A comprehensive overview of sarcoidosis covering subjects like symptoms, diagnostics, research, causes and pictures
Sarcoidosis (the Besnier-Boeck-Schumann disease) affects various organs and tissues (lungs, skin, eyes, spleen, liver, peripheral lymph nodes, parotid glands, muscles, nervous system), but heart too, causing cardiac sarcoidosis. The heart of the patients affected by sarcoidosis is affected very rare, occurring heart palpitations or cardiac insufficiency.
The heart can be affected directly, by myocardial granulomas or indirectly, by affecting the lungs causing a chronic pulmonary cord. In direct affection, the disease is more restrictive due to the granulomatous infiltration, but it can also have the characters of a dilated cardiomyopathy by decreasing the contractility and raises the cavities.
Signs and symptoms of cardiac sarcoidosis
Rhythm and driving disturbances are frequently reported. Also, the ventricular tachycardia and the complex heart block are resistant to the treatment. Syncope and sudden cardiac death can sometimes be the first manifestation of cardiac sarcoidosis.
Cardiomyopathy from sarcoidosis can lead to progressive heart failure and resistance to treatment. Heart failure can occur in chronic pulmonary cord due to pulmonary hypertension in pulmonary sarcoidosis. Most often, cardiac sarcoidosis is associated with other systemic manifestations: hepatomegaly, splenomegaly, lung damage, etc.
Cardiac manifestations occur in 5% of cases especially to young people and they are clinically asymptomatic. There is possible the appearance of arrhythmias, sudden heart arrest and dead.
Para clinical explorations
Electrocardiogram - is sensitive to approximately 50% of cases. It can be registered: driving and rhythm disturbances (most commonly), repolarization abnormalities, or Q pathological waves regressive in glucocorticoids, without significant scar.
Myocardial perfusion scintigraphy - it reveal areas or diffuse heterogeneity due to granulomatous infiltration.
Endomyocardial biopsy - is very useful in isolated cardiac sarcoidosis, it can be normal or nonspecific granulomas when are not distributed homogeneously
Cardiac sarcoidosis treatment
Treatment is with steroids and is being achieved the regression of granulomatous infiltration and it prevents the formation of new granulomas. The expected effects are obtained after ca. 3 months of treatment with prednisone (50 mg / day).The chances of recovery increase if corticotherapy is instituted early. It is also the treatment of heart failure, rhythm disturbances and other management or complications.
Untreated, cardiac sarcoidosis produces death in 2 years, usually by ventricular arrhythmias or by heart blocking.
Cardiac sarcoidosis is present in 25% of patients with systemic sarcoidosis, but symptoms related to the cardiac sarcoidosis occur only in 5% of patients with systemic sarcoidosis. 50% of patients with granulomatous inflammation of the heart have symptoms of the extra cardiac sarcoidosis. In a study that included 26 patients in the cardiac sarcoidosis was suspected based on clinical diagnostic criteria, ECG abnormalities or noninvasive imaging, granulomas being found only in 19.2% of patients, a situation that confirmsthe old reports in which were confirming the sensitivity of EMB in the sarcoidosis 20-30%. Because the heterogeneity myocardial distribution of cardiac sarcoidosis disease, it may occur sampling error, and thus the diagnose rate of the EMB decreases. In patients with pulmonary sarcoidosis proven by biopsy, MRI was used to describe the cardiac involvement in order to define the activity of the disease.
Although the rate of EMB in diagnosing cardiac sarcoidosis is small, it is important to distinguish histological between cardiac sarcoidosis and GCM, both with giant cells in the prognosis and in the therapeutic decision. Immediate and remote prognosis of sarcoidosis is favorable in most cases. Death is rarely, being mainly due to respiratory and cardiac insufficiency.