Abstract Background: The Indian Takayasu Clinical Activity Score (ITAS2010) was developed in 2010 as an assessment tool for disease activity in patients with Takayasu arteritis (TA). It has since been widely used in different studies and in clinical practice for the management of patients with TA. The present study aims to translate the ITAS2010 into Brazilian Portuguese language and to validate it for use in clinical practice in Brazil. Methods: For this cross-sectional study, the ITAS2010 was translated in accordance with the guidelines described by Beaton et al. and then applied with 27 patients with TA on three assessments by two rheumatologists working independently. To measure interrater agreement, the assessments were performed on the same day within approximately 1 hour. One of the rheumatologists performed a second evaluation of patients with TA within 7 to 14 days to measure intrarater agreement. Results: The correlation coefficient for the ITAS2010 score between the two raters was high (r =0.916; p < 0.0001), as well as the intraclass correlation coefficient (ICC) [0.918 with a 95% confidence interval (95CI): 0.828-0.962]. The correlation coefficient and the ICC for intrarater agreement were moderate for ITAS2010 (r =0.633; p < 0.0001 and ICC = 0.594; 95CI: 0.292-0.790). The ITAS2010 at baseline was compared with the physician's global assessment (PGA) and with Kerr's criteria for detecting disease activity in TA. Higher ITAS2010 scores were observed in patients with active and grumbling/persistent disease than in those presenting inactive disease according to the PGA [1.5 (0.0-3.0) vs. 0.0 (0.0-0.0); p = 0.0025]. Patients with active disease according to the Kerr's criteria had also higher ITAS2010 scores than those considered in remission [3.0 (3.0-7.0) vs. 0.0 (0.0-0.0); p = 0.0068]. Conclusions: The Brazilian Portuguese version of the ITAS2010 is a valid and reproducible tool for the assessment of disease activity in TA and it is an additional tool for the routine evaluation of Brazilian patients with TA.

Abstract: Due to mounting evidences of interaction between Hansen's bacilli with endothelial cells and the paucity of studies addressing the presence of nailfold capillaroscopic alterations in patients with Hansen's disease, a study was carried out in order to verify the presence of capillaroscopic alterations in patients with leprosy in its various forms and its correlation with clinical parameters. Ten patients were evaluated at a specialized university service. Sixty percent of those had some capillaroscopic change, such as micro-hemorrhages, ectatic, bushy and corkscrew capillaries. Such changes were unspecific, which suggests there is not a specific pattern for this disease.

Relatamos um caso de diagnóstico de Doença de Still do Adulto (DSA) em paciente feminina com febre, mialgia, rash cutâneo fugaz e linfonodomegalia inguinal bilateral, após extensa investigação para exclusão de outras doenças reumatológicas, infecciosas e neoplásicas. A paciente inicialmente apresentou resposta ao tratamento com prednisona, porém evoluiu com aumento de volume de linfonodos inguinais, cuja biópsia revelou adenocarcinoma seroso de ovário. De acordo com nosso conhecimento, esse é o primeiro relato de neoplasia ovariana associada ao diagnóstico de DSA.

We report a case of adult-onset Still's disease in a female patient with fever, myalgia, vanishing rash and bilateral inguinal lymphadenopathy, diagnosed after extensive workup to exclude other rheumatic, infectious and neoplastic diseases. The patient initially responded to corticosteroid therapy, but progressed to increased lymph nodes size that when biopsied, revealed serous ovarian adenocarcinoma. To our knowledge, this is the first report of ovarian neoplasm associated with adult-onset Still's disease.

A associação de porfiria cutânea tarda (PCT) e lúpus eritematoso sistêmico (LES) é rara. O LES, de fisiopatologia complexa e manifestações clínicas pleomórficas, assemelha-se à PCT pela fotossensibilidade. Um achado que pode diferenciar as duas doenças são as lesões cutâneas bolhosas, raras no LES, mas características da PCT. Descrevemos um caso de associação de PCT e LES e revisamos a literatura, enfatizando questões fisiopatológicas, clínicas e terapêuticas. Um dado relevante para a prática clínica concerne ao tratamento do lúpus com antimaláricos, o que pode oferecer riscos para a PCT.

The association of porphyria cutanea tarda (PCT) and systemic lupus erythematosus (SLE) is rare. Systemic lupus erythematosus, of complex pathophysiology and pleomorphic clinical manifestations, is similar to PCT regarding photosensitivity. One finding that can differentiate both diseases is the presence of cutaneous blisters, which are rare in SLE, but characteristic of PCT. We report one case of the association of PCT and SLE and revise the literature, emphasizing pathophysiological, clinical and therapeutic aspects. One relevant information for clinical practice relates to the treatment of SLE with antimalarials, which is a risk for PCT.

Os autoanticorpos possivelmente influenciam as manifestações clínicas da esclerose sistêmica (ES). Essa correlação clínico-sorológica, associada à insuficiência de casos de concomitância de autoanticorpos, originou o paradigma histórico de que seriam mutuamente excludentes. Porém, pode-se questionar essa tese. Poderia a multiplicidade de autoanticorpos significar a coexistência de duas patologias distintas? Por outro lado, se assumidos como anticorpos específicos de uma doença única, essa multiplicidade seria um evento aleatório ou representaria um subgrupo distinto de pacientes, com características clínicas, patogênicas e imunogenéticas próprias? A prevalência de autoanticorpos na ES precoce é elevada. Entretanto, a duplicidade do anticorpo anticentrômero (AAC) e do anticorpo antitopoisomerase 1 (AAT) é um evento raro. Já a coexistência de AAC, AAT e anticorpo anti-RNA polimerase (anti-RNA-P) III ainda não foi descrita em um paciente isolado. Neste relato, com positividade para AAC, AAT e anti-RNA-P III, notamos manifestações vasculares precoces e posterior comprometimento cutâneo limitado. Este parece ser o primeiro relato de concomitância de três autoanticorpos específicos em um paciente com ES. Acreditamos que essa coexistência representa um subgrupo sorológico raro de uma única doença, com possível valor clínico e prognóstico - porém, ainda há necessidade de confirmação.

Autoantibodies possibly influence clinical manifestations of systemic sclerosis (SSc). This clinical-serological correlation, associated with the paucity of autoantibodies concomitance, gave rise to the historical paradigm of autoantibodies mutual exclusivity. However, one can question this assumption. Does autoantibodies concomitance mean coexistence of two different entities? On the other hand, if considered a unique disease, is this phenomenon a random event or does it represent a distinct subgroup of patients, with peculiar clinical, pathogenic, and immunogenetic characteristics? The autoantibodies' prevalence in early SSc is high. However, anti-centromere antibody (ACA) and antitopoisomerase 1 antibody (ATA) duplicity is a rare event. Similarly, the ACA, ATA, and anti-RNA polymerase (anti-RNA-P) III coexistence have not been described yet in single patient. In the reported case, with ACA, ATA, and anti-RNA-P III positivity, we have noted early vascular manifestations and late limited cutaneous involvement. This is, to our knowledge, the first report of three concomitant specific autoantibodies in a patient with SSc. We do believe this coexistence represents a rare serologic subgroup of a unique disease, with possible clinical and prognostic value, although this remains to be confirmed.