Lung Problems in Scleroderma: Symptoms, Causes, Diagnosis, Treatment - Verywell Health
Lung Problems in Scleroderma: Symptoms, Causes, Diagnosis, Treatment - Verywell Health |
| Lung Problems in Scleroderma: Symptoms, Causes, Diagnosis, Treatment - Verywell Health Posted: 21 Jun 2021 12:00 AM PDT /GettyImages-1286701913-266fa196e6d44184b5d2187208eb9234.jpg) Lung disease is a serious potential health condition that can develop in people with systemic sclerosis (scleroderma). Interstitial lung disease occurs in most, but not all people with the condition. It is the leading cause of death among people with systemic sclerosis. Pulmonary hypertension is another less common lung problem in systemic sclerosis. It is the second leading cause of death in people with the condition. Systemic SclerosisIn some forms of scleroderma, internal organs such as the lungs are not affected. Therefore, this article uses the more specific term "systemic sclerosis" instead. Milko / Getty Images Lung Issues From Systemic SclerosisPeople with systemic sclerosis can develop specific problems with their lungs. Interstitial Lung Disease SymptomsCough and shortness of breath are common symptoms in people with interstitial lung disease from systemic sclerosis. Tiredness and weakness can also occur. However, not everyone with interstitial lung disease from systemic sclerosis has symptoms, especially if their condition is mild. When the disease is severe, respiratory failure and death are possible. Pulmonary Hypertension SymptomsSystemic sclerosis can also cause elevated blood pressure in the blood vessels of the lungs (pulmonary hypertension). This often happens along with interstitial lung disease. However, some people with systemic sclerosis may have one condition and not the other. People who have both interstitial lung disease and pulmonary hypertension tend to develop worse disease symptoms and do not live as long as people who have just one condition. Pulmonary hypertension does not always cause symptoms at first. However, over time, symptoms like shortness of breath may develop and get worse. Pulmonary hypertension increases the work that your heart must do to pump blood through the vessels of the lungs. Therefore, a person may experience symptoms like chest pain and swelling of the extremities. If severe, this can eventually lead to life-threatening symptoms. Pulmonary HypertensionHigh blood pressure (hypertension) is not the same condition as pulmonary hypertension. When you hear that a person has high blood pressure, it means that the pressure is high in the blood vessels that go out to the rest of the body, not in the vessels going to the lungs, as is the case in pulmonary hypertension. Pulmonary hypertension also creates a different set of health issues than high blood pressure. CausesSystemic sclerosis is an autoimmune disease. Genetics and environmental factors likely play a role in triggering the disease, but much about the disease is unknown. Although researchers are not sure exactly what causes systemic sclerosis, some parts of the immune system appear to be overactivated in people who have the condition. The body starts reacting to parts of itself as if they were foreign invaders, which sets off inflammation and other immune system issues. In systemic sclerosis, part of the problem is the excess formation of scar tissue (fibrosis). Although this can be a normal part of the immune response in some circumstances, in people with systemic sclerosis the fibrosis gets out of control. Excess scar tissue can lead to problems all over the body. For example, it can cause hardened, thickened skin. Systemic sclerosis can also affect the internal organs. When fibrosis affects the gastrointestinal tract, it can cause issues like reflux. Fibrosis of other internal organs, such as the kidneys, can also cause health complications. In many people with systemic sclerosis, the lungs are also affected. Excess scar tissue in the lungs leads to interstitial lung disease, which causes the lung tissue to be more constricted than usual. Excess scar tissue can also damage the blood vessels in the lungs and lead to pulmonary hypertension. Risk FactorsNot everyone with systemic sclerosis develops interstitial lung disease. However, certain factors can increase a person's risk. Factors that might make it more likely that a person will get interstitial lung disease include:
Antibodies People who have a certain antibody—anti-Scl-70 (or anti-topoisomerase I)—are more likely to get interstitial lung disease. Not having another antibody—anti-centromere—increases a person's risk for interstitial lung disease. Symptoms and RisksPeople are also more likely to develop interstitial lung disease if they have not had other symptoms for a long time. If you've had systemic sclerosis symptoms for several years already, this lowers the risk that you'll ever experience problems from lung disease. Other Types of Interstitial Lung DiseasesOther types of autoimmune diseases can cause symptoms from interstitial lung disease. These diseases also can affect connective tissue (a sort of filler tissue in the body). These conditions all have some overlapping causes. For example, interstitial lung disease is sometimes (but not always) found in people with the following conditions: DiagnosisPeople with interstitial lung disease from systemic sclerosis are most often diagnosed after their initial diagnosis with systemic sclerosis. Everyone with systemic sclerosis should be screened for the possibility of lung disease. The screening will include a careful medical history and physical exam. Your doctor will assess your basic lung function—for example, by measuring how far you can walk in six minutes. The assessment should also include a complete set of pulmonary function tests, which show how well you breathe and how well your lungs are working overall. You should also have a lung imaging test called high-resolution computed tomography (HRCT), which is a very detailed CT scan. You might need to have an echocardiogram, which will give your doctor an idea of whether you might have pulmonary hypertension. It will also show whether pulmonary hypertension might be putting strain on your heart. Sometimes, the symptoms from the interstitial lung disease are noticed before those from systemic sclerosis (which is later identified as the underlying cause of the lung disease). If new symptoms emerge, such as Raynaud's syndrome or spider veins (telangiectasia), it can point to systemic sclerosis as a diagnosis. Monitoring for Interstitial Lung Disease in Systemic SclerosisEven if you do not have interstitial lung disease at your initial diagnosis of systemic sclerosis, your doctor will want to monitor you for it. Screening is especially important in the first few years after your symptoms appear because that's when it's most likely that interstitial lung disease will develop. During this time, you may need to have certain screening tests, like pulmonary function tests, every four to six months. By getting regular screenings, your doctor is more likely to catch lung problems early on. Early diagnosis is important because the sooner lung problems are treated, the more likely it is that lung function can be preserved for a longer time. TreatmentThe treatment options for interstitial lung disease from systemic sclerosis are limited. Treatments like supplemental oxygen may help some people, while other treatments may slow—but not cure— the disease. Immunosuppressants Corticosteroids are commonly prescribed as the initial therapy. If symptoms do not get better, additional treatments to tone down the immune system are necessary. The most commonly prescribed medications are Cytoxan (cyclophosphamide) and CellCept (mycophenolate mofetil). Both are heavy-duty immunosuppressive drugs that carry the risk of certain side effects, such as potentially increasing the chance of severe infections. Recently, another type of therapy, nintedanib, was approved by the Food and Drug Administration (FDA). This treatment helps slow the scarring in the lungs, which may help keep a person's lung function from declining as quickly. Other Prescription Medications Certain treatments may help with pulmonary hypertension that is caused by systemic sclerosis. However, it's not clear if these medications are effective specifically in people with pulmonary hypertension from systemic sclerosis. Doctors sometimes treat pulmonary hypertension from different causes with treatments, such as:
Stem Cells Some people with systemic sclerosis have successfully received stem cell transplantation as a treatment for severe lung disease. However, this treatment carries a significant risk of serious side effects, such as infections. Stem cell treatment for people with lung disease from systemic sclerosis is only available at a small number of medical centers. Lung Transplant Lung transplant is also a theoretical option, but it is not done often. People with systemic sclerosis typically have other serious health issues, and only a limited number of lung transplants are available. People with systemic sclerosis might be less likely to be selected to receive a set of donated lungs because doctors may worry that people with the condition will not be able to handle the stress of surgery and follow-up immunosuppressive treatment. ResearchResearchers are actively looking for better treatments for lung disease from systemic sclerosis which will more effectively treat the disease with less significant risks and potential side effects. Several therapies are currently being investigated. PrognosisThe prognosis for people with interstitial lung disease from systemic sclerosis is usually not good. People who have interstitial lung disease along with systemic sclerosis are much more likely to die from disease complications than people who do not have lung disease. Around 80% to 90% are alive five years after being diagnosed with interstitial lung disease from systemic sclerosis. The rate is lower in people who have both interstitial lung disease and pulmonary hypertension. However, some people with lung disease stabilize and live much longer. People who are treated early on for their lung disease may also do better than people who are treated later or who are not receiving treatment.  |
| Posted: 24 Jun 2021 05:00 AM PDT
DURHAM, N.C., June 24, 2021 (GLOBE NEWSWIRE) -- Aerami Therapeutics, Inc. today announced the initiation of dosing in the Phase 1 clinical trial of its drug-device combination product candidate, AER-901, an inhaled imatinib for the treatment of pulmonary arterial hypertension (PAH). "We are pleased to announce that the dosing of subjects in our Phase 1 trial is ongoing and that we expect to have results available later this year, which, combined with the completion of our long-term toxicology program, will allow us to quickly move into our planned Phase 2/3 trials targeted for early in 2022," said Steve Thornton, chief executive officer of Aerami. "We believe that AER-901 might, for the first time, provide the opportunity to modify the course of this insidious disease, and offer another important therapeutic option for patients with limited treatment options." Oral imatinib is a tyrosine kinase inhibitor used to treat certain types of cancers which has been previously investigated in a Phase 3 clinical trial, IMPRES, as an oral formulation for the treatment of PAH, given its potential to reduce the tightening and stiffness of pulmonary arteries. Oral imatinib demonstrated clinically meaningful and statistically significant improvement in pulmonary hemodynamics and physical exercise capacity but the indication was not pursued because of substantial adverse events believed to be a result of systemic toxicity associated with the high oral doses used in the study. "By delivering AER-901, Aerami's proprietary inhaled imatinib, directly to the site of the disease, we believe it has the potential to significantly reduce the dose necessary to achieve therapeutic benefit, thereby avoiding the adverse events seen with oral imatinib," said Timm Crowder, president of Aerami, "Our nebulized formulation and delivery system has the potential to improve efficacy through deeper lung penetration and better drug uptake while reducing the potential for side effects like cough, which is commonly associated with other inhaled technologies such as dry powder formulations." Earlier this year Aerami announced an exclusive license and development agreement with Hangzhou Chance Pharmaceuticals Co. Ltd. ("Chance") to develop and commercialize AER-901 in mainland China, Hong Kong, Macau and Taiwan. Chance has recently submitted a pre-IND meeting request to the Center for Drug Evaluation (CDE). About AER-901 AER-901 has been developed as a nebulized formulation to improve drug uptake and dosing consistency and to avoid or reduce commonly seen issues related to powdered forms for inhalation including cough, which would be problematic in this group of patients who suffer severe shortness of breath. AER-901 is designed to deliver consistent, therapeutically effective, and well tolerated levels of imatinib through once-a-day inhalation via the Fox® device, licensed from Vectura Group plc, which is both 510k cleared and CE marked. A version of this nebulizer is currently marketed to patients with a form of pulmonary arterial hypertension in Europe. The Fox®, a breath actuated, smart nebulizer, is expected to enable AER-901 to be deposited more deeply in the lungs and small airways than a powdered form. A high percentage of powdered inhaled products are deposited in the throat. The Fox® achieves deep lung deposition by administering drug only during inhalation and controlling the flow rate and volume during inspiration. About Pulmonary Arterial Hypertension (PAH) PAH is a devastating disease affecting almost 68,000 patients worldwide with limited treatment options. The disease causes blood vessels in the lungs to become narrowed, blocked or destroyed. This results in slowing of blood flow through the lungs and increases the blood pressure in the lung arteries. Over time, the heart must work harder to pump blood through the lungs, eventually causing the heart muscle to weaken and fail. PAH is often idiopathic and most commonly affects young women. Current therapeutic options focus mostly on vasodilation and have significant shortcomings including limited efficacy, inconsistent survival benefits, and significant side effects. Even with current medications, median survival is only seven to 10 years. About Aerami Therapeutics A clinical stage biopharmaceutical company developing inhaled therapies to treat severe respiratory and chronic diseases. More information can be found at www.aerami.com. This press release contains "forward-looking statements" concerning the development and commercialization of Aerami's product candidates, out-licensing arrangements, timing of clinical trials, the company's business development efforts and its expectations regarding its prospects. Forward-looking statements are subject to risks, assumptions and uncertainties that could cause actual future events or results to differ materially from such statements, including risks associated with the clinical development process and the ability to obtain funding to support planned clinical activities. These statements are made as of the date of this press release. Actual results may vary. Aerami undertakes no obligation to update any forward-looking statements for any reason. 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