What is Pulmonary Sarcoidosis?

Sarcoidosis is a rare condition that causes small patches of red and swollen tissue, called granulomas, to develop in the organs of the body. It usually affects the lungs and skin. Sarcoidosis is an inflammatory disease in which the immune system overreacts, causing clusters of inflamed tissue called "granulomas" to form in different organs of the body. Sarcoidosis most commonly affects the lungs and lymph nodes, but it can also affect the eyes, skin, heart and nervous system. Sarcoidosis is a rare disease.

Sarcoidosis in the lungs is called Pulmonary Sarcoidosis. The granulomas generally heal and disappear on their own. But, if they do not heal, the lung tissue can remain inflamed and become scarred and stiff. This is called pulmonary fibrosis. It changes the structure of the lungs and can affect breathing. The cause of pulmonary sarcoidosis is unknown however some studies shows that bacteria, viruses, or chemicals might trigger the disease. It may also be genetic.

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The following are the most common symptoms of Pulmonary Sarcoidosis. However, each person may experience symptoms differently. These symptoms may include: Shortness of breath, which often gets worse with activity, dry cough that will not go away, chest pain and wheezing.

In Pulmonary Sarcoidosis, staging indicates the location of granulomas—the lungs, the lymph nodes, or both—and the nature of the disease. Staging is an easy way to categorize sarcoidosis patients, and it does not indicate severity.

Stage one sarcoidosis indicates granulomas in the lymph nodes. Stage two indicates lymph node involvement in addition to granulomas in the lungs. At first, this might appear to be a progressive diagnosis compared to stage one. However, stage three sarcoidosis indicates granulomas present in the lungs, but not in the lymph nodes. This demonstrates how the stages are not a progression. Stage four sarcoidosis is a little bit different than the other three. Stage four indicates scarring in the lungs—pulmonary fibrosis—which is irreversible.

Because of this, stage four is the most severe presentation of sarcoidosis. All stages of pulmonary sarcoidosis can be severe. Any stage could cause serious symptoms and debilitating fatigue.

The key driver for the surge in market size is the rise in number of incident cases of Pulmonary Sarcoidosis patients in 7MM.

The fundamental goals in the management of Pulmonary Sarcoidosis are to prevent or control organ damage, relieve symptoms, and improve the patient's quality of life. Many people with pulmonary sarcoidosis show mild symptoms and do not require any treatment at all. In practice, treatment is administered only when needed. Generally, the available treatments fall into two categories - maintenance of good health practices and drug treatment.

Drug treatments are used to relieve symptoms, reduce the inflammation of the affected tissues, reduce the impact of granuloma development, and prevent the development of lung fibrosis and other irreversible organ damage. The therapeutic landscape of pulmonary sarcoidosis is owned by various classes of drugs, such as Corticosteroids, Methotrexate, Anti-malarials, Tumor necrosis factor-alpha (TNF-alpha) inhibitors, Corticotropin and others. Sometimes more than one treatment is used. Most medicines used to treat sarcoidosis suppress the immune system.

Methotrexate, leflunomide, or azathioprinea have been used in place of or in addition to corticosteroids to treat pulmonary sarcoidosis and chronic sarcoidosis. Apart from these, rehab program that includes education, exercise, and support can also be joined. In severe cases, which are not common, oxygen therapy and even lung transplant may be needed. An overview of several different medications that can be prescribed to treat.

Acthar Gel, a repository corticotropin injection (Mallinckrodt), is a porcine-derived pituitary extract that was approved by the FDA for the treatment of pulmonary sarcoidosis in the 1950s, on the basis of reports of its efficacy in that era.

In the last few years, biological agents, particularly infliximab and adalimumab, have shown great promise as antigranulomatous agents for Pulmonary Sarcoidosis. Although efficacy data concerning these agents are not robust, these agents appear to be at least as effective as or possibly more effective than the aforementioned second-line agents.

Their use is mainly limited by their cost. Newer anti-granulomatous agents for Pulmonary Sarcoidosis continue to be developed. To redress the current issues, many companies are currently working toward this indication to meet the needs of the market. Pipeline drugs in clinical development are designed to address the major unmet needs in Pulmonary Sarcoidosis treatment. The other factors that shall further expedite the growth of Pulmonary Sarcoidosis market include increasing morbidity rate of Pulmonary Sarcoidosis and increasing awareness about available treatments during the forecast period (2019–2030). A better understanding of disease pathogenesis will also contribute to the development of novel therapeutics for Pulmonary Sarcoidosis.

Expected launch of the pipeline therapies shall fuel the growth of the market during the forecast period, i.e., 2020–2030. The market size shall grow during the forecast period owing to the launch of upcoming therapies. The market size is expected to increase at a CAGR of XX during the study period.

The research and development of new treatment therapies for Pulmonary Sarcoidosis are categorized into various groups. The dynamics of Pulmonary Sarcoidosis market is anticipated to change in the coming years owing to the improvement in the diagnosis methodologies, incremental healthcare spending across the world, and also the expected launch of emerging therapies such as CMK389 (Novartis Pharmaceuticals), ATYR1923 (aTyr Pharma), Selexipag (Actelion), Combination product: iNO or inhaled nitric oxide (Bellerophon) and others during the forecast period.

Aviptadil (Relief Therapeutics) is an abundant biologically active endogenous human peptide acting as a ligand on specific G-protein coupled transmembrane receptors. It is one of the signal molecules of the neuroendocrine-immune network comprising anti-proliferative, anti-inflammatory, and immune-regulatory features. Its predominant biological activity is performed in the lungs, and a vast body of experimental, pharmacological, and clinical evidence suggests aviptadil to be an attractive candidate for the treatment of sarcoidosis. Since the 1970s, a number of clinical phase I/II trials using systemic (by injection) and local (by inhalation) administration of aviptadil have been performed in humans.

To circumvent the side effects observed with systemic delivery, Relief Therapeutics will use inhalation of aviptadil as the route of administration for sarcoidosis patients, as inhaled drugs act quickly, minimize undesired negative side effects, avoid the hepatic first-pass metabolism, and act locally in the affected organ. Following a phase II trial in 20 sarcoidosis patients demonstrating a striking suppression of inflammatory mechanisms of the lung, in combination with amelioration of dry cough and of exertional dyspnea, Relief Therapeutics now conducting a phase III clinical trial.

RLF-100 (Aviptadil) is a patented formulation of Vasoactive Intestinal Polypeptide (VIP) that was originally developed and is currently marketed in Europe for the treatment of erectile dysfunction. VIP is known to be highly concentrated in the lung and to inhibit a variety of inflammatory cytokines. Aviptadil was awarded Orphan Drug Designation by the European Medicines Agency in 2007 for the treatment of Sarcoidosis. Both the US FDA and the EMEA have granted Investigational New Drug licenses for human trials of Aviptadil.