RESUMO
BACKGROUND: Systemic lupus erythematosus (SLE) and Primary Antiphospholipid Syndrome (PAPS) overlap clinical and immunological features. Therefore, misclassification of PAPS patients as SLE is a concern. The ACR/EULAR 2019 SLE classification has never been studied in PAPS. OBJECTIVE: To verify if the ACR/EULAR 2019 SLE classification can correctly classify a PAPS patient as not having SLE and compare its performance with the SLICC 2012 SLE classification. Methods: One-hundred thrombotic PAPS patients who fulfilled the Sidney criteria were consecutively screened and those who attended the inclusion criteria were submitted to ACR/EULAR 2019 and SLICC 2012 classifications. RESULTS: Sixty-seven PAPS patients were included in this study. The majority was female (89.6%) with median age at study inclusion of 45 years (35-53) and median PAPS disease duration of 13 years (8-19). PAPS correct classification was observed more often with ACR/EULAR 2019 than SLICC 2021 criteria (94.0% vs. 64.2%; p < 0.001). The 4 misclassified patients in ACR/EULAR 2019 were also misclassified in SLICC 2012. The comparison of misclassified patients to those correctly not classified as SLE resulted, for both criteria, in higher frequencies of hematological domain [ACR/EULAR 2019 (100% vs. 28.6%, p = 0.010) and SLICC 2012 (95.8% vs. 11.6%, p < 0.001)]. Further analysis of hematological manifestations revealed that for the ACR/EULAR 2019 leukopenia (100% vs. 22.2%, p = 0.004) and for the SLICC 2012 leukopenia/lymphopenia (91.7% vs. 7%, p < 0.001) were more frequent in misclassified group. Proteinuria (20.8% vs. 0%, p = 0.004) and low complement (45.8% vs. 20.9%, p = 0.033) were also more often observed in the incorrectly SLICC 2012 classified patients. CONCLUSION: ACR/EULAR 2019 had high accuracy for distinguishing PAPS from SLE, whereas the SLICC 2012 incorrectly classified more than one third of the PAPS patients as having SLE.
Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Trombose , Adulto , Síndrome Antifosfolipídica/classificação , Síndrome Antifosfolipídica/diagnóstico , Erros de Diagnóstico , Feminino , Indicadores Básicos de Saúde , Humanos , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Trombose/diagnósticoRESUMO
OBJECTIVE: To 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 the 1982/1997 ACR or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. MATERIALS AND METHODS: Patients from a Latin America, multiethnic, multicentre cohort, where SLE was defined using the physicians' diagnosis, were included. To calculate the sensitivity of the 2019 EULAR/ACR criteria, the 1982/1997 ACR criteria were considered the gold standard. Additionally, comparison of the 1982/1997 ACR criteria and the 2012 SLICC criteria with the 2019 EULAR/ACR criteria was performed. RESULTS: The sensitivity of the 2019 EULAR/ACR criteria when compared with the 1982/1997 ACR criteria as the gold standard was 91.3%. This new set of criteria allowed an earlier SLE patient classification in 7.4% (mean 0.67 years) and 0.6% (mean 1.47 years) than the 1982/1997 ACR and the 2012 SLICC criteria, respectively. Patients accruing the 2019 EULAR/ACR earlier than the 1982/1997 ACR criteria were more likely to have high anti-dsDNA titres; those accruing them later were less likely to have mucocutaneous and joint manifestations; this was not observed when comparing them with the 2012 SLICC criteria. CONCLUSIONS: The 2019 EULAR/ACR criteria classified earlier only a small proportion of Latin America patients than with the two other criteria sets in real-life clinical practice scenarios. Further studies in different patient populations are needed before these new criteria are adopted worldwide.
Assuntos
Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/diagnóstico , Reumatologia/métodos , Sociedades Médicas , Adolescente , Adulto , Europa (Continente) , Feminino , Humanos , América Latina , Masculino , Reumatologia/normas , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Estados Unidos , Adulto JovemRESUMO
OBJECTIVE: A project aimed at developing new classification criteria for systemic lupus erythematosus (SLE) is based on weighted criteria that include both laboratory and clinical items. Combinations of certain symptoms may occur commonly in SLE, which provides an argument against independently counting these items. The current study was undertaken to evaluate the interrelationship between candidate criteria items in the International Early SLE cohort and in the Euro-Lupus cohort. METHODS: The International Early SLE cohort included 389 patients, who were diagnosed within 3 years prior to the study. Data on the ACR's 1997 update of the SLE revised criteria, the Systemic Lupus International Collaborating Clinics 2012 criteria, and on 30 additional items were collected. To evaluate the interrelationship of the criteria, a tetrachoric correlation was used to assess the degree of association between different manifestations in the same organ system. The correlations identified in the International Early SLE cohort were validated in the Euro-Lupus cohort. RESULTS: A few relevant correlations were observed among specific clinical cutaneous manifestations (in particular, malar rash correlated with photosensitivity, alopecia, and oral ulcers) and serologic manifestations (anti-Sm and anti-double-stranded DNA and anti-RNA polymerase, anti-Ro and anti-La, and antiphospholipid antibodies), and these results were validated in the Euro-Lupus cohort. The associations within the mucocutaneous domain, hematologic and the specific autoantibodies suggest that within a single domain only the highest ranking item should be counted to avoid overrepresentation. CONCLUSION: Some of the candidate SLE criteria cluster within domains. Given these interrelationships, multiple criteria within a domain should not be independently counted. These results are important to consider for the structure of new SLE classification criteria.
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Lúpus Eritematoso Sistêmico/diagnóstico , Avaliação de Sintomas , Adulto , Brasil , Análise por Conglomerados , Europa (Continente) , Feminino , Humanos , Lúpus Eritematoso Sistêmico/classificação , Masculino , Pessoa de Meia-Idade , América do Norte , Filipinas , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Adulto JovemRESUMO
BACKGROUND: To 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. METHODS: We 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. RESULTS: Study 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. CONCLUSIONS: In 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.
Assuntos
Lúpus Eritematoso Sistêmico/classificação , Adolescente , Idade de Início , Anticorpos Antinucleares/análise , Estudos de Casos e Controles , Criança , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Reumatologia , Sensibilidade e Especificidade , Estados UnidosRESUMO
OBJECTIVE: Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for systemic lupus erythematosus (SLE) classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined the clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients. METHODS: Medical records of subjects enrolled in the Lupus Family Registry and Repository were reviewed for medication history and American College of Rheumatology (ACR) classification criteria to identify ILE patients (3 ACR criteria; n = 440) and SLE patients (≥4 ACR criteria; n = 3,397). Participants completed the Connective Tissue Disease Screening Questionnaire. Anticardiolipin and plasma B lymphocyte stimulator (BLyS) were measured by enzyme-linked immunosorbent assay, antinuclear antibodies (ANAs) by indirect immunofluorescence, and 13 autoantibodies by bead-based assays. RESULTS: On average, ILE patients were older than SLE patients (46.2 years versus 42.0 years; P < 0.0001), and fewer ILE patients were African American (23.9% versus 32.2%; P < 0.001). ILE patients exhibited fewer autoantibody specificities than SLE patients (1.3 versus 2.6; P < 0.0001) and were less likely to have ANA titers ≥1:1,080 (10.5% versus 19.5%; P < 0.0001). BLyS levels were intermediate in ILE patients (controls < ILE; P = 0.016; ILE < SLE; P = 0.008). Pericarditis, renal, or neurologic manifestations occurred in 12.5% of ILE patients and were associated with non-European American race/ethnicity (P = 0.012). Hydroxychloroquine use increased over time, but was less frequent in ILE than SLE patients (65.2% versus 83.1%; P < 0.0001). CONCLUSION: Although usually characterized by milder symptoms, ILE manifestations may require immunomodulatory treatments. Longitudinal studies are necessary to understand how ILE affects organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.
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Anticorpos Anticardiolipina/sangue , Fator Ativador de Células B/imunologia , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/diagnóstico , Testes Sorológicos , Terminologia como Assunto , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/uso terapêutico , Biomarcadores/sangue , Ilhas Virgens Britânicas , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Etnicidade , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Porto Rico , Grupos Raciais , Sistema de Registros , Índice de Gravidade de Doença , Inquéritos e Questionários , Estados Unidos , Ilhas Virgens AmericanasRESUMO
Objective: To study systemic lupus erythematosus in a Brazilian population using the American College of Rheumatology hematological classification criteria and report associations of the disease with serological and clinical profiles. Methods: This is a retrospective study of 460 systemic lupus erythematosus patients followed in a single rheumatologic center during the last 10 years. Hematological manifestations considered for this study were hemolysis, leukopenia, lymphocytopenia and thrombocytopenia. Results: The cumulative prevalences of leukopenia, thrombocytopenia, lymphocytopenia and hemolytic anemia were 29.8%, 21.08%, 17.7% and 8.4%, respectively. A higher percentage of patients with hemolysis had anticardiolipin IgM (p-value = 0.002). Those with leukopenia had more lymphopenia (p-value = 0.02), psychosis (p-value = 0.01), thrombocy- topenia (p-value <0.0001) and anti-double stranded DNA antibodies (p-value = 0.03). Patients with lymphopenia had more leukopenia (OR = 1.8; 95% CI = 1.01-3.29) and lupus anticoagulant antibodies (OR = 2.2; 95% CI = 1.16-4.39) and those with thrombocytopenia had more leukopenia (OR = 3.1; 95% CI = 1.82-5.44) and antiphospholipid syndrome (OR = 3.1; 95% CI = 1.28-7.87). Conclusion: The most common hematological finding was leukopenia and the least common was hemolysis. Associations of low platelet count and hemolysis were found with antiphospholipid syndrome and anticardiolipin IgM positivity, respectively. Leukopenia and lymphocytopenia are correlated and leukopenia is more common in systemic lupus erythe- matosus patients with psychosis, thrombocytopenia and anti-double stranded DNA.
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Humanos , Masculino , Feminino , Hemolíticos , Leucopenia , Lúpus Eritematoso Sistêmico/classificação , Prevalência , TrombocitopeniaRESUMO
OBJECTIVE: To evaluate the performance of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria in classifying systemic lupus erythematosus (SLE) in an uncontrolled real-life scenario. METHODS: Chart review study was performed in which each criterion from the 1997 American College of Rheumatology (ACR) and the 2012 SLICC criteria to classify SLE was applied to patients from an outpatient rheumatology clinic. The clinical diagnosis was used as the gold standard. RESULTS: The sensitivity and specificity of the 2012 SLICC criteria were 92% and 99%, respectively, compared with the 1997 ACR criteria, which were 97% and 99%, respectively. The 2012 SLICC criteria were similar to the 1997 ACR criteria in terms of positive (98.9% versus 99%) and negative (92.5% versus 97.1%) predictive values as well as positive (92 versus 97) and negative (0.08 versus 0.03) likelihood ratios. A concordance of 0.96 (95% confidence interval [95% CI] 0.921.00) was observed between clinical diagnosis and the 1997 ACR criteria, while the concordance was 0.91 (95% CI 0.850.97) for the 2012 SLICC criteria. Seven SLE patients classified by the 1997 ACR criteria did not meet the 2012 SLICC criteria because of either the new definition for lymphopenia (2 patients) or the presence of isolated cutaneous involvement (5 patients), while 2 SLE patients who were classified by the 2012 SLICC criteria did not meet the 1997 ACR criteria because of either the presence of erosive arthritis or biopsy-proven nephritis with circulating antinuclear antibodies. CONCLUSION: Overall, the 1997 ACR and the 2012 SLICC criteria are similar to classify SLE in an uncontrolled real-life scenario, although several new items contained in the 2012 SLICC criteria could represent a step forward for research purposes in selected clinical settings.
Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Adulto , Instituições de Assistência Ambulatorial , Anticorpos Antinucleares/sangue , Biomarcadores/sangue , Biópsia , Feminino , Humanos , Funções Verossimilhança , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Reumatologia , Índice de Gravidade de DoençaRESUMO
Neutrophilic dermatoses have long been known to be associated with autoinmune systemic diseases. Recently, a small number of cases of a disorder distinct from Sweet syndrome or bullous lupus erythematosus (LE) have been described as specifically related to systemic LE under diverse terms, including nonbullous neutrophilic dermatosis, nonbullous neutrophilic LE, and Sweet-like neutrophilic dermatosis. We describe 7 patients that developed urticarial lesions in the context of a known or concurrently diagnosed autoimmune connective tissue disease. Of a total of 7 patients, 6 were afflicted by systemic LE and 1 by rheumatoid arthritis and secondary Sjögren syndrome. Histological findings in all patients included an interstitial and perivascular neutrophilic infiltrate with leukocytoclasia, vacuolar alteration along the dermal-edidermal junction, and no vasculitis. Most patients had active systemic disease at the time of the cutaneous eruption. Skin lesions resolved rapidly after the administration of immunomodulating agents. In conclusion, we provide additional evidence of the existence of a recently defined nonbullous neutrophilic dermatosis in the context of autoimmune connective tissue diseases and propose the term autoimmunity-related neutrophilic dermatosis as an appropriate designation. Furthermore, we believe that this entity should prompt physicians to screen the presence of an active systemic disorder in afflicted patients.
Assuntos
Doenças Autoimunes/classificação , Lúpus Eritematoso Sistêmico/classificação , Síndrome de Sweet/classificação , Terminologia como Assunto , Adulto , Artrite Reumatoide/imunologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Biópsia , Feminino , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Síndrome de Sjogren/imunologia , Pele/imunologia , Pele/patologia , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/tratamento farmacológico , Síndrome de Sweet/imunologia , Resultado do Tratamento , Urticária/imunologiaRESUMO
Las Enfermedades del Tejido Conectivo (ETC) son entidades de baja prevalencia en la población general. Son de naturaleza inflamatoria y autoinmune, tienden a la cronicidad y al compromiso de muchos parénquimas, órganos y tejidos, dejando en ellos daño estructural y funcional. Dado lo anterior, amenazan la vida o disminuyen la expectativa y calidad de vida. El diagnóstico y tratamiento precoz de estas entidades, permite cambiar su curso y muchas veces lograr remisión. Es por lo tanto de suma importancia tenerlas en mente y sospecharlas como entidades de enfermedad e iniciar un tratamiento oportuno.
Connective Tissue Diseases have a low prevalence in the general population. They are inflammatory autoimmune diseases, chronic in nature and compromise different tissues and organs, leaving permanent and irreversible damage. They threaten live, and diminish quality and expectancy of life. Early diagnosis and treatment can change their natural course and in many cases induce remission. A high suspicion is necessary for a prompt diagnosis.