Protocol Overview

January 21, 2010

The Expert Review Panel has not reviewed this measure yet.

The System Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index for Systemic Lupus Erythematosus is a method that physician investigators use to determine the extent of permanent damage to various body organs or organ systems due to lupus or to the treatment thereof. This method is widely used and allows the physician to determine if a person has had any nonreversible changes (i.e., damage) to organ systems. These changes have occurred since a person is being diagnosed with lupus, are not related to active inflammation, and are ascertained via a clinical assessment. Also, unless stated otherwise, these changes must be present for at least six months prior to the assessment. The SLICC /ACR Damage Index is a cumulative index that records damage that occurs in individuals with lupus, regardless of whether the damage can be definitively attributed to lupus or is due to another cause, such as a comorbid condition.

Prior to completing this protocol, the PhenX Skin, Bone, Muscle and Joint Working Group (WG) notes that investigators must first confirm the presence of lupus in respondents. This can be done via the PhenX measure Autoimmune Diseases Related to Type 1 Diabetes. This measure uses one question to determine the presence of various autoimmune diseases (including lupus) in respondents or their children.

The PhenX Skin, Bone, Muscle and Joint Working Group also notes that there are Toolkit measures that can be used to assess the listed organ damage (e.g., glomerular filtration rate, diabetes, kidney disease, cognitive impairment, cataracts, etc.).

System Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index for Systemic Lupus Erythematosus

Glossary of SLICC/ACR Damage Index terms:

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 change:

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.

CVA:

Cerebovascular accident resulting in focal findings such as paresis, weakness, etc., or

surgical resection for causes other than malignancy.

Neuropathy :

Damage to either a 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/24 hours, or end-stage renal disease (regardless of dialysis or transplantation).

Pulmonary:

Pulmonary hypertension (right ventricular prominence, or loud P2), pulmonary fibrosis (physical and 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 pericardiectomy .

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 spleen, liver, or gall bladder 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, shown 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 40.

Diabetes:

Diabetes requiring therapy, but regardless of treatment.

Malignancy:

Documented by pathologic examination, excluding dysplasias.

Scoring Instructions:

Each item in the SLICC/ACR is scored as present only if it has been present for at least six months prior to the assessment. With the exception of end stage renal disease (ESRD), the presence of each item is given a score of 1 or 2. Whereas, ESRD is given a score of 3.

Of note, repeat episodes must occur at least six months apart and the same lesion cannot be scored twice, except for CVA.

Export Variables

Variable Name	Variable ID	Variable Description	Version	Mapping
PX171001_Ocular_Any_Cataract_Ever	PX171001010000	Ocular (either eye, by clinical assessment) Any cataract ever	4	N/A
PX171001_Ocular_Retinal_Change_Optic_Atrophy	PX171001020000	Ocular (either eye, by clinical assessment) Retinal change or optic atrophy	4	N/A
PX171001_Neuropsychiatric_Cognitive_Impairment	PX171001030000	Neuropsychiatric Cognitive impairment (e.g., memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance level) or major psychosis	4	N/A
PX171001_Neuropsychiatric_Seizures	PX171001040000	Neuropsychiatric Seizures requiring therapy for 6 months	4	N/A
PX171001_Neuropsychiatric_Cerebrovascular_Accident_Ever	PX171001050000	Neuropsychiatric Cerebrovascular accident ever (score 2 if > 1)	4	N/A
PX171001_Neuropsychiatric_Cranial_Or_Peripheral_Neuropathy	PX171001060000	Neuropsychiatric Cranial or peripheral neuropathy (excluding optic)	4	N/A
PX171001_Neuropsychiatric_Transverse_Myelitis	PX171001070000	Neuropsychiatric Transverse myelitis	4	N/A
PX171001_Renal_Glomerular_Filtration_Rate	PX171001080000	Renal Estimated or measured glomerular filtration rate <50%	4	N/A
PX171001_Renal_Proteinuria	PX171001090000	Renal Proteinuria > = 3.5 gm/24 hours	4	N/A
PX171001_Renal_Endstage_Renal_Disease	PX171001100000	Renal End-stage renal disease (regardless of dialysis or transplantation)	4	N/A
PX171001_Pulmonary_Hypertension	PX171001110000	Pulmonary Pulmonary hypertension (right ventricular prominence, or loud P2)	4	N/A
PX171001_Pulmonary_Fibrosis	PX171001120000	Pulmonary Pulmonary fibrosis (physical and radiograph)	4	N/A
PX171001_Pulmonary_Shrinking_Lung	PX171001130000	Pulmonary Shrinking lung (radiograph)	4	N/A
PX171001_Pulmonary_Pleural_Fibrosis	PX171001140000	Pulmonary Pleural fibrosis (radiograph)	4	N/A
PX171001_Infarction	PX171001150000	Pulmonary Pulmonary infarction (radiograph)	4	N/A
PX171001_Cardiovascular_Angina_Coronary_Artery_Bypass	PX171001160000	Cardiovascular Angina or coronary artery bypass	4	N/A
PX171001_Cardiovascular_Myocardial_Infarction_Ever	PX171001170000	Cardiovascular Myocardial infarction ever (score 2 if >I)	4	N/A
PX171001_Cardiovascular_Cardiomyopathy	PX171001180000	Cardiovascular Cardiomyopathy (ventricular dysfunction)	4	N/A
PX171001_Cardiovascular_Valvular_Disease	PX171001190000	Cardiovascular Valvular disease (diastolic, murmur, or systolic murmur >3/6)	4	N/A
PX171001_Cardiovascular_Pericarditis	PX171001200000	Cardiovascular Pericarditis for 6 months, or pericardiectomy	4	N/A
PX171001_Peripheral_Vascular_Claudication	PX171001210000	Peripheral vascular Claudication for 6 months	4	N/A
PX171001_Peripheral_Vascular_Minor_Tissue_Loss	PX171001220000	Peripheral vascular Minor tissue loss (pulp space)	4	N/A
PX171001_Peripheral_Vascular_Significant_Tissue_Loss	PX171001230000	Peripheral vascular Significant tissue loss ever (e.g., loss of digit or limb) (score 2 if >1 site)	4	N/A
PX171001_Peripheral_Vascular_Venous_Thrombosis	PX171001240000	Peripheral vascular Venous thrombosis with swelling, ulceration, or venous stasis	4	N/A
PX171001_Gastrointestinal_Infarction_Resection_Of_Bowel	PX171001250000	Gastrointestinal Infarction or resection of bowel below duodenum, spleen, liver, or gall bladder ever, for cause any (score 2 if > 1)	4	N/A
PX171001_Gastrointestinal_Mesenteric_Insufficiency	PX171001260000	Gastrointestinal Mesenteric insufficiency	4	N/A
PX171001_Gastrointestinal_Chronic_Peritonitis	PX171001270000	Gastrointestinal Chronic peritonitis	4	N/A
PX171001_Gastrointestinal_Stricture_Gastrointestinal_Tract_Surgery	PX171001280000	Gastrointestinal Stricture or upper gastrointestinal tract surgery ever	4	N/A
PX171001_Musculoskeletal_Muscle_Atrophy	PX171001290000	Musculoskeletal Muscle atrophy or weakness	4	N/A
PX171001_Musculoskeletal_Arthritis	PX171001300000	Musculoskeletal Deforming or erosive arthritis (including reducible deformities, excluding avascular necrosis)	4	N/A
PX171001_Musculoskeletal_Osteoporosis	PX171001310000	Musculoskeletal Osteoporosis with fracture or vertebral collapse (excluding avascular necrosis)	4	N/A
PX171001_Musculoskeletal_Avascular_Necrosis	PX171001320000	Musculoskeletal Avascular necrosis (score 2 if > 1 )	4	N/A
PX171001_Musculoskeletal_Osteomyelitis	PX171001330000	Musculoskeletal Osteomyelitis	4	N/A
PX171001_Skin_Scarring_Chronic_Alopecia	PX171001340000	Skin Scarring chronic alopecia	4	N/A
PX171001_Skin_Extensive_Scarring_Or_Panniculum	PX171001350000	Skin Extensive scarring or panniculum other than scalp and pulp space	4	N/A
PX171001_Skin_Ulceration	PX171001360000	Skin Skin ulceration (excluding thrombosis) for >6 months	4	N/A
PX171001_Premature_Gonadal_Failure	PX171001370000	Premature gonadal failure	4	N/A
PX171001_Diabetes	PX171001380000	Diabetes (regardless of treatment)	4	N/A
PX171001_Malignancy	PX171001390000	Malignancy (exclude dysplasia) (score 2 if > 1 site)	4	N/A

The System Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index for Systemic Lupus Erythematosus is a well-vetted method used in multiple clinical and research settings. The SLICC/ACR Damage Index determines the presence of damage to a person’s body due to the disease.

Gladman, D., Ginzler, E., Goldsmith, C., Fortin, P., Liang, M., Urowitz, M., Bacon, P., Bombardieri, S., Hanly, J., Hay, E., Isenberg, D., Jones, J., Kalunian, K., Maddison, P., Nived, O., Petri, M., Richter, M., Sanchez-Guerrero, J., Snaith, M., Sturfelt, G., Symmons, D., & Zoma, A. (1996). The Development and Initial Validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus Arthritis & Rheumatism, 39(3), 363–369.399

Adult

English

Adults, Older Adults

A trained physician is required to perform the clinical assessment associated with the Systemic Lupus International Collaborating Clinics/American College (SLICC/ACR) Damage Index.

None

Standard	Name	ID	Source
Common Data Elements (CDE)	Person Clinical Systemic Lupus Erythematosus Assessment Score	3182299	CDE Browser
Logical Observation Identifiers Names and Codes (LOINC)	Lupus SLE proto	64392-4	LOINC

Alarcon, G. S., Roseman, J. M., McGwin, G., Jr., Uribe, A., Bastian, H. M., Fessler, B. J., Baethge, B. A., Friedman, A. W., & Reveille, J. D.; the LUMINA Study Group. (2004). Systemic lupus erythmatosis in three ethnic groups. XX. Damage as a predictor of further damage. Rheumatology, 43, 202–205.

Danila, M. I., Pons-Estel, G. J. Zhang, J., Vila, L. M., Reveille, J. D., & Alarcon, G. S. (2009). Renal damage is the most important predictor of mortality within the damage index: data from LUMINA LXIV, a multiethnic US cohort Rheumatology, 48, 542–545.

Lupus in Minorities: Nature versus Nurture (LUMINA) study glossary. Available by contacting one of the study investigators, Dr. Graciela S. Alarcon or Dr. John Reveille.

Clinical Examination

None

Requirement Category	Required
Average time of greater than 15 minutes in an unaffected individual	Yes
Major equipment	No
Specialized requirements for biospecimen collection	No
Specialized training	Yes

The Expert Review Panel has not reviewed this measure yet.