Cardiovascular

Evaluating disease burden in German AAV patients using the AAV-PRO: associations with disease activity, physical function, depression, fatigue and quality of life.

TL;DR

The German AAV-PRO captures patient-perceived disease burden, complementing traditional clinical outcome measures, with its multidimensional approach allowing assessment of functional, psychosocial and symptom-related aspects of AAV.

Key Findings

Patients with active ANCA-associated vasculitis had higher AAV-PRO scores across all domains compared to those with inactive disease.

  • Disease activity was assessed by physicians using the Birmingham Vasculitis Activity Score (BVAS)
  • The finding held across all AAV-PRO subdomains: Systemic symptoms, Social and emotional impact, Concerns about the future, and Physical function
  • The cohort included 70 AAV patients with mean disease duration of 10.5 years (SD 7.9)
  • The study was a prospective cohort design

The AAV-PRO 'Physical function' subdomain showed a strong negative correlation with SF-36 physical function.

  • Correlation coefficient r = -0.9 (95% CI -0.94 to -0.84)
  • The negative direction reflects that higher AAV-PRO scores (worse disease burden) correspond to lower SF-36 physical function scores (worse function)
  • AAV-PRO 'Physical function' also strongly correlated with IMET (r = 0.79, 95% CI 0.68 to 0.87)
  • IMET assesses restrictions in participation in everyday life

The AAV-PRO 'Systemic symptoms' subdomain showed strong correlations with both SF-36 physical function and the Funktionsfragebogen-Hannover (FFbH).

  • Correlation with SF-36 physical function: r = -0.72 (95% CI -0.85 to -0.58)
  • Correlation with FFbH (which evaluates abilities and limitations in daily activities): r = -0.88 (95% CI -0.56 to -0.97)
  • Both correlations were in the negative direction, indicating higher symptom burden corresponds to lower functional scores

The AAV-PRO 'Social and emotional impact' subdomain showed strong correlations with SF-36 social role functioning, IMET, and FFbH.

  • Correlation with SF-36 social role functioning: r = -0.8 (95% CI -0.87 to -0.69)
  • Correlation with IMET: r = 0.7 (95% CI 0.55 to 0.81)
  • Correlation with FFbH: r = -0.75 (95% CI -0.94 to -0.23)

The AAV-PRO 'Concerns about the future' subdomain showed strong correlations with SF-36 social role functioning and FFbH.

  • Correlation with SF-36 social role functioning: r = -0.71 (95% CI -0.81 to -0.55)
  • Correlation with FFbH: r = -0.77 (95% CI -0.94 to -0.27)
  • The subdomain also had associations with depression screening (PHQ-9) and optimism measures (LOT-R) assessed in the study

The study cohort of 70 AAV patients was predominantly composed of granulomatosis with polyangiitis (GPA) patients, with representation of all three major AAV subtypes.

  • GPA: 45 patients (64.3%)
  • Eosinophilic granulomatosis with polyangiitis (EGPA): 16 patients (22.9%)
  • Microscopic polyangiitis (MPA): 9 patients (12.8%)
  • Mean disease duration at study inclusion was 10.5 years (SD 7.9)
  • Physicians assessed disease activity (BVAS), damage (VDI), and glucocorticoid side-effects (GTI)

The AAV-PRO demonstrated associations with fatigue, depression, and quality of life measures in AAV patients.

  • Fatigue was measured using the Fatigue Severity Scale (FSS)
  • Depressive symptoms were screened using the Patient Health Questionnaire-9 (PHQ-9)
  • Health-related quality of life was assessed using the Short Form-36 (SF-36)
  • Optimism and future expectations were assessed using the Life Orientation Test-Revised (LOT-R)
  • These associations support the AAV-PRO's multidimensional assessment of functional, psychosocial, and symptom-related aspects of AAV

What This Means

This research suggests that a disease-specific patient questionnaire called the AAV-PRO (ANCA-Associated Vasculitis Patient-Reported Outcome) is effective at measuring how much the disease affects patients' lives in Germany. The study followed 70 patients with various forms of AAV — a group of rare autoimmune diseases that cause inflammation of blood vessels — and compared their answers on the AAV-PRO to results from several other well-established questionnaires measuring physical function, fatigue, depression, social participation, and quality of life, as well as physicians' assessments of disease activity and damage. Patients whose disease was currently active scored higher (worse) on all parts of the AAV-PRO, and the questionnaire's different sections showed strong statistical agreement with the corresponding established measures — for example, the 'Physical function' section of the AAV-PRO correlated very closely (r = -0.9) with the SF-36 physical function score. The AAV-PRO covers four key areas: physical symptoms, physical function, social and emotional impact, and concerns about the future. Each of these areas was found to correlate strongly with relevant clinical and patient-reported measures, confirming that the questionnaire captures a wide range of disease effects beyond what doctors can observe directly. Notably, the questionnaire also captured aspects like depression, fatigue, and optimism about the future, which are often overlooked in routine medical assessments. This research suggests that the AAV-PRO can be a useful tool in everyday clinical care for AAV patients, helping doctors understand how the disease affects their patients' daily lives, mental health, and social functioning — aspects that standard physician assessments may miss. Since AAV is a chronic and potentially severe disease requiring long-term management, having a validated way to monitor patient-perceived burden could help guide treatment decisions and improve patient-centered care.

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Citation

Jacobs M, Filla T, Chehab G, Stütz A, Niesmann A, von Saan M, et al.. (2026). Evaluating disease burden in German AAV patients using the AAV-PRO: associations with disease activity, physical function, depression, fatigue and quality of life.. Journal of patient-reported outcomes. https://doi.org/10.1186/s41687-026-01116-y