Leon M. van den Toorn

Clinical implications of airway inflammation in mild intermittent asthma

Objective To determine whether inhaled corticosteroids should be prescribed to patients with milder forms of asthma and whether markers of airway inflammation should be considered when making therapy decisions. Data Sources A PubMed search was performed of the English-language literature published in the preceding 10 years (January 1, 1993, through December 31, 2003) concerning epidemiology, pathophysiology, therapy, and prognosis of mild intermittent asthma, with asthma , mild , and intermittent as indexing terms. Study Selection All relevant studies including authors expert opinions were selected. Results Several studies have addressed the question of a possible benefit of maintenance therapy (ie, inhaled steroids) in patients with mild intermittent asthma. Although a diminishing effect on airway inflammation has been widely demonstrated, even in patients with mild disease, the impact of inhaled steroids on the longterm prognosis is much less clear. For patients with mild disease who are longterm inhaled steroid users, alternative therapy strategies, including low-dose inhaled steroids and leukotriene receptor antagonists, have been advocated. Conclusions Mild intermittent asthma is a disease characterized not only by infrequent symptoms and normal lung function but also by chronic airway inflammation, possibly resulting in irreversible airflow limitation if left unattended. Therefore, maintenance therapy, such as (low-dose) inhaled steroids or leukotriene receptor antagonists, should be considered in patients with mild disease. Future studies should give more insight into the impact of prolonged antiinflammatory therapy on the longterm prognosis of mild intermittent asthma patients. Whether results from these studies will justify a more aggressive treatment for these patients remains to be answered.

Daily home spirometry to detect early steroid treatment effects in newly treated pulmonary sarcoidosis

Prednisone is the mainstay of sarcoidosis treatment. However, prednisone treatment optimisation is warranted, since prolonged high-dose prednisone therapy is associated with burdensome and harmful side-effects [1, 2]. Early prednisone dose tapering has the potential to reduce side-effects. Gaining insight in the early treatment response can help to determine when tapering could be initiated. To date, there are no prospective studies that look at early treatment response to prednisone in sarcoidosis by monitoring clinical symptoms and daily patient-administered lung function. Therefore, we initiated a multicentre, prospective and observational study with daily home spirometry to detect early steroid treatment effects in newly treated pulmonary sarcoidosis (Dutch National Trial Register NTR4328; www.trialregister.nl/trialreg). The major treatment effect of prednisone on FVC is reached within 2 to 3 weeks in newly treated sarcoidosis patients http://ow.ly/3A3E30h5ZuT

Lung Transplantation in Gaucher Disease : A Learning Lesson in Trying to Avoid Both Scylla and Charybdis

Gaucher disease (GD), a lysosomal storage disorder, may result in end-stage lung disease. We report successful bilateral lung transplantation in a 49-year-old woman with GD complicated by severe pulmonary hypertension and fibrotic changes in the lungs. Before receiving the lung transplant, the patient was undergoing both enzyme replacement therapy (imiglucerase) and triple pulmonary hypertension treatment (epoprostenol, bosentan, and sildenafil). She had a history of splenectomy, severe bone disease, and renal involvement, all of which were related to GD and considered as relative contraindications for a lung transplantation. In the literature, lung transplantation has been suggested for severe pulmonary involvement in GD but has been reported only once in a child. To our knowledge, until now, no successful procedure has been reported in adults, and no reports deal with the severe potential posttransplantation complications specifically related to GD.

“Tree in bud” attributable to organising pneumonia

A 19-year-old man presented with dyspnoea, a non-productive cough and subfebrile temperature. Chest radiography showed a diffuse nodular pattern and a pneumomediastinum. HR-CT revealed a diffuse ‘tree in bud’ pattern (figure 1). An infectious bronchiolitis was suspected. Despite treatment with moxifloxacin he developed hypercapnic respiratory failure requiring mechanical ventilation. Extensive microbiological investigations did not reveal a pathogenic microorganism. Continued treatment with antibiotics, low-dose steroids and neomacrolides was not effective, and an open lung biopsy was performed. Histology showed an organising pneumonia (OP) with extensive endobronchiolar granulation tissue (figure 2). The treatment was converted …

Management of recurrent malignant pleural effusions with a tunneled indwelling pleural catheter

Management of recurrent malignant pleural effusions with a tunneled indwelling pleural catheter