The Problem
Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis with heterogeneous clinical presentation. Our understanding of the condition is limited and it is unclear whether the myriad of symptoms differ among patient groups leading to differential prognosis and outcomes.
Outcome
Using Dexter, we identified patients with GPA and that GPA can be classified into three distinct clusters with different prognoses, susceptibility to recurrent infections and presence of comorbidities. The tendency of cluster 1 (patients with limited disease mainly with involvement of ENT symptoms and cough) to evolve into a more generalised disease raises questions about current immunosuppressive treatment approaches in these patients. The information we found can inform future drug discovery approaches to aid precision medicine approaches by treating specific groups of patients dependent on their symptom burden in a personalised way to optimise outcomes.
Outputs: Patient cohort identified with rare condition and novel information identified about their symptom burden.
