Systemic Lupus International Collaborating Clinics Criteria Research Articles

Identification and assessment of classification criteria attributes for systemic lupus erythematosus in a regional medical record data network

ObjectiveTo assess the application and utility of algorithms designed to detect features of SLE in electronic health record (EHR) data in a multisite, urban data network.MethodsUsing the Chicago Area Patient-Centered...

Evaluation of the EULAR/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus in a Population-Based Registry.

Using the Manhattan Lupus Surveillance Program, a multiracial/ethnic population-based registry, we aimed to compare 3 commonly used classification criteria for systemic lupus erythematosus (SLE) to identify unique cases and determine the incidence and prevalence of SLE using the EULAR/American College of Rheumatology (ACR) criteria. SLE cases were defined as fulfilling the 1997 ACR, the Systemic Lupus International Collaborating Clinics (SLICC), or the EULAR/ACR classification criteria. We quantified the number of cases uniquely associated with each and the number fulfilling all 3 criteria. Prevalence and incidence using the EULAR/ACR classification criteria and associated 95% confidence intervals (95% CIs) were calculated. A total of 1,497 cases fulfilled at least 1 of the 3 classification criteria, with 1,008 (67.3%) meeting all 3 classifications, 138 (9.2%) fulfilling only the SLICC criteria, 35 (2.3%) fulfilling only the 1997 ACR criteria, and 34 (2.3%) uniquely fulfilling the EULAR/ACR criteria. Patients solely satisfying the EULAR/ACR criteria had <4 manifestations. The majority classified only by the 1997 ACR criteria did not meet any of the defined immunologic criteria. Patients fulfilling only the SLICC criteria did so based on the presence of features unique to this system. Using the EULAR/ACR classification criteria, age-adjusted overall prevalence and incidence rates of SLE in Manhattan were 59.6 (95% CI 55.9-63.4) and 4.9 (95% CI 4.3-5.5) per 100,000 population, with age-adjusted prevalence and incidence rates highest among non-Hispanic Black female patients. Applying the 3 commonly used classification criteria to a population-based registry identified patients with SLE fulfilling only 1 validated definition. The most recently developed EULAR/ACR classification criteria revealed prevalence and incidence estimates similar to those previously established for the ACR and SLICC classification schemes.

The impact of normal serum complement levels on the disease classification and clinical characteristics in systemic lupus erythematosus

BackgroundSome patients have normal levels of complement during the diagnosis of systemic lupus erythematosus (SLE), although decreased serum levels of complement are a hallmark of the active phase of the disease. This study investigated the clinical characteristics, impact on the classification of SLE, and the prognosis of patients with SLE who had normal serum complement levels at initial diagnosis (N-com).MethodsWe evaluated 21 patients with N-com and 96 patients with hypocomplementemia at the initial diagnosis of SLE (H-com). The classification rates among the American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012, European League Against Rheumatism (EULAR)/ACR 2019 criteria, and clinical and immunological involvements were compared between SLE patients with N-com and H-com. Relapse and organ damage based on the SLICC/ACR damage index were also evaluated.ResultsThe classification rates of SLE were not significantly different in the ACR, SLICC, and EULAR/ACR criteria between the N-com and H-com groups. Patients with N-com had no significant differences in the classification rates among the three criteria, whereas patients with H-com had lower classification rates in the ACR criteria than in the SLICC criteria. A lower incidence of renal manifestation, less positivity for anti-dsDNA antibody, and a higher incidence of fever were observed in patients with N-com than in those with H-com. The occurrence of relapse and organ damage was not significantly different between patients with N-com and H-com.ConclusionPatients with N-com were less involved in renal manifestation and anti-dsDNA antibody positivity but had a higher incidence of fever than those with H-com, while having no disadvantage in SLE classification processes. Serum complement levels at the initial diagnosis of SLE may not predict prognosis.

Validation of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in ANA-positive Chinese patients.

Background:The aim of this study was to validate the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) in antinuclear antibody (ANA)-positive Chinese patients.Methods:Medical records of all adult patients who attended the rheumatology out-patient clinics between May and September 2019 were reviewed. Patients with ever ANA positive (titre ⩾1:80) were included and evaluated for the fulfilment of the 2019 EULAR/ACR, 2012 Systemic Lupus International Collaborating Clinics (SLICC) and 1997 ACR criteria for SLE classification. The performance of these criteria in predicting a clinical diagnosis of SLE as judged by an independent panel of rheumatologists was studied and compared in different subgroups.Results:A total of 1533 patients (88.2% women; age at first clinic attendance 45.5 ± 15.6 years) were studied and 562 patients were judged to be clinical SLE. The sensitivity and specificity of the EULAR/ACR (⩾10 points), SLICC and ACR criteria for a clinical diagnosis of SLE was 96.1%, 97.9% and 86.1%; and 85.8%, 86.3% and 94.3%, respectively. Applying the attribution rule to the non-SLE controls, the specificity of the three criteria increased to 95.0%, 92.5% and 98.8%, respectively. The specificity of the EULAR/ACR criteria was higher in male patients (97.9%), those aged >50 years (97.0%) and disease duration of ⩽3 years (97.6%). Using a cut-off of 12 points, the specificity of the EULAR/ACR criteria was further increased (96.6%) while a high sensitivity (95.0%) was maintained.Conclusion:In Chinese patients with a positive ANA, the EULAR/ACR criteria for clinical SLE perform equally well to the SLICC criteria. Both the EULAR/ACR and SLICC are more sensitive but less specific than the ACR criteria. The specificity of all the three criteria is enhanced by applying the attribution rule to controls. The specificity of the EULAR/ACR criteria is higher in certain patient subgroups or when the cut-off score is raised.

Impact of the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus in a Multicenter Cohort Study of 133 Women With Undifferentiated Connective Tissue Disease

We aimed to investigate the impact of applying the 2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus (SLE) in a previously described cohort of women with undifferentiated connective tissue disease (UCTD). This study included 133 women with UCTD. At the time of inclusion into the study, none of the patients met any classification criteria for other defined systemic connective tissue disease. When applying the 2019 EULAR/ACR classification criteria to the cohort, 22 patients (17%) fulfilled the classification criteria for SLE. Patients classified as having SLE had significantly higher frequencies of mucocutaneous manifestations (23% versus 5%; P = 0.007), arthritis (59% versus 17%; P < 0.001), isolated urine abnormalities (18% versus 1%; P < 0.001), and highly specific antibodies (50% versus 15%; P < 0.001) compared to the other patients with UCTD. At follow-up, these patients were statistically significantly more likely to also meet the 1997 ACR revised SLE criteria and the Systemic Lupus International Collaborating Clinics (SLICC) criteria (18.2% versus 1.8%; P < 0.001) compared to the other UCTD patients. Patients who were diagnosed as having SLE according to the ACR 1997 update of the SLE revised criteria and the SLICC criteria during the follow-up scored higher on outcome measures when classified as having SLE according to the new 2019 EULAR/ACR classification criteria when compared to the other patients with UCTD (mean ± SD score 8.3 ± 3.7 versus 4.5 ± 4; P < 0.05). When applying the 2019 EULAR/ACR criteria for SLE in a cohort of patients with UCTD, we observed that in up to 17% of cases the original classification could be challenged. New implementation will help to identify earlier patients at higher risk of developing more severe CTD manifestations.

Frequency of Histopathological patterns of Lupus Nephritis according to classification by WHO among Pakistani patients

Background: Systemic lupus erythematosus (SLE) is an autoimmune disorder, multisystemic in nature more common in females of childbearing age. There are certain risk factors which predispose to this disease. It affects various organs, kidney is among them. Almost 60% patients having SLE ultimately leads to kidney dysfunction at some stage of the life. Aim: To find out pattern of histopathological findings of lupus nephritis as per WHO classification on kidney biopsy in Pakistan. Methodology: This cross-sectional study was completed in department of Medicine, Fatima Memorial Hospital, Lahore, from March 2016 to May, 2018. Total sample size was 165 patients. Only patients who fulfilled the 2012 SLICC (Systemic Lupus International Collaborating Clinics) criteria were included in the study. SPSS version 25.0 was used data analysis. Results: Age of the patients was between 31-50 years i.e. 114(69.09%), mean and SD was 43.96±4.84 years, females were more commonly affected by calculating 99(59.70%). Patterns of lupus nephritis as per WHO classification and renal biopsy were noted which shows 18(10.91%) had Class I, 53(32.12%) Class II, 43(26.07%) Class III, 35(21.20%) Class IV, 10(6.06%) Class V and 6(3.64%) had Class VI. Conclusion: Class II and Class III Lupus Nephritis are the most common modalities found in patients of SLE. Every patient with Lupus Nephritis should undergo a Renal Biopsy for correct diagnosis of the class of this disease and further management accordingly. Keywords: Lupus Nephritis, SLE, renal biopsy

SLICC-based Phenotype of Juvenile Systemic Lupus Erythematosus in the Philippines

Objective. To describe the clinical profile of Filipino pediatric SLE patients as determined using the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria.&#x0D; Methods. We checked which among the SLICC criteria were fulfilled by Filipino pediatric SLE patients when weexamined them and their case records, as part of a nationwide genetic study on SLE conducted from October2015 to March 2017.&#x0D; Results. Ninety-seven (out of 321) who were diagnosed to have SLE before 19 years of age were evaluated.The mean age of the population at the time of evaluation was 19.8 ± 6.9 years. Females comprised 94% of ourpopulation. Mean age of onset was 14.4 ± 2.7 years, while the mean age of diagnosis was at 14.5 ± 2.6 years. Acute cutaneous rash was found in 87%; oral ulcers 65%; renal disorder 63%; non-scarring alopecia 61%; arthritis 58%; chronic cutaneous rash 36%; leukopenia 35%; hemolytic anemia 34%; serositis 25%; thrombocytopenia 23%, and neurologic disorder 8%. Anti-nuclear antibody was present in 85%; low complement 32%; anti-dsDNA 28%; direct Coombs’ 16%; antiphospholipid antibody 3%; and anti-Smith antibody 1%. Kidney biopsy was performed in only 14% (14/97) of patients, of whom 27% had class III histopathologic characteristic.&#x0D; Conclusions. Filipino pediatric SLE patients typically present with mucocutaneous, renal, and musculoskeletalinvolvement. Cardiopulmonary and neurologic manifestations are found to be less common among them. Finally, renal biopsy is not commonly performed among these patients.

Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities

ObjectivesThe European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance...

Incomplete Systemic Lupus Erythematosus: What Remains After Application of American College of Rheumatology and Systemic Lupus International Collaborating Clinics criteria?

Incomplete systemic lupus (iSLE) is an acknowledged condition of patients with clinical signs of lupus who do not fulfill classification criteria for SLE. Some patients with iSLE have persistent mild disease, but others have serious organ involvement, and up to 55% progress to established SLE. Research on this subject could reveal predictive or diagnostic biomarkers for SLE. Ideally, it would become possible to discern those patients with critical organ involvement or a high risk for progression to SLE. This high‐risk group might benefit from early treatment, which would preferably be confirmed in randomized controlled trials. This process would, however, require agreement on a definition of iSLE. The Systemic Lupus International Collaborating Clinics (SLICC) classification criteria was composed in order to diagnose SLE earlier. The present review outlines the clinical characteristics of iSLE after introduction of SLICC criteria and furthermore proposes a definition of iSLE with the aim of discriminating the high‐risk group from those with a lower risk.

Utility of the AVISE Connective Tissue Disease test in predicting lupus diagnosis and progression

BackgroundThe AVISE Connective Tissue Disease (CTD) test uses autoantibody, erythrocyte-bound C4d (EC4d) and B-cell-bound C4d (BC4d) levels to aid in diagnoses of SLE, other CTDs and fibromyalgia. We evaluated the...

Applying the 2019 EULAR/ACR lupus criteria to patients from an established cohort: a Latin American perspective

ObjectiveTo evaluate the performance of the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) criteria in terms of earlier patients’ classification in comparison to...

EULAR/ACR classification criteria for SLE

Supported by both the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) from the beginning, a large transatlantic, and in many phases worldwide, project has led to new classification criteria for systemic lupus erythematosus (SLE). The project had ambitious goals, particularly maintaining the high specificity of the ACR criteria, while reaching a sensitivity close to the Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria. The validation data suggest that the new criteria reached this goal, with a specificity of 93% (as for the ACR criteria) and a sensitivity of 96% (vs 97% for the SLICC criteria). The new EULAR/ACR classification criteria use positive antinuclear antibodies (ANA) as an entry criterion and have weighted items, ranging from 2 (for delirium, non-infectious fever and anti-phospholipid antibodies) to 10 (for class III or IV lupus nephritis). The cut-off for classification is 10, reached by class III/IV nephritis alone. Items are organized in domains, within which only the highest item is to be counted. Instead of many defined exclusions, one rule is used for all, namely that items are to be attributed to SLE and counted only if there is no more likely alternative diagnosis. These criteria are now expected to be externally validated in various other cohorts. The team spirit and collegiality characteristic for this EULAR/ACR criteria effort will hopefully facilitate future international projects on SLE.

The majority of Swedish systemic lupus erythematosus patients are still affected by irreversible organ impairment: factors related to damage accrual in two regional cohorts.

BackgroundAlthough the survival of patients with systemic lupus erythematosus (SLE) has improved, irreversible organ damage remains a critical concern. We aimed to characterize damage accrual and its clinical associations and causes of death in Swedish patients.MethodsAccumulation of damage was evaluated in 543 consecutively recruited and well-characterized cases during 1998−2017. The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI) was used to estimate damage.ResultsOrgan damage (SDI ≥ 1) was observed in 59%, and extensive damage (SDI ≥ 3) in 25% of cases. SDI ≥ 1 was significantly associated with higher age at onset, SLE duration, the number of fulfilled SLICC criteria, neurologic disorder, antiphospholipid antibody syndrome (APS), hypertension, hyperlipidemia, depression and secondary Sjögren's syndrome (SS). In addition, SDI ≥ 3 was associated with serositis, renal and haematological disorders and interstitial lung disease. A multiple regression model identified not only well-known risk factors like APS, antihypertensives and corticosteroids, but pericarditis, haemolytic anaemia, lymphopenia and myositis as being linked to SDI. Malignancy, infection and cardiovascular disease were the leading causes of death.ConclusionsAfter a mean SLE duration of 17 years, the majority of today's Swedish SLE patients have accrued damage. We confirm previous observations and report some novel findings regarding disease phenotypes and damage accrual.

Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

BackgroundTo date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort.MethodsWe conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up.ResultsStudy population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p < 0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p < 0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p < 0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods.ConclusionsIn this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Comparison of Sensitivities of American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria in Childhood-onset Systemic Lupus Erythematosus

Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort. We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids' Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar's test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either. ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2-3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies. SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.

Brief Report: How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria.

Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-β2 -glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria.

Classification of primary antiphospholipid syndrome as systemic lupus erythematosus: Analysis of a cohort of 214 patients.

To assess the limitations of the SLICC (Systemic Lupus International Collaborating Clinics) classification criteria for systemic lupus erythematosus (SLE), in patients with primary antiphospholipid syndrome (PAPS). Retrospective study of a cohort of APS patients (Sydney criteria). We successively excluded patients with (1) at least one "SLE-specific" manifestation (biopsy-proven SLE nephropathy, arthritis, cutaneous, or neurologic SLE manifestations, pericarditis, autoimmune haemolytic anaemia, oral and nasal ulcers, non-scarring alopecia, anti-dsDNA, and anti-Sm antibodies), (2) any other autoimmune connective tissue disease, and/or (3) antinuclear antibodies >1/320. Careful file review confirmed PAPS among the remaining patients. We then assessed the number of SLICC criteria each patient met. Among these 214 APS patients, we excluded 85 with at least one SLE-specific manifestation, 8 with another connective tissue disease, and 21 with antinuclear antibodies >1/320, leaving 100 patients with primary APS. Among them, 28% met at least 4 SLICC classification criteria including one clinical and one immunological criterion (antiphospholipid antibodies, aPL, by definition) and could thus theoretically be classified with SLE. Fourteen had an arterial phenotype (50%), 9 a history of catastrophic APS (32%), and 18 a triple-positive profile for aPL (64%). None had developed SLE during a median follow-up of 12 [6.5-17] years. Because 28% of our patients with longstanding and strictly defined PAPS could be mistakenly classified as SLE, they were at risk of deleterious therapeutic management. We therefore suggest that any future classification for SLE should specifically require at least one SLE-specific criterion for patients with aPL.

Evaluation of the SLICC classification criteria in patients with juvenile systemic lupus erythematosus followed up in a pediatric institution in Bogotá, Colombia

IntroductionSystemic lupus erythematosus is a multisystemic disease that is associated with an increase in morbidity. Several classification criteria, with a variable performance, have been published in the last few years. The Systemic Lupus International Collaborating Clinics (SLICC) recently developed a new version that has been validated in children and adults. The performance of these criteria in Colombian juvenile lupus patients is unknown. ObjectivesTo evaluate the sensitivity and specificity of the SLICC criteria in a group of patients with juvenile systemic lupus erythematosus in Bogotá, Colombia. Materials and methodsRetrospective evaluation of clinical and laboratory data during the first month of disease in patients with juvenile systemic lupus erythematosus and in control patients followed-up in a paediatric rheumatology clinic. ResultsThe study subjects (n=110), with a mean age of 11.1 years (2–16 years), were divided equally into cases and controls. The gender ratio in cases was F:M 5.4:1 and in controls 1.8:1. The ACR 1997 and SLICC criteria were analysed in both groups of patients. The sensitivity and specificity of ACR criteria were 78% and 96%, respectively, and in SLICC criteria were 89% and 87%, respectively. ConclusionsIn this group of paediatric patients, the sensitivity of SLICC criteria during the first month of diagnosis was higher than the ACR 1997 criteria. This was associated with a lower specificity. These results are similar to those of previous studies.

Evaluación de los criterios de clasificación SLICC en pacientes con lupus eritematoso sistémico juvenil seguidos en una clínica pediátrica de Bogotá, Colombia

IntroductionSystemic lupus erythematosus is a multisystemic disease that is associated with an increase in morbidity. Several classification criteria, with a variable performance, have been published in the last few years. The Systemic Lupus International Collaborating Clinics (SLICC) recently developed a new version that has been validated in children and adults. The performance of these criteria in Colombian juvenile lupus patients is unknown. ObjectivesTo evaluate the sensitivity and specificity of the SLICC criteria in a group of patients with juvenile systemic lupus erythematosus in Bogotá, Colombia. Materials and methodsRetrospective evaluation of clinical and laboratory data during the first month of disease in patients with juvenile systemic lupus erythematosus and in control patients followed-up in a paediatric rheumatology clinic. ResultsThe study subjects (n=110), with a mean age of 11.1 years (2-16 years), were divided equally into cases and controls. The gender ratio in cases was F:M 5.4:1 and in controls 1.8:1. The ACR 1997 and SLICC criteria were analysed in both groups of patients. The sensitivity and specificity of ACR criteria were 78% and 96%, respectively, and in SLICC criteria were 89% and 87%, respectively. ConclusionsIn this group of paediatric patients, the sensitivity of SLICC criteria during the first month of diagnosis was higher than the ACR 1997 criteria. This was associated with a lower specificity. These results are similar to those of previous studies.

The use of SLICC and ACR criteria to correctly label patients with cutaneous lupus and systemic lupus erythematosus.

The American College of Rheumatology (ACR) classification criteria for lupus and Systemic Lupus International Collaborating Clinics (SLICC) criteria are designed to classify disease. However, they have become widely used as diagnostic criteria in clinical situations. Patients may be labelled as systemic lupus erythematosus (SLE) in their medical records, when in fact they have cutaneous lupus erythematosus (CLE) without systemic symptoms. We sought to investigate how many of our cutaneous lupus patients attending a dermatology lupus clinic were mislabelled as either CLE or SLE using the ACR and SLICC criteria. Thirty-six patients with biopsy-proven cutaneous lupus were identified. Fourteen (39%) of the patients were labelled as 'SLE' in their medical notes, either by dermatology or another medical team. Of these 14 patients, 12 (86%) fulfilled the ACR and SLICC criteria; however, two (14%) did not meet the criteria for SLE. Of the remaining 22 patients who were not labelled as having SLE, four (18%) met both the SLICC and ACR criteria, one (5%) met the ACR criteria and one (5%) met the SLICC criteria. These patients had a history of discoid or subacute lupus, with very few systemic symptoms. They met the criteria for SLE primarily on their cutaneous signs and positive serology. It is important to screen patients with CLE routinely for SLE. Although the ACR and SLICC criteria can be helpful as they have a high sensitivity for systemic lupus, their use needs to be paired with the clinical context and patient evolution. We found patients were labelled as SLE when in fact they had no evidence of systemic involvement, as well as patients labelled as cutaneous lupus who fulfilled the criteria for SLE, although unlikely having any systemic involvement. It is important to correctly identify patients as 'cutaneous lupus' or 'systemic lupus erythematosus' and documentation in clinical notes should be accurate to avoid confusion and allow appropriate treatment.