Shear-Wave Elastography Evaluation of Major Salivary Glands in Primary Sjögren's Syndrome.

OBJECTIVES: Salivary glands ultrasonography has recently been shown to be useful in the diagnosis of Primary Sjögren's Syndrome (pSS). Shear-wave elastography (SWE) is a promising tool for the quantitative assessment of tissues stiffness, but studies evaluating its role in pSS diagnosis are limited. This study aimed at investigating the diagnostic performance of SWE in pSS. MATERIALS AND METHODS: Cross-sectional study including patients fulfilling the 2016 ACR/EULAR classification criteria for pSS and healthy subjects. The four major salivary glands were assessed using SGUS. B-mode scans were rated using the Hocevar score, and shear-wave velocity (SWV) values were obtained using SWE. Intraclass-correlation coefficient (ICC) estimates were used to assess reliability. Cut-off values for differentiating pSS patients from healthy subjects were calculated using Receiver-Operating Characteristics (ROC) curves. RESULTS: We included 50 pSS and 25 healthy subjects. Inter-rater reliability of SWE was moderate (ICC=0.64) and intra-rater reliability was moderate to good (ICC= 0.73 to 0.83). Total SWV (2.09 m/s (0.32); p < 0.001), parotid SWV (2.25 m/s (0.40)) and submandibular SWV (1.92 m/s (0.38)) were significantly higher in pSS patients. Total and parotid SWV presented good diagnostic performance for pSS diagnosis (AUROC= 0.80 and 0.81, respectively). The Hocevar score demonstrated excellent diagnostic performance (AUROC= 0.98) and combining it with total SWV did not result in statistically significant improvement (p=0.301). CONCLUSIONS: SWE may contribute to the diagnosis of pSS. Large prospective studies including sicca and secondary SS patients, as well as the standardisation of SWE protocols, are warranted to assess the role of SWE in pSS management.

High patient global assessment scores in patients with rheumatoid arthritis otherwise in remission do not reflect subclinical inflammation.

OBJECTIVES: To assess whether high patient global assessment (PGA) scores by patients with rheumatoid arthritis (RA) otherwise in remission reflect subclinical inflammation. METHODS: Cross-sectional, single-center study, including consecutive RA patients. Remission states were defined based on the ACR/EULAR Boolean definition: 4V-remission (tender and swollen 28-joint counts (TJC28/SJC28), C-reactive protein (CRP), and PGA all≤1), PGA-near-remission (the same, except PGA>1), and non-remission (any of TJC28, SJC28, CRP>1). A blinded expert musculoskeletal ultrasonographer scanned 44 joints, 38 tendon sheaths, 4 bursae on the same day of the clinical evaluation. Each structure was assessed for the presence of Grey Scale synovial hypertrophy (GS) and Power Doppler (PD), both scored using a semi-quantitative scale (0-3 points). The Global OMERACT-EULAR Synovitis Score (GLOESS, 0-132, primary outcome), and a global tenosynovitis/bursitis score (GTBS) were compared between remission states, using non-parametric tests. Different sensitivity analyses comparing GS and PD subscores were performed. RESULTS: In total, 130 patients (mean age 63 years, 86% female, average disease duration 14 years) were included 40 being in 4V-remission, 40 in PGA-near-remission, 50 in non-remission. 4v-remission and PGA-near-remission presented similar median (IQR) GLOESS, [6 (5-11) and 4 (1-7), P>0.05, respectively] and GTBS [0 (0-1) and 0 (0-2), P>0.05, respectively]. The same was observed in GS, PD scores, and in global synovitis score considering only the 16 joints not included in 28-joint counts. These observations were confirmed in patients with≤5 years disease duration. CONCLUSIONS: Subclinical inflammation is not present among persons with elevated PGA who are otherwise in remission. PGA-near-remission patients would be exposed to the risk of overtreatment if current treatment recommendations were strictly followed. This study supports the need to reconsider the role of PGA in definitions used to target immunosuppressive therapy and to provide a separate and enhanced focus to the patient's experience of the disease.

Sun Exposure: Beyond the Risks.

Excessive solar exposure presents well-recognized risks and side effects, solar radiation being the most important environmental factor concerning skin cancer. In the last few years, several connections between solar exposure and prevention and/or treatment of several diseases have been discussed, with studies suggesting that regular solar exposure may be beneficial for conditions such as colorectal, breast, prostate, and pancreatic cancer; non-Hodgkin lymphoma; arterial hypertension; obesity; type 2 diabetes mellitus and metabolic syndrome; nonalcoholic hepatic steatosis; multiple sclerosis; Alzheimer disease; and several psychiatric disturbances. In most cases, UV radiation's beneficial effects are mediated through vitamin D; however, studies show that in other instances other mediators are responsible for these associations, specifically nitric oxide. Moderation is therefore essential, as a strict strategy of total sun avoidance may be inadequate.

Assessment of the vaccination status in rheumatic disease patients treated with disease-modifying antirheumatic drugs.

Patients with rheumatic diseases are at a higher risk for infections associated to the underlying pathology and immunosuppressive therapy. This fact leads to an increased morbidity and/or mortality. Effective vaccination is essential for the prevention of a significant number of these infections, namely influenza and pneumococcal vaccination. In our cohort, and despite current recommendations, vaccination rates are still low among patients with autoimune diseases, which is in agreement with the available literature. A greater effort from all physicians is required to improve these results and to make the difference.

Cutaneous Manifestations of Diabetes Mellitus and Prediabetes.

Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially to health care costs. In this article the authors review the most common and sensitive skin manifestations that can be present on patients with diabetes and prediabetes. The prompt recognition of these frequently underestimated entities is extremely important as it may trigger not only an adequate metabolic evaluation but also a timely referral and appropriate treatment, minimizing the secondary effects of long-term diabetes and improving the prognosis of diabetic patients.

A diabetes mellitus é uma doença crónica, com uma prevalência crescente a nível mundial. As complicações da diabetes são uma causa major de morbilidade e mortalidade, condicionando custos importantes na área da saúde. Neste artigo é efetuada uma revisão das manifestações cutâneas mais frequentes presentes em doentes com diabetes e pré-diabetes. O reconhecimento atempado destas entidades é fulcral, levando não só a uma avaliação atempada do perfil metabólico como a uma referenciação e tratamento precoces. Desta forma, é possível minimizar os efeitos secundários da diabetes a longo prazo, melhorando significativamente o prognóstico dos doentes.

[Clinical and Laboratory Factors Associated with Prolonged Hospital Stay among Patients with Cellulitis/Erysipelas]. / Fatores Clínico-Laboratoriais Associados ao Internamento Prolongado em Doentes com Celulite/Erisipela.

INTRODUCTION: Cellulitis and erysipelas represent the most frequent cause of hospitalization in the dermatology department of Santa Maria Hospital in Lisbon, Portugal. The aim of this study was to investigate whether patient demographics, comorbidities, previous episodes of cellulitis/erysipelas, the presence of complications, laboratory markers at admission, microbial isolation or previous use of antibiotics, are associated with prolonged stays. MATERIAL AND METHODS: Retrospective analysis, including patients admitted with cellulitis/erysipelas in the inpatient dermatology department of Santa Maria Hospital between July 1st 2012 and June 30th 2017. RESULTS: There were 372 admissions, corresponding to 348 patients. The median length of stay was 11 days. Increased age (p = 0.002, OR 1.03, 95% CI 1.01 - 1.04), previous episode of cellulitis/erysipelas requiring hospitalization (p = 0.005, OR 4.81, 95% CI 1.63 - 14.23), the presence of cellulitis/erysipelas-associated complications (p = 0.001, OR 3.28, 95% CI 1.63 - 6.59), leukocytosis (p = 0.049, OR 1.81, 95% CI 1.00 - 3.30), high levels of C-reactive protein (p = 0.035, OR 1.03, 95% CI 1.00 - 1.06) and a positive culture result (p = 0.002, OR 2.59, 95% CI 1.41 - 4.79) were associated with prolonged hospitalization. DISCUSSION: Prolonged hospitalization for cellulitis/erysipelas is associated with higher costs, additional clinical investigation, invasive treatments, prolonged courses of antibiotic therapy, risk of nosocomial infections, and delayed return to activities of daily living. Thus, the investigation of clinical-laboratory factors associated with prolonged hospitalization for cellulitis / erysipelas is essential and may be useful for the construction of a severity score. CONCLUSION: The knowledge of the characteristics that are associated with prolonged stay among patients with cellulitis/erysipelas may be relevant to improve health care, by reducing the length of hospital stay and associated risks and costs.

Introdução: A celulite e a erisipela constituem a causa mais frequente de internamento no Serviço de Dermatologia do Hospital Santa Maria. Este estudo teve como objetivo investigar se as características demográficas, as comorbilidades, a existência de episódios prévios de celulite/erisipela, a presença de complicações associadas, os parâmetros laboratoriais na admissão, o isolamento de microrganismo em cultura ou o uso prévio de antibióticos estão associados a internamentos prolongados.Material e Métodos: Estudo retrospetivo, incluindo os doentes internados no Serviço de Dermatologia do Hospital Santa Maria com o diagnóstico de celulite/erisipela, entre 1 de julho de 2012 e 30 de junho de 2017.Resultados: Existiram 372 internamentos, correspondendo a 348 doentes. A mediana do tempo de internamento foi de 11 dias. A idade (p = 0,002, OR 1,03, 95% IC 1,01 ­ 1,04), a existência de internamento prévio por celulite/erisipela (p = 0,005, OR 4,81, 95% IC 1,63 ­ 14,23), a presença de complicações associadas à celulite/erisipela (p = 0,001, OR 3,28, 95% IC 1,63 ­ 6,59), a leucocitose (p = 0,049, OR 1,81, 95% IC 1,00 ­ 3,30), valores elevados de proteína C reativa (p = 0,035, OR 1,03, 95% IC 1,00 - 1,06) e o isolamento de microrganismo em cultura (p = 0,002, OR 2,59, 95% IC 1,41 ­ 4,79) estiveram associados a internamentos prolongados.Discussão: A par dos maiores custos associados, o internamento prolongado por celulite/erisipela está frequentemente associado à necessidade de investigação clínica adicional, a tratamentos invasivos, a cursos prolongados de antibioterapia, ao risco de infeções nosocomiais e ao atraso no retorno às atividades da vida diária. Assim, o estudo dos fatores clínico-laboratoriais associados ao internamento prolongado por celulite/erisipela é fundamental e poderá ser útil para a construção de um score de gravidade.Conclusão: O conhecimento de características clínicas e laboratoriais associadas ao internamento prolongado poderá ser relevante para melhorar os cuidados de saúde, através da redução dos tempos de internamento e dos seus riscos e custos associados.

Portuguese Recommendations for the use of biological therapies in patients with rheumatoid arthritis- 2016 update.

OBJECTIVE: To update the recommendations for the treatment of Rheumatoid Arthritis (RA) with biological therapies, endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. At a national meeting the 10 recommendations were discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the recommendations. RESULTS: These recommendations cover general aspects as shared decision, prospective registry in Reuma.pt, assessment of activity and RA impact and treatment objective. Consensus was also achieved regarding specific aspects as initiation of biologic therapy, assessment of response, switching and definition of persistent remission. CONCLUSION: These recommendations may be used for guidance of treatment with biological therapies in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated.

Schnitzler Syndrome: A Dramatic Response to Anakinra.

Schnitzler syndrome is a rare acquired systemic disease with a chronic evolution and difficult treatment. We report a 50-year-old woman with Schnitzler syndrome for 10 years, with major impact on her quality of life and refractory to conventional therapies. The patient was started on anakinra, an IL-1 receptor antagonist, with a rapid and sustained remission of the syndrome manifestations.

Oxidative stress in chronic hepatitis C: the effect of interferon therapy and correlation with pathological features.

AIMS: To evaluate the oxidative stress parameters before, during and after interferon treatment. PATIENTS/METHODS: Twenty patients were treated with interferon a2b 5 MU, three times a week, subcutaneously, for 12 months. Liver biopsy was performed six months before treatment and at the six month follow-up. Chromosomal breakage studies were evaluated by the adjusted clastogenic score (ACS, normal value [nv] 1.1 +/- 2.4%). Plasma malondialdehyde (MDA) was measured according to the Yagi method (nv 6.6 +/- 1.4 nmol/mL) and total thiols using the Ellman's reagent (DTNB) (nv 9.8 +/- 1.3 micromol/g protein). A serum marker of fibrogenesis, the amino-terminal propeptide of Procollagen type III (PIIIP), was quantified by radioimmunoassay (nv 0.37 +/- 0.18 U/L). RESULTS: Compared with reference samples, the plasma of patients before treatment showed an increase of ACS (9.2 +/- 3.2%, P<0.001); higher MDA values (12.6 +/- 2.7 nmol/mL, P<0.001) and total plasma sulfhydryl groups (t-SH) were decreased (6.3 +/- 1.1 micromol/g protein, P<0.001). During treatment and at the follow-up, a decrease in ACS was noticed in all patients (P<0.001), but without normalization; a decrease in MDA was seen, with progressive normalization until the end of the follow up only in sustained responders (P<0.003), while an increase of t-SH was seen, with progressive normalization until the end of follow up in all patients (P<0.005). A positive correlation of ACS with grading of inflammation was found (r=0.52, P<0.03) but not with fibrosis staging. In contrast, plasma MDA correlates with fibrosis staging (r=0.51, P<0.03) and with PIIIP (r=0.57, P<0.03) but without grading of inflammation. CONCLUSIONS: The present study confirmed the presence of oxidative stress in chronic hepatitis C patients. Interferon promotes a long term inhibition of oxidative stress with concomitant improvement of activity and fibrosis. In the management of chronic hepatitis C, adjuvant therapy with antioxidants could be useful.

Mucocutaneous manifestations of Behçet's disease.

Behçet's disease is a relapsing multisystem polysymptomatic disease with exacerbations and remissions defined by the presence of the major symptom, recurrent oral aphthous ulcers, plus two of the following: recurrent genital ulceration, eye lesions, skin lesions or a positive pathergy test. Mucocutaneous manifestations like oral and genital ulcers, and cutaneous lesions (papulopustular lesions, erythema nodosum-like lesions, cutaneous ulcers, superficial thrombophlebitis), are considered the \"fingerprint\" of the disease, being the most common and often the first signs to appear. Although the exact etiopathogenesis is still not known, genetic predispose and environmental factors may influence and contribute to the development of this disease. Diagnosis is based in the International Study Group criteria. During the last years, this disease has been largely studied and new immunological data and treatment strategies have been postulated. Despite that, further studies and attention to new data are needed.

Cutaneous manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.