NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 1

NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 1

@SJIA_Foundation
@SJIA_Foundation
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2 months ago 224

Presentation from Dr. Claudia Bracaglia: Monitoring and Treating Lung Disease in Still's Disease

NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 1

@SJIA_Foundation2 months ago

Lung Disease in Still's Disease

Monitoring & Treating Lung Disease in Still's Disease

Claudia Bracaglia Division of Rheumatology Ospedale Pediatrico Bambino Gesù Roma - Italy claudia.bracaglia@opbg.net

        61 SD-LD patients from 21 Ped Rheum Centres European Registry on SD associated LD

              European Registry on sJIA associated LD

              61 SJIA-LD patients from 21 Ped Rheum Centres

                    SD associated LD Bambino Gesù experience

                          SD associated LD Bambino Gesù experience

                          *The reaction occurred after the first and the third administration of TCZ in 1 pt. LD developed before the drug reaction in 2 pts.

                                SD associated LD Bambino Gesù experience

                                • *2 pts were already on cyclosporine before LD diagnosis
                                • **3 pts switched from cyclosporine to MMF after LD diagnosis

                                      SD associated LD Bambino Gesù experience

                                      • *2 pts were already on cyclosporine before LD diagnosis
                                      • **3 pts switched from cyclosporine to MMF after LD diagnosis

                                      Outcome

                                      • -1 pt improved with IL-1i and MMF
                                      • -2 pts improved with MAS825
                                      • -1 pt stable with emapalumab
                                      • -1 pt worsened despite MAS825 and she now on emapalumab
                                      • -1 pt developed LD on sirolimus
                                      • -No pt developed LD complication
                                      • -No pt died
Monitoring & Treating 
Lung Disease in Still’s Disease
Claudia Bracaglia
Division of Rheumatolog…
1/7
61 SD-LD patients from 21 Ped Rheum Centres 
European Registry on SD associated LD
Clinical chara…
2/7
61 SJIA-LD patients from 21 Ped Rheum Centres 
European Registry on sJIA associated LD
Patients w…
3/7
SD associated LD
Bambino Gesù experience
SJIA-LD (n=6)
Female 3 (50%)
Aget at SD onset (range) …
4/7
SD associated LD 
Bambino Gesù experience
SJIA-LD (n=6)
Exposure to IL-1/IL-6 inhibitors before …
5/7
SD associated LD 
Bambino Gesù experience
Treatment after LD 
diagnosis
SJIA-LD 
(n=6)
- IV G…
6/7
SD associated LD 
Bambino Gesù experience
Treatment after LD 
diagnosis
SJIA-LD 
(n=6)
- IV G…
7/7


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