Chest MRI with CT in the assessment of interstitial lung disease progression in patients with systemic sclerosis.

OBJECTIVE: To describe the performance of CT and MRI in the assessment of the progression of interstitial lung disease (ILD) associated with SSc and demonstrate the correlations of MRI with pulmonary function test (PFT) and CT scores. METHODS: This prospective single-centre observational study included patients with SSc diagnoses, and magnetic resonance (MR) images were assessed visually using the Scleroderma Lung Study (SLS) I system. Differences in the median scores were assessed with Student's t-test and the Wilcoxon rank-sum test. Pearson's and Spearman's rank correlation coefficients were calculated to correlate imaging scores and PFT results. Using disease progression as the gold standard, we calculated the area under the curve (AUC) of the CT and MRI scores with Harrel's c-index. The best thresholds for the prediction of disease progression were determined by receiver operating characteristic curve analysis with maximum Youden's Index (P < 0.05). The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of the scores were calculated. RESULTS: The AUCs for MRI and CT scores were 0.86 (0.72-0.98; P = 0.04) and 0.83 (0.70-0.99; P = 0.05), respectively. CT and MRI scores correlated with Forced vital capacity (%FVC) (MRI: r = -0.54, P = 0.0045; CT: r = -0.44; P = 0.137) and diffusing capacity of the lung for carbon monoxide (MRI: r = -0.39, P = 0.007; CT r = -0.36, P = 0.006). The sensitivity, specificity, PPV and NPV were 85%, 87.5%, 88.34% and 86.11% (MRI score) and 84.21%, 82.35%, 84.14% and 82.4% (CT score), respectively. CONCLUSIONS: MRI scores from patients with SSc may be an alternative modality for the assessment of ILD progression in patients with SSc.

Magnetic resonance imaging of interstitial lung diseases: A state-of-the-art review.

Magnetic resonance imaging (MRI) has been emerging as an imaging modality to assess interstitial lung diseases (ILD). An optimal chest MRI protocol for ILDs should include non-contrast breath-holding sequences, steady-state free-precession sequences, and contrast-enhanced sequences. One of the main MRI applications in ILDs is the differentiation between areas of active inflammation (i.e. reversible stage) and fibrosis. Alveolitis presents high signal intensity on T2-weighted sequences (WS) and early-enhancement on contrast-enhanced MR sequences, while fibrotic-predominant lesions present low signal and late-enhancement in these sequences, respectively. MRI can be useful in connective tissue diseases, idiopathic pulmonary fibrosis, and sarcoidosis. The aim of this state-of-the-art review was to perform a state-of-the-art review on the use of MRI in ILDs, and propose the optimal MRI protocols for imaging ILDs.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy.

Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

Necrose digital em paciente com lúpus eritematoso sistêmico e esclerose sistêmica tratada com inibidores da fosfodiesterase / Digital necrosis treated with phosphodiesterase inhibitors in a patient with connective tissue disease

Os inibidores da fosfodiesterase têm sido introduzidos, nos últimos anos, como novos agentes farmacológicos no tratamento dos pacientes com fenômeno de Raynaud e isquemia digital. Será descrito o caso de uma paciente com lúpus eritematoso sistêmico e esclerose sistêmica limitada apresentando fenômeno de Raynaud grave e necrose digital refratária à terapia. A paciente obteve excelente resposta à associação de imunossupressão e sildenafil.

The phosphodiesterase inhibitors have been used recently for the treatment of Raynaud's phenomenon and digital ischaemia. We report the case of a patient affected by systemic lupus erythematosus and limited systemic sclerosis who presented severe Raynaud's phenomenon with digital necrosis despite treatment. The patient presented an excellent response to the association of immunosuppressant therapy and sildenafil.

Autoantibodies to NOR 90/hUBF: longterm clinical and serological followup in a patient with limited systemic sclerosis suggests an antigen driven immune response.

We describe the clinical and serological followup of a 9-year-old girl with anti-nucleolar organizing region 90/human upstream-binding factor (anti-NOR 90/hUBF) who had features of systemic sclerosis over a period of 17 years, from childhood into adulthood. We review the associations of anti-UBF autoantibodies, and provide evidence that anti-NOR 90/UBF immune response is antigen driven.

Há associaçäo entre vírus da hepatite C e fenômeno de Raynaud? / Is there a link between hepatitis C virus and Raynaud's phenomenon?

Introduçäo: Na literatura médica, destaca-se a associaçäo entre o vírus da hepatite C (HCV) e a crioglobulinemia, principalmente do tipo II, em que há alta prevalência de alteraçöes hepáticas. O fenômeno de Raynaud (FRy) também ocorre em resposta ao frio ou a estresse emocional, associado ou näo à presença de crioglobulinas. Objetivo: Verificar se há associaçÝo entre infecçäo pelo HCV e FRy, controlando para presença de crioglobulinas. Material e métodos: Avaliamos 46 pacientes com FRy primário ou secundário, 10 homens e 36 mulheres, idade média = 40 anos. Presença de anticorpos anti-HCV totais testada por ELISA. Crioglobulinas pesquisadas por precipitaçäo ao frio. Populaçöes-controles: 1) para HCV: a) doadores de sangue(n=408.477), sendo aproximadamente 1 por cento HCV+; e b) 33 pacientes internados sem FRy, sendo 9 (27,6 por cento) HCV+; 2) para crioglobulinas: pacientes testados no laboratório de imunologia (1993 a 1995, n= 99), com três casos positivos (3 por cento). Resultados: De 46 pacientes com FRy, 5 (11 por cento) tinham anti-HCV+ e 8 (17 por cento), crioglobulinas. Dois pacientes eram simultaneamente anti-HCV+ e crioglobulinas+ (4 por cento). Dos 5 pacientes anti-HCV+, 3 tinham procedimentos prévios de hemoterapia e todos foram internados no passado. Aplicando-se teste z (proporçÝo populacional), obteve-se p<0,0005 na prevalência de anti-HCV em pacientes com FRy, quando comparados à populaçäo de doadores de sangue. Entretanto, quando comparados ao grupo dos pacientes internados sem FRy, näo houve diferença estatística (p<0,1). Dos pacientes internados sem FRY e anti-HCV+ (n= 9), 3 haviam recebido transfusÝo de sangue e 8 tinham internaçöes prévias. Pacientes com FRy tiveram prevalência maior de crioglobulinas+, quando comparados aos grupo-controle (p<0,05). Pacientes com FRy primário tiveram maior prevalência de anti-HCV+ que o grupo com FRy secundário (p<0,03). Conclusöes: Houve maior prevalência de anti-HCV em pacientes com FRy que na populaçäo em geral, quando testados por ELISA. A maior prevalência do HCV na populaçäo estudada parece se dever a vício de seleçäo, eis que os pacientes tinham internaçöes prévias, sofriam de doenças crônicas e tinham recebido transfusöes de sangue ou plasmaferese. A concomitância de anti-HCV+ e crioglobulinemia+ (2/5 pacientes HCV+) ocorreu na proporçäo vista na literatura para crioglobulinemia tipo II (>40 por cento). Casos com FRy primário podem ter maior contato ou responder de forma diversa ao HCV...