Extract
Spontaneous pneumothorax is one of the most common disorders affecting the pleura. A large epidemiological study in France from 2008 to 2011 reported an estimated annual rate of non-traumatic pneumothoraces of 22.7 cases per 100 000 inhabitants, with a male to female ratio of 3.3 to 1 [1]. 85% of the nearly 60 000 hospital admission episodes corresponded to primary spontaneous pneumothoraces (PSP) [1], a categorisation which implies that the person does not have a known lung disease. Despite spontaneous pneumothorax being frequent and first recognised as a distinct entity two centuries ago [2], only about 20 randomised controlled trials have been published to date, the vast majority of which comprised a small number of patients. Consequently, gaps still remain in the fundamental pathophysiological mechanisms and preferred management options. For instance, the assumption that the underlying lung is normal in PSP is debatable. PSP predominantly (∼90%) occurs in tobacco smokers [3]. Airway inflammation is produced by tobacco and cannabis smoking and, thus, both contribute to the development of subclinical lung disease, particularly upper lobe bullous emphysema [3]. The general consensus is that rupture of subpleural blebs or bullae (usually located in the lung apices) into the pleural space plays a major role in PSP development. Most patients with a PSP who are thought to be free of parenchymal disease are found to have blebs, bullae (termed emphysema-like pulmonary changes) or apical opacities (i.e. linear opacities, focal subpleural consolidations or pleural thickening) on high-resolution computed tomography (HRCT) [4–6]. Abnormal regions of the visceral pleura detected by fluorescein-enhanced autofluorescence thoracoscopy, so-called “pleural porosity”, have also been put forward as a potential cause to explain the occurrence of PSP [7]. However, pleural porosity, which hypothetically implies slow air leakage through the visceral pleura, is difficult to reconcile with the sudden start of PSP symptoms. According to the previous text, the distinction between PSP and secondary spontaneous pneumothorax is viewed increasingly as artificial. In the same line, the need to routinely obtain an HRCT to detect an underlying occult disease for clinical decision-making purposes during a first episode of a PSP should be considered, though not currently explicitly recommended by scientific guidelines [7–9]. In fact, HRCT imaging in patients presenting with an apparent PSP may be cost-effective even if only for the uncovering of infrequent diffuse cystic lung diseases, such as lymphangioleiomyomatosis, Birt−Hogg−Dubé syndrome or Langerhans cell histiocytosis [10]. Spontaneous pneumothorax occurs in around 60% [11], 75% [12] and 15% [13] of these entities, respectively, albeit with a very high likelihood of recurrence [14].
Abstract
In the era of personalised medicine, there is a need to “phenotype” primary spontaneous pneumothorax in order to tailor the most appropriate treatment for each patient http://ow.ly/slNX30lizXz
Footnotes
Conflict of interest: J.M. Porcel has nothing to disclose.
- Received July 31, 2018.
- Accepted August 1, 2018.
- Copyright ©ERS 2018