Abstract
Background
Generalized pustular psoriasis (GPP) is a rare, severe inflammatory disease with limited evidence on long-term prognosis and treatment–mortality associations.
Objective
To compare mortality and major comorbidities in GPP, psoriasis vulgaris (PV), and non-psoriatic individuals, and treatment–mortality associations in GPP.
Methods
Adults with GPP, psoriasis vulgaris (PV) without pustular psoriasis, and non-psoriatic controls were identified. GPP patients were 1:1 propensity score–matched to PV and non-psoriatic controls by age, sex, and race. Among GPP patients, those receiving biologics, conventional anti-psoriatic drugs, or small-molecule inhibitors were separately matched to patients treated with systemic corticosteroids alone. Primary outcome was all-cause mortality; secondary outcomes included major mortality-related comorbidities based on the ten leading causes of death per US CDC. Kaplan–Meier and Cox models estimated cumulative incidence and hazard ratios.
Results
After matching, 6,959 GPP–PV and 28,459 GPP–non-psoriatic pairs were analyzed. Over 5 years, GPP patients had higher 5-year mortality than PV (HR, 1.83; 95% CI, 1.28–2.59) and non-psoriatic controls (HR, 2.42; 95% CI, 1.99–2.94). Major comorbidity risks were higher in GPP than in PV, notably ischemic heart disease (HR, 1.34; 95% CI, 1.01–1.78), cerebrovascular disease (HR, 1.62; 95% CI, 1.06–2.47), and neurodegenerative disease (HR, 2.94; 95% CI, 1.47–5.89), and were elevated across all evaluated comorbidities compared with non-psoriatic controls. Mortality risks were particularly pronounced in patients >60 years and males. Among GPP patients, biologic therapy was associated with lower mortality than systemic corticosteroids alone (HR, 0.71; 95% CI, 0.52–0.96), whereas conventional anti-psoriatic drugs and small-molecule inhibitors were not.
Conclusion
GPP is associated with higher mortality and comorbidity risk than PV and non-psoriatic individuals. Biologic therapy was associated with lower mortality.