Job's syndrome, or hyperimmunoglobulinemia E, is a rare immunodeficiency condition with no established etiology, characterized by recurrent lower and upper respiratory tract and skin infections associated with high levels of immunoglobulin E, eosinophilia, and a characteristic facial alteration. The authors report on a 22-year-old man who carried this disease complicated by pleural empyema and lung cysts, showing a good evolution.

Keywords: Job's syndrome. Respiratory tract infections. Recurrence.

Sporadic cases of patients with neuromyelitis (Devic's syndrome) in association with tuberculosis restricted to the lungs have come out in recent years. The case of a patient with sudden visual loss and a further diagnosis of optic neuritis and pulmonary tuberculosis is reported. Based on the description of this case, the possible relationship between these two pathologies and their implications are discussed.

Keywords: Tuberculosis. Optical neuritis. Devic's syndrome.

The hyperlucent lung syndrome, first described by Swyer and James, is considered a post-infectious complication related to bronchiolitis in infancy. The authors report a case of Swyer-James-MacLeod syndrome which was diagnosed in an adult male, and make a brief review of pertinent literature.

Keywords: Swyer-James-MacLeod. Syndrome. Bronchiolitis. Lung disease.

Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. The diagnosis can usually be made by measuring the tracheal diameter. We report the case of a 40-year-old black man with refractory lower respiratory tract infection. Tracheobronchomegaly was confirmed through computed tomography.

Keywords: Tracheobronchomegaly/diagnosis; Tomography, X-ray computed; Tracheobronchomegaly/rehabilitation;

Lung cancer is now the main cause of blood flow obstruction in the superior vena cava and of its clinical manifestations. History tells that in the 18th Century, syphilis and tuberculosis were responsible for 40% of the known cases. The knowledge of hemodynamic changes seen in this syndrome and the improvement of diagnostic and cytopathologic techniques allow for a safer and more comfortable treatment of the patient than 10 years ago. Contrast CT and MR added to the identification and location of the obstruction, and older techniques such as cavography could be abandoned. SVC obstruction diagnosis and Doppler studies carried out at the bed of the patient contribute to deobstruction procedures such as transluminal percutaneous angioplasty in cases of thrombosis and venous stenosis. In addition, the use of prostheses is of great importance in the management of cases utilization of traumatic lesion to the SVC during lung or mediastinal cancer surgeries. In the realm of radiotherapy, the fractionating technique allows for the administration of high doses of irradiation to cancer patients with benefits to 70% of the cases on the average.

We report the case of a 25-year-old woman with Churg-Strauss syndrome, the symptoms of which had first appeared soon after she began taking oral contraceptive at the age of sixteen. The clinical profile evolved rapidly to severe persistent asthma, nasal polyposis, perennial obstructive rhinitis, eosinophilia (peripheral/tissue) and mononeuritis. Churg-Strauss syndrome is the type of disease that demands early detection, accurate diagnosis, aggressive treatment and periodic monitoring. It should be considered in the differential diagnosis of moderate and severe persistent asthma. The case reported calls attention to possibility that there is a hormonal component and that the disease can present early onset.

Keywords: Asthma; Churg-Strauss syndrome; Eosinophilia; Nasal polyps; Vasculitis; Case reports [publication type]

Yellow nail syndrome is a rare disorder characterized by three main features: discoloration of the nails, together with dystrophic alterations; lymphedema; and pleural effusion. It is often accompanied by bronchiectasis and chronic rhinosinusitis. Herein, we report a case of the complete syndrome with bilateral pleural effusion in a patient under treatment for pulmonary tuberculosis for nine months. There was a family history of two similar cases in siblings.

Keywords: Nail diseases; Lymphedema; Pleural effusion; Antitubercular; Syndrome; Case report

A rare case of Pancoast's syndrome caused by non-Hodgkin's lymphoma is reported. The diagnosis was made on the grounds of examination of the tissue obtained by pulmonary needle biopsy. The necessity of accurate histologic diagnosis and immunohistochemical study is emphasized.

Keywords: lymphoma; Pancoast's syndrome; lung neoplasms

Hantavirus pulmonary and cardiovascular syndrome is a recently identified and often fatal disease, which presents as acute respiratory distress syndrome (ARDS). Since the first outbreak, in Nov/Dec 1993, in Juquitiba, Brazil, 226 cases have been registered by FUNASA (National Health Foundation).(4) The disease occurs in previously healthy subjects, presenting with fever and symptoms similar to the common cold, and may rapidly evolve to pulmonary edema, respiratory failure and shock. Hemoconcentration and thrombocytopenia are common features, and the typical radiological finding is a bilateral diffuse interstitial infiltrate that evolves to alveolar consolidations in parallel to the worsening of the clinical condition. Initially, mortality was around 75%, but it declined to approximately 35% in the last few years. Patients who survive usually recover completely, about a week after the onset of the respiratory symptoms. The causal agent is a previously unrecognized hantavirus whose natural reservoirs are rodents of the family Muridae, sub-family Sigmodontinae. Specific antiviral treatment for hantavirus pulmonary and cardiovascular syndrome has not yet been well established, and the efficacy of ribavirin is currently being studied. Intensive care, including mechanical ventilation and invasive hemodynamic monitoring, is required for the more severe presentations of the disease. These measures may improve the prognosis and survival of patients with hantavirus pulmonary and cardiovascular syndrome if started early in the course of the disease.

Keywords: Hantavirus pulmonary syndrome/diagnoses. Hantavirus infections/diagnoses. Brazil.

1. Pulmonary Function Laboratory and Respiratory Investigation Unit, Division of Respirology, Kingston Health Science Center & Queen’s University, Kingston (ON) Canada. 2. Unidade de Fisiologia Pulmonar, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre (RS) Brasil.

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.

The greatest causes of morbidity and mortality in patients infected with human immunodeficiency virus are opportunistic infections and neoplastic diseases. Most of them are lung diseases. Its correct diagnosis and proper treatment improve the quality of life as well as the survival in this group of patients.

Keywords: lung disease; acquired immunodeficiency syndrome; bronchoscopy

Different kinds of pulmonary impairment have been described in Sjögren syndrome, including rare cases of bronchiectasis. The authors report a female patient with a history of episodes of respiratory infections and progressive breathlessness whose high resolution computerized tomography revealed bronchiectasis. A former open lung biopsy showed bronchiolar inflammatory and fibrotic changes. The diagnosis of Sjögren syndrome was made only late in the evolution, although sicca syndrome symptoms had been present for years. The authors discuss the potential pathogenic mechanisms involved in the development of the bronchiectasis and the need for a high degree of clinical medical skill for the early diagnosis of such conditions.

Keywords: Sjögren syndrome; bronchiectasis; bronchiolitis

Postemetic rupture of the esophagus, also known as spontaneous rupture or Boerhaave's syndrome, was first described by Herman Boerhaave in 1724. This is a severe disease that causes high mortality rates and is difficult to diagnose not only because it is rare but also because it is frequently confused with other severe clinical conditions, such as acute myocardial infarction, perforated peptic ulcer, and acute pancreatitis. Herein, we describe three cases of patients with this syndrome. Two underwent primary repair of the rupture, and one underwent esophagectomy followed by reconstruction. There was one death due to septic shock in the immediate postoperative period. The other two patients presented favorable long-term evolution.

Keywords: Esophageal diseases; Rupture, spontaneous; Mediastinitis; Esophagectomy.