In a nutshell, Swyer-James syndrome, also known as Swyer-James-Macleod syndrome (SJMS), is an incurable respiratory disease that has a unilaterally bright * and hyper-transparent lung. Even if the discovery of this clinical picture was more than 60 years ago, the Swyer-James syndrome and its origin are still quite unexplored. The syndrome is still completely unknown to a large number of doctors, so treatment by a specialized specialist is therefore imperative for those affected.
1953 - The Discovery In 1953, Swyer and James first reported "a 6-year-old boy with unilateral pulmonary emphysema" **. This is a non-recoverable enlargement of the smallest bronchi (bronchioles) and the associated alveoli. Because of this enlargement, there is pathological bloating of the lungs, as a result of which the inhaled oxygen can no longer pass properly into the blood. The actual gas exchange is therefore severely restricted. The lungs as a complex tree structure Our lungs can easily be imagined as an upside down tree. Very similar to its model from nature, the lungs also have a large number of branched branches, the bronchi, and sprouting leaves, the alveoli. When we breathe in, the inhaled air passes through the bronchi to the alveoli, where the gas exchange between blood and alveolar air takes place. To ensure that not every pollutant from the environment gets into our airways and thus to the alveoli, the bronchi have fine hairs, the so-called cilia. They ensure that the inhaled air is moistened and possible pollutants such as dust or pollen are transported outside again. As part of this breathing process, a bronchial secretion is produced by the tissue structures and the airways themselves. This serves to moisten the airways and bind particles that have entered the airways. Depending on the temperature, moisture and particle content of the breathing air, the amount produced can fluctuate greatly. **** With SJMS, the removal of the bronchial mucus by the cilia is impaired due to the irreversible expansion of the bronchioles. The mucus remains in the enlarged bronchi and is therefore more easily colonized by bacteria. Chronic inflammation is the result. *** In this context, strong parallels are often drawn with post-viral bronchiectasis disease. 1954 - The second discovery Just a year after the discoveries of Swyer and James, Macleod described "a series of 9 adult patients with unilaterally weakened breathing and radiologically increased transparency of a lung" ** Today, classic SJMS is known as a full-page lung disease. However, some speak of individual segments. How does Swyer-James-Macleod syndrome develop? Assumption 1: There are different assumptions about the development of Swyer-James-Macleod syndrome. The syndrome is probably the most common assumption at the moment as a result of undetected or treated bronchiolitis in early childhood. "SJMS has been described as a result of infections with adenoviruses, measles viruses, mycoplasmas, mycobacteria and Bordetella pertussis. The infection leads to an inflammatory process with [obliteration] of the peripheral airways and the vascular bed of the lungs [lying on the same side of the body]." ** The growth of the alveoli stopped at the age of 8. It is therefore assumed that the undetected bronchiolitis must have occurred beforehand and thus impaired the development of the lungs. "The narrowness of the airways leads to air trapping with the resulting hyper transparency of the affected ... lungs" **
Assumption 2: The SJMS makes another assumption in connection with serious complications at birth. For example, if a baby is born with an umbilical cord tightly wrapped around the neck or by other means that it does not take a first breath without support, this can also affect lung growth. Other assumptions: Most of the studies on the subject of Swyer-James syndrome consistently point out that there have not been enough long-term studies on the disease so far, and that it cannot be ruled out that the syndrome is genetically passed on or acquired by circumstances other than those listed here can be. My next blog entries will therefore focus more on the topic of "studies" in order to collect as much information as possible.
* In some articles there is also the description of the unilateral dark lung.
** Source: Seltene Lungenerkrankungen, Michael Kreuter, Ulrich Costabel, Felix Herth, Detlef Kirsten, Springer-Verlag Berlin Heidelberg 2016
**** Source: https://flexikon.doccheck.com/de/Bronchialsekret