{"id":7146,"date":"2025-11-19T10:08:35","date_gmt":"2025-11-19T09:08:35","guid":{"rendered":"https:\/\/alpha1-deutschland.org\/?p=7146"},"modified":"2026-04-20T15:07:15","modified_gmt":"2026-04-20T13:07:15","slug":"bronchiektasen-bei-aat-mangel","status":"publish","type":"post","link":"https:\/\/alpha1-deutschland.org\/en\/bronchiektasen-bei-aat-mangel","title":{"rendered":"Bronchiectasis and its association with alpha-1 antitrypsin deficiency (AATD)"},"content":{"rendered":"<div id=\"fws_6a8ea3d59c1ca\"  data-column-margin=\"default\" data-midnight=\"dark\"  class=\"wpb_row vc_row-fluid vc_row full-width-section\"  style=\"padding-top: 0px; padding-bottom: 50px; \"><div class=\"row-bg-wrap\" data-bg-animation=\"none\" data-bg-animation-delay=\"\" data-bg-overlay=\"false\"><div class=\"inner-wrap row-bg-layer\" ><div class=\"row-bg viewport-desktop\"  style=\"\"><\/div><\/div><\/div><div class=\"row_col_wrap_12 col span_12 dark left\">\n\t<div  class=\"vc_col-sm-12 wpb_column column_container vc_column_container col no-extra-padding inherit_tablet inherit_phone flex_gap_desktop_10px\"  data-padding-pos=\"all\" data-has-bg-color=\"false\" data-bg-color=\"\" data-bg-opacity=\"1\" data-animation=\"\" data-delay=\"0\" >\n\t\t<div class=\"vc_column-inner\" >\n\t\t\t<div class=\"wpb_wrapper\">\n\t\t\t\t<div class=\"vc_separator wpb_content_element vc_separator_align_center vc_sep_width_100 vc_sep_border_width_5 vc_sep_pos_align_center vc_sep_color_grey wpb_content_element vc_separator-has-text\" ><span class=\"vc_sep_holder vc_sep_holder_l\"><span class=\"vc_sep_line\"><\/span><\/span><h4>Author<\/h4><span class=\"vc_sep_holder vc_sep_holder_r\"><span class=\"vc_sep_line\"><\/span><\/span>\n<\/div>\n<div class=\"wpb_text_column wpb_content_element\" >\n\t<p style=\"font-weight: 400;\"><strong>Marion Wilkens, as appeared in <a href=\"https:\/\/alpha1-deutschland.org\/en\/alpha1-journal\/\">Alpha1 Journal 2\/2025<\/a>.<\/strong><\/p>\n<\/div>\n\n\n\n<div class=\"divider-wrap\" data-alignment=\"default\"><div style=\"margin-top: 25px; height: 5px; margin-bottom: 25px;\" data-width=\"100%\" data-animate=\"\" data-animation-delay=\"\" data-color=\"default\" class=\"divider-border\"><\/div><\/div>\n<div class=\"wpb_text_column wpb_content_element\" >\n\t<p><strong>Bronchiectasis refers to a persistent widening of the airways (bronchi) with impaired self-cleaning mechanisms of the lungs. This leads to persistent mucus production, chronic cough, recurrent infections, inflammation, and often, over time, a deterioration of lung function.<\/strong><\/p>\n<p>Alpha-1 antitrypsin deficiency (AATD), like other causes, can play a contributing role in the development of bronchiectasis. If the body lacks sufficient amounts of the protective protein alpha-1 antitrypsin, lung tissue is significantly more susceptible to damage from the body&#039;s own enzymes (e.g., neutrophil elastases). Over time, this can weaken the bronchial walls, impair the clearing of accumulating mucus from the airways, and ultimately contribute to the development of bronchiectasis.<\/p>\n<p>For patients with AATTM, this means: Bronchiectasis is <strong>not uncommon,<\/strong> but a possible consequence \u2013 and should therefore be investigated by a doctor at an early stage if symptoms such as chronic cough, increased sputum production or frequent infections occur.<\/p>\n<h2><span style=\"color: #004267;\">Why bronchiectasis in AAT deficiency deserves special attention <\/span><\/h2>\n<p>The typical symptoms \u2013 cough, sputum production, shortness of breath, and susceptibility to infections \u2013 often overlap with those of &quot;conventional&quot; COPD, asthma, or pulmonary emphysema. The underlying cause (e.g., an autoimmune thyroid disease) is therefore frequently overlooked. By the time bronchiectasis is diagnosed, the structural changes are usually already irreversible. Early detection can help slow its progression through appropriate therapies, physiotherapy and respiratory therapy, infection prophylaxis, and regular checkups.<br \/>\nFor people with AAT deficiency, there is an established, specific therapy: replacement therapy, which can slow the progression of pulmonary emphysema and theoretically also help to limit bronchial damage. Therefore, it is very important that affected individuals and treating physicians keep bronchiectasis in mind as a possible complication of AAT deficiency and not only assume classic emphysema or COPD scenarios.<\/p>\n<h2>New developments: Drug approval for bronchiectasis<\/h2>\n<p>A major advance for the treatment of bronchiectasis, including for patients with AATD, is the recent approval of the first specific drug: The orally administered drug brensocatib (trade name BRINSUPRI\u00ae) was approved in November 2025. <a href=\"https:\/\/www.aerzteblatt.de\/search\/result\/989de876-be45-4695-aeb2-14cc53204911?q=Erstes+Medikament+zur+Therapie+von+Bronchiektasen+in+EU+zugelassen\" target=\"_blank\" rel=\"noopener\">European Commission approval for the treatment of non-cystic bronchiectasis (NCFB)<\/a> received. This is therefore <strong>for the first time an approved, causal therapy option<\/strong> in fibrosis bronchiectasis for many affected individuals.<\/p>\n<p>The approval applies to patients aged 12 years and older with at least two exacerbations (infection-related or worsening episodes) in the previous year. In studies \u2013 including the large-scale phase 3 ASPEN trial \u2013 brensocatib demonstrated an approximately 201-fold reduction in the annual exacerbation rate, longer time to the next episode, and a slower decline in lung function. This drug could therefore be a valuable new option, especially for people with severe disease, frequent infections, or limited responses to standard therapy. For people with AATD and concurrently diagnosed bronchiectasis, this approval offers hope \u2013 naturally, the treatment decision must be made individually by the doctor and patient.<\/p>\n<p><strong>Since this topic affects many Alphas, we have launched a new patient information leaflet on AATM and bronchiectasis.<\/strong><\/p>\n<p>Visit our new <a href=\"https:\/\/alpha1-deutschland.org\/en\/alpha-1-bronchiektasen\/\" target=\"_blank\" rel=\"noopener\">Information page<\/a>, which specifically deal with the topic of \u201eAlpha-1 &amp; Bronchiectasis\u201c <strong>for those affected, relatives and interested parties<\/strong> This section deals with the topic. There you will find clearly presented explanations, background information and helpful advice on diagnosis and therapy.<\/p>\n<p>The content of this page is currently being reviewed by Dr. Pontus Mertsch. We are pleased to announce that he will soon be reporting on new developments \u2013 especially important at a time of significant progress due to regulatory approvals and increased research. Particularly valuable: On this page, you will find practical tips on how to better manage AAT deficiency and the associated risk of bronchiectasis, as well as how to effectively plan treatment and preventative care.<\/p>\n<p>Therefore, it is important that patients, relatives and treating physicians inform themselves now and think together about an optimal therapy plan.<\/p>\n<p>If you or a close relative suffers from AAT deficiency: Please note that <strong>Bronchiectasis is a frequently underestimated but serious complication.<\/strong> We can present this. The new information page from Alpha1-Germany and the approval of the drug Brensocatib open up new avenues \u2013 and we hope that you can benefit from them.<\/p>\n<p>Stay vigilant, stay informed \u2013 and talk to your pulmonologist about options for diagnosis, treatment and prevention.<\/p>\n<p><em>As of December 2025<\/em><\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>","protected":false},"excerpt":{"rendered":"Author Marion Wilkens, as published in Alpha1-Journal 2\/2025. Bronchiectasis refers to a persistent dilation of the airways (bronchi) with reduced self-cleaning mechanisms of the lungs. This leads to persistent mucus production, chronic cough, recurrent infections,...","protected":false},"author":1,"featured_media":7147,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1,24,108],"tags":[],"class_list":["post-7146","post","type-post","status-publish","format-standard","has-post-thumbnail","category-allgemein","category-alpha1-journal","category-bronchiektasen"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.3 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Bronchiektasen bei AAT-Mangel erkennen<\/title>\n<meta name=\"description\" content=\"Untersch\u00e4tztes Risiko: Bronchiektasen bei AAT-Mangel. 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