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Thursday, 02/25/2021 9:18:10 AM

Thursday, February 25, 2021 9:18:10 AM

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Sarcoidosis is a systemic inflammatory disease with a predilection for the respiratory system. Although most patients enter remission and have good long-term outcomes, up to 20% develop fibrotic lung disease, whereby granulomatous inflammation evolves to pulmonary fibrosis.

https://pubmed.ncbi.nlm.nih.gov/23952859/

But for many types, a definite cause cannot be found, for example idiopathic pulmonary fibrosis. Although we do not always know what causes pulmonary fibrosis, we do know it is not a form of cancer or cystic fibrosis, and it is not contagious. Cystic fibrosis is also not a type of ILD or pulmonary fibrosis.

https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/what-is-pulmonary-fibrosis

Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. ———————-

In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dynamic sfunctional. When the protein is not working correctly, it’s unable to help move chloride -- a component of salt -- to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.
In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, minimizing contact with germs is a top concern for people with CF.

https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/

Mucus is secreted from two distinct areas within the lung tissue. In the surface epithelium, which is part of the tissue lining of the airways, there are mucus-producing cells called goblet cells. The connective tissue layer beneath the mucosal epithelium contains seromucous glands which also produce mucus.

https://www.nursingtimes.net/clinical-archive/respiratory-clinical-archive/the-physiology-of-mucus-and-sputum-production-in-the-respiratory-system-10-06-2003/

Recent Findings: As a neurotransmitter, VIP exerts a potent bronchodilatory and vasodilatory effect and also is supposed to induce the house-keeping mucus secretion by submucosal glands. On the other hand, it has immunomodulatory functions which include humoral immune response suppression, inhibition of vascular and bronchial remodeling and inflammation and attenuation of the cigarette smoke extract-induced apoptotic death of alveolar L2 cells. Recent research on a wide spectrum of lung diseases including asthma, chronic obstructive pulmonary disease, cystic fibrosis, pulmonary hypertension, and sarcoidosis indicates a potential therapeutic role of a VIP agonist. Simultaneously, novel stabilized inhaled VIP agonists and drug delivery systems have been proposed as a promising candidate alternative drug with minimized side effects. These data are supported by the results of certain, limited clinical trials which have already been conducted.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3738270/

As part of a new agreement with AdVita Lifescience, Relief Therapeutics will acquire the intellectual rights for the specifications and potential applications of an inhaled formulation of RLF-100 (aviptadil).
Relief has been collaborating with NeuroRx to advance the development of RLF-100 to treat patients with severe COVID-19 respiratory complications and other lung disorders, including pulmonary sarcoidosis.
RLF-100 is an artificial form of a peptide hormone, called vasoactive intestinal peptide (VIP), that is naturally produced throughout the body and has been found to have potent anti-viral and anti-inflammatory properties.
By acquiring the rights to AdVita’s therapy formulation, Relief also will accelerate and secure RLF-100’s future development as an inhaled therapy for lung diseases, such as pulmonary sarcoidosis, that are associated with COVID-19. Other lung diseases associated with COVID-19, which also could potentially be treated by RLF-100, include checkpoint inhibitor-induced pneumonitis, and acute respiratory distress syndrome (ARDS).

https://sarcoidosisnews.com/2021/01/28/deal-gives-relief-therapeutics-intellectual-rights-potential-sarcoidosis-therapy-rlf-100/

The global respiratory diseases drugs market is expected to decline from $90.32 billion in 2020 to $79.82 billion in 2021 at a compound annual growth rate (CAGR) of -11.6%. The change in growth trend is mainly due to the companies stabilizing their output after catering to the demand that grew exponentially during the COVID-19 pandemic in 2020. The market is expected to reach $98.81 billion in 2025 at a CAGR of 5%.

https://www.thebusinessresearchcompany.com/report/respiratory-diseases-drugs-global-market-report-2020-30-covid-19-implications-and-growth