Early onset, recurrent illness tied to DADA2 disease damage

In patients with deficiency of adenosine deaminase 2 (DADA2) vasculitis, early age of onset and periodic illness are associated with disease-related damage, but prompt initiation of a tumour necrosis factor inhibitor (TNFi) may prevent complications and severe progression, suggests a study.

A team of investigators conducted this retrospective cohort study to describe the clinical and laboratory findings, disease course, and treatment effectiveness of a large homogenous group of patients with DADA2, as well as to identify factors linked to disease-related damage. Forty-five patients with long-term follow-up were included in the analysis.

Data obtained from patient charts included demographic information, clinical presentations, laboratory findings, and treatment. Finally, the investigators used the Vasculitis Damage Index to determine the extent of disease-related damage.

Based on the assessment, a young age at disease onset and periodic illness, including recurrent episodes of fever, myalgia, and abdominal pain, significantly correlated with disease-related damage. However, patients with severe DADA2 vasculitis showed a favourable response to treatment with TNFi.

“Early age of onset and the periodic nature of the disease warrant prompt initiation of TNFi treatment to prevent later complications and severe disease progression,” the investigators said.