RESUMO A classificação de Sapporo revisada para a síndrome antifosfolipídica definida de 2006 incluiu como critérios laboratoriais aqueles testes para anticorpos antifosfolípides cuja acurácia era considerada satisfatória de acordo com a evidência então disponível. Porém, na prática, a sensibilidade e especificidade desses anticorpos antifosfolípides “critério” são por vezes insuficientes para identificar ou descartar a síndrome antifosfolípide. Tem-se estudado se a acurácia do diagnóstico laboratorial da síndrome poderia ser melhorada por meio da testagem de anticorpos antifosfolípides não critério. Neste trabalho revisamos a evidência a respeito das associações clínicas e valor diagnóstico dos anticorpos não critério mais estudados, nomeadamente: anticorpos antifosfatidiletanolamina, antianexina A5, antiprotrombina, anticomplexo fosfatidilserina/protrombina, IgA anticardiolipina e IgG antidomínio I da anti-β2 glicoproteína I.

SUMMARY The 2006 Revised Sapporo Classification Criteria for Definite Antiphospholipid Syndrome included as laboratory criteria the tests for antiphospholipid antibodies whose accuracy was regarded as satisfactory according to the evidence available at that time. In practice, however, the sensitivity and specificity of these “criteria” of antiphospholipid antibodies are sometimes insufficient for identifying or ruling out antiphospholipid syndrome. It has been studied whether the accuracy of the laboratory diagnosis of the syndrome could be improved by testing for non-criteria antiphospholipid antibodies. In this work, we review evidence on the clinical associations and diagnostic value of the most commonly studied non-criteria antibodies, namely: antiphosphatidylethanolamine, anti-annexin A5, anti-prothrombin, anti-phosphatidylserine/prothrombin complex, IgA anticardiolipin, and IgG anti-domain I of the β2 glycoprotein antibodies.

Abstract Background: Imbalance and disfuntion in regulatory T-cells (Tregs) and IL-17 producer lymphocytes (Th17) have been implicated in the pathogenesis of rheumatoid arthritis (RA). Gray scale synovial proliferation (GS), power Doppler signal (pD) and bone erosions seen on high resolution muskuloskeletal ultrasound (MSUS) are hallmarks of destructive articular disease. Objective: To evaluate the association of peripheral Tregs and Th17 with MSUS findings in RA. Methods: RA patients (1987 ACR criteria) treated with disease-modifying antirheumatic drugs (DMARDs) were included. Lymphocytes were isolated and immunophenotyped by flow cytometry to investigate regulatory FoxP3+ T cells and IL-17+ cells. MSUS (MyLab 60, Esaote, Genova, Italy, linear probe 6-18 MHz) was performed on hand joints, and a 10-joint US score was calculated for each patient. Results: Data on lymphocytes subsets were avaiable for 90 patients. The majority of patients were Caucasian women with a median disease duration of 6 years (interquartile range: 2-13 years). Mean DAS28 was 4.28 (SD ± 1.64) and mean HAQ score was 1.11 (SD ± 0.83). There was no significant correlation of 10-joint GS score (rS = 0.122, 95% CI: - 0.124 to 0.336, P = 0.254) and 10-joint pD score (rS = 0.056, 95% CI: - 0.180 to 0.273, P = 0.602) with the mean percentage of peripheral Treg cells. Also, 10-joint GS score (rS = 0.083, 95% CI: - 0.125 to 0.302, P = 0.438) and 10-joint pD score 10 (rS = - 0.060, 95% CI: - 0.271 to 0.150, P = 0.575); did not correlate to Th17 profile. No association of bone erosions on MSUS with Treg and Th17 profiles (P = 0.831 and P = 0.632, respectively) was observed. Conclusion: In this first study addressing MSUS features and lymphocytes subtypes in established RA, data did not support an association of circulating Tregs and Th17 lymphocytes with inflammatory and structural damage findings on MSUS.

Abstract High-resolution musculoskeletal ultrasound (MSUS) has been increasingly employed in daily rheumatological practice and in clinical research. In rheumatoid arthritis (RA), MSUS can be now considered a complement to physical examination. This method evaluates synovitis through gray-scale and power Doppler and it is also able to identify bone erosions. The utilization of MSUS as a marker of RA activity has received attention in recent literature. Current data account for good correlation of MSUS with classical measures of clinical activity; in some instances, MSUS appears to perform even better. Diagnosis of subclinical synovitis by MSUS might help the physician in RA management. With some variation, interobserver MSUS agreement seems excellent for erosion and good for synovitis. However, lack of MSUS score standardization is still an unmet need. In this review, we describe several MSUS scores, as well as their correlation with clinical RA activity and response to therapy. Finally, we look at the relationship of MSUS with synovial tissue inflammation and discuss future perspectives for a better interpretation and integration of this imaging method into clinical practice.

Abstract: BACKGROUND: The nail involvement in psoriasis is related to psoriatic arthritis and may represent a predictor of the disease. OBJECTIVES: To analyze, through nail clipping, clinically normal and dystrophic nails of patients with cutaneous psoriasis and psoriatic arthritis. METHODS: This is a cross-sectional multicenter study, conducted between August 2011 and March 2012. Patients were divided into four groups: patients with cutaneous psoriasis and onychodystrophy, patients with cutaneous psoriasis and clinically normal nails, patients with psoriatic arthritis and onychodystrophy and patients with psoriatic arthritis and clinically normal nails. We calculated NAPSI (Nail Psoriasis Severity Index) of the nail with more clinically noticeable change. After collection and preparation of the nail clipping, the following microscopic parameters were evaluated: thickness of the nail plate and subungual region, presence or absence of parakeratosis, serous lakes, blood, and fungi. RESULTS: There were more layers of parakeratosis (p=0.001) and a greater thickness of the subungual region in patients with cutaneous psoriasis and onychodystrophy (p=0.002). Serous lakes were also more present in the same group (p=0.008) and in patients with psoriatic arthritis and normal nails (p=0.047). The other microscopic parameters showed no significant difference between normal and dystrophic nails or between patients with psoriatic arthritis or cutaneous psoriasis. STUDY LIMITATIONS: Small sample size and use of medications. CONCLUSIONS: Nail clipping is a simple and quick method to assess the nails of patients with nail psoriasis although does not demonstrate difference between those with joint changes or exclusively cutaneous psoriasis.

Resumo Introdução: Foi postulada uma ligação entre psoríase e aterosclerose subclínica. A rede de citocinas pode intermediar essa associação. Poucos dados estão disponíveis em pacientes com psoríase leve. Avaliamos a espessura íntima-média carotídea (cIMT) em psoriáticos e controles livres de medicação. Paralelamente, pesquisamos a associação de cIMT com os índices de atividade de doença e interleucinas séricas (IL) em pacientes com psoríase. Método: Um radiologista experiente procedeu à análise do cIMT. As concentrações de citocinas foram avaliadas por citometria de fluxo. A atividade da doença foi avaliada pelo índice de gravidade (PASI) e pela área de superfície corporal (BSA). Resultados: Sessenta e cinco (65) pacientes e 64 controles foram estudados. A idade média dos pacientes (50,9 anos) não diferiu dos controles (p=0,362). PASI e BSA baixos (< 10) prevaleceram (69,2% e 56,9%, respectivamente). As medianas de IL-12p70, TNF-α, IL-1β e IL-10 foram significativamente menores nos casos do que nos controles (p<0,05 ajustado), enquanto as medianas de IL-6 e IL-8 não diferiram nos grupos (p>0,05 ajustado). Tabagismo e diabetes mellitus predominaram nos casos (p=0,002). Um cIMT alterado (≥ 0,9 mm) foi mais frequente nos casos do que nos controles (23,8% versus 8,5%, p=0,045 ajustado). A média de cIMT foi maior nos casos com significância borderline (p=0,057). Os escores de cIMT não se correlacionaram com o PASI (rs=0,066; p=0,250) ou o BSA (rs=0,175; p=0,185), mas se correlacionaram significativamente com a IL-6 sérica (rs=0,26; p=0,005). Conclusão: A aterosclerose subclínica foi mais frequente em pacientes com psoríase leve do que nos controles. Em psoriáticos, cIMT correlacionou-se com níveis de IL-6 no soro, apontando para um eventual papel pró-aterogênico para a IL-6 nesses pacientes. Novos estudos devem esclarecer a ligação da aterogênese com citocinas na psoríase.

Summary Introduction: A link of psoriasis with subclinical atherosclerosis has been postulated and cytokine network might intermediate this association. Few data are available in patients with mild psoriasis. We evaluated carotid intima-media thickness (cIMT) in drug-free psoriatic individuals and controls. In parallel, we searched for associations of cIMT with disease activity indexes and serum interleukins (IL) in psoriatic patients. Method: An experienced radiologist performed the cIMT analyses. Cytokine concentrations were assessed by flow cytometry. Disease activity was evaluated based on psoriasis area and severity index (PASI) as well as body surface area (BSA). Results: Sixty-five (65) patients and 64 controls were studied. Mean age of patients (50.9 years) did not differ from controls (p=0.362). A low PASI and BSA (< 10) prevailed (69.2% and 56.9%, respectively). Median levels of IL-12p70, TNF-α, IL-1β and IL-10 were significantly lower in cases than in controls (adjusted p<0.05), while IL-6 and IL-8 medians did not differ between groups (adjusted p>0.05). Smoking habit and diabetes mellitus predominated in cases (p=0.002). An altered cIMT (≥ 0.9 mm) was more frequent in cases than in controls (23.8% versus 8.5%, adjusted p=0.045). Mean cIMT was higher in cases with a borderline significance (p=0.057). cIMT scores did not correlate to PASI (rs=0.066; p=0.250) or BSA (rs=0.175; p=0.185), but did correlate significantly with serum IL-6 (rs=0.26; p=0.005). Conclusion: Subclinical atherosclerosis was more frequent in patients with mild psoriasis than controls. cIMT in psoriatic individuals correlated with serum IL-6, pointing to an eventual proatherogenic role of IL-6 in these patients. Newer studies should clarify the connection of atherogenesis with cytokines in psoriasis.

Histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum.Primary infection occurs through inhalation of spores from the air. Immunocompetent individuals are usually asymptomatic, but may develop pulmonary disease. Immunocompromised patients tend to present systemic histoplasmosis with cutaneous lesions occurring by secondary invasion. In this case report, we describe a probable primary cutaneous histoplasmosis (PCH) in a patient with adult onset Still's disease under immunosuppression.

Introdução: A depleção de células T CD4+ CD25+ Foxp3+ regulatórias (células Treg) foi descrita em pacientes com lúpus eritematoso sistêmico (LES) e, recentemente, na síndrome antifosfolípide (SAF) primária; até o momento, o tópico não tinha sido estudado em pacientes com LES e com SAF secundária (LES/SAFS). Objetivo: Quantificar linfócitos totais, células Treg, células T CD3+ CD19- e células B CD3- CD19+ em pacientes com LES/SAF e em controles saudáveis. Métodos: Subtipos celulares foram imunofenotipados utilizando anticorpos monoclonais específicos (antiCD3CY5, antiCD4FITC, antiCD25, antiFoxp3, antiCD19PE) e citometria de fluxo. Resultados: Participaram do estudo 25 pacientes com LES/SAF (média de idade 43,5 anos, 96% mulheres, 96% da raça branca, duração média da doença 9,87 anos, SLEDAI médio 10±5,77) e 25 controles compatibilizados para idade e gênero. Foi constatado que os números de células Treg e de células B CD3- CD19+ estavam significativamente mais baixos em pacientes com LES/SAF, em comparação com controles (todos p<0,05). As células Treg e as células B CD3 CD19+ permaneceram numericamente baixas em seguida ao controle (ANCOVA) para percentual de linfócitos totais (p<0,05). Níveis decrescentes de células Treg e células B CD3-CD19+ circulantes tiveram correlação com escores mais elevados de atividade lúpica (rs=0,75, p<0,0001; rs=0,46, p=0,021, respectivamente). Os números de células Treg e de células B CD3 CD19+ não diferiam significativamente em usuários ou não usuários de cloroquina, azatioprina e corticosteroides (todos p>0,05). Conclusões: Nesse estudo preliminar, pacientes com LES e com SAF secundária demonstraram depleção de células Treg e de células B CD3 CD19+; a redução numérica dos dois subtipos teve correlação com aumento de SLEDAI. A depleção de células Treg pode contribuir para a lesão autoimune observada em pacientes com LES/SAFS. O número reduzido de células B CD3 CD19+ observado nesses pacientes está a merecer estudos objetivando um aprofundamento em sua elucidação.

Introduction: CD4+CD25+Foxp3+ regulatory T (Treg) cell depletion has been reported in systemic lupus erythematosus (SLE) and, recently, in primary antiphospholipid syndrome (APS); the issue has not been studied in SLE patients with secondary APS (SLE/APS) so far. Objective: To quantify total lymphocytes, Treg cells, CD3+CD19- T cells and CD3-CD19+ B cells in SLE/APS patients and healthy controls. Methods: Cell subtypes underwent immunophenotyping using specific monoclonal antibodies (anti-CD3 CY5, anti-CD4 FITC, anti-CD25, anti-Foxp3, anti-CD19 PE) and flow cytometry. Results: Twenty-five patients with SLE/APS (mean age 43.5 years, 96% females, 96% caucasians, mean duration of disease 9.87 years, mean SLEDAI 10 ± 5.77) and 25 age and sex-matched controls entered the study. It was realized that the numbers of Treg and CD3- CD19+ B cells were significantly lower in SLE/APS patients than in controls (all p < 0.05).Treg and CD3-CD19+ B cells remained numerically low after controlling (ANCOVA) for percentage of total lymphocytes (p < 0.05). Decreasing levels of circulating Treg and CD3-CD19+ B cells correlated to higher scores of lupus activity (rs = -0.75, p < 0.0001; rs = -0.46, p = 0.021, respectively). Number of Treg cells and CD3-CD19+ B lymphocytes did not significantly differ in users or nonusers of chloroquine, azathioprine and corticosteroids (all p > 0.05). Conclusions: In this preliminary study, patients with SLE and secondary APS showed depletion of Treg and CD3-CD19+ B cells; decreasing numbers of both subtypes correlated to a higher SLEDAI. Treg cells depletion might contribute to the autoimmune lesion seen in patients with SLE/APS. The reduced number of CD3-CD19+ B cells seen in these patients deserves more studies in order to get further elucidation.

Introdução: A ultrassonografia (US) tem uso recente na reumatologia, e a confiabilidade do método em pacientes com artrite reumatoide (AR) ainda está por ser definida. Objetivo: Testar, em uma pesquisa de AR, a reprodutibilidade da US musculosquelética realizada por reumatologistas com treinamento de um ano por meio da reanálise por um reumatologista com experiência no método. Pacientes e métodos: Esse estudo transversal incluiu pacientes de AR consecutivos de nosso centro terciário. O exame US foi realizado nas articulações metacarpofalângicas, articulações interfalângicas proximais e punhos. Os parâmetros avaliados foram: presença de sinovite, sinal de power Doppler (PD), erosões ósseas e alterações cartilaginosas. Um valor Kappa entre 0,20 e 0,40 foi considerado razoável; entre 0,41 e 0,60, moderado; entre 0,61 e 0,80, bom; e acima de 0,81, excelente. Resultados: Analisamos 1380 articulações de pacientes com AR (78% mulheres, 78% caucasoides). Média de idade = 58 ± 11,56 anos, duração média da doença = 9,98 ± 7,79 anos, DAS28 média = 3,82 ± 1,53 e HAQ média = 0,91 ± 0,67. A concordância de Kappa para sinovite variou de 0,30-0,70; para sinal PD, de 0,53 até a concordância absoluta; para erosões, de 0,70-0,97; para alterações cartilaginosas, de 0,28-0,63. Conclusão: Embora tenha sido obtida concordância interobservadores boa, moderada e excelente para erosões e PD, a concordância para sinovite e alterações cartilaginosas foi me-nos substancial em nossos pacientes com AR ativa. Há necessidade de novos estudos sobre a padronização da técnica de análise, objetivando a melhora da reprodutibilidade da US musculosquelética.

Introduction: Ultrasonography (US) has a recent use in Rheumatology, and the reliability of the method in rheumatoid arthritis (RA) patients has yet to be clarified. Objective: To test, in a RA survey, the reproducibility of musculoskeletal US performed by rheumatologists with one-year training through re-analysis by a Rheumatologist experienced in the method. Patients and methods: This cross-sectional study included consecutive RA patients from our tertiary center. US exam was performed in metacarpophalangeal joints, proximal interphalangeal joints, and wrists. Presence of synovitis, power Doppler (PD) signal, bone erosions, and cartilage changes comprised the US parameters evaluated. A kappa value in-between 0.20 and 0.40 was considered fair; in-between 0.41 and 0.60 was moderate; in-between 0.61 and 0.80 was good; and above 0.81 was excellent. Results: We analyzed 1,380 joints of 60 RA patients (78% females, 78% caucasoids). Mean age was 58 ± 11.56 years, mean disease duration was 9.98 ± 7.79 years, mean DAS28 was 3.82 ± 1.53, and mean HAQ was 0.91 ± 0.67. Kappa agreement for synovitis ranged from 0.30 to 0.70; for PD signal, from 0.53 to absolute agreement; for erosions, from 0.70 to 0.97; for cartilage changes, from 0.28 to 0.63. Conclusion: Although good, moderate and excellent interobserver agreement were obtained for erosions and PD, concordance for synovitis and cartilage changes were less impressive in our patients with active RA. Further studies on standardization of scanning technique are necessary to improve musculoskeletal US reproducibility.

INTRODUÇÃO: Enquanto os critérios de neurolúpus estão bem-estabelecidos, manifestações psiquiátricas globais são de frequência variável em pacientes com lúpus eritematoso sistêmico (LES); suas relações com atividade da doença e prognóstico são desconhecidas. OBJETIVO: Avaliar a frequência de sintomas psiquiátricos no LES utilizando o Questionário de Morbidade Psiquiátrica em Adultos (QMPA); correlacionar alterações no QMPA com atividade da doença e variáveis socioeconômicas. MATERIAIS E MÉTODOS: Este estudo transversal avaliou pacientes com LES ativo ou inativo quanto à prevalência de sintomas psiquiátricos utilizando, pela primeira vez, o QMPA. Oito ou mais respostas afirmativas entre 45 perguntas definiram um QMPA como anormal. Os pacientes foram classificados de acordo com os critérios do American College of Rheumatology de 1997, e o grau de atividade da doença foi mensurado pelo SLEDAI. RESULTADOS: Participaram do estudo 72 pacientes com LES, dos quais 68 eram do sexo feminino (94,4%). A média de idade foi de 46,1 anos (± 12 DP). A frequência de QMPA anormal foi de 89%. Entre os 64 pacientes lúpicos com QMPA alterado, 60 (93,7%) apresentavam distúrbios mentais comuns, a maioria ansiedade e somatização. Não houve correlação de sintomas psiquiátricos com atividade da doença (P = 0,46; rs = 0,09) ou com história de psicose e/ou convulsões atribuíveis ao LES (P = 1,00). Sintomas psiquiátricos também não se correlacionaram com idade de início da doença (rs = -0,16) ou duração da doença (rs = -0,11). Houve associação de QMPA anormal com baixo nível educacional (P=0,02), mas não com renda familiar destinada ao paciente (P = 0,24). CONCLUSÃO: A frequência de sintomas psiquiátricos medidos pelo QMPA foi alta em nossa população com LES. Um QMPA anormal esteve dissociado da atividade do LES, mas se associou com baixo nível educacional.

INTRODUCTION: While the neurolupus criteria are well-established, global psychiatric manifestations are of variable frequency in patients with systemic lupus erythematosus (SLE); their relation with disease activity is unknown. OBJECTIVE: To evaluate the frequency of psychiatric symptoms in SLE using the Adult Psychiatric Morbidity Questionnaire (APMQ); to correlate APMQ changes with disease activity and socio-economic variables. MATERIALS AND METHODS: This cross-sectional study evaluated patients with active or inactive SLE as to the prevalence of psychiatric symptoms utilizing, for the first time, the APMQ. Eight or more affirmative replies out of 45 questions defined the APMQ as abnormal. Patients were classified according to the American Collge of Rheumatology 1997 criteria, and disease activity was measured by the SLEDAI. RESULTS: Seventy-two SLE patients entered the study, being 68 females (94.4%). Mean age was 46.1 years (± 12 SD). The frequency of abnormal APMQ was of 89%. Out of the 64 SLE patients with altered APMQ, 60 (93.7%) had common mental disorders, mostly anxiety and somatization. There was no correlation of psychiatric symptoms with active disease (rs = 0.09; P = 0.46), or with history of psychosis and/or seizures attributable to SLE (P = 1.00). Psychiatric symptoms also did not correlate with age at disease onset (rs = -0.16) or disease duration (rs = -0.11). There was an association of abnormal APMQ with low education level (P = 0.02), but not with family income allotted to the patient (P = 0.24). CONCLUSION: The frequency of psychiatric symptoms measured by the APMQ was high in our SLE population. An abnormal APMQ was disconnected from SLE activity, but it did associate with low education level.

A síndrome da encefalopatia posterior reversível (PRES) é uma entidade nova clinicamente caracterizada por cefaleia, alterações sensoriais, convulsões e perda visual. A patogenia da PRES ainda não foi esclarecida. A PRES pode estar associada a uma variedade de condições clínicas, principalmente hipertensão, insuficiência renal e terapia imunossupressora. Uma possível associação de doenças autoimunes com PRES foi recentemente sugerida. Aqui descrevemos dois casos de lúpus eritematoso sistêmico nos quais a PRES foi deflagrada por diferentes fatores.

The posterior reversible encephalopathy syndrome (PRES) is a novel entity clinically manifested by headache, changes of sensorium, seizures, and visual loss. PRES pathogenesis has not been fully clarified. The entity can be associated to a variety of clinical conditions, mainly hypertension, renal insufficiency and immunosuppressive therapy. A possible link of autoimmune disorders with PRES has been recently hypothesized. We herein describe two cases of systemic lupus erythematosus whereby PRES was triggered by different factors.

Recentemente, vem-se atribuindo uma influência benéfica dos antimaláricos no perfil lipídico de pacientes com lúpus eritematoso sistêmico (LES). Neste estudo transversal, avaliamos o efeito da cloroquina nos níveis de colesterol de uma população brasileira com LES. O estudo avaliou 60 pacientes, 95% dos quais, mulheres, sendo a idade média 48,7 anos (DP 13,3 anos). Em 27 casos (45%), sobrepeso ou obesidade estava presente. Trinta e quatro pacientes (56,6%) usavam cloroquina em uma dosagem padrão, enquanto 33 (55%) usavam corticosteroides. Hipercolesterolemia foi detectada em 26 pacientes (43,3%), e baixos níveis de colesterol HDL em 18 (30%). Colesterolemia normal foi observada igualmente em usuários e não usuários de antimaláricos (P > 0,20). Após ajuste para a ingestão de estatina e corticosteroide através de análise multivariada, os níveis de colesterol total e colesterol HDL não diferiram significativamente em usuários e não usuários de cloroquina (P > 0,05). A ingestão de cloroquina não se associou a um baixo índice de massa corporal (P = 0,314). Nossos achados sugerem que a ingestão de antimaláricos por si não distingue perfis lipídicos em pacientes com LES.

A benefi cial influence of antimalarials on lipid profile of systemic lupus erythematosus (SLE) patients has been recently claimed. In this cross-sectional study, we evaluated the effect of chloroquine on cholesterol levels of a Brazilian population with SLE. Sixty patients were studied, 95% females. Mean age was 48.7 years (SD 13.3 years). Overweight or obesity was documented in 27 cases (45%). Thirty-four patients (56.6%) were using chloroquine in standard dosage, while 33 (55%) were on corticosteroids. Hypercholesterolemia was present in 26 patients (43.3%), while low HDLcholesterol levels were seen in 18 cases (30%). Normal cholesterolemia was documented equally in users and non-users of antimalarials (P > 0.20). After adjustment for statin and corticosteroid intake by multivariate analysis, cholesterol and HDL-cholesterol levels did not significantly differ in users or non-users of chloroquine (P > 0.05). There was no association of chloroquine intake with low body mass index (P = 0.314). Our findings suggest that antimalarial intake by itself does not distinguish cholesterol profiles in SLE patients.

A esclerose lupóide é uma entidade polêmica. A afecção engloba achados de ambos: lúpus eritematoso sistêmico e esclerose múltipla. Os critérios diagnósticos são matéria de debate, assim como o papel de anticorpos antinucleares e antifosfolípides. Reportamos aqui cinco casos nos quais o diagnóstico de esclerose lupóide foi postulado. Os achados clínicos e laboratoriais são discutidos, e uma atualização da literatura acerca da esclerose lupóide é efetuada.

Lupoid sclerosis (LS) is a polemic entity. LS comprises features of both systemic lupus erythematosus (SLE) and multiple sclerosis (MS). Diagnostic criteria are a matter of debate, as well as the role of antinuclear and antiphospholipid antibodies. We here report 5 cases whereby LS could be postulated. Clinical and laboratory findings are discussed, and an updating of the literature on LS is brought about.