Previous reported cases of anakinra treatment of ECD
| . | Sex, age (years) . | ECD localizations and previous treatments . | ECD duration (years) . | Anakinra duration (months) . | CRP . | Tolerance . | Efficacy . |
|---|---|---|---|---|---|---|---|
| Aouba 113 | F, 46 | Bones, retroperitoneal, hydronephrosis, xanthelasma | 7 | 25 | Normalized | Pruritus | Disappearance of bone pain and fever |
| Steroids | Regression of hydronephrosis and retroperitoneal infiltration | ||||||
| Zoledronic acid | Normalization of bone scintigraphy | ||||||
| Cladribine | |||||||
| Aouba 213 | M, 55 | Bones, coated aorta and renal artery stenosis, retroperitoneal, hydronephrosis | 9 | 26 | Normalized | Pain at injection sites | Disappearance of bone pain and fever |
| Steroids | Regression of hydronephrosis | ||||||
| IFN-α | No improvement of scintigraphic bone uptake | ||||||
| Tran14 | F, 10 | Bones, retroperitoneal | ? | 10 | Normalized | Pain at injection sites | Disappearance of bone pain and fever |
| Vinblastine | No changes in bone lesions nor retroperitoneal infiltration | ||||||
| Steroids | |||||||
| IFN-α | |||||||
| Aubert20 | F, 32 | Bones, retroperitoneal, adrenal infiltration | 10 | 12 | Normalized | Slight injection sites reaction | Disappearance of bone pain and fever |
| Steroids | Improvement of bone hypermetabolism in PET | ||||||
| Vesanoid | No change in retroperitoneal infiltration | ||||||
| Killu22 | M, 71 | Right atrial mass, bilateral pleura infiltration, temporal artery infiltration | 0 | 6 | ? | ? | Improvement of 18 FDG uptake in right atrial mass |
| No prior treatment | Heart MRI evaluation not done | ||||||
| Courcoul23 | M, 57 | Bones | 1 | 12 | Normalized | No side effects | Disappearance of bone pain |
| Steroids | No changes in 18 FDG uptake, bone scan and bone MRI | ||||||
| Pamidronate | |||||||
| IFN-α | |||||||
| Darstein24 | M, 48 | Bones, retroperitoneal, hydronephrosis, CNS | 3 | 5 | Rapid decrease | Gram-negative urosepsis controlled under antibiotics | Improvement of neurological symptoms (not assessed by MRI) |
| No prior treatment | |||||||
| Cohen25 | F, 43 | Bones, pituitary involvement, sinuses, lichen planus | 4 | 3 | NA | Injection site reaction | Cutaneous lesions, desmopressin need and bone pain improvement |
| . | Sex, age (years) . | ECD localizations and previous treatments . | ECD duration (years) . | Anakinra duration (months) . | CRP . | Tolerance . | Efficacy . |
|---|---|---|---|---|---|---|---|
| Aouba 113 | F, 46 | Bones, retroperitoneal, hydronephrosis, xanthelasma | 7 | 25 | Normalized | Pruritus | Disappearance of bone pain and fever |
| Steroids | Regression of hydronephrosis and retroperitoneal infiltration | ||||||
| Zoledronic acid | Normalization of bone scintigraphy | ||||||
| Cladribine | |||||||
| Aouba 213 | M, 55 | Bones, coated aorta and renal artery stenosis, retroperitoneal, hydronephrosis | 9 | 26 | Normalized | Pain at injection sites | Disappearance of bone pain and fever |
| Steroids | Regression of hydronephrosis | ||||||
| IFN-α | No improvement of scintigraphic bone uptake | ||||||
| Tran14 | F, 10 | Bones, retroperitoneal | ? | 10 | Normalized | Pain at injection sites | Disappearance of bone pain and fever |
| Vinblastine | No changes in bone lesions nor retroperitoneal infiltration | ||||||
| Steroids | |||||||
| IFN-α | |||||||
| Aubert20 | F, 32 | Bones, retroperitoneal, adrenal infiltration | 10 | 12 | Normalized | Slight injection sites reaction | Disappearance of bone pain and fever |
| Steroids | Improvement of bone hypermetabolism in PET | ||||||
| Vesanoid | No change in retroperitoneal infiltration | ||||||
| Killu22 | M, 71 | Right atrial mass, bilateral pleura infiltration, temporal artery infiltration | 0 | 6 | ? | ? | Improvement of 18 FDG uptake in right atrial mass |
| No prior treatment | Heart MRI evaluation not done | ||||||
| Courcoul23 | M, 57 | Bones | 1 | 12 | Normalized | No side effects | Disappearance of bone pain |
| Steroids | No changes in 18 FDG uptake, bone scan and bone MRI | ||||||
| Pamidronate | |||||||
| IFN-α | |||||||
| Darstein24 | M, 48 | Bones, retroperitoneal, hydronephrosis, CNS | 3 | 5 | Rapid decrease | Gram-negative urosepsis controlled under antibiotics | Improvement of neurological symptoms (not assessed by MRI) |
| No prior treatment | |||||||
| Cohen25 | F, 43 | Bones, pituitary involvement, sinuses, lichen planus | 4 | 3 | NA | Injection site reaction | Cutaneous lesions, desmopressin need and bone pain improvement |