Érica Vieira Serrano

Primary Sjögren's syndrome prevalence in a major metropolitan area in Brazil

There has been no previous prevalence study about of Sjögrens syndrome (SS) in Brazil. The aim was to evaluate the SS prevalence in a general population in Vitória, ES, Brazil. This was an epidemiological, observational, and cross-sectional study conducted on 1,205 randomized people, aged 18-65 years, who lived in Vitória. The subjects were screened for xerostomia and xerofphthalmia through home interviews. Those with sicca symptoms were asked to report to a hospital for further medical evaluation, unstimulated salivary flow, Schirmer I test, blood analysis and minor labial salivary biopsy. Sicca symptoms were found in 18% (217 subjects) of the sample. Of the 217 subjects with sicca symptoms, 127 (58%) were available for examination. In this sample, 61.7% were female and 46.8% were under medication. Sicca syndrome was confirmed in 12% by at least one examination (salivary flow or Schirmer I). Two patients (0.17%) matched four criteria according to American-European Criteria (95% CI = 0.020-0.5983).

Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report.

Acute fibrinous and organizing pneumonia (AFOP), recently described, is a histologic pattern characterized by the presence of fibrin “balls” within alveolar spaces. The term undifferentiated connective tissue disease (UCTD) is used to identify autoimmune systemic diseases that do not fulfill the criteria to be classified as a definitive connective tissue disease. The AFOP has never been reported in association with UCTD. The present reported case is a 39-year-old Caucasian, female with dry cough and progressive dyspnea. Eight months later, she was diagnosed with “organizing pneumonia” based on clinical history and radiologic images. She manifested Raynauds Phenomenon, sicca syndrome, boot and gloves neuropathic pain, and previous hypothyroidism. Antinuclear antibody, rheumatoid factor, and specific autoantibodies were negative. Salivary gland biopsy and electroneuromyiography were normal. The capillaroscopy showed a “scleroderma” pattern with capillary deletion and ectasia. She experienced clinical and radiologic worsening. Despite being submitted to cyclophosphamide pulse, she developed hemorrhage and then died. Thoracotomy pulmonary specimen showed histological pattern of AFOP. This paper shows a rare association of AFOP with UCTD.

Clinical characteristics and biopsy accuracy in suspected cases of Sjögren's syndrome referred to labial salivary gland biopsy.

BackgroundLabial salivary gland biopsy (LSGB) is the most important diagnostic tool for the diagnosis of Sjögren’s syndrome (SS), but its diagnostic value is rarely studied. This study assessed the sensibility and specificity of LSGB, and the clinical profiles of patients who were referred for biopsy.MethodsRetrospective analysis of the histopathological reports from LSGB and medical report data from patients who underwent LSGB between 2008 and 2011 was conducted.ResultsAbout 290 biopsies were performed and 74 were excluded due to insufficient clinical data. Of the 216 patients, 0.46% was carrier of hepatitis C virus, 30.1% had primary SS (pSS), and 8.8% had secondary SS (sSS). Of the samples, 94.3% presented dryness symptoms, 51.6% experienced dryness only, 42.7% had systemic manifestations, and 66.9% presented low unstimulated salivary flow and/or Schirmer’s test. LSGB was necessary in 67.6% to confirm the presence of SS based on the American-European Consensus Group 2002 criteria (AECG). Based on specialist’s opinion, sensibility level was 86.57%, and specificity was 97.43%. Positive predictive value (PPV) was 95%, and negative predictive value (NPV) was 92.6%. Determined accuracy was 93.3%. Concordance (kappa coefficient) of LSGB and specialist’s opinion was 0.851, and LSGB with AECG criteria was 0.806. Of the 98 patients referred with fibromyalgia and dryness, 36.7% had SS and LSBG focus score of ≥ 1. Patients with SS were older, and showed more severe lachrymal and salivary dysfunctions, greater frequency of fibromyalgia, anti-nuclear antibodies (ANA), anti-SSA-Ro, and anti-SSB-La.ConclusionsLabial salivary gland biopsy has high sensibility, specificity, positive and negative predictive values for diagnosis of pSS. In the clinical practice, it is useful, especially for those patients with glandular dysfunctions and negative antibodies.

Prevalência da síndrome de Sjögren primária em importante área metropolitana no Brasil

There has been no previous prevalence study about of Sjogrens syndrome (SS) in Brazil. The aim was to evaluate the SS prevalence in a general population in Vitoria, ES, Brazil. This was an epidemiological, observational, and cross-sectional study conducted on 1,205 randomized people, aged 18-65 years, who lived in Vitoria. The subjects were screened for xerostomia and xerofphthalmia through home interviews. Those with sicca symptoms were asked to report to a hospital for further medical evaluation, unstimulated salivary flow, Schirmer I test, blood analysis and minor labial salivary biopsy. Sicca symptoms were found in 18% (217 subjects) of the sample. Of the 217 subjects with sicca symptoms, 127 (58%) were available for examination. In this sample, 61.7% were female and 46.8% were under medication. Sicca syndrome was confirmed in 12% by at least one examination (salivary flow or Schirmer I). Two patients (0.17%) matched four criteria according to American-European Criteria (95% CI = 0.020-0.5983).

Recomendações para o tratamento da síndrome de Sjögren

The recommendations proposed by the Sjögrens Syndrome Committee of the Brazilian Society of Rheumatology for the treatment of Sjögrens syndrome were based on a systematic review of literature in Medline (PubMed) and the Cochrane databases until October 2014 and on expert opinion in the absence of studies on the subject. 131 items classified according to Oxford & Grade were included. These recommendations were developed in order to guide the appropriate management and facilitate the access to treatment for those patients with an appropriate indication, considering the Brazilian socioeconomic context and pharmacological agents available in this country.

Avaliação da fadiga e da secura na síndrome de Sjögren primária: versão brasileira do “Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (Profad-SSI-SF)”

OBJECTIVE To perform a cross-cultural adaptation and validation of the Profile of Fatigue and Discomfort - Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF) questionnaire assessing the subjective aspects of the symptoms of primary Sjögren syndrome (pSS), for the Brazilian Portuguese language. METHOD Conceptual, of the item, semantic and operational equivalences were evaluated. The Brazilian version of PROFAD-SSI-SF was administered to 62 women with pSS according to the European-American consensus 2002 to assess measurement equivalence. α-Cronbach was used for internal consistency; intraclass correlation coefficient (ICC) for intraobserver reproducibility; and Spearman correlation coefficient for validity by comparing with Patient Global Assessment (PaGA), EULAR Sjögrens Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F) and EuroQOL (EQ-5D). RESULTS The internal consistency of PROFAD, SSI and total score was 0.80; 0.78; and 0.87, respectively. The intraobserver reproducibility of total PROFAD was 0.89; of total SSI of 0.86; and total score of 0.89. In terms of validity, PROFAD correlated significantly with PaGA (r = 0.50), FACIT-F (r = 0.59), ESSPRI (r = 0.58) and all domains of EQ-5D, with the exception of Mobility. On the other hand, SSI correlated significantly with PaGA (r = 0.43), FACIT-F (r = 0.57), ESSPRI (r = 0.55) and most areas of EQ-5D. The total score of PROFAD-SSI-SF had a non-statistically significant correlation only with Mobility domain and with 1-100 range of EQ-5D. CONCLUSION The Portuguese version of PROFAD-SSI-SF proved to be an adaptable, reproducible and valid tool for the Brazilian Portuguese language.

Artigo originalValidação e propriedades psicométricas do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesaValidation and psychometric properties of the Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) into Brazilian Portuguese☆

OBJECTIVE To carry out the cross-cultural adaptation of Eular Sjögrens Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties. METHOD Cross-sectional study of patients with primary Sjögrens syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patients Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were:Cronbachs alpha, intraclass correlation coefficient (ICC), Bland-Altman method and Spearman coefficient. A value of p ≤ 0.05 was considered significant. RESULTS There was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4 ± 11.6 years, with onset of symptoms of 7.2 ± 5.4 years, and time of diagnosis of 3.0 ± 3.3 years. The mean ESSPRI was 6.87 ± 1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland-Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed. CONCLUSION The Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

Validação e propriedades psicométricas do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesa

OBJECTIVE To carry out the cross-cultural adaptation of Eular Sjögrens Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties. METHOD Cross-sectional study of patients with primary Sjögrens syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patients Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were:Cronbachs alpha, intraclass correlation coefficient (ICC), Bland-Altman method and Spearman coefficient. A value of p ≤ 0.05 was considered significant. RESULTS There was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4 ± 11.6 years, with onset of symptoms of 7.2 ± 5.4 years, and time of diagnosis of 3.0 ± 3.3 years. The mean ESSPRI was 6.87 ± 1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland-Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed. CONCLUSION The Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

AB0533 Recommendations of Brazilian Society of Rheumatology for the Treatment of SjÖGren's Syndrome

Objectives Recommendations of Brazilian Society of Rheumatology for the treatment of Sjögrens syndrome (SS) were developed to guide management of SS considering the Brazilian social and economic context. Methods It was based on specialists opinion and systematic review on MEDLINE (PubMed) and Cochrane database until October 2014, including 127 articles classified according Oxford & Grade. Results Forty-four recommendations were organized in 3 main topics: Part 1. General recommendations and patient education: The management of SS should be conducted by multidisciplinary team. Systemic treatment should be according disease severity measured by EULAR Sjögrens Syndrome Disease Activity (ESSDAI). Patients should avoid caffeine, tobacco, alcohol, toothpaste with abrasive, and mouthrinses with alcohol. Patient should be educated about preventive measures for oral health and hydration. Aerobic exercise improves fatigue and quality of life. Immunization to influenza and pneumococo are indicated. Serum levels of vitamin D should be evaluated and supplemented if it is necessary. Part 2. Symptomatic treatment of dryness: Topical treatment for dry mouth includes saliva substitutes, sugar-free candies and gums. Topical treatments for dry eye are lubricants (glucanes or carboximethylcelulose), cyclosporine 0.05%, and punctual occlusion. Topical glucocorticoids may be used for severe dry eye for short time avoiding complications. Pilocarpine and cevimeline should be used for dry mouth and for severe dry eye. N-acetylcysteine may be used for dryness symptoms, including patients showing intolerance for muscarinic agonists. Omega-3 supplementation may be used to dry eye. Part 3. Systemic treatment: Immunosupressant and/or biological therapy are not indicated to dryness treatment. Hydroxichloroquine, glucocorticoid, and immunossupressants (azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine) should be indicated according severity of systemic involvement. Rituximab is indicated to treat systemic manifestations without improvement with immunosuppressive therapy. Abatacept and belimumab may be considered in patients not responding to rituximab and with high level of disease activity. Acknowledgements Sociedade Brasileira de Reumatologia (SBR) Disclosure of Interest None declared