Segmentation-Based Analysis of T2- and T1-Weighted Dynamic Magnetic Resonance Images Provides Adequate Observer Agreement in the Evaluation of Interstitial Lung Disease.

OBJECTIVE: The aim of the study is to quantify observer agreement in the magnetic resonance imaging (MRI) classification of inflammatory or fibrotic interstitial lung disease (ILD). METHODS: Our study is a preliminary analysis of a larger prospective cohort. The MRI images of 18 patients with ILD (13 females; mean age, 65 years) were acquired in a 1.5 T scanner and included axial fat-saturated T2-weighted (T2-WI, n = 18) and coronal fat-saturated T1-weighted images before and 1, 3, 5, and 10 minutes after gadolinium administration (n = 16). The MRI studies were evaluated with 2 different methods: a qualitative evaluation (visual assessment and measurement of few regions of interest; evaluations were performed independently by 5 radiologists and 3 times by 1 radiologist) and a segmentation-based analysis with software extraction of signal intensity values (evaluations were performed independently by 2 radiologists and twice by 1 radiologist). Interstitial lung disease was classified as inflammatory or fibrotic, based on previously described imaging criteria. RESULTS: Regarding the qualitative evaluation, intraobserver agreement was excellent (κ = 0.92, P < 0.05) for T2-WI and fair (κ = 0.29, P < 0.05) for T1 dynamic study, while interobserver agreement was moderate (κ = 0.56, P < 0.05) and poor (κ = 0.11, P = 0.18), respectively. In contrast, upon segmentation-based analysis, intraobserver and interobserver agreement were excellent for T2-WI (κ = 0.886, P < 0.001; κ = 1.00, P < 0.001; respectively); for T1-WI, intraobserver agreement was excellent (κ = 0.87, P < 0.05) and interobserver agreement was good (κ = 0.75, P < 0.05). CONCLUSIONS: Segmentation-based MRI analysis is more reproducible than a qualitative evaluation with visual assessment and measurement of few regions of interest.

Intestinal Hemangiomatosis: Case Report of an Uncommon Cause of Rectal Bleeding.

A four-month-old girl presented with recurrent low gastrointestinal hemorrhage. Abdominal ultrasound showed diffuse parietal thickening and hyperemia of the colon. Computed tomography (CT) demonstrated diffuse thickening of the colon but also intense arterial globular mural enhancement with diffuse filling in the portal phase. Colonoscopy revealed multiple pseudopolipoid lesions along the colon which were histologically diagnosed as hemangiomas. The infant was diagnosed with gastrointestinal hemangiomatosis and was treated with propranolol resulting in complete resolution of symptoms. Teaching point: Although rare, the possibility of intestinal hemangiomatosis should be considered in the setting of rectal bleeding in an infant.

Lung Magnetic Resonance Imaging for Prediction of Progression in Patients With Nonidiopathic Pulmonary Fibrosis Interstitial Lung Disease: A Pilot Study.

PURPOSE: Correlate magnetic resonance imaging (MRI) parameters at baseline with disease progression in nonidiopathic pulmonary fibrosis interstitial lung disease (ILD). MATERIALS AND METHODS: Prospective observational cohort study, in which patients with non-idiopathic pulmonary fibrosis ILD underwent MRI at baseline (1.5 T). T2-weighted images (T2-WI) were acquired by axial free-breathing respiratory-gated fat-suppressed "periodically rotated overlapping parallel lines with enhanced reconstruction" and T1-weighted images (T1-WI) by coronal end-expiratory breath-hold fat-suppressed "volumetric interpolated breath-hold examination" sequences, before and at time points T1, T3, T5, and T10 minutes after gadolinium administration. After MRI segmentation, signal intensity values were extracted by dedicated software. Percentage of the ILD volume and a ratio between signal intensity of ILD (SIILD) and normal lung (SInormal lung) were calculated for T2-WI; percentage of signal intensity (%SI) at each time point, time to peak enhancement, and percent relative enhancement of ILD in comparison with normal lung (%SIILD/normal lung) were calculated for T1-WI. MRI parameters at baseline were correlated with diagnosis of disease progression and variation in percent predicted forced vital capacity (%FVC) and diffusing capacity of the lung for carbon monoxide after 12 months. RESULTS: Comprehensive MRI evaluation (T2-WI and T1-WI) was performed in 21 of the 25 patients enrolled (68% females; mean age: 62.6 y). Three of the 24 patients who completed follow-up fulfilled criteria for disease progression. Baseline T2-WI SIILD/SInormal lung was higher for the progression group (P = 0.052). T2-WI SIILD/SInormal lung and T1-WI %SIILD/normal lung at T1 were positively correlated with the 12-month variation in %FVC (r = 0.495, P = 0.014 and r = 0.489, P= 0.034, respectively). CONCLUSIONS: Baseline MRI parameters correlate with %FVC decline after 12 months.

Biological Therapy in Patients with Rheumatoid Arthritis in a Tertiary Center in Portugal: A Cross-Sectional Study.

INTRODUCTION: Clinical outcomes in rheumatoid arthritis have greatly improved with therapeutic advances. Despite the availability of substantial clinical trial evidence, there is a lack of real-life data. The aim of this study was to assess disease status and quality of life in an outpatient population treated with biological disease-modifying anti-rheumatic drugs. MATERIAL AND METHODS: Cross-sectional study recalling all patients ever treated in our unit with biological disease-modifying antirheumatic drugs. Clinical and demographic data, compliance, disease activity, functional status, joint deformities, and comorbidities were documented, and patients queried on occupational status, education, marital status and generic health related quality of life questionnaires. RESULTS: Recall was attended by 77 of the original 94 patients. At recall, median age was 63 years old, 82% of the patients were female and the median disease duration was 12 years. Biological therapy was started at a median of four years following disease onset. According to the disease activity score (DAS28), the percentage of patients with high, moderate, low disease activity or remission changed from 50, 45, 0 and 5 (pre-therapy) to 11, 37, 25 and 26 at recall, respectively; functional status was significantly improved. Seventy-five per cent of the patients retained the original treatment with good compliance. Lower Short Form-36 domain scores accompanied a low EQ-5D-3L score. Deceased patients (n = 6) had a lower estimated 10-year survival rate. In this group, biological therapy was discontinued at a higher frequency during follow-up. DISCUSSION: A high disease activity and a high HAQ disability index characterized most patients at pre-bDMARD onset. CONCLUSION: Despite therapy switches and regular follow-up, a significant percentage of patients still presented with moderate disease activity, functional impairment and a poor health-related quality of life.

Introdução: Avanços no tratamento da artrite reumatóide contribuiram para uma evolução favorável. Apesar de evidências substanciais provenientes de ensaios clínicos, são menos conhecidos os dados provenientes da vida real. O objetivo do estudo foi caracterizar a doença e a qualidade de vida em doentes sob fármacos biotecnológicos. Material e Métodos: Trata-se de um estudo transversal com recolha de dados clínicos relativos à adesão terapêutica, atividade da doença, capacidade funcional, deformidades articulares, comorbilidades e questionários de qualidade de vida relacionada com a saúde, estado civil, situação profissional e escolaridade. Resultados: Foram recrutados 77 doentes do grupo original de um total de 94. A mediana da idade foi 63 anos, 82% do sexo feminino e início de biológico cerca de quatro anos após o início da doença, com uma mediana de duração de 12 anos. De acordo com o disease activity score (DAS28), a percentagem de doentes com atividade alta, moderada, baixa ou em remissão mudou, respectivamente, de 50, 45, 0 e 5 pré- biológico para 11, 37, 25 e 26 na altura da re-avaliação, com melhoria funcional. Setenta e cinco por cento dos doentes mantiveram o tratamento original com boa adesão. Pontuações mais baixas do short form-36 associaram-se a uma baixa pontuação no EQ-5D-3L. No grupo de doentes que viriam a falecer (n = 6), foi observada uma menor esperança de vida aos 10 anos, assim como uma maior discontinuação da terapêutica biológica. Discussão: Pré-biológico, uma elevada percentagem dos doentes apresentava elevada atividade da doença e incapacidade funcional. Conclusão: Não obstante ajustes terapêuticos e seguimento regular, uma percentagem significativa de doentes mantinha atividade moderada e limitação funcional com baixa qualidade de vida relacionada com a saúde.

Clinical Presentation and Long-Term Outcomes of Systemic Sclerosis Portuguese Patients from a Single Centre Cohort: A EUSTAR Registration Initiative.

INTRODUCTION: Systemic sclerosis is a complex disorder that requires systematic screening. Our objective is to report the European Scleroderma Trials and Research group centre affiliation and its impact in our clinical practice. MATERIAL AND METHODS: The European Scleroderma Trials and Research group affiliation process, database update and current patient evaluation, with respect to demographic and clinical features. Cumulative mortality was analysed. RESULTS: We identified 19 female patients (which met all the American College of Rheumatology/ European League Against Rheumatism 2013 criteria for systemic sclerosis) under current follow-up, divided according to the LeRoy classification into diffuse cutaneous (n = 5), limited cutaneous (n = 11) and limited (n = 3) types, followed for a median period of 5, 12 and 6 years, respectively. Raynaud´s phenomenon and abnormal nailfold capillaries were universally present. Interstitial lung disease was absent in the limited cutaneous form but present in 100% of the diffuse subtype. Pitting scars were more common in the diffuse form. Active disease was also more frequent in the diffuse form, and most patients with active disease were treated with anti-endothelin receptor antagonists. Over 21 years (from 1994 to 2015) the mortality rate was 55% (n = 23/42). Age at time of death was significantly lower in the diffuse subtype. DISCUSSION: Our single centre cohort shares many features with larger and international reports and more specifically is in accordance with patient characteristics described in the European Scleroderma Trials and Research group registries. CONCLUSION: The European Scleroderma Trials and Research group registration motivated our systematic patient characterization and may be used as a tool for homogenous disease registries.

Introdução: A esclerose sistémica é uma doença complexa que requer uma vigilância regular e sistemática. Este estudo teve como objetivo divulgar a afiliação da nossa Unidade no registo Europeu Scleroderma Trials and Research group e o seu impacto na prática clínica. Material e Métodos: Processo de afiliação Scleroderma Trials and Research group, atualização da base de dados, avaliação sistemática das características demográficas e clínicas dos doentes em seguimento e análise da mortalidade cumulativa. Resultados: Foram identificados 19 doentes do sexo feminino (com preenchimento completo dos critérios de classificação de esclerose sistémica do American College of Rheumatology/ European League Against Rheumatism 2013), seguidas no momento atual e divididas pela classificação de LeRoy em três formas: cutânea difusa (n = 5), cutânea limitada (n = 11) e limitada (n = 3), com um período de seguimento com uma duração mediana de 5, 12 e 6 anos, respetivamente. O fenómeno de Raynaud e as anomalias nos capilares peri-ungueais estavam presentes em todas as doentes. Todas as doentes com a forma difusa apresentavam doença intersticial pulmonar, ausente na forma cutânea limitada. As pitting scars foram mais frequentes na forma difusa. A doença ativa foi mais frequente na forma difusa, na sua maioria tratada com antagonistas dos recetores da endotelina. Num período de 21 anos (de 1994 a 2015), a mortalidade foi de 55% (n = 23/42). A expectativa de vida nos doentes com a forma difusa estava significativamente reduzida quando comparada com a forma localizada. Discussão: O nosso grupo de doentes é semelhante a outros de maiores dimensões e cariz internacional e, mais especificamente, enquadra-se nas características dos doentes registados nos registos Scleroderma Trials and Research group. Conclusão: O registo Scleroderma Trials and Research group incentivou uma caracterização sistemática e pode revelar-se um veículo para a criação de registos mais homogéneos.

Ultrasound detects subclinical joint inflammation in the hands and wrists of patients with systemic lupus erythematosus without musculoskeletal symptoms.

OBJECTIVES: To assess the prevalence and severity of ultrasonographic abnormalities of the hand and wrist of asymptomatic patients with systemic lupus erythematosus (SLE) and compare these findings with those from patients with SLE with musculoskeletal signs or symptoms and healthy controls. METHODS: We conducted a prospective cross-sectional study that evaluated bilaterally, with grey-scale and power Doppler (PD) ultrasound (US), the dorsal hand (2nd to 5th metacarpophalangeal and 2nd to 5th proximal interphalangeal joints) and wrist (radiocarpal, ulnocarpal and intercarpal joints) of 30 asymptomatic patients with SLE, 6 symptomatic patients with SLE and 10 controls. Synovial hypertrophy (SH) and intra-articular PD signal were scored using semiquantitative grading scales (0-3). Individual scores were graded as normal (SH≤1 and PD=0) or abnormal (SH≥2 or PD≥1). Global indexes for SH and PD were also calculated. US findings were correlated with clinical and laboratory data and disease activity indexes. RESULTS: US detected SH (score ≥1) in 77% asymptomatic patients with SLE, mostly graded as minimal (score 1: 63%). 23% of the asymptomatic patients with SLE showed abnormal US PD findings (SH≥2 or PD≥1). SH was present in all symptomatic patients with SLE, mostly graded as moderate (grade 2: 67%), and with associated PD signal (83%). SH (score 1) was identified in 50% of controls, however, none presented abnormal US PD findings. SH index in the asymptomatic SLE group was higher than in the control group (2.0 (0-5) vs 0.5 (0-2), median (range), p=0.01) and lower than in the symptomatic SLE group (7.0 (4-23), median (range), p<0.001). No significant correlation was demonstrated between US PD findings and clinical or laboratory variables and disease activity indexes. CONCLUSION: A small subgroup of asymptomatic patients with SLE may present subclinical joint inflammation. Global US scores and PD signal may be important in disease evaluation and therapeutic monitoring.

Congenital Thoracic Venous Anomalies in Adults: Morphologic MR Imaging.

Congenital anomalies of the thoracic veins are rare yet important developmental abnormalities, usually classified into systemic and pulmonary. They may be encountered incidentally; as such the radiologist must be aware of their imaging presentation and clinical relevance. Furthermore, to understand these anomalies, knowledge of the embryological development and of the normal anatomy of the thoracic veins is required. In the age of non-invasive imaging modalities, magnetic resonance is paramount for the characterization of these developmental abnormalities.

Thoracic manifestations of connective tissue diseases.

Connective tissue diseases (CTDs) comprise several immunologic systemic disorders, each of which associated with a particular set of clinical manifestations and autoimmune profile. CTDs may cause numerous thoracic abnormalities, which vary in frequency and pattern according to the underlying disorder. The CTDs that most commonly involve the respiratory system are progressive systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, polymyositis, dermatomyositis, and mixed connective tissue disease. Pulmonary abnormalities in this group of patients may result from CTD-related lung disease or treatment complications, namely drug toxicity and opportunistic infections. The most important thoracic manifestations of CTDs are interstitial lung disease and pulmonary arterial hypertension, with nonspecific interstitial pneumonia being the most common pattern of interstitial lung disease. High-resolution computed tomography is a valuable tool in the initial evaluation and follow-up of patients with CTDs. As such, general knowledge of the most common high-resolution computed tomographic features of CTD-related lung disease allows the radiologist to contribute to better patient management.