Biological therapy of traditional therapy-resistant adult-onset Still’s disease: an evidence-based review

Figures & data

Table 1 Summary of patient characteristics at the start of the biologics therapy

Characteristics	Results
Age at biologics start, median (range) (years)	35 (16–80)
Sex (N)	Female: 269, male: 142, unknown: 9
Duration from onset to the start of biologics median (range)	27 (<1–163)
Follow-up median (range) (months)	13.5 (<1–84).
Symptoms
Fever	N=281, 68.7%
Rash	N=223, 54.5%
Sore throat	N=113, 27.6%
Arthritis/arthralgia	N=303, 74.1%
Myalgia	N=57, 13.9%
Serositis/pleuritis	N=62, 15.2%
Lymphadenopathy	N=90, 22%
Hepatosplenomegaly	N=77, 18.8%
Leukocytosis median (range) (10^9/L)	N=198, 48.8%, 16.4 (10.2–96.0)
ESR median (range) (mm/1 h)	N=261, 63.8%, 72 (4–144)
CRP median (range) (µg/dL)	N=262, 64.06%, 11.1 (2.3–40)
Hyperferritinemia median (range) (mg/dL)	N=193, 47.19%, 3,875 (500–155,000)

Abbreviations: CRP, C-reactive protein; ESR, erythrocyte sedimentation rate.

Table 2 Side effects reported on biological therapy of refractory AOSD

Biologics	Rash	MAS	Infection	Others	Total
Adalimumab	1	2	0	0	3
Etanercept	2	0	3 (1 Listeria Meningoencephalitis, 1 Pneumocystis jirovecii pneumonia, 1 thigh abscess)	2 (1 allergy; 1 shortness of breath)	7
Infiliximab	4	2	3 (1 bacterial pneumonia, 1 hepatitis B virus, 1 recurrent bronchitis)	3 (1 massive edema of the bilateral periorbital region, 1 lupus rash + optic neuritis, 1 cardiac failure)	12
Anakinra	18	0	13 (5 urinary tract infections, 2 soft tissue abscess, 1 phalanx osteomyelitis, 1 gastroenteritis with fever, 1 influenza A virus infection of the upper respiratory tract, 1 trachiobronchitis, 2 respiratory tract infection by Pseudomonas aeruginosa, 1 herpes zoster)	8 (3 mild leukopenia, 1 cardiac death, 1 escape phenomenon, 1 myopathy, 1 severe respiratory distress and hemodynamic shock, 1 subacute liver failure, 2 trombocytopenia)	39
Canakinumab	0	1	0	0	1
Tocilizumab	0	2	15 (4 upper respiratory tract infection, 2 herpes zoster virus infection, 1 Epstein–Barr virus infection, 1 dental infection, 1 acute enterocolitis, 1 pyelonephritis, 3 bacterial pneumonia, 1 urinary infection, 1 abscess in the psoas muscle caused by Staphylococcus aureus)	23 (2 facial swelling, 2 high blood pressure, 1 deep vein thrombosis and massive hematochezia, 1 chest pain and chills, 5 increased alanine aminotransferase levels, 5 leukopenia or neutropenia, 1 liver failure, 1 liver Injury, 2 hyperlipidaemia, 2 hyperlipidemia, 1 malignant melanoma)	40

Abbreviations: AOSD, adult-onset Still’s disease; MAS, macrophage activation syndrome.