Macrophage activation syndrome is a rare and potentially life-threatening disease. It occurs due to immune dysregulation manifested as excessive macrophage proliferation, typically causing hepatosplenomegaly, pancytopenia and hepatic dysfunction. Here, we report an unusual case of macrophage activation syndrome presenting as dyspnea, as well as (reported here for the first time) high resolution computed tomography findings of an excavated nodule, diffuse ground glass opacities and consolidations (mimicking severe pneumonia or alveolar hemorrhage). The patient was successfully treated with human immunoglobulin. We recommend that macrophage activation syndrome be considered in the differential diagnosis of respiratory failure. Rapid diagnosis and treatment are essential to achieving favorable outcomes in patients with this syndrome.

Keywords: Macrophage activation; Lymphohistiocytosis, hemophagocytic; Tomography, X-ray computed; Lung diseases, interstitial.

Abstract Objective: To present the findings of computed tomography (CT) scans of the chest in patients with laryngotracheobronchial papillomatosis. Methods: We retrospectively analyzed CT scans of eight patients, five males and three females, ranging from 5 to 18 years of age with a mean age of 10.5 years. Images were independently reviewed by two radiologists. In discrepant cases, a consensus was reached. Results: The most common CT findings were intratracheal polypoid lesions and pulmonary nodules, many of which were cavitated. Conclusions: In patients with laryngotracheobronchial papillomatosis, the most common tomographic finding was the combination of intratracheal polypoid lesions and multiple pulmonary nodules, many of which were cavitated.

Keywords: Tomography, X-ray computed; Papilloma; Tracheal neoplasms.

Objective: To present findings on computed tomography scans of the chest indicative of extramedullary hematopoiesis in six patients. Methods: We retrospectively analyzed computed tomography scans of six adult patients-five males and one female-with a mean age of 36.5 years. Two radiologists independently reviewed the scans, and a consensus was reached in discrepant cases. Results: The most common finding in the scans was lower paravertebral masses with heterogeneous content (four patients). The scans of two patients showed a solitary parietal and pleural mass. Conclusions: There are findings in computed tomography scans that are highly suggestive of extramedullary hematopoiesis, especially when those findings correlate with underlying blood diseases. Such findings, in most of the cases, allow physicians to dispense with histopathological confirmation.

Keywords: Tomography, X-ray computed; Hematopoiesis, extramedullary; Mediastinum/physiopathology; Anemia, sickle cell.

Objective: To evaluate, using computerized tomography, the frequency of paranasal sinus involvement in patients with allergic rhinitis. Methods: From among outpatients diagnosed with rhinitis and complaining of nasal obstruction, 60 were selected for evaluation. The patients were submitted to anterior rhinoscopy, skin prick test for reactivity to aeroallergens and computed tomography of the paranasal sinuses. In addition, questionnaires designed to evaluate symptom severity were administered. The Lund score was used to evaluate paranasal sinus involvement on computed tomography scans. Results: Computed tomography scans of the paranasal sinuses were abnormal in 31 patients (52%). The sum of the largest diameters of cutaneous reactions to the aeroallergens, symptom severity and anterior rhinoscopy findings did not differ between patients with paranasal sinus involvement and those without. All the patients with paranasal sinus abnormalities also presented osteomeatal complex abnormalities, whereas only 11 patients (38%) without paranasal sinus involvement presented such abnormalities (p < 0.01). Conclusion: In a sample of patients with allergic rhinitis, the frequency of paranasal sinus abnormalities on computed tomography scans was elevated and did not correlate with symptom severity or skin prick test reactivity but was correlated with osteomeatal complex obstruction.

Keywords: Rhinitis; Sinusitis; Tomography, X-ray computed; Paranasal sinuses/radiography

Here, we report high-resolution computed tomography (HRCT) findings in a patient who developed sudden hemoptysis, dyspnea and chest pain after smoking crack cocaine. Chest X-rays showed consolidations, primarily in the upper lobes, and HRCT scans showed ground glass attenuation opacities, consolidations and air-space nodules. A follow-up CT, after drug use discontinuation and administration of corticosteroids, showed partial resolution of pulmonary lesions and the appearance of cavitations. Clinical, imaging and laboratory findings led to a diagnosis of 'crack lung'.

Keywords: Crack cocaine/adverse effects; Cocaine-related disorders; Tomography, X-ray computed; Street drugs/adverse effects;

Emphysema is a condition of the lung, characterized by the abnormal increase in the size of the airspace distal to the terminal bronchioles. Currently, emphysema is the fourth leading cause of death in the USA, affecting 14 million people. The present article describes the principal tools in the imaging diagnosis of emphysema, from the early days until the present. We describe traditional techniques, such as chest X-ray, together with the evolution of computed tomography (CT) to more advanced forms, such as high resolution CT, as well as three-dimensional CT densitometry and volumetric assessment.

Keywords: Emphysema; Radiology; Tomography, X-Ray computed; Radiography, thoracic.

Objective: Obstructive sleep apnea syndrome (OSAS) has a high prevalence and carries significant cardiovascular risks. It is important to study new therapeutic approaches to this disease. Positional therapy might be beneficial in reducing the apnea-hypopnea index (AHI). Imaging methods have been employed in order to facilitate the evaluation of the airways of OSAS patients and can be used in order to determine the effectiveness of certain treatments. This study was aimed at determining the influence that upper airway volume, as measured by cervical CT, has in patients diagnosed with OSAS. Methods: This was a quantitative, observational, cross-sectional study. We evaluated 10 patients who had been diagnosed with OSAS by polysomnography and on the basis of the clinical evaluation. All of the patients underwent conventional cervical CT in the supine position. Scans were obtained with the head of the patient in two positions (neutral and at a 44° upward inclination), and the upper airway volume was compared between the two. Results: The mean age, BMI, and neck circumference were 48.9 ± 14.4 years, 30.5 ± 3.5 kg/m2, and 40.3 ± 3.4 cm, respectively. The mean AHI was 13.7 ± 10.6 events/h (range, 6.0-41.6 events/h). The OSAS was classified as mild, moderate, and severe in 70%, 20%, and 10% of the patients, respectively. The mean upper airway volume was 7.9 cm3 greater when the head was at a 44° upward inclination than when it was in the neutral position, and that difference (17.5 ± 11.0%) was statistically significant (p = 0.002). Conclusions: Elevating the head appears to result in a significant increase in the caliber of the upper airways in OSAS patients.

Keywords: Sleep apnea, obstructive/prevention and control; Sleep apnea, obstructive/therapy; Tomography.

Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.

Keywords: Lymphohistiocytosis, hemophagocytic; Ferritins; Sarcoidosis, pulmonary; Macrophage activation syndrome.

Chronic dyspnea has a wide range of causes. The aim of the authors was to evaluate a diagnostic approach applied to patients with unexplained chronic (> 30 days) dyspnea, or when multiple causes were present. Methods: An algorithm for the work-up was applied: 1) complete history and physical examination; 2) bronchoprovocation test (BPT) and serial measurements of echocardiography; 3) cardiopulmonary exercise testing (CPET) and doppler echocardiography; 4) special tests: FRCT, V/Q scan, DCO, lung volumes, MIP, and others, as indicated. Results: Ninety patients were included, mean age of 51 ± 16 yr. Hyperventilation symptoms were present in 48, but associated with BHR or asthma in 19. Obstructive defect was present in 24 (asthma, 11; COPD, 8). Reduced FVC with normal FEV1/FVC was found in 10; explanations were asthma in 4, heart disease in 4. BPT was performed in 71 and was verified to be normal in 20; PEF variation was evaluated in 71, and was abnormal in 28, isolated in 16, and in 7 with asthma as final diagnosis. Echocardiography was performed in 44, and showed diastolic dysfunction in 11 out of 16 patients with final diagnosis of heart disease. CPET showed cardiovascular limitation in 19, hyperventilation in 19, and ventilatory defect in 12. CPET was decisive in 33 (38%), specially for heart diseases and to exclude causes suspected by clinical data. Respiratory disease was the explanation for dyspnea in 51 (59%): asthma (31), BHR (8), COPD (8), interstitial lung disease (4); other causes found were: heart disease (16), primary hyperventilation syndrome (8), obesity (5), neuromuscular disease (6), psychogenic dyspnea (2), and others (7). Multiple causes were found in 13 patients. Eight patients had non-recognizable etiology. Conclusion: An algorithm approach to chronic dyspnea resulted in diagnosis of 91% of the cases; spirometry, tests for BHR including PEF measurements, echocardiography, and CPET are essential for the investigation.

Keywords: Chronic dyspnea. Exercise test. Spirometry. Respiratory function tests. Bronchoprovocation tests. Peak expiratory flow. Echocardiography. Chest X-ray. Heart failure, congestive.

Pulmonary capillary hemangiomatosis is a rare disorder characterized by a proliferation of capillaries that invade the pulmonary interstitium and alveolar septae. Herein, we report the first Brazilian case of pulmonary capillary hemangiomatosis. A 21-year-old man presented with severe pulmonary hypertension that eventually resulted in his death. Upon admission, a computed tomography scan of the chest revealed diffuse ill-defined bilateral pulmonary nodules. A postmortem lung biopsy revealed pronounced multifocal proliferation of capillaries in the alveolar walls, interlobular septa and peribronchial connective tissue. A diagnosis of pulmonary capillary hemangiomatosis should be considered in patients presenting pulmonary hypertension and suspicious changes on high-resolution computed tomography scans.

Keywords: Hemangioma, capillary/etiology; Hemangioma capillary/diagnosis; Hypertension, pulmonary/complications; Tomography, Emission-Computed; Case report

The aim of this pictorial essay was to present the main computed tomography findings seen in diffuse diseases of the trachea. The diseases studied included amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, lymphoma, neurofibromatosis, relapsing polychondritis, Wegener's granulomatosis, tuberculosis, paracoccidioidomycosis, and tracheobronchomalacia. The most common computed tomography finding was thickening of the walls of the trachea, with or without nodules, parietal calcifications, or involvement of the posterior wall. Although computed tomography allows the detection and characterization of diseases of the central airways, and the correlation with clinical data reduces the diagnostic possibilities, bronchoscopy with biopsy remains the most useful procedure for the diagnosis of diffuse lesions of the trachea.

Keywords: Thoracic diseases; Tracheal diseases; Tomography, X-ray computed; Trachea.

Madelung's disease, or multiple symmetric lipomatosis, is a rare disease, characterized by accumulation of unencapsulated fat, generally located symmetrically around the neck and shoulders. Here, we present the case of a patient with diffuse lipomatosis accompanied by obstructive sleep apnea due to cervical involvement and facial deformity, which made it necessary to use nasal pillows for ventilation. The patient was hospitalized with a diagnosis of pneumonia and required noninvasive ventilation due to severe hypercapnia. A brief review of the literature was made, and we describe and discuss the investigation of this rare clinical case.

Keywords: Lipomatosis, multiple symmetrical; Sleep apnea, obstructive; Continuous positive airway pressure; Masks.