The heart in sarcoidosis: Difference between revisions
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*Genetic factors are also believed to have a role in the pathogenesis of cardiac sarcoidosis | *Genetic factors are also believed to have a role in the pathogenesis of cardiac sarcoidosis | ||
*positive association with cardiac sarcoidosis has been reported with HLA‐DQB1*0601 | *positive association with cardiac sarcoidosis has been reported with HLA‐DQB1*0601 | ||
*ACE gene polymorphism as well as tumour necrosis factor α promoter gene polymorphism have also been evaluated as a potential marker for an increased risk of sarcoidosis<br /> | *ACE gene polymorphism as well as tumour necrosis factor α promoter gene polymorphism have also been evaluated as a potential marker for an increased risk of sarcoidosis | ||
*No portion of the heart is immune to infiltration by sarcoid granulomas.<br /> | |||
==Differentiating Cardiac sarcoidosis from Other Diseases== | ==Differentiating Cardiac sarcoidosis from Other Diseases== | ||
Revision as of 14:23, 6 November 2019
| The heart in sarcoidosis | |
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| The heart in sarcoidosis: Moderately dilated left ventricle. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor: Cafer Zorkun, M.D., Ph.D. [2] ; Huda A. Karman, M.D.
Keywords: cardiac sarcoidosis, sarcoidosis cordis
Overview
Pericarditis as a manifestation of sarcoidosis has been frequently described and necropsy studies have documented cardiac involvement in 27% of patients, but clinically significant pericarditis is uncommon. In addition, sarcoidosis has been rarely documented in children. The granulomatous infiltrative disease of the myocardium is often asymptomatic, but can cause arrhythmias, conduction disease and, rarely, otherwise unexplained congestive heart failure. Early diagnosis can be very important because it's generally believed aggressive steroid treatment may decrease mortality. Granulomatous infiltration may be patchy, with a predilection toward involvement of the left ventricle, particularly the upper septal area. This distribution influences the likelihood of obtaining a diagnostic right sided endomyocardial biopsy. Use of gallium or thallium imaging may be helpful in determining the need for and duration of immunosuppressive therapy, but this approach has not been proved in any formal trial[1] [2][3] [4]. Sarcoid dilated cardiomyopathy may be difficult to distinguish from idiopathic cardiomyopathy or occasionally from giant cell myocarditis. Conduction disease is more common than pump dysfunction in patients with sarcoidosis. Biopsy may help distinguish sarcoidosis from idiopathic or giant cell myocarditis, but the diagnostic yield of endomyocardial biopsy is low. Active sarcoidosis is generally believed to be steroid responsive. However, myocardial involvement with sarcoid can result in large patches of fibrotic scar that may be arrhythmogenic but no longer respond to steroids. Scar is often significantly underestimated by imaging studies and biopsy. Pulmonary artery hypertension and cor pulmonale can occur in sarcoidosis, generally as a result of pulmonary fibrosis.Systemic vasculitis is an uncommon complication of sarcoidosis. Its prevalence remains unknown. Sarcoid vasculitis can affect small to large caliber vessels, including the aorta. The latter presentation can be easily confused with Takayasu arteritis. African American patients appear predisposed to developing large vessel involvement. [5] [6] [7] [8] [9]
Historical perspective
- In 1869 Jonathan Hutchinson described the first case of cutaneous sarcoid [10]
- In 1899 the disease was named by Boeck, a Norwegian dermatologist, who thought that the nodular skin lesions of epithelioid cells resemble sarcoma cells and descried them as sarcoid
- In 1929, Bernstein was the first to recognise cardiac involvement in a patient with systemic sarcoidosis
- In 1952, Longcope and Freiman were the first to describe myocardial involvement in 20% of 92 necropsied cases of sarcoidosis
Pathophysiology
- It is though that antigens, non-self and self trigger primarily the helper inducer T cells leading to the formation of granuloma lesions
- During the early stage of the disease, sarcoid infiltrates consist of mononuclear phagocytes and CD4 positive T cells with a T helper type I response, secreting interleukin‐2 and interferon‐γ.
- During the late of the disease, it shifts to T helper type 2 response which has been demonstrated during the fibroproliferative phase of the granuloma and is believed to exert anti‐inflammatory effects and result in tissue scarring
- high concentrations of interleukin‐6 were found in the circulation at the onset of the disease and before the initiation of immunosuppressive therapy, but not thereafter
- Interleukin‐6 is thought to be involved in the maintenance of inflammation by inducing the proliferation of T cells
- Genetic factors are also believed to have a role in the pathogenesis of cardiac sarcoidosis
- positive association with cardiac sarcoidosis has been reported with HLA‐DQB1*0601
- ACE gene polymorphism as well as tumour necrosis factor α promoter gene polymorphism have also been evaluated as a potential marker for an increased risk of sarcoidosis
- No portion of the heart is immune to infiltration by sarcoid granulomas.
Differentiating Cardiac sarcoidosis from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Diagnosis
Cardiac MRI
Common MRI findings in patients with cardiac sarcoidosis include:
- Delayed enhancement, which may be either mid wall or transmural
- Nodular mid wall hyperintense foci on black blood T2-weighted imaging
- Areas of focal myocardial thickening.
- Disease may involve either the left or the right ventricle but more commonly involves the left ventricle.
Treatment
References
- ↑ Braunwald's Heart Disease 8th Ed, 2007, Libby P
- ↑ "Sarcoidosis: eMedicine Pediatrics: General Medicine".
- ↑ Mayo Clinic Cardiology, Concise Textbook, 3rd edition, 2007
- ↑ Hurst's The Heart, Fuster V, 11th (printed) and 12th (online) editions, 2004-2008
- ↑ Harris: Kelley's Textbook of Rheumatology, 7th ed. 2005
- ↑ Robbins and Cotran PATHOLOGIC BASIS OF DISEASE, 7th Edition, 2005
- ↑ Washington Manual of Medical Therapeutics, The, 32nd Edition, 2007
- ↑ Cecil Textbook of Medicine, 23rd Edition, 2007
- ↑ Harrison's Principals of Internal Medicine, 16th Edition, 2005
- ↑ Doughan AR, Williams BR (2006). "Cardiac sarcoidosis". Heart. 92 (2): 282–8. doi:10.1136/hrt.2005.080481. PMC 1860791. PMID 16415205.