In cases of primary pulmonary hypertension or secondary to scleroderma, endothelin (which has a vasoconstrictive action) is increased. In such cases, there is an increase in pressure inside the blood vessels that carry blood from the heart to the lungs.
Endothelin is a substance present in the body, essential for the regulation of blood vessel activity. Its action is mainly related to the contraction of blood vessels.
In healthy people, the concentration of the substance is small and its action occurs in a balanced way. It then acts by regulating the caliber of blood vessels so that blood flow is adequate for the whole body in different situations, for example, at rest and during physical activity.
However, if there is a change in the organism, with an increase in the presence of endothelin, there is a greater action of vasoconstriction and proliferation of smooth muscle cells.
When exaggerated vasoconstriction occurs, blood flow through the pulmonary arteries may be reduced, limiting the arrival of blood to the lungs and, consequently, the distribution of oxygen to the body is reduced.
Patients then suffer from symptoms such as shortness of breath and extreme tiredness . For treatment, among other options available, there is the drug Bosentana, which can bring significant improvements to the lives and well-being of patients.
What is the medicine Bosentana for?
According to the package leaflet, the substance Bosentana is indicated to treat people diagnosed with pulmonary arterial hypertension, classified in group I, according to WHO, who are in functional classes II, III and IV, also according to WHO.
In these cases, the medication helps to improve physical capacity and to reduce the clinical worsening of the symptoms.
The package insert also indicates the use for reducing the occurrence of new digital ulcers in people with systemic sclerosis and active digital ulcers. Learn more about each condition:
Decrease the worsening of pulmonary arterial hypertension
Pulmonary arterial hypertension is a progressive disease that causes increased pressure in the pulmonary artery due to the high constriction of arterial vessels. This, over time, leads to overload and damages the right ventricle.
The pulmonary artery carries oxygen-poor (venous) blood from the heart to the lungs. Thus, when there is a dysfunction in this system, a series of symptoms can manifest.
The causes can be secondary, arising from other conditions or diseases, idiopathic or hereditary. Considering this variety of factors, the most recent classificatory adoption by the World Health Organization indicates that the diagnosis of Pulmonary Hypertension is performed in 5 groups.
However, only patients in group I are indicated, according to the package leaflet, for the use of Bosentana.
In this group are the cases of idiopathic, hereditary pulmonary arterial hypertension, induced by drugs or toxins, associated with connective tissue diseases, HIV infection , portal hypertension, congenital heart disease and schistosomiasis .
In addition, the picture must belong to class II, III or IV. Level I indicates the presence of pulmonary hypertension (PH), but without compromising daily activities, with little or slight limitation to the routine. But only patients from the other classes receive indication of use. These tables are characterized by:
- Class II : patients with PH, who have few symptoms at rest, but common activities generate excessive fatigue and difficulties in performing;
- Class III : patients with PH with significant limitation of physical activities. In general, there are no symptoms during rest, but small movements can cause dyspnea (shortness of breath or difficult breathing) or excessive fatigue, chest pain or pre-syncope;
- Class IV : patients with PH who are severely limited and unable to perform any physical activity due to symptoms. They can even occur during rest.
Reduction of new digital ulcers in systemic sclerosis
Systemic sclerosis is a chronic inflammatory disease that affects connective tissue and is related to autoimmune factors. In general, it consists of a thickening of the skin, which becomes stiff and darker.
However, the disease can affect several tissues besides the skin, including blood vessels. In these cases, the vascular wall is less dilated, reducing blood flow.
External factors, such as cold, cause the vessels to naturally contract. However, in people affected by scleroderma, this and other external changes can severely compromise the already reduced blood circulation.
This causes the ends of the fingers and toes to receive less blood and become whitish or purplish.
With the repetition of blood loss, wounds or changes in the region of the fingers, called digital ulcers, may occur.
Studies indicate that, although Bosentana does not present improvements in digital ulcers already present, its use can reduce the manifestation of new ones.
What is the mechanism of action?
In people with pulmonary arterial hypertension, there is a high presence of endothelins that bind to their receptors and trigger the contraction of blood vessels.
Bosentana is a medication called Endothelin receptor blocker, this causes the endothelin to not bind to the receptors and does not maintain or cause vasoconstriction, which allows the dilation of the vessel and the reduction of pulmonary arterial pressure.
In studies conducted on the effects of Bosentana in patients with digital ulcers resulting from systemic sclerosis, it was observed that the treatment helped to reduce the appearance of new digital ulcers, although there was no improvement in the healing time of those already manifested.
Generic or reference?
According to Anvisa, the reference drug based on Bosentana is the one supplied by the Actelion industry, registered in 2013, in the available presentations of 62.5mg and 125mg.
The choice of generic medication or reference is always based on the medical assessment and the person’s responses to treatment. Therefore, it is important to talk to professionals who are accompanying the disease, to clarify doubts and make the necessary choice or modification.
Is Bosentana Actelion generic or reference?
According to the December 2019 update of Anvisa, the regulatory body, the Actelion laboratory is registered, under the Tracleer brand, as a Bosentana Reference.
What side effects are on the label?
Treatment with Bosentana, like any other drug therapy, may involve adverse effects. These manifestations can be variable and will not necessarily occur. However, it is important to know that, in clinical studies, the following adverse effects have been reported in common or very common frequency:
- Abnormal liver function tests;
- Edemas (such as swelling or signs of fluid retention);
- Anemia (reduction in the rate of red blood cells) or decrease in hemoglobin;
- Flushed appearance;
- Hypersensitivity reactions (such as skin inflammation, itching and rash);
- Acid reflux (gastroesophageal reflux disease);
- Redness of the skin.
In addition, the package leaflet indicates that some symptoms of common manifestation were reported after the drug was marketed, namely:
- Syncope (fainting);
- Palpitations (rapid or irregular heartbeat);
- Low blood pressure;
- Nasal congestion.
It is also important to mention that the manifestation of some specific symptoms indicates the need for emergency medical assistance, as liver function may be impaired. Are they:
- Nausea (feeling like vomiting);
- Fever (high temperature);
- Pain in the stomach (abdomen);
- Jaundice (yellowing of the skin or bleaching of your eyes);
- Dark colored urine;
- Itchy skin;
- Lethargy or fatigue (unusual tiredness or exhaustion);
- Flu syndrome (joint pain and muscle pain with fever).
Contraindications: what are the precautions with Bosentana?
Some conditions must be reported to the doctor before starting treatment with Bosentana. It is important that professionals are aware of the entire clinical condition of the person being treated, in order to avoid aggravating factors.
According to the package insert, the use of the bosentan drug is contraindicated when there is:
- Hypersensitivity to the active ingredient or to any component of the formula;
- Concomitant use of cyclosporine A;
- Women of childbearing potential who are not using reliable contraceptive methods (the use of hormonal contraceptives exclusively for contraception is not effective for treatment with Bosentana).
The label highlights that the use of hormonal contraceptives alone is not safe and effective to prevent pregnancy during treatment, and this includes the oral, injectable, transdermal and implant types.
Bearing in mind that a pregnancy can worsen the condition of pulmonary arterial hypertension, then it is very important that women talk to professionals in gynecology, adopting appropriate methods of protection.
In addition, the drug is contraindicated for children under 3 years old, and the safety and efficacy of the drug have not been established in this age group.
What is the price of Bosentana 62.5mg and 125mg?
The Bosentana substance can vary in price according to the generic category or reference. On average, the generic drug from Actelion can be found from R $ 4,970, while the reference Tracleer is from R $ 16,070 *.
* Prices consulted in January 2020. Prices may change.
Does SUS provide?
Yes. Bosentan is part of the National List of Medicines of the Specialized Component of Pharmaceutical Assistance, updated in 2018, being made available to patients who meet the necessary criteria for supply.
It is worth remembering that patients need to seek information from the medical team, as each state may have specific criteria and procedures for free drug release.
How to make a budget of Bosentana?
Patients with a prescription for Bosentana can use the public health system, SUS, or health plans to carry out treatment free of charge.
The substance is part of Rename, which is the National List of Medicines of the Specialized Component of Pharmaceutical Assistance, which means that, in principle, it can be obtained free of charge by the person being treated, being paid by the government.
However, if for any reason (lack of medication from the supplying unit or rejection of the therapeutic request), the substance is not dispensed, patients may resort to legal remedies for a new attempt. This also includes purchasing via private health insurance.
In order to open the process, it is necessary to present some documents, such as medical report and supporting exams, in addition, it is necessary to attach 3 quotes from different pharmacies.
Budgets are a fundamental part of the documentation, and it often becomes a difficult or laborious process for patients.
To make it easier, the Consulta Remédios group offers a simple personalized legal quote service. Just access the link and fill out the form. In a practical way, you obtain the necessary budgets for the continuation of the judicial process.
Bosentana is a substance indicated for pulmonary vascular resistance, in cases of pulmonary arterial hypertension and, also, to control the appearance of digital ulcers resulting from systemic sclerosis.
The medicine comes in tablets, with dosages of 62.5mg and 125mg, which should be taken according to medical advice, combining routine examinations and constant monitoring.
Studies indicate good responses in therapy, with the best tolerance to physical exercises being observed due to the reduction of vascular constriction.
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