Respiratory Medicine Extra最新文献

Background

Hypocomplementemic urticarial vasculitis (HUV) with pulmonary emphysema is associated with a poor prognosis, despite high-dose anti-inflammatory treatment including steroids.

Objective

A female former marathon runner, age 47 years, was diagnosed with HUV with skin, renal and pulmonary involvement. Pulmonary arterial pressure was increased during exercise, a novel finding in this disease entity. Complement consumption was associated with the presence of an anti-C1q autoantibody (IgG).

Methods

The initial treatment included high-dose steroids and chloroquine. In analogy to other systemic inflammatory lung diseases, increased oxidant levels were postulated and high-dose acetylcysteine (NAC) was initiated. Later on, dapsone as an inhibitor of pathologically increased neutrophil function was added.

Results

Within 3 months, this therapy resulted in a significant increase of the reduced serum complement levels (CH50, APH50, C4A, and C4B), diffusing capacity, decrease of the anti-C1q autoantibody titer, and normalization of the increased granulocyte count in the broncho-alveolar lavage. Additionally, a stepwise improvement of exercise tolerance (6-min walk test, oxygen consumption during exercise) was seen. Dapsone had no further effect on diffusing capacity or (did not affect) complement serum levels, which remained slightly reduced but stable despite the presence of the anti-C1q autoantibody. The effects have remained stable for the following 36 months. The therapy was well tolerated.

Conclusions

Antioxidant therapy with high-dose NAC might be a potential addition to the conventional anti-inflammatory therapy in patients with HUV and pulmonary involvement. It should be considered for investigation in a larger patient population.

A case report of a 26-year-old Caucasian male with a medical history of chronic sinusitis and a “difficult to treat” asthma bronchiale is described. After a period of 6 years intensive medical treatment and diagnostic procedures, a diffuse panbronchiolitis (DPB) was diagnosed, confirmed by histological examination. This case report describes the successful outcome of macrolide treatment in this patient. Despite the low incidence of DPB in western countries, physicians must be aware of this orphan disorder in order to provide adequate therapy.

An unusual cavitary lung lesion in an asymptomatic and nonsmoker patient is uncommon. In these cases, infectious pathologies should be included in the differential diagnosis. Then, to correctly conduct an investigation, it is important to consider the clinical picture, the radiological images and the epidemiology. This presentation may need a wide variety of tests to establish the final diagnosis. Such a presentation occurs in the following case report.

In this report, we highlight the association of sarcoidosis and testicular cancer in 3 patients and comment on the potential impact of this association on the interpretation of the radiological and pathological findings in suspected cancer. Sarcoidosis, a condition that can coexist with testicular cancer, should always be considered in the differential diagnosis of suspected metastases, particularly as intrathoracic lymphadenopathy may be assumed to represent metastatic disease, which can have radical implications for patient therapy.

Idiopathic pulmonary fibrosis (IPF) is a progressive fatal lung disease characterised by persistent pulmonary inflammation. Approximately 2% of cases are familial. Herein, we describe a family in which four members over two generations developed IPF. Of particular interest, the age of onset was lower in the second generation affected by the disease. Familial IPF offers a unique opportunity to study the proinflammatory processes implicated in IPF.

We report a rare case of thoracic empyema due to Salmonella enteritidis infection in a 79-year-old woman with breast cancer and diarrhea. The presence of the bacteria was determined by culturing the stool, blood, and pleural fluid. Although it is a rare occurrence, Salmonella empyema should be excluded in a febrile cancer patient with pleural effusion and a recent history of enteritis.

An unusual case of smoking-related centrilobular emphysema with normal spirometry.

A 64-year-old man presented with severe dyspnoea and respiratory failure. Pulmonary function and mechanics were normal except for a marked reduction in diffusing capacity of the lung.

High-resolution CT scan showed diffuse centrilobular emphysema also involving lower lobes. Pulmonary embolism, cardiac or pulmonary shunt and immunopathologically based vasculitis were excluded. Pulmonary pressure was at the upper limit of normality but within few months he developed a severe pulmonary hypertension.

Although spirometry is the only physiologic measure recommended by the updated Global Initiative for Chronic Obstructive Lung Disease guidelines for confirming the diagnosis it should be recognized that diffuse emphysema may occur with only abnormalities in gas exchange without airflow obstruction. The identification of different phenotypes within COPD is important for understanding disease heterogeneity and progression.

We report a case of an unusual presentation of a complicated community-acquired pneumonia resembling lung cancer in an adult male with a long history of fever and consumption syndrome. Chest X-ray showed a right lower lobe consolidation and CT of the chest confirmed those findings. The bronchoscopy demonstrated a mass obstructing the right lower lobe orifice and narrowing the intermedius and right bronchus. Although the picture resembled a lung cancer, two biopsies were consistent with chronic inflammation and features of squamous metaplasia. He received several courses of empiric antibiotics (moxifloxacin and macrolides) without improvement. Cultures yielded Streptococcus pneumoniae and the antibiogram indicated exhibited resistance to moxifloxacin. We started with Amoxicilin–Clavulanic with a clear clinical and radiological improvement. This is the first case report in USA of a community-acquired pneumonia resistant to moxifloxacin.

A case of Mycoplasma pneumoniae pneumonia presenting with severe neutropenia, and diagnosed by immunoflourecence test is reported here. The patient was admitted to our clinic with severe neutropenia, and fever. During follow up a pneumonic consolidation was detected by thorax computerized tomography (CT) and anti-IgM was positive for M. pneumoniae serologically. It was concluded that neutropenia can develop due to M. pneumoniae.