CNS, central nervous system; F, female; IFN, interferon-alpha (standard or pegylated); M, male; New, appearance of a new localisation; PMR, partial metabolic remission.