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Protocol - Systemic Lupus Erythematosus

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Protocol Name from Source:

The Expert Review Panel has not reviewed this measure yet.

Availability:

Publicly available

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.

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
Average time of greater than 15 minutes in an unaffected individualYes
Major equipmentNo
Specialized requirements for biospecimen collectionNo
Specialized trainingYes

Mode of Administration

Self-administered

Life Stage:

Adult, Senior

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.).

Research Domain Information

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.

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 Score3182299CDE Browser
Logical Observation Identifiers Names and Codes (LOINC)Lupus SLE proto64392-4LOINC

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_Ocular_Any_Cataract_EverPX171001010000Ocular (either eye, by clinical assessment) Any cataract ever4N/A
PX171001_Ocular_Retinal_Change_Optic_AtrophyPX171001020000Ocular (either eye, by clinical assessment) Retinal change or optic atrophy4N/A
PX171001_Neuropsychiatric_Cognitive_ImpairmentPX171001030000Neuropsychiatric Cognitive impairment (e.g., memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance level) or major psychosis4N/A
PX171001_Neuropsychiatric_SeizuresPX171001040000Neuropsychiatric Seizures requiring therapy for 6 months4N/A
PX171001_Neuropsychiatric_Cerebrovascular_Accident_EverPX171001050000Neuropsychiatric Cerebrovascular accident ever (score 2 if > 1)4N/A
PX171001_Neuropsychiatric_Cranial_Or_Peripheral_NeuropathyPX171001060000Neuropsychiatric Cranial or peripheral neuropathy (excluding optic)4N/A
PX171001_Neuropsychiatric_Transverse_MyelitisPX171001070000Neuropsychiatric Transverse myelitis4N/A
PX171001_Renal_Glomerular_Filtration_RatePX171001080000Renal Estimated or measured glomerular filtration rate <50%4N/A
PX171001_Renal_ProteinuriaPX171001090000Renal Proteinuria > = 3.5 gm/24 hours4N/A
PX171001_Renal_Endstage_Renal_DiseasePX171001100000Renal End-stage renal disease (regardless of dialysis or transplantation)4N/A
PX171001_Pulmonary_HypertensionPX171001110000Pulmonary Pulmonary hypertension (right ventricular prominence, or loud P2)4N/A
PX171001_Pulmonary_FibrosisPX171001120000Pulmonary Pulmonary fibrosis (physical and radiograph)4N/A
PX171001_Pulmonary_Shrinking_LungPX171001130000Pulmonary Shrinking lung (radiograph)4N/A
PX171001_Pulmonary_Pleural_FibrosisPX171001140000Pulmonary Pleural fibrosis (radiograph)4N/A
PX171001_InfarctionPX171001150000Pulmonary Pulmonary infarction (radiograph)4N/A
PX171001_Cardiovascular_Angina_Coronary_Artery_BypassPX171001160000Cardiovascular Angina or coronary artery bypass4N/A
PX171001_Cardiovascular_Myocardial_Infarction_EverPX171001170000Cardiovascular Myocardial infarction ever (score 2 if >I)4N/A
PX171001_Cardiovascular_CardiomyopathyPX171001180000Cardiovascular Cardiomyopathy (ventricular dysfunction)4N/A
PX171001_Cardiovascular_Valvular_DiseasePX171001190000Cardiovascular Valvular disease (diastolic, murmur, or systolic murmur >3/6)4N/A
PX171001_Cardiovascular_PericarditisPX171001200000Cardiovascular Pericarditis for 6 months, or pericardiectomy4N/A
PX171001_Peripheral_Vascular_ClaudicationPX171001210000Peripheral vascular Claudication for 6 months4N/A
PX171001_Peripheral_Vascular_Minor_Tissue_LossPX171001220000Peripheral vascular Minor tissue loss (pulp space)4N/A
PX171001_Peripheral_Vascular_Significant_Tissue_LossPX171001230000Peripheral vascular Significant tissue loss ever (e.g., loss of digit or limb) (score 2 if >1 site)4N/A
PX171001_Peripheral_Vascular_Venous_ThrombosisPX171001240000Peripheral vascular Venous thrombosis with swelling, ulceration, or venous stasis4N/A
PX171001_Gastrointestinal_Infarction_Resection_Of_BowelPX171001250000Gastrointestinal Infarction or resection of bowel below duodenum, spleen, liver, or gall bladder ever, for cause any (score 2 if > 1)4N/A
PX171001_Gastrointestinal_Mesenteric_InsufficiencyPX171001260000Gastrointestinal Mesenteric insufficiency4N/A
PX171001_Gastrointestinal_Chronic_PeritonitisPX171001270000Gastrointestinal Chronic peritonitis4N/A
PX171001_Gastrointestinal_Stricture_Gastrointestinal_Tract_SurgeryPX171001280000Gastrointestinal Stricture or upper gastrointestinal tract surgery ever4N/A
PX171001_Musculoskeletal_Muscle_AtrophyPX171001290000Musculoskeletal Muscle atrophy or weakness4N/A
PX171001_Musculoskeletal_ArthritisPX171001300000Musculoskeletal Deforming or erosive arthritis (including reducible deformities, excluding avascular necrosis)4N/A
PX171001_Musculoskeletal_OsteoporosisPX171001310000Musculoskeletal Osteoporosis with fracture or vertebral collapse (excluding avascular necrosis)4N/A
PX171001_Musculoskeletal_Avascular_NecrosisPX171001320000Musculoskeletal Avascular necrosis (score 2 if > 1 )4N/A
PX171001_Musculoskeletal_OsteomyelitisPX171001330000Musculoskeletal Osteomyelitis4N/A
PX171001_Skin_Scarring_Chronic_AlopeciaPX171001340000Skin Scarring chronic alopecia4N/A
PX171001_Skin_Extensive_Scarring_Or_PanniculumPX171001350000Skin Extensive scarring or panniculum other than scalp and pulp space4N/A
PX171001_Skin_UlcerationPX171001360000Skin Skin ulceration (excluding thrombosis) for >6 months4N/A
PX171001_Premature_Gonadal_FailurePX171001370000Premature gonadal failure4N/A
PX171001_DiabetesPX171001380000Diabetes (regardless of treatment)4N/A
PX171001_MalignancyPX171001390000Malignancy (exclude dysplasia) (score 2 if > 1 site)4N/A