Vasculitis Damage Index (VDI)

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

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