Is Sarcoidosis A Rare Disease – The heart can be affected by sarcoidosis in two ways. First, sarcoidosis can occur in the heart muscle itself (cardiac sarcoidosis). Second, the heart may be indirectly affected by sarcoidosis in the lungs (pulmonary hypertension). Both of these conditions can have serious consequences.
The information on this page was compiled with the help of sarcoidosis experts Dr V. Kouranos, Dr. L. Williams & Dr. S. Agarwal, Consultant Cardiologists, Dr. K. Tweed, Consultant Radiologist and Dr. M. Thillai, Consultant Chest Physician (All Cardiac Sarcoidosis Stations, Royal Papworth Hospital) and additional comments from Dr. M. Wickremasinghe, Consultant Respiratory Physician and Dr. A. Varnava, Consultant Cardiologist (both Imperial College Healthcare NHS Trust).
Is Sarcoidosis A Rare Disease
The heart can be affected by sarcoidosis in two ways. First, sarcoidosis can affect the heart muscle itself (cardiac sarcoidosis). Second, the heart may be indirectly affected by sarcoidosis in the lungs (pulmonary hypertension). Both of these conditions can have serious consequences.
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Cardiac sarcoidosis occurs when the heart muscle itself is affected. Accumulation of immune cells causes clusters of cells called granulomas. Myocardial granulomatous inflammation can involve the heart in different areas:
Cardiac sarcoidosis has been reported in a third of all sarcoidosis patients, but it only causes certain symptoms (arrhythmias, heart failure, etc.) in about 5 percent of cases.
International guidelines suggest the performance of a 12-lead ECG in all patients with sarcoidosis as a diagnostic tool in addition to obtaining a history of cardiac symptoms. A Holter monitor and echocardiogram will be performed on a case-by-case basis. All patients with suspected cardiac sarcoidosis should undergo cardiac MRI and/or cardiac FDG-PET. The available diagnostic tests are described below:
Most patients with newly diagnosed sarcoidosis will have an ECG, Holter monitor and echocardiogram. Patients with suspected cardiac sarcoidosis may continue to undergo additional diagnostic tests. Most of them are described below.
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ECG (Electrocardiogram): This provides information about the heart’s electrical system, including heart rate and rhythm. Abnormalities of the conduction system and ventricular tachycardia can be identified in cardiac patients with sarcoidosis.
Holter monitor: A Holter monitor is a small, portable ECG device that takes a continuous record of your heart rhythm over a long period of time (usually 24 hours but sometimes longer). It is very important to identify any rhythm disturbances that may or may not be related to your symptoms (for example, a very fast or slow heartbeat or any irregular heartbeat).
Echocardiogram (Echo): This uses ultrasound waves to create images that show the pumping action and structure of the heart muscle and the function of the heart valves. It also allows measuring the blood pressure in the arteries of the heart (looking for pulmonary blood pressure).
Magnetic Resonance Imaging (MRI): This is the most important diagnostic tool for detecting sarcoidosis involvement in the heart. As part of the protocol you will be injected with a special dye. Delayed dye enhancement in myocardial tissue suggests damaged areas (fibrosis and/or inflammation) of the heart muscle. MRI images also provide accurate information (even higher than echocardiography) about myocardial structure and function.
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Cardiac Flurodeoxyglucose – Positron Emission Tomography (FDG-PET): FDG-PET scans create images that can identify active inflammation in the heart muscle and can help guide immunosuppressive therapy. Special food preparation and long-term fasting (18 hours) are required for cardiac FDG-PET scan. (this will be explained to you beforehand).
Nuclear Medicine Scans: Thallium scintigraphy or Rubidium perfusion scans are used to detect myocardial damage in the heart muscle. They are often combined with FDG-PET cardiac imaging.
Clinical Electrophysiology Studies: A catheter is used to map the cardiac system. This can reveal abnormalities that may cause an abnormally slow heart rate, as well as any abnormally fast or dangerous heart rhythms that may occur in areas of inflammation or scarring.
Biopsy of the Heart: This option will be chosen in rare cases when the diagnosis of sarcoidosis of the heart is not supported by normal signs or imaging.
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Timely diagnosis and medical treatment are important for a good prognosis. Your doctor may prescribe medications to prevent the inflammation associated with sarcoidosis. These drugs prevent inflammation but do not treat the heart itself.
You may be given medication to help control your heart rhythm or treat heart failure. Some basic medications include:
Patients with severe heart failure or severe ventricular arrhythmias will require a defibrillator (ICD) as secondary prophylaxis. An ICD can also be implanted in patients with abnormal conduction patterns or who are at high risk of a major arrhythmic event.
Pulmonary hypertension refers to high blood pressure in the pulmonary arteries; vessels that supply blood to the lungs. It is a serious condition that can damage the right side of the heart. It is more common in sarcoidosis patients with advanced lung or heart disease as well as patients with large or persistent bleeding in the lungs. However, it can also occur as a result of direct inflammation of the pulmonary vessels as well as obstruction of blood flow in the pulmonary arteries from enlarged mediastinal lymph nodes. Increased pressure in the blood vessels in the lungs can overload the right ventricle and result in right ventricular heart failure. This can occur in up to 15% of all patients with sarcoidosis.
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Echocardiogram (Echo): This uses ultrasound waves to create images that show the pumping function and structure of the heart’s chambers and the function of the heart’s valves. It also allows measuring the blood pressure in the coronary arteries (for pulmonary hypertension). The decision to perform a right heart catheterization depends largely on the level of suspicion of pulmonary hypertension based on an echocardiogram.
Right Heart Catheterization: This test is the gold standard test for diagnosing pulmonary hypertension. It uses a device to measure the pressures in the heart and nearby blood vessels.
Timely diagnosis and medical treatment are important for a good prognosis. It is very important to know the exact cause of sarcoidosis associated with pulmonary hypertension as this can have many factors. Treatment decisions depend on the underlying cause and may include specific treatment for sarcoidosis, treatment for heart failure or even anticoagulation.
Want to find a mentor? Use our directory to find a sarcoidosis specialist or clinic near you. Sarcoidosis (also known as Besnier–Boeck–Schaumann disease) is a disease associated with an abnormal collection of inflammatory cells that form lumps known as granulomata.
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Others may have Löfgr’s syndrome with fever, enlarged lymph nodes, arthritis, and a rash known as erythema nodosum.
Others believe that it may be due to the immune system’s response to a stimulus such as an infection or chemicals in those who are naturally allergic.
Findings that make it possible include enlarged lymph nodes at the base of the lung on both sides, high blood calcium with a normal parathyroid hormone level, or high levels of angiocin-converting zyme in the blood .
Medicines such as methotrexate, chloroquine, or azathioprine can sometimes be used to try to reduce the side effects of steroids.
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In 2015, pulmonary sarcoidosis and interstitial lung disease affected 1.9 million people worldwide and caused 122,000 deaths.
Sarcoidosis is a systemic inflammatory disease that can affect any organ, although it may not have symptoms and is found by accident in about 5% of cases.
Common, vague symptoms include fatigue (unrelieved by sleep; occurs in up to 85% of cases.
Arthritis (14-38% cases), dry eyes, swollen knees, blurred vision, difficulty breathing, dry cough, or skin lesions.
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Sarcoidosis is also accompanied by stress and symptoms of anxiety and depression, which are associated with fatigue.
Skin symptoms vary, and range from a rash with noduli (small lumps) to erythema nodosum, granuloma annulare, or lupus pernio. Sarcoidosis and cancer can mimic each other, making the distinction difficult.
The combination of erythema nodosum, bilateral hilar lymphadopathy, and joint pain is called Löfgr syndrome, which has a relatively good prognosis.
This type of disease occurs more often in Scandinavian patits than in non-Scandinavian ones.
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Overall, about 50% develop chronic lung problems, and 5 to 15% have progressive “fibrosis” of the lung parchyma. Pulmonary sarcoidosis is mainly an interstitial lung disease where the inflammatory process affects the alveoli, small bronchi and small blood vessels.
The upper respiratory tract (including the larynx, pharynx and sinuses) may be affected, occurring in between 5 and 10% of cases.
Four stages of pulmonary involvement are based on the radiological status of the disease, which helps in prognosis:
The use of the Scadding scale only provides general information about the behavior of pulmonary disease over time. Caution is recommended,
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Sarcoidosis affects the skin in 9 and 37% of cases and is more common in African Americans than in European Americans.
Treatmt is not required, as the lesions usually heal on their own after 2-4 weeks. Although it can be disfiguring, cutaneous sarcoidosis rarely causes serious problems.
Histologically, cardiac sarcoidosis is a granulomatous inflammation surrounded by active edema. The distribution of affected areas is not dynamic and is localized
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