✓
Alopecia (extensive, for 6 months or more)
+1
✓
Extensive scarring (burns, chemical, surgical - more than 1% body area)
+1
✓
Skin ulceration (non-healed, for 6 months or more)
+1
✓
Sensorineural hearing loss
+1
✓
Nasal blockage/chronic sinusitis/crusting (no surgery required, for 6 months or more)
+1
✓
Nasal bridge collapse or septal perforation
+1
✓
Chronic laryngeal or tracheal stenosis (with or without surgery)
+1
✓
Salivary gland damage
+1
✓
Pulmonary hypertension (right heart failure or echocardiographic diagnosis)
+1
✓
Pulmonary fibrosis (clinical or radiological)
+1
✓
Pulmonary infarction or resection
+1
✓
Respiratory impairment (FEV1 or FVC less than 75% predicted or requiring O2)
+1
✓
Angina pectoris (symptom-based) or coronary artery bypass grafting
+1
✓
Myocardial infarction (ever)
+1
✓
Cardiomyopathy (ventricular dysfunction)
+1
✓
Valvular disease (diastolic murmur greater than grade 3 or diastolic)
+1
✓
Pericarditis for 6 months or more, or pericarditis requiring surgery
+1
✓
Hypertension (diastolic greater than 95 mmHg or requiring antihypertensive therapy)
+1
✓
Absence of pulses in 2 or more vessels (excluding aorta)
+1
✓
Loss of major vessel (aorta or other large vessel)
+1
✓
Intermittent claudication (for 3 months or more)
+1
✓
Significant tissue loss (loss of digit or limb)
+1
✓
Non-healing chronic ulcer (for 6 months or more)
+1
✓
Significant cognitive impairment (including dementia)
+1
✓
Cerebrovascular accident (ever) or surgical resection
+1
✓
Peripheral neuropathy (motor neuropathy or mononeuritis multiplex)
+1
✓
Transverse myelitis or cord damage
+1
✓
Seizure disorder (requiring treatment for 6 months or more)
+1
✓
Treatment failure (ever required cyclophosphamide or other second-line agent)
+1
✓
Gonadal failure from treatment
+1
✓
Diabetes mellitus (not previously present)
+1
✓
Drug-related cystitis (requiring treatment or causing long-term bladder dysfunction)
+1
✓
Malignancy (excluding dysplasia)
+1
📊 Interpreting the VDI
The VDI is a count of distinct items of irreversible damage present (0 to 64 items maximum, though the theoretical maximum is rarely reached). Unlike BVAS, there is no linear threshold - any VDI accumulation is clinically meaningful because damage items have been shown to independently predict mortality in systemic vasculitis cohort studies.
| VDI Score | Interpretation | Clinical Significance |
|---|
| 0 | No Damage | No irreversible organ damage from disease or treatment. Unusual in long-standing vasculitis. |
| 1 to 3 | Mild Damage | Limited damage accrual. Monitor progression over time. |
| 4 to 7 | Moderate Damage | Significant irreversible damage. Associated with impaired quality of life and higher mortality risk. |
| 8 or more | Severe Damage | Substantial damage burden. High mortality risk in long-term cohort data. Minimize further damage accrual aggressively. |
In long-term AAV cohort studies, each unit increase in VDI score is associated with approximately 24% higher mortality risk (Exley et al. 1997, Flossmann et al. 2011). Annual VDI tracking is recommended in active vasculitis patients.
💡 Pearls and Pitfalls
✓
Track VDI at every visit, not just at diagnosis. The VDI is designed for longitudinal monitoring. The delta VDI (change from baseline) is more prognostically informative than absolute VDI at a single time point. New damage items appearing under treatment suggest inadequate disease control or treatment toxicity.
✓
Treatment toxicity items are scored the same as disease damage. Diabetes mellitus from glucocorticoids, gonadal failure from cyclophosphamide, and bladder damage from cyclophosphamide are scored in the VDI. This feature makes VDI uniquely useful for comparing harm profiles of different treatment regimens.
⚠
VDI does not capture activity - use BVAS for active disease. VDI items must be irreversible. A renal impairment that improves with treatment should not be scored. The most common scoring error is including features that reflect current inflammation (reversible) rather than permanent damage (irreversible).
🔮 Evidence
The VDI was developed by Exley, Bacon, Luqmani and colleagues and published in Annals of the Rheumatic Diseases in 1997. Development used a methodology similar to the SLICC/ACR damage index in SLE, adapted for the specific damage patterns seen in systemic vasculitis. Long-term cohort data from the European Vasculitis Study Group (EUVAS) have validated the prognostic importance of VDI accumulation.
View References▾
1
Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40(2):371-380.
2
Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-494. VDI independently predicts mortality.