Protocol - Systemic Lupus Erythematosus
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
|Ocular (either eye, by clinical assessment)|
|Any cataract ever||1|
|Retinal change or optic atrophy||1|
|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|
|Estimated or measured glomerular filtration rate <50%||1|
|Proteinuria ≥3.5 gm/24 hours||1|
|End-stage renal disease (regardless of dialysis or transplantation)||3|
|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|
|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|
|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|
|Infarction or resection of bowel below duodenum, spleen, liver, or gall bladder ever, for cause any (score 2 if > 1 site)||1 (2)|
|Stricture or upper gastrointestinal tract surgery ever||1|
|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)|
|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:
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.
A lens opacity (cataract) in either eye, ever, whether primary or secondary to steroid therapy, documented by ophthalmoscopy.
Documented by ophthalmoscopic examination, may result in field defect, legal blindness.
Documented by ophthalmoscopic examination.
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.
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.
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.
Cerebovascular accident resulting in focal findings such as paresis, weakness, etc., or
surgical resection for causes other than malignancy.
Damage to either a cranial or peripheral nerve, excluding optic nerve, resulting in either motor or sensory dysfunction.
Lower-extremity weakness or sensory loss with loss of rectal and urinary bladder sphincter control.
Estimated or measured glomerular filtration rate < 50%, proteinuria ≥ 3.5 gm/24 hours, or end-stage renal disease (regardless of dialysis or transplantation).
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.
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 .
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.
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.
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.
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 requiring therapy, but regardless of treatment.
Documented by pathologic examination, excluding dysplasias.
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.
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.
|Specialized requirements for biospecimen collection||No|
|Average time of greater than 15 minutes in an unaffected individual||Yes|
Mode of Administration
Adults, Older Adults
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.
|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|
Process and Review
The Expert Review Panel has not reviewed this measure yet.
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
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.
|Variable Name||Variable ID||Variable Description||Version||dbGaP 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_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|
Systemic Lupus Erythematosus
January 21, 2010
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.
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.
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