Treating Refractory Adult Still's Patients

From sclence

health

Bruno Fautrel , MD PhD

Sorbonne University - Assistance Publique Hôpitaux de Paris Rheumatology Department, GH Pitié Salpêtrière CRI - IMIDIATE Clinical Research Network Pierre Louis Institute of Epidemiology and Public Health

INSERM UMRS 1136, Team 5

· Financial interests

• -None

· Permanent links

• -None

· Interventions

• -AbbVie, Amgen, Biogen, BMS, Celltrion, Fresenius Kabi, Galapagos, Gilead, Janssen, Lilly, Medac, MSD, NORDIC Pharma, Novartis, Pfizer, Roche, Sandoz, Sanofi-Genzyme, SOBI, UCB, Viatris.

· Indirect links

• -Research grants: AbbVie, Lilly, MSD, Pfizer



Adult Still's disease (MSA)

· Still's disease in children

• - Systemic form of AJI (FS-AJI) Systemic onset JIA (SoJIA)
• - 1 ere description in 1897 by Sir G Still
• · Fever + Rash + Arthritis triad
• - Evolution by recurrent attacks
• · Prevalence 3.1 / 100000 [0.5-0.7]
• · Incidence 0.6 / 100,000 [2.7-3.6]

• · Adult form :
• - Adult Still's disease Adult onset Still's Disease (AoSD)
• - 1st description by Bywaters in 1971
• · Triad: Fever + Rash + Arthralgia/itis
• - Recurrent attacks, young adults
• · Prevalence: 0.16 to 1.5 / 100,000

Magadur-Joly 1995, Wakai 1997

Thierry S, 2011

• · No gender predominance
• · No family aggregation
• · No clear comorbid association



« Refractory » Adult Still's Patients

• · Refractory or « D2T » : lack of definition
• · Several options
• - Drug dependency or Inadequate response
• · Prednisone? Or bDMARD?
• - Life threatening complications?
• · MAS, lung disease, fulminant hepatitis
• - Non-inflammatory residual symptoms
• · Fatigue, residual pain, consequences of high dose steroids …



Current Views amongst adult Still's Experts

Clinical heterogeneity -> Need for stratification




EULAR / PReS Recommendations

Important message for the risk to evolve towards hyperinflammatory complications




For non-inflammatory D2T patients

Comprehensive, multidisciplinary and coordinated care



REGULAR EVALUATION + PREVENTIVE MEDICINE :

• Vaccination status (DTP; influenza, pneumococcus, and SARS-COV-2 if applicable)
• Cancer screening
• Oral health
• Osteoporosis prevention
• Lung involvement screening (if confirmed, clinical monitoring, PFT with DLCO every 6-12 months, PEC)
• Depression prevention


Conclusion



EULAR / PReS Recommendations




· Merci!