Impact of Azathioprine use in chronic hypersensitivity pneumonitis patients.

INTRODUCTION: Systemic corticosteroids are widely used in chronic hypersensitivity pneumonitis (CHP); however, there is not much evidence to support their use, besides being associated with significant side effects. Azathioprine (AZA) use is common in CHP, although not prospectively tested in randomized controlled trials. Our objective was to evaluate the lung function trajectory of CHP patients after AZA initiation, as well as to assess the safety profile of this drug. METHODS: Retrospective analysis of patients initiated on AZA following a multidisciplinary team diagnosis of CHP. The longitudinal trajectory of lung function in the first 2 years of treatment was assessed. RESULTS: Thirty-five out of 62 patients (56.5%) remained on treatment after 2 years. AZA treatment was associated with a significant improvement in forced vital capacity (FVC) at 12 and 24 months (p = 0.015 and p < 0.001, respectively). A slight increase in total lung capacity (TLC) and 6-min walking test (6MWT) were also reported, although it did not reach statistical differences at the end of 2 years. No changes in diffusion capacity for carbon monoxide (DLCO) were observed. CONCLUSIONS: This is the first study identifying an improvement in lung function (FVC) of CHP patients on AZA treatment for 2 years. Prospective studies are needed to confirm these results and to more adequately select CHP patients who may benefit from AZA.

Peripheral inflammatory pseudotumor of the liver and extensive thrombosis of the portal venous system in a child.

We report a case of a peripheral inflammatory pseudotumor of the right lobe of the liver with extensive thrombosis of the portal venous system in a 9-year-old boy. Local thrombosis of the portal vein is a known complication of the inflammatory pseudotumor, especially in the hepatic hilum. The extent of the thrombosis in this case was unexpected, considering the peripheral location and the benign nature of the lesion. To our knowledge, thrombosis of this degree has not been described.

[The Impact of the COVID-19 Pandemic on the Intensive Care Residency Program in Portugal]. / Impacto da Pandemia COVID-19 na Formação Especializada do Internato Médico de Medicina Intensiva em Portugal.

INTRODUCTION: In 2020, critical care departments underwent profound changes imposed by the COVID-19 pandemic. The aim of this study was to evaluate the impact of the pandemic on the intensive care residency program in Portugal. MATERIAL AND METHODS: The Association of Critical Care Residents (AIMINT) prepared a questionnaire using the Google Forms® tool, which was applied during August 2020 to the Critical Care residents in Portugal. A descriptive analysis was performed with the data collected. RESULTS: Eighty-five residents participated in the questionnaire, yieldinga response rate of 62%. Three-quarters of all participants provided care to COVID-19 patients. More than 80% of the surveyed participants were on rotations, and these were canceled in 59% of cases. Seventy-eight percent reported a workload greater than 40 hours per week. CONCLUSION: The COVID-19 pandemic had an impact on the Critical Care Residency program in Portugal. Most residents surveyed provided care to COVID-19 patients and not only saw their rotations suspended but also experienced difficulties in rescheduling them.

Introdução: No ano de 2020, os serviços de medicina intensiva sofreram profundas adaptações e reestruturações impostas pela pandemia de COVID-19. Este estudo teve como objetivo avaliar o impacto desta pandemia na formação especializada do internato médico de medicina intensiva em Portugal.Material e Métodos: A Associação de Internos de Medicina Intensiva elaborou um questionário usando a ferramenta Google Forms®, e que foi aplicado durante o mês de agosto de 2020 aos internos de formação especializada de medicina intensiva, em Portugal. Com base na informação recolhida realizou-se uma análise descritiva.Resultados: Oitenta e cinco médicos internos responderam ao questionário, perfazendo uma taxa de resposta de 62%. Três quartos dos participantes no estudo contactaram com doentes com COVID-19. Oitenta e seis por cento dos médicos internos inquiridos encontravam-se em estágios, tendo os mesmos sido cancelados em 59% dos casos. Setenta e oito por cento referiram uma carga assistencial superior a 40 horas semanais.Conclusão: A pandemia de COVID-19 teve impacto na formação especializada do internato médico de medicina intensiva em Portugal. A maioria dos internos inquiridos contactaram com doentes com COVID-19, com suspensão dos seus estágios e com prejuízo na remarcação dos mesmos.

Non-COVID-19 respiratory viral infection.

Implemented control measures brought about by the coronavirus disease 2019 (COVID-19) pandemic have changed the prevalence of other respiratory viruses, often relegating them to a secondary plan. However, it must not be forgotten that a diverse group of viruses, including other human coronaviruses, rhinoviruses, respiratory syncytial virus, human metapneumoviruses, parainfluenza and influenza, continue to be responsible for a large burden of disease. In fact, they are among the most common causes of acute upper and lower respiratory tract infections globally. Viral respiratory infections can be categorised in several ways, including by clinical syndrome or aetiological agent. We describe their clinical spectrum. Distinctive imaging features, advances in microbiological diagnosis and treatment of severe forms are also discussed. Educational aims: To summarise the knowledge on the spectrum of disease that respiratory viral infections can cause and recognise how often they overlap.To learn the most common causes of respiratory viral infections and acknowledge other less frequent agents that may target certain key populations (e.g. immunocompromised patients).To improve awareness of the recent advances in diagnostic methods, including molecular assays and helpful features in imaging techniques.To identify supportive care strategies pivotal in the management of severe respiratory viral infections.

Mortality risk prediction with ILD-GAP index in a fibrotic hypersensitivity pneumonitis cohort.

BACKGROUND: Fibrotic hypersensitivity pneumonitis (fHP) is associated with significant morbidity and mortality. Interstitial lung disease-gender-age-physiology (ILD-GAP) performance in fHP outside the initial cohort was never performed. AIM: To assess the ILD-GAP index's ability to predict mortality in a Portuguese cohort of patients with fHP and analyse whether other clinical variables add value. METHODS: Retrospective analysis of fHP cohort in two Portuguese ILD centres. The baseline ILD-GAP index was calculated. Survival was analysed in months; mortality was the primary outcome. Univariate and multivariate analyses to identify mortality risk factors were performed. RESULTS: A total of 141 patients were included. Fifty-three patients (37.6%) died during the follow-up. The usual interstitial pneumonia (UIP) pattern was found in 49.6%, and their survival was inferior to non-UIP [32 months (interquartile range, IQR = 19, 60) versus 52 months (IQR = 28, 98), p = 0.048]. Patients with an ILD-GAP index higher than three double their risk of mortality [hazard ratio (HR) = 6.48, 95% confidence interval (CI) = (3.03-13.96)] when compared with the patients with an index between 2 and 3 [HR = 3.04, 95% CI = (1.62-5.71)] adjusting for acute exacerbation history. Even though UIP patients had worse survival, it did not reach statistical significance when UIP pattern was added to this model. Acute exacerbation history was an independent risk factor for mortality; however, ILD-GAP still predicted mortality after adjusting for this factor. PaO2 and 6-minute walk test desaturation were not significant risk factors. CONCLUSION: ILD-GAP index is a good predictor for mortality in fHP, even after adjusting for other mortality risk factors.

Kikuchi-Fujimoto Disease: Diagnosis in a Relapsing Case.

The differential diagnosis of cervical lymphadenopathy is varied. Different age groups require different approaches. Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare but important diagnosis to consider after excluding more common aetiologies. We present the case of a 21-year-old female with painful left cervical swelling, lasting over a week. Physical examination revealed multiple cervical lymphadenopathies, elastic, non-adherent to deep tissues which were tender to touch. Blood tests showed elevated acute phase proteins. Cytomegalovirus, Epstein-Barr, toxoplasmosis, and human immunodeficiency virus serologies were negative. Computed tomography of the neck revealed multiple cervical lymphadenopathies which were round-shaped, some with necrosis and with extracapsular extent. These features could be compatible with tuberculous lymphadenitis. However, interferon-gamma release assay was negative. Excisional biopsy was scheduled, but spontaneous regression did not allow it. Two weeks later she relapsed. Excisional biopsy revealed histiocytic necrotizing lymphadenitis. Kikuchi-Fujimoto's diagnosis demands high clinical suspicion and histological documentation. This case represents a rare diagnosis of a relapsing disease.

Insights on chronic hypersensitivity pneumonitis' treatment: Factors associated with a favourable response to azathioprine.

BACKGROUND: The use of immunosuppressive and antifibrotic agents for the treatment of chronic hypersensitivity pneumonitis (CHP) appears promising, but there is still no evidence supporting the clinical decision regarding the implementation of each specific pharmacological strategy. METHODS: Patients diagnosed with CHP and treated with azathioprine (AZA) were retrospectively selected from a single centre for Interstitial Lung Diseases. Baseline clinical data, as well as functional, imaging, bronchoalveolar lavage (BAL) and histology features were assessed. Longitudinal data on functional parameters were collected and comparatively analysed with patients' characteristics. RESULTS: In this cohort of 80 patients, of those who reached 12 months of treatment, 78.3% presented a preserved forced vital capacity, with 59 being eligible to be classified as AZA responders (n = 36) or non-responders (n = 23). BAL lymphocytosis was associated with a favourable response to AZA treatment (OR 1.051; 95% CI 1.015-1.089), although it didn't identify all responders. CONCLUSIONS: AZA revealed to be effective in disease stabilisation in most patients, while ineffective for a subset. BAL lymphocytosis appears as a potentially valuable strategy to identify AZA responders, although with limited accuracy. Further studies are needed to clarify other response markers to immunosuppressive agents, in order to optimize the therapeutic options for this condition.

Currarino triad: A case report of a 48-year-old patient with a neuroendocrine tumor.

Currarino triad is a rare syndrome, with less than 250 cases reported, and it includes a combination of sacrococcigeal bony abnormalities, anorectal malformations and the presence of a presacral mass. Here we present a case of a 48-year-old male patient with history of severe chronic constipation, who was incidentally diagnosed with a presacral during imagiological investigation, which was histologically proven to be a neurendocrine tumor.

Pulmonary Sarcoidosis: Prognostic Factors at Diagnosis in Patients from North of Portugal.

BACKGROUND: Sarcoidosis is a multisystemic granulomatous disease that affects the lungs in more than 90% of the patients. It is associated with a variable clinical course and considering all the different forms of disease presentation, there are an absence of reliable clinical prognostic markers that can predict the outcome at diagnosis. OBJECTIVE: The aim of our study was to investigate prognostic factors at diagnosis in a population of sarcoidosis patients from Northern Portugal. METHODS: A group of 110 patients with chronic evolution was compared with 129 patients with disease resolution regarding their clinical, radiologic and laboratorial features. RESULTS: We found a positive association between the chronic forms and lung function impairment, radiologic stage II, lower lymphocyte CD4/CD8 and extrapulmonary disease. Löfgren syndrome and asthenia instead had a protective significant association to chronicity. Our final logistic regression model found a significant independent association between age (adjusted OR=1.06), extrapulmonary involvement (adjusted OR=2.68), Löfgren's syndrome (adjusted OR=0.15) with outcome toward chronicity. CONCLUSIONS: In this first study searching for prognostic factors at diagnosis in a Northern Portuguese population, we found clinical prognosis factors that have been described in other populations that should be considered whenever sarcoidosis is identified.

Epiploic appendagitis: an entity frequently unknown to clinicians--diagnostic imaging, pitfalls, and look-alikes.

OBJECTIVE: Epiploic appendagitis is an ischemic infarction of an epiploic appendage caused by torsion or spontaneous thrombosis of the epiploic appendage central draining vein. When it occurs on the right side of the abdomen, it can mimic appendicitis and right-sided diverticulitis; whereas when it occurs on the left side of the abdomen, it is often mistaken for sigmoid diverticulitis. The purpose of this article is to review the diagnostic imaging of this entity. CONCLUSION: Epiploic appendagitis is self-limited and spontaneously resolves without surgery within 5-7 days. Therefore, it is imperative for radiologists to be familiar with this entity.

T2-Weighted endorectal magnetic resonance imaging of prostate cancer after external beam radiation therapy.

PURPOSE: To retrospectively determine the accuracy of T2-weighted endorectal MR imaging in the detection of prostate cancer after external beam radiation therapy and to investigate the relationship between imaging accuracy and time since therapy. MATERIALS AND METHODS: Institutional review board approval was obtained and the study was HIPPA compliant. We identified 59 patients who underwent 1.5 Tesla endorectal MR imaging of the prostate between 1999 and 2006 after definitive external beam radiation therapy for biopsy-proven prostate cancer. Two readers recorded the presence or absence of tumor on T2-weighted images. Logistic regression and Fisher's exact tests for 2x2 tables were used to determine the accuracy of imaging and investigate if accuracy differed between those imaged within 3 years of therapy (n = 25) and those imaged more than 3 years after therapy (n = 34). Transrectal biopsy was used as the standard of reference for the presence or absence of recurrent cancer. RESULTS: Thirty-four of 59 patients (58%) had recurrent prostate cancer detected on biopsy. The overall accuracy of T2-weighted MR imaging in the detection cancer after external beam radiation therapy was 63% (37/59) for reader 1 and 71% for reader 2 (42/59). For both readers, logistic regression showed no difference in accuracy between those imaged within 3 years of therapy and those imaged more than 3 years after therapy (p = 0.86 for reader 1 and 0.44 for reader 2). CONCLUSION: T2-weighted endorectal MR imaging has low accuracy in the detection of prostate cancer after external beam radiation therapy, irrespective of the time since therapy.

Evaluation of Small-Bowel Patency in Crohn's Disease: Prospective Study with a Patency Capsule and Computed Tomography.

BACKGROUND AND PURPOSE: Patency capsule (PC) examination is usually performed - previously to capsule endoscopy - to evaluate small-bowel patency in patients with established Crohn's disease (CD). The reported PC retention rate is significantly higher than expected. Our aims were to assess small-bowel patency, to determine the precise location of the retained PC in patients with CD, and to determine the false positive rate of evaluation with a radiofrequency identification tag (RFIT) scanner. METHODS: This is a prospective single-center study including CD patients with clinical indication for small-bowel capsule endoscopy. PillCam® PC examination was performed on all patients to assess small-bowel patency. On all patients with a positive identification of the PC using an RFIT scanner, 30 h after ingestion, an abdominal CT was performed in order to identify its precise location. RESULTS: Fifty-four patients were included. The PC retention rate, according to evaluation with the RFIT scanner, was 20% (in 11 patients) 30 h after ingestion. These patients were then submitted to abdominal CT, which revealed that there was small-bowel retention in 5 cases (9%). Higher CRP levels, penetrating disease, and a history of abdominal surgery were associated with an increased risk of PC retention (p = 0.007, p = 0.011, and p = 0.033, respectively). On multivariate analysis, there was an independent association between small-bowel PC retention and CRP levels >5 mg/dL (OR = 15.5; p = 0.03). DISCUSSION: The small-bowel PC retention rate (9%) was considerably lower than those found in previous reports. Our results show that, with this protocol, the false-positive cases of RFIT scans or plain abdominal X-rays may be avoided. This may contribute to more extensive application of capsule endoscopy without the risk of small-bowel retention.

Diffuse smoking-related lung diseases: insights from a radiologic-pathologic correlation.

Cigarettes are well-recognized risk factors responsible for the emergence of a variety of pathologic conditions affecting both the airways and the lungs. Smoking-related lung diseases can be classified as chronic obstructive pulmonary disease (COPD) and several types of interstitial diseases, such as pulmonary Langerhans cell histiocytosis, bronchiolitis, desquamative interstitial pneumonitis, acute eosinophilic pneumonia, and interstitial fibrosing lung diseases. The evidence of combined lower lung fibrosis and predominant upper lung emphysema is renowned as a distinct clinical entity, named combined pulmonary fibrosis and emphysema. Although computerized tomography permits an adequate classification and distinction of these diseases, the clinical, imaging, and histological features often overlap and coexist in a single patient. Therefore, a combined radiologic and pathologic approach, in the appropriate clinical setting, is useful for best comprehension and distinction of these entities. Our goals are to describe the imaging features in smoking-related lung diseases and how the pathological manifestations translate on high-resolution computerized tomography.

Diagnostic yield and safety of transbronchial cryobiopsy in sarcoidosis.

INTRODUCTION: Transbronchial lung cryobiopsy (TBLC) is an endoscopic technique proven to be useful in diagnostic approach to interstitial lung disease (ILD), but its role in sarcoidosis is not fully established. The aim of the present study was to assess the diagnostic yield of TBLC in sarcoidosis and its safety profile. METHODS: Retrospective analysis of patients, evaluated in a tertiary hospital ILD outpatient clinic, who underwent TBLC in the diagnostic work-up. TBLC was performed in accordance with the 2018 expert statement from the Cryobiopsy Working Group. RESULTS: 32 patients were included (mean±sd age 47.7±12.6 years, 59.4% male) and divided into three groups: highly likely sarcoidosis (n=21), possible sarcoidosis (n=6) and unlikely sarcoidosis (n=5). A mean of 2.8±0.8 TBLCs were performed. The definitive diagnosis was established by TBLC in 20 out of 27 patients with suspected sarcoidosis. Two patients were diagnosed with sarcoidosis by other methods performed afterwards. TBLC leaded to other diagnosis as well, such as fungal infection (n=1), hypersensitivity pneumonitis (n=1) and silicosis (n=3), making the diagnostic yield for suspected sarcoidosis of TBLC of 92.6%. TBLC was also able to show compatible histological features in five patients whom sarcoidosis was not previously considered. The complications reported overall were pneumothorax in five (15.6%) patients and moderate bleeding in one (3.1%) case. CONCLUSION: In this cohort, TBLC was a safe, reliable and useful procedure in sarcoidosis diagnosis. These results suggest that TBLC can be used successfully in those cases where a definitive diagnosis could not be reached with the usual and less-invasive diagnostic tools.

Monitoring Crohn's disease activity: endoscopy, fecal markers and computed tomography enterography.

Background: The treatment goal of Crohn's disease (CD) has moved towards achieving mucosal healing, resolution of transmural inflammation, and normalization of biomarkers. The purpose of this study was to evaluate how well computed tomography enterography (CTE) and fecal calprotectin (FC) correlated with endoscopic activity in newly diagnosed patients with CD and after 1 year of therapy. Methods: Consecutive patients with newly diagnosed CD were evaluated by endoscopy, CTE, and FC at diagnosis and 12 months after beginning immunosuppression. Endoscopic severity was assessed using the Simplified Endoscopic Score for Crohn's Disease (SES-CD). Biomarkers, clinical indexes, and FC were recorded on the day of ileocolonoscopy at diagnosis and 1 year after diagnosis. We adapted a CTE score for disease activity based on radiological signs of inflammation (i.e. mural thickness, mural hyperenhancement, mesenteric fat proliferation, mesenteric fat densification, comb sign, presence of strictures, fistulas, abscesses, ascites, and lymphadenopathy). Correlations between endoscopy, CTE, and FC were assessed using Spearman's rank correlation. Results: A total of 29 patients (48% women; median age 30 (24.5-35.5) years) were included in this prospective cohort. CTE findings significantly correlated with endoscopic findings. Endoscopic remission (ER) at 1-year follow up significantly correlated with improvement in mural hyperenhancement (p = 0.004), mesenteric fat densification (p = 0.001), comb sign (p = 0.004), and strictures (p = 0.008) in CTE. None of the CTE findings improved in patients without ER. FC correlated with SES-CD (rs = 0.696, p < 0.001) and with CTE features of inflammation (rs = 0.596, p < 0.001). A cut-off of 100 µg/g predicted ER with 92% sensitivity, 65% specificity, and 83% accuracy (area under curve 0.878, p < 0.001). Conclusions: CTE findings and FC levels correlated with endoscopic activity in CD both at diagnosis and at 1-year follow up. These two noninvasive markers of disease activity may be used as an alternative to endoscopy to monitor disease response to therapy.

Acute Anaemia: A Challenging Diagnosis.

Acute anaemia is characterized by a reduction in the number of red blood cells, haemoglobin levels or haematocrit. By far the most common aetiology is haemorrhage, but in its absence other less frequent causes should be considered. The authors present the case of a 42-year-old man with a diagnosis of glucose-6-phosphate deficiency and progressing gastric signet ring cell carcinoma, who was admitted to the internal medicine department for symptomatic back pain control. During his hospitalization, the patient developed acute anaemia with schistocytes on peripheral blood smear with no concurrent hyperbilirubinaemia or decreased haptoglobin. Bone metastatic disease was documented. The case was revised with the transfusion medicine department and malignancy-associated microangiopathic haemolytic anaemia (Ma-MAHA) was suggested. The patient was transferred to the oncology department and later discharged, dying at home shortly afterwards. LEARNING POINTS: The possibility of multiple aetiologies for anaemia in the same patient should be considered.Pathophysiological mechanisms are important in the differential diagnosis of anaemia.Malignancy-associated microangiopathic haemolytic anaemia can present as a paraneoplastic syndrome.

Bottom Up Ethics - Neuroenhancement in Education and Employment.

Neuroenhancement involves the use of neurotechnologies to improve cognitive, affective or behavioural functioning, where these are not judged to be clinically impaired. Questions about enhancement have become one of the key topics of neuroethics over the past decade. The current study draws on in-depth public engagement activities in ten European countries giving a bottom-up perspective on the ethics and desirability of enhancement. This informed the design of an online contrastive vignette experiment that was administered to representative samples of 1000 respondents in the ten countries and the United States. The experiment investigated how the gender of the protagonist, his or her level of performance, the efficacy of the enhancer and the mode of enhancement affected support for neuroenhancement in both educational and employment contexts. Of these, higher efficacy and lower performance were found to increase willingness to support enhancement. A series of commonly articulated claims about the individual and societal dimensions of neuroenhancement were derived from the public engagement activities. Underlying these claims, multivariate analysis identified two social values. The Societal/Protective highlights counter normative consequences and opposes the use enhancers. The Individual/Proactionary highlights opportunities and supports use. For most respondents these values are not mutually exclusive. This suggests that for many neuroenhancement is viewed simultaneously as a source of both promise and concern.

Cross-sectional imaging of extranodal involvement in abdominopelvic lymphoproliferative malignancies.

Extranodal lymphoproliferative diseases are common, and their prevalence is increasing. Non-Hodgkin lymphomas and Hodgkin disease, in particular, frequently involve extranodal structures in the abdomen and pelvis, including both the solid organs (liver, spleen, kidneys, and pancreas) and the hollow organs of the gastrointestinal tract. Because virtually any abdominopelvic tissue may be involved, many different imaging manifestations are possible, and lymphoproliferative diseases may mimic other disorders. Familiarity with the imaging manifestations that are diagnostically specific for extranodal lymphoproliferative diseases is important because imaging plays an important role in the noninvasive management of disease. However, a definitive diagnosis requires a biopsy (of bone marrow, a lymph node, or a mass), a peripheral blood analysis, and other laboratory tests. In patients with known disease, the goals of imaging are staging, evaluation of response to therapy, and identification of new or recurrent disease or of complications of therapy. In patients without known disease, imaging permits a provisional diagnosis.

Sarcoidosis: An unusual presentation.

A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported.