Autoinflammatory & rare systemic diseases
Pulmonary arterial hypertension in adults with Still’s disease: another pulmonary manifestation associated with HLA-DRB1*15
Authors' conclusion
These results reinforce the association between the HLA-DRB1*15 allele and severe forms of SD and raise the question of therapeutic optimisation in such patients.
Study context
- Population
- Adults with Still's disease, CRI-IMIDIATE network and French pulmonary hypertension network registry; controls from a reference centre84 with genetic typing
- Participants
- 127 adults
- Comparison
- Adult Still's disease with pulmonary arterial hypertension (PAH) vs Still's disease without PAH
- PAH+n = 16
- PAH (PAH-)n = 111reference
- Outcome
- HLA-DRB1*15 allele prevalence (patients with genetic typing)
Methodological detailsmulticentre call for observations and national registry search, controls from a reference centre
- Multicentre call for observations and national registry search
- Controls from a reference centre
Main finding
As reported in the abstract
P = 0.014
Observational data: associations, not effects.
Further findings
Female sex
P = 0.006
Black ethnicity
P < 0.0001
Macrophage activation syndrome
P < 0.0001
Eosinophilia during disease course
P < 0.0001
Mortality · all deaths related to SD flare
P < 0.0001
Disease activity and treatment
- PAH+ had more active SD both at diagnosis and throughout the disease course
- PAH+ more frequently received canakinumab and immunosuppressants
Safety
Drug reactions to IL-1 and/or IL-6 inhibitors
P = 0.002
Original publication
Pulmonary arterial hypertension in adults with Still’s disease: another pulmonary manifestation associated with HLA-DRB1*15. Ann Rheum Dis. Published 15 May 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →