SLE Damage Index | Systemic Lupus Erythematosus

Damage

Nonreversible change, not related to active inflammation, occurring since diagnosis of lupus, ascertained by clinical assessment and present for at least 6 months, unless otherwise stated. Repeat episodes must occur at least 6 months apart to score 2. The same lesion cannot be scored twice.

Cataract

A lens opacity (cataract) in either eye, ever, whether primary or secondary to steroid therapy, documented by ophthalmoscopy

Retinal damage

Documented by ophthalmoscopic examination, may result in field defect, legal blindness

Optic atrophy

Documented by ophthalmoscopic examination.

Cognitive impairment

Memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance level, documented on clinical examination or by formal neurocognitive testing.

Major psychosis

Altered ability to function in normal activity due to psychiatric reasons. Severe disturbance in 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 6 months are counted as damage.

Cerebrovascular accident (CVA)

Cerebrovascular accident resulting in focal findings such as paresis, weakness, etc., or surgical resection for causes other than malignancy.

Neuropathy

Damage to either cranial or peripheral nerve, excluding optic nerve, resulting in either motor or sensory dysfunction.

Transverse myelitis

Lower extremity weakness or sensory loss with loss of rectal and urinary bladder sphincter control

Renal

Estimated or measured glomerular filtration rate < 50%, proteinuria >= 3.5 gm per 24 hours, or end-stage renal disease (regardless of dialysis or transplantation)

Pulmonary

Pulmonary hypertension (right ventricular prominence, or loud P2); pulmonary fibrosis (physical or radiograph); shrinking lung (radiograph); pleural fibrosis (radiograph); pulmonary infarction (radiograph); resection for cause other than malignancy)

Cardiovascular

Angina or coronary artery bypass; myocardial infarction (documented by electrocardiograph and enzyme studies), ever; cardiomyopathy (ventricular dysfunction documented clinically); valvular disease (diastolic murmur, or systolic murmur > 3/6); pericarditis for 6 months, or pericardectomy

Peripheral vascular

Claudication, persistent for 6 months, by history; minor tissue loss, such as pulp space, ever; significant tissue loss, such as loss of digit or limb, or resection, ever; venous thrombosis with swelling, ulceration or clinical evidence of venous stasis

Gastrointestinal

Infarction or resection of bowel below duodenum, by history, resection of liver, spleen, or gallbladder, ever, for whatever cause; mesenteric insufficiency, with diffuse abdominal pain on clinical examination; chronic peritonitis with persistent abdominal pain and peritoneal irritations on clinical examination; esophageal stricture on endoscopy, upper gastrointestinal tract surgery, such as correction of stricture, ulcer surgery, etc. , ever, by history; pancreatic insufficiency requiring enzyme replacement or with a pseudocyst

Musculoskeletal

Muscle atrophy or weakness, demonstrated on clinical examination; deforming or erosive arthritis, including reducible deformities (excluding avascular necrosis) on clinical examination; osteoporosis with fracture or vertebral collapse (excluding avascular necrosis) demonstrated radiographically; avascular necrosis, demonstrated by any imaging technique; osteomyelitis, documented clinically, and supported by culture evidence; tendon ruptures

Skin

Scarring, chronic alopecia, documented clinically; extensive scarring or panniculum other than scalp and pulp space, documented clinically; skin ulceration (excluding thrombosis) for more than 6 months

Premature gonadal failure

Secondary amenorrhea prior to age of 40

Diabetes

Diabetes requiring therapy, but regardless of treatment.

Malignancy

Documented by pathologic examination, excluding dysplasia

Ocular (either eye, by clinical assessment)

any cataract ever

1

retinal damage or optic atrophy

1

Neuropsychiatric

cognitive impairment (e.g., memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance level) or major psychosis

1

seizures requiring therapy for 6 months

1

cerebrovascular accident ever (score 2 if more than 1), or surgical resection for causes other than malignancy

1 or 2

cranial or peripheral neuropathy (excluding optic)

1

transverse myelitis

1

Renal

estimated or measured glomerular filtration rate < 50%, or

1

proteinuria >= 3.5 g per 24 hours,or

1

end-stage renal disease (regardless of dialysis or transplantation)

3

Pulmonary

pulmonary hypertension (right ventricular prominence, or loud P2)

1

pulmonary fibrosis (physical and radiograph)

1

shrinking lung (on radiograph)

1

pleural fibrosis (on radiograph)

1

pulmonary infarction (on radiograph), OR pulmonary resection for cause other than malignancy

1

Cardiovascular

angina or coronary artery bypass

1

Myocardial infarction ever (score 2 if more than 1)

1 or 2

cardiomyopathy (ventricular dysfunction)

1

valvular disease (diastolic murmur, or systolic murmur > 3/6)

1

pericarditis for 6 months, or pericardiectomy

1

Peripheral vascular

claudication for 6 months

1

minor tissue loss (pulp space)

1

significant tissue loss ever (e.g., loss of digit or limb, resection) (score 2 if more than one site)

1 or 2

venous thrombosis with swelling, ulceration or venous stasis

1

Gastrointestinal

infarction or resection of bowel below duodenum, spleen, liver or gallbladder, for any cause (score 2 if more than 1 site)

1 or 2

mesenteric insufficiency

1

chronic peritonitis

1

esophageal stricture or upper gastrointestinal tract surgery ever

1

pancreatic insufficiency, requiring enzyme replacement, or pseudocyst

1

Musculoskeletal

muscle atrophy or weakness

1

deforming or erosive arthritis (including reducible deformities, excluding avascular necrosis)

1

osteoporosis with fracture or vertebral collapse (excluding avascular necrosis)

1

avascular necrosis (score 2 if more than 1)

1 or 2

osteomyelitis

1

Skin

scarring chronic alopecia

1

extensive scarring or panniculum other than scalp and pulp space

1

skin ulceration (excluding thrombosis) for more than 6 months

1

Other

premature gonadal failure

1

diabetes (regardless of treatment)

1

malignancy (exclude dysplasia) (score 2 if more than 1 site)

1 or 2