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Protocol - Systemic Lupus Erythematosus

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Description:

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.

Protocol:

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

Item Score
Ocular (either eye, by clinical assessment)
Any cataract ever 1
Retinal change 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 > 1) 1 (2)
Cranial or peripheral neuropathy (excluding optic) 1
Transverse myelitis 1
Renal
Estimated or measured glomerular filtration rate <50% 1
Proteinuria ≥3.5 gm/24 hours 1
or
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 (radiograph) 1
Pleural fibrosis (radiograph) 1
Pulmonary infarction (radiograph) 1
Cardiovascular
Angina or coronary artery bypass 1
Myocardial infarction ever (score 2 if > 1) 1 (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) (score 2 if >1 site) 1 (2)
Venous thrombosis with swelling, ulceration, or venous stasis 1
Gastrointestinal
Infarction or resection of bowel below duodenum, spleen, liver, or gall bladder ever, for cause any (score 2 if > 1 site) 1 (2)
Mesenteric insufficiency 1
Chronic peritonitis 1
Stricture or upper gastrointestinal tract surgery ever 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 > 1 ) 1 (2)
Osteomyelitis 1
Skin
Scarring chronic alopecia 1
Extensive scarring or panniculum other than scalp and pulp space 1
Skin ulceration (excluding thrombosis) for >6 months 1
Premature gonadal failure 1
Diabetes (regardless of treatment) 1
Malignancy (exclude dysplasia) (score 2 if > 1 site) 1 (2)

 

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.

Protocol Name from Source:

The Expert Review Panel has not reviewed this measure yet.

Availability:

Publicly available

Personnel and Training Required

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

Equipment Needs

None

Requirements
Requirement CategoryRequired
Major equipment No
Specialized training Yes
Specialized requirements for biospecimen collection No
Average time of greater than 15 minutes in an unaffected individual Yes
Mode of Administration

Clinical Examination

Life Stage:

Adult, Senior

Participants:

Adults, Older Adults

Specific Instructions:

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.).

Selection Rationale

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.

Language

English

Standards
StandardNameIDSource
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
Derived Variables

None

Process and Review

The Expert Review Panel has not reviewed this measure yet.

Source

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

General References

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.

Protocol ID:

171001

Variables:
Export Variables
Variable NameVariable IDVariable DescriptionVersiondbGaP Mapping
PX171001_Cardiovascular_Angina_Coronary_Artery_Bypass PX171001160000 Cardiovascular Angina or coronary artery bypass 4 N/A
PX171001_Cardiovascular_Cardiomyopathy PX171001180000 Cardiovascular Cardiomyopathy (ventricular dysfunction) 4 N/A
PX171001_Cardiovascular_Myocardial_Infarction_Ever PX171001170000 Cardiovascular Myocardial infarction ever (score 2 if >I) 4 N/A
PX171001_Cardiovascular_Pericarditis PX171001200000 Cardiovascular Pericarditis for 6 months, or pericardiectomy 4 N/A
PX171001_Cardiovascular_Valvular_Disease PX171001190000 Cardiovascular Valvular disease (diastolic, murmur, or systolic murmur >3/6) 4 N/A
PX171001_Diabetes PX171001380000 Diabetes (regardless of treatment) 4 N/A
PX171001_Gastrointestinal_Chronic_Peritonitis PX171001270000 Gastrointestinal Chronic peritonitis 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_Stricture_Gastrointestinal_Tract_Surgery PX171001280000 Gastrointestinal Stricture or upper gastrointestinal tract surgery ever 4 N/A
PX171001_Infarction PX171001150000 Pulmonary Pulmonary infarction (radiograph) 4 N/A
PX171001_Malignancy PX171001390000 Malignancy (exclude dysplasia) (score 2 if > 1 site) 4 N/A
PX171001_Musculoskeletal_Arthritis PX171001300000 Musculoskeletal Deforming or erosive arthritis (including reducible deformities, excluding avascular necrosis) 4 N/A
PX171001_Musculoskeletal_Avascular_Necrosis PX171001320000 Musculoskeletal Avascular necrosis (score 2 if > 1 ) 4 N/A
PX171001_Musculoskeletal_Muscle_Atrophy PX171001290000 Musculoskeletal Muscle atrophy or weakness 4 N/A
PX171001_Musculoskeletal_Osteomyelitis PX171001330000 Musculoskeletal Osteomyelitis 4 N/A
PX171001_Musculoskeletal_Osteoporosis PX171001310000 Musculoskeletal Osteoporosis with fracture or vertebral collapse (excluding avascular necrosis) 4 N/A
PX171001_Neuropsychiatric_Cerebrovascular_Accident_Ever PX171001050000 Neuropsychiatric Cerebrovascular accident ever (score 2 if > 1) 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_Cranial_Or_Peripheral_Neuropathy PX171001060000 Neuropsychiatric Cranial or peripheral neuropathy (excluding optic) 4 N/A
PX171001_Neuropsychiatric_Seizures PX171001040000 Neuropsychiatric Seizures requiring therapy for 6 months 4 N/A
PX171001_Neuropsychiatric_Transverse_Myelitis PX171001070000 Neuropsychiatric Transverse myelitis 4 N/A
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_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_Premature_Gonadal_Failure PX171001370000 Premature gonadal failure 4 N/A
PX171001_Pulmonary_Fibrosis PX171001120000 Pulmonary Pulmonary fibrosis (physical and radiograph) 4 N/A
PX171001_Pulmonary_Hypertension PX171001110000 Pulmonary Pulmonary hypertension (right ventricular prominence, or loud P2) 4 N/A
PX171001_Pulmonary_Pleural_Fibrosis PX171001140000 Pulmonary Pleural fibrosis (radiograph) 4 N/A
PX171001_Pulmonary_Shrinking_Lung PX171001130000 Pulmonary Shrinking lung (radiograph) 4 N/A
PX171001_Renal_Endstage_Renal_Disease PX171001100000 Renal End-stage renal disease (regardless of dialysis or transplantation) 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_Skin_Extensive_Scarring_Or_Panniculum PX171001350000 Skin Extensive scarring or panniculum other than scalp and pulp space 4 N/A
PX171001_Skin_Scarring_Chronic_Alopecia PX171001340000 Skin Scarring chronic alopecia 4 N/A
PX171001_Skin_Ulceration PX171001360000 Skin Skin ulceration (excluding thrombosis) for >6 months 4 N/A
Research Domain Information
Measure Name:

Systemic Lupus Erythematosus

Release Date:

January 21, 2010

Definition

This measure is a clinical assessment of individuals with systemic lupus erythematosus (SLE, or lupus) to determine the level of organ damage due to the disease.

Purpose

Systemic lupus erythematosus (SLE, or lupus) is a chronic inflammatory autoimmune disease that can affect the skin, joints, lungs, kidneys, nervous system, and other body organs.

Keywords

Systemic Lupus International Collaborating Clinics, SLICC Damage Index, SDI, American College of Rheumatology, ACR, Systemic Lupus Erythematosus, SLE, Autoimmune disorder, Autoimmune disease, Inflammatory, Skin Bone Muscle and Joint