Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing. Methods In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS). Systematic literature reviews were conducted accordingly. Results The TF based their recommendations on four overarching principles, highlighting notably that sJIA and AOSD are one disease, to be designated by one name, Still’s disease. Fourteen specific recommendations were issued. Two therapeutic targets were defined: clinically inactive disease (CID) and remission, that is, CID maintained for at least 6 months. The optimal therapeutic strategy relies on early use of interleukin (IL-1 or IL-6 inhibitors associated to short duration glucocorticoid (GC). MAS treatment should rely on high-dose GCs, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors. A specific concern rose recently with cases of severe lung disease in children with Still’s disease, for which T cell directed immunosuppressant are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients. All overarching principles and recommendations were agreed by over 80% of the TF experts with a high level of agreement. Conclusion These recommendations are the first consensus for the diagnosis and management of children and adults with Still’s disease.

Despite their limited licensed indications, anti–interleukin-1 (anti–IL-1) agents are often used in clinical practice for an increasing number of auto-inflammatory diseases. We conducted a national cross-sectional observational study from January 2011 to January 2013 to record the off-label use of such agents in France. We aimed to estimate the off-label use of anti–IL-1 treatments in France, assess their efficacy in rare diseases, and increase the reporting of their possible side effects. Physicians answered a questionnaire that covered patient and disease data, anti–IL-1 agent use, efficacy and adverse events. The study involved adult or paediatric patient who had received an anti–IL-1 agent after January 2005 in France. In total, 189 patients from 38 centres were included. The main diseases were adult-onset Still’s disease (AOSD) (35), gout (28), systemic juvenile idiopathic arthritis (27), cryopyrin-associated periodic syndrome (CAPS) (21), familial Mediterranean fever (14) and mevalonate kinase deficiency (12). The main off-label used agent was anakinra, used at least once for 185 patients, with canakinumab used for 25. Anakinra was effective in most patients (90%), with higher complete clinical response rates for Schnitzler’s syndrome, gout, CAPS and AOSD. Overall, 58% of patients showed at least one adverse event, mainly minor injection-site reactions. The main reported serious adverse event was severe infection. Injection-site reactions and liver toxicity were significantly more frequent in children than adults. The main non-cutaneous adverse event was liver toxicity, significantly associated with treatment duration. Weight gain was reported in about 10% of patients and was associated with treatment duration and CAPS. Canakinumab was rarely used and showed better cutaneous tolerance than anakinra but similar rates of non-cutaneous and severe adverse events. Anakinra was well tolerated and effective in most patients with various inflammatory diseases. The main adverse events were mild injection-site reactions, especially in children. The survey allowed for collecting limited information on the off-label use of canakinumab.

Objective To evaluate the efficacy of tocilizumab, an antibody against IL-6 receptor, in patients with hand osteoarthritis. Methods This was a multicentre, 12-week, randomised, double-blind, placebo-controlled study from November 2015 to October 2018. Patients with symptomatic hand osteoarthritis (pain ≥40 on a 0–100 mm visual analogue scale (VAS) despite analgesics and non-steroidal anti-inflammatory drugs; at least three painful joints, Kellgren-Lawrence grade ≥2) were randomised to receive two infusions 4 weeks apart (weeks 0 and 4) of tocilizumab (8 mg/kg intravenous) or placebo. The primary endpoint was changed in VAS pain at week 6. Secondary outcomes included the number of painful and swollen joints, duration of morning stiffness, patients’ and physicians’ global assessment and function scores. Results Of 104 patients screened, 91 (45 to tocilizumab and 46 to placebo; 82% women; mean age 64.4 (SD 8.7) years) were randomly assigned and 79 completed the 12-week study visit. The mean change between baseline and week 6 on the VAS for pain (primary outcome) was −7.9 (SD 19.4) and −9.9 (SD 20.1) in the tocilizumab and placebo groups (p=0.7). The groups did not differ for any secondary outcomes at weeks 4, 6, 8 or 12. Overall, adverse events were slightly more frequent in the tocilizumab than placebo group. Conclusion Tocilizumab was no more effective than placebo for pain relief in patients with hand osteoarthritis.

Objective.: To date, early diagnosis of stress fractures depends on magnetic resonance imaging (MRI) or bone scan scintigraphy, as radiographs are usually normal at onset of symptoms. These examinations are expensive or invasive, time-consuming, and poorly accessible. A recent report has shown the ability of ultrasonography (US) to detect early stress fractures. Our objective was to evaluate sensitivity and specificity of US versus dedicated MRI (0.2 Tesla), taken as the gold standard, in early diagnosis of metatarsal bone stress fractures.Methods.: A case-control study from November 2006 to December 2007 was performed. All consecutive patients with mechanical pain and swelling of the metatarsal region for less than 3 months and with normal radiographs were included. US and dedicated MRI examinations of the metatarsal bones were performed the same day by experienced rheumatologists with expertise in US and MRI. Reading was undertaken blind to the clinical assessment and MRI/US results.Results.: Forty-one feet were analyzed on US and dedicated MRI from 37 patients (28 women, 9 men, mean age 52.7 ± 14.1 yrs). MRI detected 13 fractures in 12 patients. Sensitivity of US was 83%, specificity 76%, positive predictive value 59%, and negative predictive value 92%. Positive likehood ratio was 3.45, negative likehood ratio 0.22.Conclusion.: In cases of normal radiographs, US is indicated in the diagnosis of metatarsal bone stress fractures, as it is a low cost, noninvasive, rapid, and easy technique with good sensitivity and specificity. From these data, we propose a new imaging algorithm including US.