The Vasculitis Damage Index (VDI) is a standardized clinical assessment of damage caused by a systemic vasculitis, where damage is considered irreversible change resulting from scars. It consists of 65 items divided into 11 organ or system groups. The index was developed at the University of Birmingham in England.
No. |
Organ or System |
Item |
---|---|---|
1.1 |
Musculoskeletal |
significant muscle atrophy or weakness |
1.2 |
|
deforming or erosive arthritis |
1.3 |
|
avascular necrosis |
1.4 |
|
osteoporosis with fractures or vertebral collapse |
1.5 |
|
osteomyelitis |
2.1 |
Skin |
alopecia |
2.2 |
|
skin ulcerations |
2.3 |
|
oral ulcerations |
3.1 |
Ear, nose and throat |
hearing loss |
3.2 |
|
nasal blockage or chronic discharge or crusting |
3.3 |
|
nasal bridge collapse or septal perforation |
3.4 |
|
chronic sinusitis or radiologic evidence of bone destruction |
3.5 |
|
subglottal stenosis without surgery |
3.6 |
|
subglottal stenosis with surgery |
4.1 |
Pulmonary |
pulmonary hypertension |
4.2 |
|
pulmonary fibrosis/cavity |
4.3 |
|
pleural fibrosis |
4.4 |
|
pulmonary infarction |
4.5 |
|
chronic asthma |
4.6 |
|
significant chronic breathlessness |
4.7 |
|
impaired pulmonary function tests |
5.1 |
Cardiovascular |
angina or coronary artery bypass |
5.2 |
|
myocardial infarction |
5.3 |
|
second myocardial infarction |
5.4 |
|
cardiomyopathy |
5.5 |
|
valvular disease |
5.6 |
|
pericarditis |
5.7 |
|
hypertension |
6.1 |
Renal |
estimated or measured GFR < 50% |
6.2 |
|
proteinuria > 0.5 g per 24 hours |
6.3 |
|
end-stage renal failure |
7.1 |
Gastrointestinal |
gut infarction |
7.2 |
|
mesenteric insufficiency or pancreatitis |
7.3 |
|
chronic peritonitis |
7.4 |
|
esophageal structure or upper GI tract surgery |
8.1 |
Peripheral vascular |
absent peripheral pulse in 1 limb |
8.2 |
|
second episode of absent pulse in 1 limb |
8.3 |
|
absent peripheral pulses in >= 2 limbs |
8.4 |
|
major vessel stenosis |
8.5 |
|
claudication of the extremities |
8.6 |
|
complicated venous thrombosis |
8.7 |
|
minor tissue loss |
8.8 |
|
major tissue loss |
8.9 |
|
second episode of major tissue loss |
9.1 |
Ocular |
cataract |
9.2 |
|
retinal change |
9.3 |
|
optic atrophy |
9.4 |
|
visual impairment/diplopia |
9.5 |
|
blindness in 1 eye |
9.6 |
|
blindness in second eye |
9.7 |
|
orbital wall destruction |
10.1 |
Neuropsychiatric |
cognitive impairment |
10.2 |
|
major psychosis |
10.3 |
|
seizures |
10.4 |
|
cerebrovascular accident |
10.5 |
|
second cerebrovascular accident |
10.6 |
|
cranial nerve lesion |
10.7 |
|
peripheral neuropathy |
10.8 |
|
transverse myelitis |
11.1 |
Other damage |
premature gonadal failure |
11.2 |
|
marrow failure |
11.3 |
|
diabetes mellitus |
11.4 |
|
chronic chemical cystitis |
11.5 |
|
malignancy |
11.6 |
|
other feature not listed |
Item |
Description |
---|---|
significant muscle atrophy or weakness |
demonstrated on clinical examination, not attributable to a cerebrovascular accident |
deforming or erosive arthritis |
demonstrated on clinical examination, confirmed by radiographs, excludes avascular necrosis |
avascular necrosis |
demonstrated by appropriate radiographic techniques; ever, since onset of vasculitis |
osteoporosis with fractures or vertebral collapse |
by history, confirmed on radiographs, excludes avascular necrosis; ever, since onset of vasculitis |
osteomyelitis |
demonstrated clinically; confirmed by radiographs and/or culture |
alopecia |
major chronic hair loss (e.g., requires a wig) with or without scars; documented clinically |
skin ulcerations |
open sore on skin surface, excluding that caused by venous thrombosis |
oral ulcerations |
recurrent crops or persistent mouth ulcers requiring therapy |
hearing loss |
any hearing loss due to middle ear involvement or to auditory nerve/cochlear damage, preferably confirmed by audiometry |
nasal blockage or chronic discharge or crusting |
difficulties with breathing through the nose and/or with purulent discharge and/or with crust formation usually requiring nasal lavage |
nasal bridge collapse or septal perforation |
saddle nose deformity and/or perforation of nasal septum |
chronic sinusitis or radiologic evidence of bone destruction |
chronic purulent nasal discharge with sinus pain and/or radiologic evidence of sinusitis with or without bone destruction |
subglottal stenosis without surgery |
persistent hoarseness and/or stridor, preferably confirmed by endoscopy and/or radiographs |
subglottal stenosis with surgery |
confirmed by otolaryngology surgeon |
pulmonary hypertension |
right ventricular prominence or loud P2; confirmed by cardiologic investigation if appropriate |
pulmonary fibrosis/cavity |
according to physical signs and radiographs and confirmed by relevant tests if necessary; this may include patients who require pulmonary resection |
pleural fibrosis |
according to chest radiographs |
pulmonary infarction |
according to chest radiographs or ventilation/perfusion scan |
chronic asthma |
significant reversible airway obstruction |
significant chronic breathlessness |
significant symptomatic breathing difficulties and/or shortness of breath without hard signs on radiography or lung function tests |
impaired pulmonary function tests |
forced expiratory volume in 1 second (FEV1) or forced vital capacity (VC) <= 70%, or transfer coefficient for carbon monoxide (KCO) <= 70% |
angina or coronary artery bypass |
on history, confirmed at least by electrocardiographic changes |
myocardial infarction |
on history, confirmed by ECG changes or cardiac enzyme elevation; ever, since the onset of vasculitis |
second myocardial infarction |
at least 3 months after first event |
cardiomyopathy |
chronic ventricular dysfunction, documented clinically or on appropriate investigation |
valvular disease |
significant diastolic or systolic murmur, confirmed by cardiologic tests if appropriate |
pericarditis |
symptomatic pericardial inflammation or constriction for at least 3 months or pericardectomy |
hypertension |
with diastolic blood pressure > 95 mm Hg or requiring antihypertensive drugs |
estimated or measured GFR < 50% |
by any locally used method |
proteinuria > 0.5 g per 24 hours |
according to locally determined method |
end-stage renal failure |
failure of native kidneys for > 3 months regardless of subsequent dialysis or transplantation |
gut infarction |
infarction or resection of bowel below duodenum or of gallbladder, spleen or liver; ever, since the onset of vasculitis |
mesenteric insufficiency or pancreatitis |
typical abdominal pain confirmed on angiography or enzyme changes |
chronic peritonitis |
typical abdominal pain and peritoneal irritation on clinical examination |
esophageal structure or upper GI tract surgery |
documented endoscopically or radiologically; GI surgery ever, since the onset of vasculitis |
absent peripheral pulse in 1 limb |
in 1 limb, detected clinically |
second episode of absent pulse in 1 limb |
in 1 limb, detected clinically, at least 3 months after the first event |
absent peripheral pulses in >= 2 limbs |
in at least 2 limbs, detected clinically |
major vessel stenosis |
such as carotid or renal stenosis, documented on Doppler echocardiography or angiography |
claudication of the extremities |
exercise-related ischemic pain in peripheral large vessel, present at least 3 months |
complicated venous thrombosis |
with persistent swelling, ulceration or clinical evidence of venous stasis |
minor tissue loss |
such as loss of fingertip pulp space; ever, since the onset of vasculitis |
major tissue loss |
such as loss of digit(s) or limb(s), including by surgical resection; ever, since onset of vasculitis |
second episode of major tissue loss |
at least 3 months after first event |
cataract |
a lens opacity (cataract) in either eye, documented by ophthalmoscopy |
retinal change |
any significant change documented by ophthalmoscopic examination, may result in field defect, legal blindness |
optic atrophy |
documented by ophthalmoscopic examination |
visual impairment/diplopia |
restricted eye movements (not due to nerve palsies), reduced visual acuity, double vision or tunnel vision |
blindness in 1 eye |
complete loss of vision in 1 eye |
blindness in second eye |
at least 3 months after first event |
orbital wall destruction |
determined by plain radiographs or CT scan |
cognitive impairment |
memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance level, as documented on clinical examination (e.g., short mental test score) or by formal neurocognitive testing |
major psychosis |
altered ability to function in normal activity due to psychiatric reasons. Severe disturbance of the perception of reality characterized by the following features: delusions, hallucinations (auditory, visual), incoherence, marked loose associations, impoverished thought content, marked illogical thinking, bizarre, disorganized, or catatonic behavior |
seizures |
paroxysmal electrical discharge occurring in the brain and producing characteristic physical changes including tonic and clonic movements and certain behavioral disorders. Only seizures requiring therapy for more than 3 months are counted as damage. |
cerebrovascular accident |
Resulting in focal findings such as paresis, weakness, etc., or surgical resection for causes other than malignancy; ever, since the onset of vasculitis |
second cerebrovascular accident |
at least 3 months after the first event |
cranial nerve lesion |
cranial neuropathy, excluding optic nerve or sensorineural deafness |
peripheral neuropathy |
resulting in either motor or sensory dysfunction |
transverse myelitis |
lower extremity weakness or sensory loss with loss of sphincter control (rectal and urinary bladder) |
premature gonadal failure |
onset of menopause before the age of 40 years |
marrow failure |
leukopenia (WBC count < 4,000 per µL) or thrombocytopenia (platelet count < 140,000 per µL), or anemia (hemoglobin < 10 g/dL), preferably confirmed by marrow aspiration |
diabetes mellitus |
requiring any type of therapy |
chronic chemical cystitis |
persistent hematuria or shrunken bladder. This does not include acute hemorrhagic cystitis, which should be scored in the adverse drug reactions. |
malignancy |
documented by pathology, excluding dysplasias |
other feature not listed |
any other item of damage that the assessor wants to list that is not listed above; this is not included in the VDI score |
Items shared with other indices
(1) Systemic Necrotizing Vasculitis Damage Index (SNV): 1.3, 1.4, 1.5, 2.2, 4.2, 4.4, 4.7, 5.1, 5.2, 5.3, 5.4, 5.5, 5.7, 6.1 & 6.3 (single item), 7.1, 7.2, 8.5, 8.6, 8.7 & 8.8 (single item), 8.9, 9.1, 9.2, 9.5, 10.1, 10.3, 10.4, 10.5, 10.6, 10.7 (as 2 items), 11.3, 11.5
(2) Systemic Lupus International Cooperating Clinics/American College of Rheumatology Damage Index (SLICC/ACR): 1.1, 1.2, 1.3, 1.4, 1.5, 2.1, 2.2, 4.1, 4.2, 4.3, 4.4, 4.7, 5.1, 5.2, 5.3, 5.4, 5.5, 5.6, 6.1 & 6.2 & 6.3 (single item), 7.1, 7.2 (as 2 items), 7.3, 7.4, 8.5, 8.6, 8.7, 8.8, 8.9, 9.1, 9.2 & 9.3 (single item), 10.1 & 10.2 (single item), 10.3, 10.4, 10.5, 10.6 & 10.7 (single item), 10.8, 11.1, 11.3, 11.5
vasculitis damage index =
= SUM(items ever present)
increase in damage score =
= (vasculitis damage index now) – (vasculitis damage index in past)
where:
• If a finding has ever occurred, it is permanently set to present (1 point). This reflects the definition that damage is irreversible change resulting from scars.
• The final item (11.6) is not scored.
• A standard reference point for the increase in damage score is the VDI at time of initial presentation.
Interpretation:
• minimum score: 0 (which would raise doubts about the diagnosis of vasculitis)
• maximum score: 64
• Since "once positive always positive", the score can only stay unchanged or increase; it can never decrease.
• The higher the total score, the greater the total damage.
• The higher the increase in score, the greater the interval damage.
Purpose: To evaluate a person with vasculitis using the Vasculitis Damage Index (VDI).
Specialty: Immunology/Rheumatology
Objective: severity, prognosis, stage
ICD-10: L95, M05.2,