The heart in sarcoidosis: Difference between revisions
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* 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 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 recognize [[cardiac]] involvement in a patient with systemic sarcoidosis | * In 1929, Bernstein was the first to recognize [[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<br /> | * In 1952, Longcope and Freiman were the first to describe [[myocardial]] involvement in 20% of 92 necropsied cases of sarcoidosis | ||
*The Japanese Ministry of Health and Welfare (JMH) published [[diagnostic]] guidelines for [[cardiac]] sarcoidosis in 1993 and were updated in 2006<ref name="pmid191754483">{{cite journal| author=Soejima K, Yada H| title=The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. | journal=J Cardiovasc Electrophysiol | year= 2009 | volume= 20 | issue= 5 | pages= 578-83 | pmid=19175448 | doi=10.1111/j.1540-8167.2008.01417.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19175448 }}</ref>. The guidelines require [[myocardial]] [[biopsy]] for [[histologic]] confirmation of [[cardiac]] involvement or clinical confirmation via a of major and minor criteria combination<ref name="pmid161693243">{{cite journal| author=Ardehali H, Howard DL, Hariri A, Qasim A, Hare JM, Baughman KL et al.| title=A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. | journal=Am Heart J | year= 2005 | volume= 150 | issue= 3 | pages= 459-63 | pmid=16169324 | doi=10.1016/j.ahj.2004.10.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16169324 }}</ref><br /> | |||
==Pathophysiology== | ==Pathophysiology== | ||
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*Japanese Ministry of Health and Welfare created guidelines to diagnose cardiac sarcoidosis: | *Japanese Ministry of Health and Welfare created guidelines to diagnose cardiac sarcoidosis: | ||
*The guidelines are based on the histologic and clinical diagnosis | *The guidelines are based on the histologic and clinical diagnosis | ||
* | *Serum markers that have been reported as markers of sarcoidosis in general are: | ||
** Serum amyloid A (SAA) | |||
** Soluble interleukin-2 receptor (sIL-2R) | |||
** Lysozyme | |||
** Angiotensin-converting enzyme (ACE) | |||
** Gycoprotein KL-6 | |||
** Hypercalcemia | |||
** Hypercalciuria | |||
** (noncaseating granulomas secrete 1,25 vitamin D) | |||
*Electrocardiography | *Electrocardiography | ||
*electrocardiographic changes that may mimic transmural myocardial infarction | *electrocardiographic changes that may mimic transmural myocardial infarction | ||
*Echocardiography | *Echocardiography | ||
*Gold standard test: | *Gold standard test: Endomyocardial biopsy | ||
* | * | ||
* | * | ||
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**left ventricular end-diastolic diameter | **left ventricular end-diastolic diameter | ||
**Sustained VT | **Sustained VT | ||
**Greater survival rates are in patients with preserved left ventricular function and good NYHA functional class<br /> | **Greater survival rates are in patients with preserved left ventricular function and good NYHA functional class | ||
**Treatment | |||
*** Corticosteroid treatment | |||
*** Antiarrhythmic treatment | |||
*** Pacemakers and defibrillators | |||
*** Cardiac transplantation <br /> | |||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;" | [[Cardiac amyloidosis]]'''<ref name="pmid25997029">{{cite journal| author=Fontana M, Banypersad SM, Treibel TA, Abdel-Gadir A, Maestrini V, Lane T et al.| title=Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study. | journal=Radiology | year= 2015 | volume= 277 | issue= 2 | pages= 388-97 | pmid=25997029 | doi=10.1148/radiol.2015141744 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25997029 }}</ref>'''<ref name="pmid29700090">{{cite journal| author=Martinez-Naharro A, Hawkins PN, Fontana M| title=Cardiac amyloidosis. | journal=Clin Med (Lond) | year= 2018 | volume= 18 | issue= Suppl 2 | pages= s30-s35 | pmid=29700090 | doi=10.7861/clinmedicine.18-2-s30 | pmc=6334035 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29700090 }}</ref> | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;" | [[Cardiac amyloidosis]]'''<ref name="pmid25997029">{{cite journal| author=Fontana M, Banypersad SM, Treibel TA, Abdel-Gadir A, Maestrini V, Lane T et al.| title=Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study. | journal=Radiology | year= 2015 | volume= 277 | issue= 2 | pages= 388-97 | pmid=25997029 | doi=10.1148/radiol.2015141744 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25997029 }}</ref>'''<ref name="pmid29700090">{{cite journal| author=Martinez-Naharro A, Hawkins PN, Fontana M| title=Cardiac amyloidosis. | journal=Clin Med (Lond) | year= 2018 | volume= 18 | issue= Suppl 2 | pages= s30-s35 | pmid=29700090 | doi=10.7861/clinmedicine.18-2-s30 | pmc=6334035 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29700090 }}</ref> | ||
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*** Diffuse deposition of amorphous hyaline material (nodular pattern - 8 to15 nm in diameter), in mesangium (weakly staining with periodic acid-Schiff (PAS) | *** Diffuse deposition of amorphous hyaline material (nodular pattern - 8 to15 nm in diameter), in mesangium (weakly staining with periodic acid-Schiff (PAS) | ||
*** <br /> | *** <br /> | ||
| style="padding: 5px 5px; background: #F5F5F5;" |Imaging findings: | | style="padding: 5px 5px; background: #F5F5F5;" |Imaging findings: | ||
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* Dexamethasone plus Cyclophosphamide-thalidomide | * Dexamethasone plus Cyclophosphamide-thalidomide | ||
|- | |- | ||
|} | |} | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* Sarcoidosis granuloma formation involves most commonly the lungs<ref name="pmid9012596">{{cite journal| author=Rybicki BA, Major M, Popovich J, Maliarik MJ, Iannuzzi MC| title=Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. | journal=Am J Epidemiol | year= 1997 | volume= 145 | issue= 3 | pages= 234-41 | pmid=9012596 | doi=10.1093/oxfordjournals.aje.a009096 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9012596 }}</ref> | * Sarcoidosis [[granuloma]] formation involves most commonly the [[lungs]]<ref name="pmid9012596">{{cite journal| author=Rybicki BA, Major M, Popovich J, Maliarik MJ, Iannuzzi MC| title=Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. | journal=Am J Epidemiol | year= 1997 | volume= 145 | issue= 3 | pages= 234-41 | pmid=9012596 | doi=10.1093/oxfordjournals.aje.a009096 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9012596 }}</ref> | ||
*70% of sarcoidosis occurs in patients 25 to 60 years of age | *70% of sarcoidosis occurs in patients 25 to 60 years of age | ||
*Sarcoidosis is rare in people <15 or >70 years of age | |||
*The [[prevalence]] of sarcoidosis is about 4.7 to 64 in 100,000 | |||
*Sarcoidosis highest rates are reported in northern Europeans and African Americans, mainly in [[women]] | |||
*Clinically manifest [[cardiac]] involvement occurs in about 5% of patients with sarcoidosis | |||
*Studies say that [[cardiac]] sarcoidosis seems to be becoming more [[Prevalence|prevalent]] than before | |||
*In Finland, the rate of sarcoidosis has increased more than 20-fold between 1988 and 2012 <ref name="pmid27443438">{{cite journal| author=Birnie DH, Nery PB, Ha AC, Beanlands RS| title=Cardiac Sarcoidosis. | journal=J Am Coll Cardiol | year= 2016 | volume= 68 | issue= 4 | pages= 411-21 | pmid=27443438 | doi=10.1016/j.jacc.2016.03.605 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27443438 }}</ref> | |||
*In the United States, the [[incidence]] of patients with sarcoidosis-induced [[cardiomyopathy]] who underwent [[transplantation]] increased from 0.1% (1994 to 1997) to 0.5% (2010 to 2014)<ref name="pmid25527698">{{cite journal| author=Kandolin R, Lehtonen J, Airaksinen J, Vihinen T, Miettinen H, Ylitalo K et al.| title=Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. | journal=Circulation | year= 2015 | volume= 131 | issue= 7 | pages= 624-32 | pmid=25527698 | doi=10.1161/CIRCULATIONAHA.114.011522 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25527698 }}</ref> | |||
* | |||
*In patients with clinically evident sarcoidosis, cardiac involvement has been reported in at least 2-7% of them<ref name="pmid20665393">{{cite journal| author=Nunes H, Freynet O, Naggara N, Soussan M, Weinman P, Diebold B et al.| title=Cardiac sarcoidosis. | journal=Semin Respir Crit Care Med | year= 2010 | volume= 31 | issue= 4 | pages= 428-41 | pmid=20665393 | doi=10.1055/s-0030-1262211 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20665393 }}</ref> | |||
*Occult cardiac involvement has a much higher rate (> 20%)<ref name="pmid1067031">{{cite journal| author=Matsui Y, Iwai K, Tachibana T, Fruie T, Shigematsu N, Izumi T et al.| title=Clinicopathological study of fatal myocardial sarcoidosis. | journal=Ann N Y Acad Sci | year= 1976 | volume= 278 | issue= | pages= 455-69 | pmid=1067031 | doi=10.1111/j.1749-6632.1976.tb47058.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1067031 }}</ref> | |||
*In the United States and United Kingdom, several necropsy series cited pathological evidence for cardiac involvement in 19.5 to 28% of sarcoid patients<ref name="pmid7848065">{{cite journal| author=Perry A, Vuitch F| title=Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations. | journal=Arch Pathol Lab Med | year= 1995 | volume= 119 | issue= 2 | pages= 167-72 | pmid=7848065 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7848065 }}</ref> | |||
*The incidence of cardiac sarcoidosis is higher in Japanese patients with sarcoidosis (50–78% in necropsy studies)<ref name="pmid9723651">{{cite journal| author=Yazaki Y, Isobe M, Hiramitsu S, Morimoto S, Hiroe M, Omichi C et al.| title=Comparison of clinical features and prognosis of cardiac sarcoidosis and idiopathic dilated cardiomyopathy. | journal=Am J Cardiol | year= 1998 | volume= 82 | issue= 4 | pages= 537-40 | pmid=9723651 | doi=10.1016/s0002-9149(98)00377-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9723651 }}</ref> | |||
*In Japan, cardiac involvement is the leading cause of death due to sarcoidosis, accounting for 77 to 85% of deaths<ref name="pmid3014961">{{cite journal| author=Tachibana T, Ohmori F, Ueda E| title=Clinical study on cardiac sarcoidosis. | journal=Ann N Y Acad Sci | year= 1986 | volume= 465 | issue= | pages= 530-42 | pmid=3014961 | doi=10.1111/j.1749-6632.1986.tb18530.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3014961 }}</ref> | |||
*In the United States, 13 to 50% of sarcoid deaths have been attributed to myocardial involvement<ref name="pmid8610729">{{cite journal| author=Gideon NM, Mannino DM| title=Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data. | journal=Am J Med | year= 1996 | volume= 100 | issue= 4 | pages= 423-7 | pmid=8610729 | doi=10.1016/S0002-9343(97)89518-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8610729 }}</ref> | |||
* | |||
<br /> | <br /> | ||
==Risk Factors== | ==Risk Factors== | ||
* There are no established [[risk factors]] for [[cardiac]] sarcoidosis | |||
*Ethnicity: African Americans and people of European descent ,particularly Scandinavian. | |||
==Screening== | ==Screening== | ||
* There is insufficient [[evidence]] to recommend routine [[screening]] for [[cardiac]] sarcoidosis | |||
*Patients with sarcoidosis can be screened for possible [[cardiac]] involvement by [[ECG]], [[Holter monitoring]], DE, and [[cardiac]] symptoms (i.e., [[palpitations]], [[syncope]], or [[presyncope]]). If [[symptoms]] or [[screening]] tests are with positive, patients can undergo [[MRI]] and [[PET scan]].<br /> | |||
==Natural History, Complications, and Prognosis== | |||
== | * Studies have concluded that [[Survival rate|survival]] in most patients with [[symptomatic]] [[cardiac]] sarcoidosis was limited to about two years | ||
* later studies have concluded better [[outcome]] where five year [[Survival rate|survival]] was 40–60%<ref name="pmid117039972">{{cite journal| author=Yazaki Y, Isobe M, Hiroe M, Morimoto S, Hiramitsu S, Nakano T et al.| title=Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. | journal=Am J Cardiol | year= 2001 | volume= 88 | issue= 9 | pages= 1006-10 | pmid=11703997 | doi=10.1016/s0002-9149(01)01978-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11703997 }}</ref> | |||
==Diagnosis== | ==Diagnosis== | ||
* The definitive diagnosis of isolated cardiac sarcoidosis is difficult. | |||
*Noninvasive imaging should be added to the endomyocardial biopsy in order to diagnose and follow cardiac sarcoidosis | |||
*Treatment of cardiac sarcoidosis can be started even in the absence of histologic proof | |||
* The diagnosis can be confirmed histologically by [[myocardial]] [[biopsy]] or clinically via a of major and minor criteria combination [The Japanese Ministry of Health and Welfare guidelines (JMH)]<ref name="pmid191754483" /><ref name="pmid161693243" /> | |||
* [[Echocardiography]] | |||
*[[CMR]] [[imaging]] | |||
*[[Nuclear]] [[imaging]] | |||
*[[Cardiac]] [[PET]]<ref name="pmid22228794">{{cite journal| author=Youssef G, Leung E, Mylonas I, Nery P, Williams K, Wisenberg G et al.| title=The use of 18F-FDG PET in the diagnosis of cardiac sarcoidosis: a systematic review and metaanalysis including the Ontario experience. | journal=J Nucl Med | year= 2012 | volume= 53 | issue= 2 | pages= 241-8 | pmid=22228794 | doi=10.2967/jnumed.111.090662 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22228794 }}</ref> | |||
*[[Hybrid]] [[imaging]]—[[PET]] and [[CMR]] | |||
*[[Coronary Angiography]]<br /> | |||
===Cardiac MRI=== | ===Cardiac MRI=== | ||
Common [[MRI]] findings in patients with [[cardiac]] sarcoidosis include: | Common [[MRI]] findings in patients with [[cardiac]] sarcoidosis include: | ||
*Delayed enhancement, which may be either mid wall or transmural | *Delayed enhancement, which may be either mid wall or transmural | ||
*[[Nodular]] mid wall hyperintense foci on black blood T2-weighted [[imaging]] | *[[Nodular]] mid wall hyperintense foci on black blood T2-weighted [[imaging]] | ||
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==Treatment== | ==Treatment== | ||
*[[Corticosteroid]] treatment | |||
*[[Antiarrhythmic]] treatment | |||
*[[Pacemaker|Pacemakers]] and [[defibrillators]] | |||
*[[Cardiac transplantation]] | |||
* | |||
* | |||
==References== | ==References== |
Latest revision as of 15:17, 27 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, sarcoid heart
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 recognize 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
- The Japanese Ministry of Health and Welfare (JMH) published diagnostic guidelines for cardiac sarcoidosis in 1993 and were updated in 2006[11]. The guidelines require myocardial biopsy for histologic confirmation of cardiac involvement or clinical confirmation via a of major and minor criteria combination[12]
Pathophysiology
- The exact pathogenesis of cardiac sarcoidosis is not fully understood[13]
- It is thought that antigens, non-self and self, trigger the helper inducer T cells primarily and lead to the formation of granuloma lesions
- During the early stage of the disease, sarcoid infiltrates consist of:[13][14]
- Mononuclear phagocytes
- CD4 positive T cells with a T helper type I response
- Secret interleukin‐2 and interferon‐γ.
- During the late of the disease, the infiltrates shift to:
- High concentrations of interleukin‐6 can be found in the circulation at the onset of the disease and before the initiation of immunosuppressive therapy, but can't be found thereafter
- Interleukin‐6 is believed to induce the proliferation of T cells and hence be involved in the maintenance of inflammation[14]
- Genetic factors are also believed to have a role in the pathogenesis of cardiac sarcoidosis
- The heart has no portion that is immune to infiltration by sarcoid granulomas
- The most frequently involved heart portion is the myocardium. However, the granuloma can involve the pericardium and the endocardium.
- The predominant sites of myocardial involvement, in decreasing order of frequency, are:[13]
- The left ventricular free wall and papillary muscles
- The basal aspect of the ventricular septum
- The right ventricular free wall
- The atrial walls
- Microscopic appearance: samples of myocardium involved with sarcoidosis reveal the presence of:
- Numerous lymphocytes located at the border zones around the granulomas
- A dense band of fibroblasts, collagen fibers, and proteoglycans usually encase this aggregate of inflammatory cells [15]
Differentiating Cardiac sarcoidosis from Other Diseases
Cardiac sarcoidosis must be differentiated from other diseases such as cardiac amyloidosis
Disease | History and Symptoms | Physical Examination | Diagnosis | Imaging findings | Therapy and prognosis |
---|---|---|---|---|---|
Cardiac sarcoidosis[10][13][15][16][17][18][19] |
|
|
|
|
|
Cardiac amyloidosis[20][21] |
|
Periorbital purpura: Often occurs with sneezing, coughing or with minor trauma. Indicates capillary involvement of AL type amyloidosis.
|
Lab findings:
|
Imaging findings:
|
|
Epidemiology and Demographics
- Sarcoidosis granuloma formation involves most commonly the lungs[22]
- 70% of sarcoidosis occurs in patients 25 to 60 years of age
- Sarcoidosis is rare in people <15 or >70 years of age
- The prevalence of sarcoidosis is about 4.7 to 64 in 100,000
- Sarcoidosis highest rates are reported in northern Europeans and African Americans, mainly in women
- Clinically manifest cardiac involvement occurs in about 5% of patients with sarcoidosis
- Studies say that cardiac sarcoidosis seems to be becoming more prevalent than before
- In Finland, the rate of sarcoidosis has increased more than 20-fold between 1988 and 2012 [23]
- In the United States, the incidence of patients with sarcoidosis-induced cardiomyopathy who underwent transplantation increased from 0.1% (1994 to 1997) to 0.5% (2010 to 2014)[24]
- In patients with clinically evident sarcoidosis, cardiac involvement has been reported in at least 2-7% of them[25]
- Occult cardiac involvement has a much higher rate (> 20%)[26]
- In the United States and United Kingdom, several necropsy series cited pathological evidence for cardiac involvement in 19.5 to 28% of sarcoid patients[27]
- The incidence of cardiac sarcoidosis is higher in Japanese patients with sarcoidosis (50–78% in necropsy studies)[28]
- In Japan, cardiac involvement is the leading cause of death due to sarcoidosis, accounting for 77 to 85% of deaths[29]
- In the United States, 13 to 50% of sarcoid deaths have been attributed to myocardial involvement[30]
Risk Factors
- There are no established risk factors for cardiac sarcoidosis
- Ethnicity: African Americans and people of European descent ,particularly Scandinavian.
Screening
- There is insufficient evidence to recommend routine screening for cardiac sarcoidosis
- Patients with sarcoidosis can be screened for possible cardiac involvement by ECG, Holter monitoring, DE, and cardiac symptoms (i.e., palpitations, syncope, or presyncope). If symptoms or screening tests are with positive, patients can undergo MRI and PET scan.
Natural History, Complications, and Prognosis
- Studies have concluded that survival in most patients with symptomatic cardiac sarcoidosis was limited to about two years
- later studies have concluded better outcome where five year survival was 40–60%[31]
Diagnosis
- The definitive diagnosis of isolated cardiac sarcoidosis is difficult.
- Noninvasive imaging should be added to the endomyocardial biopsy in order to diagnose and follow cardiac sarcoidosis
- Treatment of cardiac sarcoidosis can be started even in the absence of histologic proof
- The diagnosis can be confirmed histologically by myocardial biopsy or clinically via a of major and minor criteria combination [The Japanese Ministry of Health and Welfare guidelines (JMH)][11][12]
- Echocardiography
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
- Corticosteroid treatment
- Antiarrhythmic treatment
- Pacemakers and defibrillators
- Cardiac transplantation
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
- ↑ 10.0 10.1 Doughan AR, Williams BR (2006). "Cardiac sarcoidosis". Heart. 92 (2): 282–8. doi:10.1136/hrt.2005.080481. PMC 1860791. PMID 16415205.
- ↑ 11.0 11.1 Soejima K, Yada H (2009). "The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities". J Cardiovasc Electrophysiol. 20 (5): 578–83. doi:10.1111/j.1540-8167.2008.01417.x. PMID 19175448.
- ↑ 12.0 12.1 Ardehali H, Howard DL, Hariri A, Qasim A, Hare JM, Baughman KL; et al. (2005). "A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy". Am Heart J. 150 (3): 459–63. doi:10.1016/j.ahj.2004.10.006. PMID 16169324.
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