Bronchiectasis: what it is, symptoms, diagnosis and new therapies

QAlmost no one knows about them, but according to the latest data they bronchiectasis concern well over 500 adults out of 100 thousand and they are an underestimated pathology. This chronic inflammatory disease can in fact also manifest itself from early life and can have genetic components and aggravating factors due to achronic infection by particular microorganisms. Hardly diagnosed and increasing, bronchiectasis they don’t have therapy yet approved drug. But the new hope comes from a better understanding of immunology. More than 200 specialists from all over Europe are talking about it these days Simulation Center of Humanitas University, home of Italian and international knowledge on the immune system. We interviewed one of the leading experts in bronchiectasis: Stefano Aliberti, head of Pneumology at the IRCCS Humanitas Research Hospital in Rozzano and Full Professor of Respiratory Diseases at Humanitas University and coordinator of the workshop.

Bronchiectasis: the international workshop at Humanitas University

From 23 to 25 February it is held in Humanitas University the third European Bronchiectasis Workshop: an opportunity for discussion between over 200 European specialists to share expertise on the management of bronchiectasis. New hopes come from one better immunological understanding of the disease. Pulmonologists, physiotherapists, nurses, paediatricians, general practitioners, radiologists, immunologists, infectious disease specialists and clinical microbiologists from all over Europe involved in the treatment of bronchiectasis, gather at the Simulation Center of Humanitas University. The workshop is introduced by lecture masterful of Alberto MantovaniScientific Director of Humanitas and professor emeritus of Humanitas University, on the role of immunity and inflammation as a meta-narrative of Medicine.

Bronchiectasis: why we need to talk about it

The data say that, in Italy, up to 500 adults out of 100,000 suffer from bronchiectasis, but it is one underestimated figure. The European community of scientists and industry experts confirms that bronchiectasis and their comorbidity they continue to be often misdiagnosed And treated inappropriately. L’epidemiology is still incomplete and varies from country to country, management is not uniform and satisfactory. Also i specific drugs are not yet availablealthough new therapies may be on the market in the near future.

Pneumonia: how to recognize it, treatments and the importance of prevention

“In this rapidly changing landscape it is Fundamental to bring together experts to continuously improve knowledge and management of this disease not only in the short term but also in the long term. This is especially true in the post-Covid reality and within the complexity of different healthcare systems across Europe. It is necessary focus on new research to help small but active communities of specialists studying bronchiectasis continue the clinical networking and research that, in recent years, has led many improvements in understanding and managing the disease. Always keeping our attention on the well-being and quality of life of patients», explains Professor Aliberti.

Great experts in comparison

Together with Professor Aliberti, the third edition of the workshop is coordinated by Francesco Blasifull professor of Respiratory Diseases at the University of Milan and director of Pneumology at the IRCCS Ca’ Granda Foundation, Ospedale Maggiore Policlinico of Milan, and by James D. Chalmersprofessor at the University of Dundee (UK), assisted by an international scientific committee with professionals from the United Kingdom, Greece, Belgium, Spain, Germany and France.

Bronchiectasis: what are they?

Bronchiectasis is a chronic respiratory disease characterized by cough with expectoration of catarrh newspapers, with frequent infections respiratoryappearance of pneumoniabronchitis and blood loss (emoptysis or hemoptysis) also consistent. Everything is due to one irreversible dilatation of the bronchiwhich is very difficult to diagnose early”, explains Professor Aliberti.

What are the symptoms of bronchiectasis?

«There are 3 most common symptoms that should help doctors in the diagnosis: the presence of chronic cough, even daily, the presence of phlegm and frequent episodes of respiratory infections, i.e. bronchitis or pneumonia. The diagnosis necessarily passes from the CT scan of the chest, preferably with high resolution. The chest film is often not sensitive enough to detect bronchiectasis. In addition to these three conditions there are other symptoms: the presence of hemoptysis or hemoptysis (blood in the spit), profuse tiredness, shortness of breath.

Why is diagnosis so difficult?

«There are no Italian guidelines on bronchiectasis that give indications on management, so we rely on a European protocol drawn up in 2017 and which is now under revision. THE centers dealing with this pathology they have very similar procedures. It starts from the confirmation of the diagnosis with the High resolution chest CT, blood tests And instrumental to find a cause, functional assessment of the breath with the global spirometry. Then you make one microbiological evaluation through the sputum culture analysis to understand if there is a chronic infection of bacteria, fungi, etc. Very important the assessment of comorbidities it’s a gravity stratification of illness», explains Professor Aliberti.

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A new approach to personalized therapy

«A very recent approach is then the evaluation of clinical phenotypesie patients who have common characteristics of the disease and the endotypes, i.e. the biological characteristics related to the type of inflammation. This approach allows for a personalized medicine, in order to be able to choose the correct drug or therapy on the basis of not only clinical but also biological characteristics», specifies the expert.

Who to contact for diagnosis

«There are no specialized centers recognized by institutional bodies, but theItalian Bronchiectasis Association He has been posting on his website for years the most up-to-date map we have to date», explains Professor Aliberti. For diagnosis, it is advisable to contact these qualified Centers which, thanks to the work of the Association, its members and the most active doctors, are spread throughout the national territory: at this link you will find the list of structures to contact to be followed by pulmonologists specialized in this pathology.

Respiratory physiotherapy: essential to get better

Respiratory physiotherapy is the key treatment of this disease, which has a dignity equal to any other pharmacological intervention. The important thing is that physiotherapy is tailor-made for each patient. «The respiratory physiotherapy strategy must be shared with the respiratory physiotherapist and individualized on the radiological, clinical and quality of life characteristics of the patient. The equipment available to the physiotherapist is very broad and foresees techniques with and without toolsalso for the effort retraining. The best technique is chosen to expectorate the patient and what he will do at home every day to reduce infection and symptoms. The point is that the percentage of patients who follow this path of physiotherapy is still very low for various reasons, including the lack of knowledge of the disease, the methods to manage it and the number of respiratory physiotherapists», confirms Aliberti.

Bronchiectasis and children

“This pathology can strike even children from the first months or years of life. The management is different compared to adults, both in respiratory physiotherapy and in the use of antibiotics (which we try to use as little as possible), and in the diagnosis because we try not to do high-resolution CT scans as often as for adults. It goes through first MRI of the lung which does not emit radiation and if the doubt remains, then we move on to the CAT scan. Compared to adults, children with bronchiectasis do not have related pathologies such as anxiety, depression, asthma, heart disease, chronic sinusitis, osteopenia, osteoporosis, reflux.

There transitioni.e. the transition from the pediatrician to the pulmonologist when the patient is 14 years old, is a fundamental moment to continue managing bronchiectasis in the best possible way», recommends the expert.

Inflammation of the bronchi and the new drugs on the way

«To date there are no approved and specific pharmacological therapies to treat patients with bronchiectasis, and the management passes above all through daily respiratory physiotherapy and a careful use of antibiotics. However, the latest evidence says that we are dealing with a disease supported by aimportant inflammatory component of the bronchi. Until a few years ago it was thought that the therapies should be sought only on infection control, but now they are opening up new horizons for bronchiectasis patients. Some of study drugsin fact, they are gods modulators of the immune system which reduce inflammation at the bronchial level», explains Professor Stefano Aliberti.

Who is Professor Stefano Aliberti

Prof. Stefano Aliberti

Stephen Aliberti he is head of Pneumology at the IRCCS Humanitas Research Hospital in Rozzano and Full Professor of Respiratory Diseases at Humanitas University. He is a national and international reference in the field of bronchiectasis (he participated in the drafting of the European guidelines on this pathology), pulmonary infections with non-tubercular mycobacteria, and chronic and acute infections, including pneumonia. He is the founder of the European register (EMBARC; bronchiectasis.eu) and the Italian registry (IRIDE) of bronchiectasis, as well as the Italian registry of non-tubercular pulmonary mycobacteriosis (IRENE; registerirene.it). Finally, he chairs the scientific committee of the Italian Bronchiectasis Association (bronchiectasis.org). He is the author of more than 360 publications in international journals in the field of respiratory infections.

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