The Spectrum of Still's Disease: A Comparative Analysis of Phenotypic Forms in a Cohort of 238 Patients.

Still's disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juvenile idiopathic arthritis (sJIA). We identified 238 patients, 152 (64%) of whom had AOSD while 86 (36%) had sJIA. The median age at SD onset was 26.6 years. In patients with identifiable patterns, the course of SD was systemic in 159 patients (74%), chronic in 55 (26%). Sore throat and myalgia were more frequent in patients with AOSD. Abnormal liver tests, serum ferritin and C-reactive protein levels were higher in AOSD group. Fever and skin rash were predictive of complete remission or recovery and high lactate dehydrogenase level was a poor prognosis factor. Symptoms such as splenomegaly, skin rash, high polymorphonuclear neutrophils count and macrophage activation syndrome were predictive of a systemic phenotype. Overall, there were no major differences between sJIA and AOSD. Our results are consistent with the "biphasic" model of an autoinflammatory disease that can progress to chronic arthritis if not treated early.

[Prise en charge médicale du Covid-19, hors réanimation]. / Medical management of covid-19, excluding intensive care.

MEDICAL MANAGEMENT OF COVID-19, EXCLUDING INTENSIVE CARE Management of Covid-19 is primarily guided by the severity of the respiratory involvement. Covid-19 is mostly asymptomatic or pauci-symptomatic allowing the ambulatory setting associated with isolation measures going from 5 to 10 days depending on the circumstances. Nirmatrelvir/ritonavir (Paxlovid) is currently the oral treatment for Covid-19 in adult patients not requiring oxygen therapy and at risk of severe disease in the absence of contraindications. For hospitalized patients requiring oxygen support, corticosteroid therapy and anticoagulation are the main supportive treatments in association with measures preventing secondary cases. The antiviral approach is limited in this context, consisting in passive immunotherapy (mostly neutralizing monoclonal antibodies) or direct-acting antivirals active on the dominant variant and that have demonstrated clinical efficacy.

PRISE EN CHARGE MÉDICALE DU COVID-19, HORS RÉANIMATION La prise en charge médicale de l'infection par le SARS-CoV-2 est principalement guidée par la sévérité de l'atteinte respiratoire. Dans la population générale, l'infection est le plus souvent asymptomatique ou paucisymptomatique, autorisant une prise en charge ambulatoire, sous réserve de mesures d'isolement de cinq à dix jours selon les circonstances. La prévention préexposition par anticorps monoclonaux doit être proposée chaque fois que possible chez les patients immunodéprimés. En l'absence de contre-indication, l'association nirmatrelvir-ritonavir (Paxlovid) est actuellement le traitement curatif oral ambulatoire chez les patients adultes ne nécessitant pas d'oxygénothérapie et à risque de forme grave. Pour les patients hospitalisés oxygénorequérants, la corticothérapie et l'anticoagulation sont les principaux traitements complémentaires, en association aux mesures de prévention pour l'éviction des cas secondaires. L'approche antivirale est limitée dans ce contexte, reposant sur l'immunothérapie passive (anticorps monoclonaux neutralisants essentiellement) ou des antiviraux actifs sur le variant dominant et ayant démontré une efficacité clinique.

Osteocutaneous-flap-related osteomyelitis following mandibular reconstruction: a cohort study of an emerging and complex bone infection.

Osteocutaneous flap (OCF) mandible reconstruction is at high risk for surgical site infection. This study aimed to describe diagnosis, management, and outcome of OCF-related osteomyelitis. All patients managed at our institution for an OCF-related osteomyelitis following mandible reconstruction were included in a retrospective cohort study (2012-2019). Microbiology was described according to gold-standard surgical samples, considering all virulent pathogens, and potential contaminants if present on at least two samples. Determinants of treatment failure were assessed by logistic regression and Kaplan-Meier curve analysis. The 48 included patients (median age 60.5 (IQR, 52.4-66.6) years) benefited from OCF mandible reconstruction mostly for carcinoma ( n = 27 / 48 ; 56.3 %) or osteoradionecrosis ( n = 12 / 48 ; 25.0 %). OCF-related osteomyelitis was mostly early ( ≤ 3 months post-surgery; n = 43 / 48 ; 89.6 %), presenting with local inflammation ( n = 28 / 47 ; 59.6 %), nonunion (wound dehiscence) or sinus tract ( n = 28 / 47 ; 59.6 %), and/or bone or device exposure ( n = 21 / 47 ; 44.7 %). Main implicated pathogens were Enterobacteriaceae ( n = 25 / 41 ; 61.0 %), streptococci ( n = 22 / 41 ; 53.7 %), Staphylococcus aureus ( n = 10 / 41 ; 24.4 %), enterococci ( n = 9 / 41 ; 22.0 %), non-fermenting Gram-negative bacilli ( n = 8 / 41 ; 19.5 %), and anaerobes ( n = 8 / 41 ; 19.5 %). Thirty-nine patients (81.3 %) benefited from surgery, consisting of debridement with implant retention (DAIR) in 25 / 39 (64.1 %) cases, associated with 93 (IQR, 64-128) days of antimicrobial therapy. After a follow-up of 18 (IQR, 11-31) months, 24 / 48 (50.0 %) treatment failures were observed. Determinants of treatment outcomes were DAIR (OR, 3.333; 95 % CI, 1.020-10.898) and an early infectious disease specialist referral (OR, 0.236 if ≤ 2  weeks; 95 % CI, 0.062-0.933). OCF-related osteomyelitis following mandibular reconstruction represents difficult-to-treat infections. Our results advocate for a multidisciplinary management, including an early infectious-disease-specialist referral to manage the antimicrobial therapy driven by complex microbiological documentation.

Detection and Prediction of Macrophage Activation Syndrome in Still's Disease.

Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still's disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14-11.2; p = 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08-15.3; p = 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 µg/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (p < 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS.