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INTRODUCTION

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Although the presentation, clinical symptoms, and laboratory findings of pediatric systemic lupus erythematosus (SLE) are similar to those that are seen in adults, children and adolescents with SLE differ from adults in terms of the frequency and severity of disease activity and damage features as well as in the treatment approaches used by their attending physicians. Furthermore, assessment of pediatric patients with SLE should take into account the disease and physical/mental age–related issues that are associated with growth and development. For these reasons, it cannot be assumed a priori that the clinical measures developed for adults are suitable for children and adolescents. Therefore, outcome measures used in adults need to be subjected to critical evidence-based evaluation of their measurement properties in children and adolescents.

Because the general characteristics of disease activity and damage measures used in SLE are addressed in another article in this issue of Arthritis Care & Research, our review will focus on the available information specific to pediatric SLE and the critical appraisal of the value of each instrument to pediatric rheumatologists dealing with children and adolescents with SLE. As a general rule, in all measures scored items and abnormalities must be attributable to SLE.

BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

In a prospective observational 12-month study of 21 patients with systemic lupus erythematosus (SLE), the renal BILAG score was found to be able to differentiate between patients with nephritis (n = 10) and patients without nephritis (n = 11) (1).

Ability to detect change.

Excellent responsiveness to change in disease activity was documented, by means of effect size, effect size index, standardized response mean, responsiveness statistic, and relative efficiency index, in a comparative study with the Systemic Lupus Erythematosus Activity Measure and the Systemic Lupus Erythematosus Disease Activity Index that involved 35 newly-diagnosed patients (2). In a study of 98 patients who were seen every 3 months for up to 7 visits (n = 623 total visits), the minimum clinically important difference (MCID) for clinically important improvement or worsening, based on physician's or parent's rating of the disease course between visits, was small. Using the standard error of measurement approach, the MCID value was 2 (3). The MCID is defined as “the smallest difference in a score of a disease measure of interest that patients perceive as beneficial and that would mandate, in the absence of side effects, a change in the patient management” (4).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

The BILAG is the most comprehensive of the SLE activity measures. It is the only SLE activity index that aims specifically to show activity in individual organs/systems. It is the only transitional index, with each item that is present being recorded as new, the same, worse or improving, rather than just present or absent (5). Although initially the developers of the index did not intend to create a cumulative score, a numerical scoring scheme was developed for the 2004 version (6).

Caveats and cautions.

With 86 items, it is the longest of the lupus activity tools. It remains to be established whether a different numeric conversion table should be developed for the pediatric population because of the differences in the extent of disease features between pediatric and adult SLE. It does not include immunologic tests.

Clinical usability.

The BILAG is a reliable and valid instrument for measuring clinical disease activity in pediatric patients with SLE in standard clinical practice. It enables an accurate assessment of disease activity in individual organs/systems and detection of new activity or flare in one or more systems, and in which system(s). Furthermore, it helps determine when a change in therapy is needed. However, performing the BILAG is time consuming and requires training, which may limit its applicability in routine care.

Research usability.

The BILAG is best suited when the assessment of the actual level or change over time in disease activity in individual organs/systems is the primary objective of the study. The excellent responsiveness to change seen in pediatric studies supports its use as a response measure in clinical trials in children and adolescents with lupus, particularly when the efficacy of a medication on single-organ involvement (e.g., nephritis, skin disease) is under scrutiny.

Advantages/disadvantages of the different versions of the BILAG.

There are 2 versions of the BILAG: the original BILAG and the BILAG-2004 (5, 6). An advantage of the 2004 version is that its numerical scoring system may overcome the inability to give an overall score in the original BILAG (7). All studies performed in pediatric SLE have used the original version of the index.

EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

The ECLAM (8) was found to have construct validity and to perform similarly to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) in predicting disease damage and the need for steroids in 66 newly diagnosed patients with pediatric systemic lupus erythematosus (SLE) (9). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the ECLAM was found to possess significant ability to discriminate patients who were improved or not improved at 6 months, based on the physician's or parent's assessment of the child's response to therapy. In the same study, the ECLAM was found to have good construct validity, i.e., it was moderately correlated with the Physician's Global Assessment of Disease Activity and predicted strongly the child's response to therapy (10). The ECLAM was subsequently found to predict improvement according to the evaluation of the participants in the consensus conference that led to the development of the provisional criteria for the evaluation of response to therapy in pediatric SLE (11).

Ability to detect change.

The ECLAM was found to be very responsive to change in disease activity and slightly more responsive than the SLEDAI in 66 newly diagnosed patients with pediatric SLE. Responsiveness statistics included effect size, effect size index, standardized response mean (SRM), and relative efficiency index (9). In a study of 98 patients who underwent a total of 623 visits, the minimum clinically important difference (MCID) for clinically important improvement or worsening, based on physician's or parent's rating of the disease course between visits, was small. Using the standard error of measurement approach, the MCID value was 1 (3). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the ECLAM was found to be strongly responsive to change in disease activity (SRM 1.3). In the same study, the ECLAM was found to be slightly more responsive and less skewed than the SLEDAI (10).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

It has been suggested that the ECLAM may be preferable to the SLEDAI for measuring disease activity because of its superior quantitative properties (9). The ECLAM has the potential advantage over the Systemic Lupus Activity Measure and SLEDAI of using the entire range of possible scores, which means that scores are less skewed.

Caveats and cautions.

It has been argued that face validity of the ECLAM may be inferior to that of the SLEDAI because items are not exactly defined, the time frame during which symptoms are regarded as “evolving” is unclear, the decrease in the complement levels necessary to be scored as “significantly reduced compared to the last observation” (attribute 12b) is not well defined, and there is a lack of a clearly defined time frame during which symptoms have to occur to be included in a certain measurement of disease activity (9).

Clinical usability.

The ECLAM is reasonably short and simple, which makes it feasible for use in standard clinical practice. Factors potentially hampering its application include unclear definition of items and time frames, and complexity of calculation of the total disease activity score, which is not equal to the simple sum of the domains scores.

Research usability.

The ECLAM has demonstrated good construct, discriminative and predictive ability, and excellent responsiveness to change over time in patients with pediatric SLE and is, therefore, a valid instrument for the assessment of disease activity in both clinical research and therapeutic trials.

SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

SLAM (12) use in childhood-onset systemic lupus erythematosus (SLE) was assessed in a comparative study using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), British Isles Lupus Assessment Group (BILAG), and SLAM in a pediatric population of 35 patients (2). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the SLAM was found to possess significant ability to discriminate patients who were improved or not improved at 6 months based on the physician's or parent's assessment of the child's response to therapy (10).

Ability to detect change.

Excellent responsiveness to change in disease activity has been documented, by means of effect size, effect size index, standardized response mean (SRM), responsiveness statistic, and relative efficiency index in a comparative study with BILAG and SLEDAI that involved 35 newly-diagnosed patients (2). In a study of 98 patients who underwent a total of 623 visits, the minimum clinically important difference (MCID) for clinically important improvement or worsening, based on physician's or parent's rating of the disease course between visits, was small. Using the standard error of measurement approach, the MCID value was 4 (3). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the SLAM was found to be strongly responsive to change in disease activity (SRM 1.3) (10).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

The SLAM and the latest version, SLAM-R, include more systemic features than the SLEDAI. They assess subjective symptoms (fatigue, arthralgias, myalgias), which may increase their correlation with parent/patient self assessment of function and general health. All items are weighted, which enables an accurate grading of clinical and laboratory abnormalities by severity. A comprehensive set of laboratory tests is incorporated.

Caveats and cautions.

Inclusion of many subjective items, which may not be related directly to disease activity, may detract from the validity of the index. The SLAM gives equal weighting to different levels of severity of organ disease activity without considering the significance of the organ involved. It does not include immunologic tests.

Clinical usability.

The SLAM was found to be very user friendly in a comparative study with BILAG and SLEDAI (2). Item grading makes this index potentially more flexible than the SLEDAI for monitoring of changes in disease over time in standard clinical practice. However, the SLAM is longer and somewhat more complex than the SLEDAI.

Research usability.

The SLAM has shown excellent psychometric properties in validation analyses in patients with pediatric SLE and is therefore well suited for use in clinical research and therapeutic trials.

SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

The SLEDAI (13) was found to have construct validity and to perform similarly to the European Consensus Lupus Activity Measurement (ECLAM) in predicting disease damage and the need for steroids in 66 newly diagnosed patients with pediatric systemic lupus erythematosus (SLE). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the SLEDAI was found to possess significant ability to discriminate patients who were improved or not improved at 6 months based on the physician's or parent's assessment of the child's response to therapy (10).

Ability to detect change.

Excellent responsiveness to change in disease activity has been documented, by means of effect size, effect size index, standardized response mean (SRM), responsiveness statistic, and relative efficiency index, in a comparative study with the British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Activity Measure (SLAM) that involved 35 newly-diagnosed patients (2). In a study of 98 patients who underwent a total of 623 visits, the minimum clinically important difference (MCID) for clinically important improvement or worsening, based on physician's or parent's rating of the disease course between visits, was small. Using the standardized error of measurement approach, the MCID value was 2 (3). In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the ECLAM was found to be strongly responsive to change in disease activity (SRM 1.1) (10).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

The SLEDAI includes only 24 items, which makes it the shortest of the lupus activity tools. The SLEDAI gives more points to renal disease than does the SLAM (up to 16 points versus a maximum of 8 points), which makes it potentially more responsive in patients who relapse with renal disease primarily.

Caveats and cautions.

The SLEDAI is the only lupus activity tool that does not include subjective items, such as fatigue, joint pain, etc. This makes it potentially less suitable in capturing patient-relevant disease changes. Individual items are not graded for severity. It has been argued that the SLEDAI may not capture sufficiently worsening of an already existing feature or detect partial improvement. However, the 2000 modification of the index (SLEDAI-2K) enables recording ongoing disease activity, as well as new or deteriorating disease activity (14).

Clinical usability.

The SLEDAI was found to be the quickest measure to complete in a comparative study with BILAG and SLAM (2). In the clinical setting, it may be the preferable lupus activity tool because it is concise and easy to complete.

Research usability.

The SLEDAI has shown excellent psychometric properties in validation analyses in patients with pediatric SLE and is, therefore well suited for use in clinical research and therapeutic trials.

Advantages/disadvantages of the different versions of the SLEDAI.

There are 3 versions of the SLEDAI: the original SLEDAI (13), the SLEDAI-2K (14), and the MEX-SLEDAI (15). In the original version, the items rash, alopecia, mucous membrane lesions, and proteinuria are scored only if they represent their first occurrence or a recurrence (or a recent increase for proteinuria), whereas in the SLEDAI-2K version these items are simply scored when present. This change in the 2K version was made to reflect ongoing disease activity in the affected organ systems. The MEX-SLEDAI has the advantage of avoiding the cost of immunologic laboratory tests because it does not include anti–double-stranded DNA antibodies and complement levels. All studies performed in pediatric SLE have used the original version of the SLEDAI, except for the study by Brunner et al (3), which was based on the SLEDAI-2K version.

PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the MD Global demonstrated a strong ability in discriminating patients who were improved or not improved at 6 months based on the physician's or parent's assessment of the child's response to therapy. The discriminative ability of the MD Global as well as its ability to predict response to therapy were comparable to that of the European Consensus Lupus Activity Measurement (ECLAM). The baseline-to-6 month change in the MD Global was moderately correlated with the change in the ECLAM, physical summary score of the Child Health Questionnaire, and parent global assessment of the patient's overall well-being, and poorly correlated with the change in the 24-hour proteinuria (10). The MD Global was subsequently found to predict improvement according to the evaluation of the participants in the consensus conference that led to the development of the provisional criteria for the evaluation of response to therapy in pediatric systemic lupus erythematosus (SLE) (11).

Ability to detect change.

In a multinational study that included 557 patients who underwent a baseline visit, at the time of an active phase of disease requiring a major therapeutic intervention, and a subsequent visit after 6 months, the MD Global was found to be the most responsive measure, together with the ECLAM and the Systemic Lupus Activity Measure (SLAM; standardized response mean 1.3 for all 3 measures) (10). In a study of 98 patients who underwent a total of 623 visits, the minimum clinically important difference (MCID) for clinically important improvement or worsening, based on physician's or parent's rating of the disease course between visits, was small. Using the standard error of measurement approach, the MCID value was 1. The change-corrected agreement of activity index with a stable course was greater for the MD Global than for the ECLAM, SLAM, and Systemic Lupus Erythematosus Disease Activity Index (3).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

The MD Global is the simplest and most feasible of the physician-reported disease activity measures. Furthermore, its statistical performances were found to be comparable to those of the composite disease activity tools.

Caveats and cautions.

The MD Global is a broad measure of SLE activity and, therefore, may not detect with sufficient reliability improvement or worsening of disease activity in individual organs/systems. Use of the 10-cm horizontal line visual analog scale (VAS) to rate the MD Global may lead to inaccuracies in assessing disease remission. Due to the relative aversion to extremes that is often seen when using such VAS, very low values (0.1 or 0.2 cm) are frequently obtained when the assessor actually intended to mark the end of the line. It has been suggested that the 21-circle VAS format may be less skewed and less affected by ceiling effect and has the potential advantage of increasing the accuracy of assessment of clinical remission (16).

Clinical usability.

The simplicity and ease of the MD Global makes it well suited for use in monitoring the course of disease activity over time in standard clinical practice.

Research usability.

The MD Global has shown excellent psychometric properties in patients with pediatric SLE and is therefore well suited for use in clinical research and therapeutic trials.

SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

Psychometric Information

Validity.

Several studies have shown that the SDI is a valid and reliable instrument to capture damage in patients with pediatric systemic lupus erythematosus (SLE) (17–21). Accumulated damage, measured with the SDI, was found to be predicted by cumulative disease activity over time and to be correlated with the frequency of severe disease flares in the first 3 years of followup. Item weightings for the SDI using Rasch analysis were not found to lead to an important clinical improvement (22). Rasch analysis is a psychometric approach that has been used to assess and improve rheumatology scales by generating appropriate item weightings (23). The presence of accumulated damage, measured with the SDI, was found to affect significantly the health-related quality of life, particularly in the physical domain in patients with pediatric SLE (24).

Critical Appraisal of Overall Value to the Pediatric Rheumatology Community

Strengths.

The SDI (25, 26) enables a detailed and comprehensive assessment and cumulative organ/system damage in pediatric patients with SLE. It constitutes an important tool to monitor over time the development of damage due to active inflammation, medication side effects, and comorbid conditions. Regular use of the SDI ensures harmonization of long-term studies of pediatric patients into adulthood.

Caveats and cautions.

It has been argued that the SDI does not cover all forms of damage that children or adolescents with SLE may develop over time, particularly effects on growth and development (27). Incorporation of growth retardation and pubertal delay in a modified pediatric version of the SDI has been advised. Furthermore, a redefinition of the item cognitive impairment has been proposed to facilitate its applicability in younger patients. Since some SDI items, such as myocardial infarction, pancreatic insufficiency, claudication, gastrointestinal stricture, ruptured tendons, and malignancy, are rarely seen in children and adolescents with SLE, the utility of their assessment in pediatric SLE has been questioned (27). To avoid confusion between active inflammation and irreversible damage, in order to be scored in the SDI an item needs to be present for 6 months (except damage items that are theoretically nonreversible). Thus, the SDI covers, by definition, only irreversible damage and does not take into account the ability of children to recover and regenerate to a greater degree than adults. For instance, avascular necrosis may have a potential for regeneration and remodeling of bone lesions in children if better control of disease without the use of steroids is achieved and if normal growth velocity is restored. Furthermore, children have an exceptional capacity for neurologic recovery that adults lack, and full recovery may occur months to years later (28). It has also been argued that since steroid-induced diabetes mellitus may be reversible, the presence of steroid-induced diabetes mellitus for 6 months or more may not represent a true permanent damage (29).

To overcome the limitations of the SDI, a modified pediatric version, (Ped-SDI) has been proposed (27). As compared with the original SDI, the pediatric version includes 2 additional items: growth failure and delayed puberty. The glossary of terms for the items of the original SDI was maintained, with the sole exception of the indication that in younger children proteinuria should be adjusted for height and weight. A specific definition for the new items growth failure and delayed puberty was provided. At variance from the original SDI, which defines damage as an irreversible change in an organ or system, it was taken into account that some forms of damage are potentially reversible in pediatric patients.

Modifications to the pediatric version of the SDI suggested subsequently are to rename the item growth failure as “reduced final height,” to reflect the really irreversible outcome, and to alter the definition of pubertal delay to mean a significant lack of pubertal progression to indicate permanent damage to the hypothalamic-pituitary axis. Furthermore, the modification of the definition of the item of gonadal failure has been advised, as gonadal failure defined as secondary amenorrhea before the age of 40 years is not easily applied to adolescent girls, whose menstrual cycles may be irregular as a normal physiologic variant for the first 2 years after menarche (29).

Clinical usability.

The pediatric version of the SDI enables an accurate assessment and monitoring of the main forms of cumulative organ/system damage that can occur in pediatric patients with SLE. It is simple and easy to complete and score. Regular (i.e., yearly) completion of the SDI provides clinicians with an important tool to follow the course of organ/system damage from the pediatric age into adulthood (30).

Research usability.

Application of the SDI and its pediatric version in pediatric patients with SLE has shown that the index is valid for use in observational cohort studies and long-term outcome surveys. It may also be valuable in the prediction of outcome.

DISCUSSION

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES

All global measures of disease activity have been found to be reliable and valid for use in children and adolescents with SLE, and none of them has shown clearly superior metrologic properties. The choice of a specific tool may largely depend on the purposes of the study, the investigational setting (standard clinical practice or research), or the personal preference of the investigator. Owing to its simplicity, feasibility, and good psychometric properties, the MD Global should always be incorporated in the assessment of disease activity either in standard clinical practice and research. Although the SDI has proved suitable to assess damage in patients with pediatric SLE, it was found to have some important limitations for use in the pediatric age group, the chief of which is the inability to capture some forms of damage that are unique to children and adolescents, namely growth failure and delayed puberty. Use of the modified pediatric version of the SDI in pediatric patients with SLE is, therefore, advised.

REFERENCES

  1. Top of page
  2. INTRODUCTION
  3. BRITISH ISLES LUPUS ASSESSMENT GROUP (BILAG)
  4. EUROPEAN CONSENSUS LUPUS ACTIVITY MEASURMENT (ECLAM)
  5. SYSTEMIC LUPUS ACTIVITY MEASURE (SLAM)
  6. SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEX (SLEDAI)
  7. PHYSICIAN'S GLOBAL ASSESSMENT OF DISEASE ACTIVITY (MD GLOBAL)
  8. SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS/AMERICAN COLLEGE OF RHEUMATOLOGY DAMAGE INDEX (SLICC/ACR DI; SDI)
  9. DISCUSSION
  10. AUTHOR CONTRIBUTIONS
  11. REFERENCES
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  • 2
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