Table 1.
Clinical features of patients with hyperinflammatory syndrome treated with anakinra: four cases from our Unit and three from the literature.
| 1 | 2 | 3 | 4 | Leone F et al. Lancet Rheumatol, 2021 1 | Baicus C et al. Rom J. Intern Med, 2021 2 | Salzman MB et al. Emerg Infect Dis, 2021 3 | |
|---|---|---|---|---|---|---|---|
| Gender, age | Male, 65 | Female, 57 | Female, 53 | Female, 50 | Male, 36 | Male, 22 | Male, 18 |
| Previous diseases | Psoriatic arthritis | Adult-onset Still’s disease | Psoriasis Hashimoto’s thyroiditis Vitiligo |
Psoriasis Non-Hodgkin’s lymphoma (2008) Hypothyroidism |
None | None | Asthma |
| SARS-CoV2 vaccine | ChAdOx1 (AstraZeneca) | BNT162b2 (Pfizer-BioNTech) | BNT162b2 (Pfizer-BioNTech) | BNT162b2 (Pfizer-BioNTech) | ChAdOx1 (AstraZeneca) | BNT162b2 (Pfizer-BioNTech) | BNT162b2 (Pfizer-BioNTech) |
| Body temperature (°C) | 39.0 | 37.5 | 39.5 | 39.0 | >39 | na | 40 |
| Time from vaccination to symptoms onset (days) | 1 (first dose) | 18 (first dose) | 10 (second dose) | 3 (second dose) | 1 (first dose) | 13 (first dose) | 18 (first dose) |
| Previous SARS-CoV-2 infection | No | No | No | No | No | No | Yes |
| Duration of fever (days) until therapy | 18 | 15 | 29 | 32 | 10 | 14 | 15 |
| CRP (mg/L) | 480 | 354.7 | 51.5 | 208 | 188 | High increase | 185.5 |
| Ferritin (µg/L) | 1550 | 813 | 223.400 | 6.000 | 946 | 9.834 | 3.002 |
| WBC (*106 mmc) | 15.5 | 24.6 | 8.34 | na | 30.830 | High increase | 7.000 |
| Neutrophils (*106 mmc) | 13.2 | 22.2 | 3.31 | na | 26.500 | High increase | 6280 |
| Autoantibodies | No | No | ANA 1:160 fine speckled | ANA 1:160 | No | No | No |
| Liver dysfunction | No (ALP, GGT, bilirubin) | Yes (LDH) | Yes (GOT, GPT, LDH, TGL) | Yes (GOT, GPT) | Yes (GOT, GPT) | Yes | Yes (GOT/GPT) |
| Splenomegaly | Yes | No | Yes | No | No | No | No |
| Lymphadenopathy | Yes | No | Yes | No | No | No | No |
| Skin rash | Yes | No | Yes | Yes | Yes | Yes | No |
| Arthritis/arthralgias | Yes | Yes | Yes | Yes | No | Yes | No |
| Treatment | Prednisone 75 mg daily i.v. anakinra 100 mg BD |
Prednisone 25 mg daily s.c. anakinra 100 mg daily |
Dexamethasone 10 mg BD i.v. anakinra 200 mg BD |
Prednisone 25 mg daily s.c. anakinra 100 mg daily Cyclosporine 150 mg/day After a flare i.v. anakinra 200 mg BD |
Methylprednisolone 0.75 mg/kg and then s.c. anakinra 100 mg/day | Dexamethasone i.v. 16 mg/day, IVIg 1–2 g/kg, 3 g pulses methylprednisolone, then s.c. anakinra 100 mg every other day/2/3 days | IVIg 100 mg, methylprednisolone 1 g/day for 3 days, s.c. anakinra 100 mg/day for 3 days |
ALP: alkaline phosphatase; BD: bis-in-die; CRP: C-reactive protein; GGT: gamma-glutamyl transpeptidase; GPT: glutamic pyruvic transaminase; GOT: glutamic oxaloacetic transaminase; IVIg: intravenous immunoglobulin; i.v.: intravenous; LDH: lactate dehydrogenase; s.c.: subcutaneous; WBC: white blood cell count; na: not available.