Fotios Drakopanagiotakis

Apoptosis in lung injury and fibrosis

Pulmonary fibrosis is characterised by fibroblast accumulation and alveolar epithelium denudation. Increased apoptosis of alveolar epithelial cells and decreased apoptosis of fibroblasts may play an important role in the pathogenesis of disease. Inflammatory cells can modulate apoptosis of other cell types, both by removal of apoptotic debris and by cytokine production, thus preserving a pro-fibrotic environment. In the present review, some of the mechanisms by which apoptosis may contribute to the pathogenesis of idiopathic pulmonary fibrosis are described.

Cryptogenic and Secondary Organizing Pneumonia: Clinical Presentation, Radiographic Findings, Treatment Response, and Prognosis

BACKGROUND Organizing pneumonia (OP) is a distinct clinical and pathologic entity. This condition can be cryptogenic (COP) or secondary to other known causes (secondary OP). In the present study, we reviewed the features associated with COP and secondary OP in patients from two teaching hospitals. METHODS The medical records of 61 patients with biopsy-proven OP were retrospectively reviewed. Forty patients were diagnosed with COP and 21 patients with secondary OP. The clinical presentation, radiographic studies, pulmonary function tests (PFTs), laboratory data, BAL findings, treatment, and outcome were analyzed. RESULTS The mean age at presentation was 60.46 ± 13.57 years. Malaise, cough, fever, dyspnea, bilateral alveolar infiltrates, and a restrictive pattern were the most common symptoms and findings. BAL lymphocytosis was observed in 43.8% of patients with OP. The relapse rate and mortality rate after 1 year of follow-up were 37.8% and 9.4%, respectively. The in-hospital mortality was 5.7%. The clinical presentation and radiographic findings did not differ significantly between patients with COP and secondary OP. A mixed PFT pattern (obstructive and restrictive physiology) and lower blood levels of serum sodium, serum potassium, platelets, albumin, protein, and pH were observed among patients with secondary OP. Higher blood levels of creatinine, bilirubin, Paco₂, and BAL lymphocytes were also more common among patients with secondary OP. There were no differences in the relapse rate or mortality between patients with COP and secondary OP. The 1-year mortality correlated with an elevated erythrocyte sedimentation rate, low albumin, and low hemoglobin levels. CONCLUSIONS The clinical and radiographic findings in patients with COP and secondary OP are similar and nonspecific. Although certain laboratory abnormalities are more common in secondary OP and can be associated with worse prognosis, they are likely due to the underlying disease. COP and secondary OP have similar treatment response, relapse rates, and mortality.

Acute exacerbations of interstitial lung diseases.

Purpose of review This review aims to highlight recent advances in pathogenesis, clinical presentation and treatment of interstitial lung diseases (ILDs). Recent findings Acute exacerbation is increasingly recognized as a major complication in the course of idiopathic pulmonary fibrosis. It is precipitated by a variety of intrinsic and extrinsic factors. Moreover, acute exacerbation is an apparently equally frequent event in hypersensitivity pneumonitis and collagen–vascular diseases associated ILDs, especially the rheumatoid pulmonary fibrosis. Treatment of acute exacerbations is unsatisfactory and prognosis extremely poor. Summary In a critically ill patient proper recognition of an acute exacerbation and of the underlying chronic ILD is warranted because treatment approach varies with the type of ILD. Advances in the understanding of the pathogenesis and treatment of this ‘idiopathic’ phenomenon are reviewed.

Ein Bewertungssystem zur Vorhersage der Erhöhung des mittleren Pulmonalarteriendrucks bei idiopathischer Lungenfibrose

Ein erhöhter mittlerer pulmonaler arterieller Druck (mPAP ≥ 21 mmHg) hat eine negative Auswirkung auf das Überleben von Patienten mit idiopathischer Lungenfibrose (idiopathic pulmonary fibrosis, IPF). Es gibt bis dato kein etabliertes nicht invasives Screeningtool für die Diagnose der präkapillären pulmonalen Hypertonie (PH). Die Daten von 273 Patienten mit IPF - die einem Rechtsherzkatheter (right heart catheterisation, RHC) unterzogen wurden - wurden bezüglich ihrer Aussagekraft für die nicht invasive Diagnose einer PH retrospektiv analysiert. Bei 55 Patienten wurde mittels RHC ein erhöhter mittlerer Pulmonalarteriendruck (mean pulmonary artery pressure, mPAP) (definiert als mPAP ≥ 21 mmHg), bei 18 Patienten eine präkapilläre PH (definiert als mPAP > 25 mmHg) diagnostiziert. In einer multivariablen Analyse zeigten sich die Kohlenmonoxid-Diffusionskapazität (diffusing capacity of the lung for carbon monoxide, DLCO) <50% vom Soll, ein Sauerstoffpartialdruck (pO2) <80 mmHg und ein PA/AO ≥0,9 im CT-Thorax als unabhängige Faktoren für die Diagnose einer PH. Es wurde jedem der 3 statistisch signifikanten Variablen ein einziger Punkt zugewiesen, sodass eine 4-Punkte-Skalierung entstand. Die Prävalenz des erhöhten mPAP mit einer Punktzahl von 0, 1, 2 oder 3 Punkten stieg von 6,7% auf 16,0%, 29,1% und schließlich 65,4% an.

Endobronchiale Ultraschall-gesteuerte transbronchiale Nadelaspiration bei Sarkoidose: Eine prospektive Studie zur Anzahl der zu punktierenden Lymphknotenstationen

Background: While endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is widely used as an initial diagnostic procedure for pathological confirmation of sarcoidosis, it is unclear how many passes are required to obtain diagnostic materials. Objectives: The aim of this study was to determine the number of needle passes needed for the diagnosis of stage I/II sarcoidosis using EBUSTBNA. Methods: At three institutions, 109 patients with suspected stage I/II sarcoidosis were recruited and underwent 6 passes of EBUS-TBNA for the main target lesion. Additional EBUS-TBNA for other lesions was permitted. The cumulative yields of needle passes for detecting noncaseating epithelioid cell granulomas were analyzed. Results: A total of 109 patients underwent EBUS-TBNA for 184 lesions. EBUS-TBNA identified specimens containing granulomas in 81 of 92 patients (88%) with a final diagnosis of sarcoidosis. The cumulative yields through the first, second, third, fourth, fifth, and sixth passes for the main target lesion were 63, 75, 82, 85, 86 and 88%, respectively. In the 55 patients that underwent EBUS-TBNA for multiple lesions, the cumulative yields of 2 passes per lesion for 2 lesions (total of 4 passes) and of 4 passes for single lesions were 86 and 84%, respectively (p = 1.00). Conclusions: If rapid on-site cytological evaluation is not available, we recommend at least 4 passes per patient for either single or multiple lesions with EBUS-TBNA for pathological diagnosis of stage I/II sarcoidosis.

Grundlagen der medikamentösen Therapie

In diesem Kapitel werden verschiedene medikamentose Verfahren zur Behandlung seltener Lungenerkrankungen, mit Fokus auf die interstitiellen Lungenerkrankungen, dargestellt. Die vorgeschlagenen Verabreichungswege und Dosierungen sowie die Nebenwirkungen und notwendige Kontrolluntersuchungen werden diskutiert.