Respiratory Tract Amyloidosis. State-of-the-Art Review with a Focus on Pulmonary Involvement.

Amyloidosis is a constellation of disease entities characterized by abnormal extracellular deposition and accumulation of protein and protein derivatives, which show apple-green birefringence when stained with Congo red and viewed under polarized light. Amyloid can infiltrate virtually all organ systems and can display multiple and diverse imaging findings. Pathologically, respiratory involvement occurs in 50 % of patients with amyloidosis, and its clinical signs and symptoms vary depending on whether the disease is systemic or localized. The four main patterns of respiratory tract involvement are tracheobronchial, nodular parenchymal, diffuse alveolar septal, and lymphatic. Imaging findings of amyloidosis are nonspecific and vary in each pattern; knowledge about the disease impairment type is thus very important, and amyloidosis should be considered in the differential diagnosis of other very common diseases, such as infectious diseases, neoplasms, and vasculitis. This literature review describes the main clinical and imaging manifestations of amyloidosis, focusing on respiratory tract involvement and differential diagnosis.

High-resolution computed tomographic findings of cocaine-induced pulmonary disease: a state of the art review.

Cocaine is the most commonly used illicit drug among patients presenting at hospital emergency departments and the most frequent cause of drug-related deaths reported by medical examiners. Various respiratory problems temporally associated with cocaine use have been reported. Acute and chronic uses also are responsible for lung complications, such as pulmonary edema, alveolar hemorrhage, pulmonary hypertension, organizing pneumonia, emphysema, barotrauma, infection, cancer, eosinophilic disease, and aspiration pneumonia. Although most imaging findings are nonspecific, they may raise suspicion of a cocaine-related etiology when considered together with patients' profiles and medical histories. This literature review describes cocaine-induced diseases with pulmonary involvement, with an emphasis on high-resolution chest computed tomographic findings and patterns.

Pulmonary alveolar microlithiasis. State-of-the-art review.

Pulmonary alveolar microlithiasis (PAM) is a rare genetic lung disease characterized by calcifications within the alveoli. Mutations in the SLC34A2 gene, which encodes a type IIb sodium-phosphate cotransporter, are responsible for this disease, leading to intra-alveolar accumulation of phosphate that favors the formation of microliths. The hallmark of this disorder is clinical-radiological dissociation, with typical imaging findings that correlate well with specific pathological findings. The long-term prognosis is poor and no treatment has been discovered to date. The aim of this review is to describe the main pathological, clinical, and imaging aspects of PAM, ranging from its genetic basis to treatment.

Thoracic textilomas: CT findings / Textilomas intratorácicos: achados tomográficos

OBJECTIVE: The aim of this study was to analyze chest CT scans of patients with thoracic textiloma. METHODS: This was a retrospective study of 16 patients (11 men and 5 women) with surgically confirmed thoracic textiloma. The chest CT scans of those patients were evaluated by two independent observers, and discordant results were resolved by consensus. RESULTS: The majority (62.5%) of the textilomas were caused by previous heart surgery. The most common symptoms were chest pain (in 68.75%) and cough (in 56.25%). In all cases, the main tomographic finding was a mass with regular contours and borders that were well-defined or partially defined. Half of the textilomas occurred in the right hemithorax and half occurred in the left. The majority (56.25%) were located in the lower third of the lung. The diameter of the mass was ≤ 10 cm in 10 cases (62.5%) and > 10 cm in the remaining 6 cases (37.5%). Most (81.25%) of the textilomas were heterogeneous in density, with signs of calcification, gas, radiopaque marker, or sponge-like material. Peripheral expansion of the mass was observed in 12 (92.3%) of the 13 patients in whom a contrast agent was used. Intraoperatively, pleural involvement was observed in 14 cases (87.5%) and pericardial involvement was observed in 2 (12.5%). CONCLUSIONS: It is important to recognize the main tomographic aspects of thoracic textilomas in order to include this possibility in the differential diagnosis of chest pain and cough in patients with a history of heart or thoracic surgery, thus promoting the early identification and treatment of this postoperative complication. .

OBJETIVO: Analisar retrospectivamente os aspectos encontrados em TCs de tórax com textiloma torácico. MÉTODOS: Estudo retrospectivo de 16 pacientes (11 homens e 5 mulheres) com diagnóstico de textiloma torácico confirmado cirurgicamente. As TCs de tórax foram avaliadas, de modo independente, por dois observadores, e os casos discordantes foram resolvidos por consenso. RESULTADOS: Na maioria dos casos (62,5%), o fator causal foi a cirurgia cardíaca prévia. Os sintomas mais frequentes foram dor torácica (em 68,75%) e tosse (em 56,25%). Em todos os casos, o principal achado tomográfico foi de massa com contornos regulares e limites bem definidos ou parcialmente definidos. A localização dos textilomas ocorreu na mesma proporção no hemitórax direito e esquerdo, mas foi mais comum no terço inferior (em 56,25%). O tamanho das massas foi ≤ 10 cm e > 10 cm, respectivamente, em 10 (62,5%) e em 6 pacientes (37,5%). A maioria dos textilomas apresentou densidade heterogênea (81,25%), observando-se no seu interior calcificações, gás, marcador radiopaco ou material da compressa. A impregnação periférica da lesão foi observada em 12 (92,3%) dos 13 pacientes que receberam o meio de contraste. A cirurgia demonstrou acometimento do espaço pleural e pericárdico, respectivamente, em 14 (87,5%) e em 2 pacientes (12,5%). CONCLUSÕES: É importante reconhecer os principais aspectos tomográficos dos textilomas intratorácicos a fim de incluir essa possibilidade no diagnóstico diferencial em pacientes com dor torácica e tosse e história de cirurgia cardíaca ou torácica, contribuindo assim para o tratamento precoce dessa complicação cirúrgica. .

Applicability of the 12-Item Short-Form Health Survey in patients with progressive systemic sclerosis.

OBJECTIVE: To evaluate the applicability of the 12-Item Short-Form Health Survey (SF-12) as an instrument to measure health-related quality of life in a sample of patients with progressive systemic sclerosis (PSS) through the analysis of its reproducibility and its correlation with functional and clinical parameters. METHODS: A test-retest reproducibility study for the comparative analysis of the intraclass correlation coefficients (ICCs) of the SF-12 and the SF-36. A total of 46 patients diagnosed with PSS were studied, regardless of the presence of respiratory symptoms. RESULTS: The physical component summary 12 (PCS-12) score had an ICC of 0.47 (95%CI: 0.05-0.71; p < 0.02), whereas the mental component summary (MCS-12) score had an ICC of 0.72 (95%CI: 0.49-0.84; p < 0.001). The PCS-36 score had an ICC of 0.88 (95%CI: 0.78-0.93; p < 0.001), and the MCS-36 score also had an ICC of 0.88 (95%CI: 0.78-0.93; p < 0.001). CONCLUSION: The SF-12 is a reliable instrument for measuring health-related quality of life in patients with PSS, since it has been proven to be reproducible. However, this version of the SF-12 should only be used in clinical research settings.

Recomendações para o manejo da tromboembolia pulmonar, 2010 / Recommendations for the management of pulmonary thromboembolism, 2010

A tromboembolia pulmonar constitui, juntamente com a trombose venosa profunda, a condição denominada tromboembolismo venoso. Apesar dos avanços, a morbidade e a mortalidade atribuídas a essa doença ainda são elevadas, pois os pacientes apresentam doenças mais complexas, são submetidos a um maior número de procedimentos invasivos e sobrevivem por mais tempo. Embora existam inúmeras diretrizes internacionais disponíveis, optou-se por redigir estas recomendações para sua aplicação na prática médica nacional, embasadas nas melhores evidências na literatura e na opinião do grupo de consultores. Este documento é apenas uma ferramenta para o atendimento dos pacientes, e, embora possa ser aplicado na maioria das situações, o médico deve adaptar as informações a sua realidade local e ao caso específico. O diagnóstico de tromboembolia pulmonar é realizado através da combinação da probabilidade clínica pré-teste (escores) com o resultado dos exames de imagem, sendo atualmente o método de eleição a angiotomografia computadorizada. É fundamental a estratificação do risco de desfecho desfavorável, sendo a instabilidade hemodinâmica o preditor mais importante. Pacientes de baixo risco devem ser tratados com heparina, comumente as de baixo peso molecular. Pacientes de alto risco requerem vigilância intensiva e uso de trombolíticos em alguns casos. A longo prazo, os pacientes devem receber anticoagulantes por no mínimo três meses, sendo sua manutenção decidida pela presença de fatores de risco para a recorrência e a probabilidade de sangramento. A profilaxia é altamente eficaz e deve ser amplamente utilizada, tanto em pacientes clínicos como cirúrgicos, conforme os grupos de risco. Finalmente, são feitas recomendações relacionadas ao diagnóstico, tratamento e prevenção da tromboembolia pulmonar.

Pulmonary thromboembolism and deep vein thrombosis together constitute a condition designated venous thromboembolism. Despite the advances, the morbidity and the mortality attributed to this condition are still high, because the patients present with more complex diseases, are submitted to a greater number of invasive procedures and survive longer. Although there are various international guidelines available, we decided to write these recommendations for their application in medical practice in Brazil. These recommendations are based on the best evidence in the literature and the opinion of the advisory committee. This document is only a tool for use in the management of patients. Although the recommendations it contains can be applied to most situations, physicians should adapt its content depending on their local context and on a case-by-case basis. Pulmonary thromboembolism is diagnosed by evaluating pre-test clinical probability (scores) together with the results of imaging studies, the current method of choice being CT angiography. Stratification of the risk for an unfavorable outcome is fundamental. Hemodynamic instability is the most important predictor. Low-risk patients should be treated with heparin, commonly low-molecular-weight heparins. High-risk patients require intensive monitoring and, in some cases, thrombolytic therapy. In the long term, patients should receive anticoagulants for at least three months. The decision to prolong this treatment is made based on the presence of risk factors for the recurrence of the condition and the probability of bleeding. Prophylaxis is highly effective and should be widely used in clinical and surgical patients alike, according to their risk group. Finally, we include recommendations regarding the prevention, diagnosis and treatment of pulmonary thromboembolism.