The heart in sarcoidosis

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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

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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

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]
  • Asymptomatic conduction abnormalities
  • Chest pain
  • Congestive heart failure symptoms:
    • Fatigue
    • Syncope
    • Dyspnea
    • Chest pain.
    • Irregular heartbeats
    • Palpitations
    • edema
  • Fatal ventricular arrhythmias
  • Complete heart block
  • First degree heart block
  • Bundle branch block
  • Re‐entrant tachyarrhythmias
  • Ventricular tachycardia (VT)Rhythm disturbances
  • Cor pulmonale caused by pulmonary hypertension
  • Valvar regurgitation,
  • Pericardial abnormalities such as pericardial effusion, constrictive pericarditis, or tamponade.
  • Sudden death due to arrhythmia
  • Supra-ventricular arrhythmias
  • Ventricular aneurysms
  • Japanese Ministry of Health and Welfare created guidelines to diagnose cardiac sarcoidosis:
  • 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
  • electrocardiographic changes that may mimic transmural myocardial infarction
  • Echocardiography
  • Gold standard test: Endomyocardial biopsy
  • Radionuclide examinations
    • Thallium‐201 scintigraphy
    • Gallium‐67 scintigraphy
    • Positron emission tomography
  • Magnetic resonance imaging
  • Samples of myocardium with sarcoidosis shows the following:
  • Non‐caseating, multinucleated giant cell granuloma in the subendocardium
  • Trichrome stain can show a dense band of collagen fibers, encasing aggregate of granulomas and inflammatory cells
  • The prognosis of cardiac sarcoidosis is not well defined
  • Early necropsy series studies showed that in most patients with symptomatic cardiac sarcoidosis, survival was about two years
  • later studies showed better outcome where five year survival was 40–60%
  • It is unclear if this improvement in prognosis is a result of lead time bias or translates a milder cardiac sarcoidosis form v versus early introduction of corticosteroid therapy
  • Independent mortality predictors are:
    • New York Heart Association (NYHA) functional class
    • left ventricular end-diastolic diameter
    • Sustained VT
    • 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
Cardiac amyloidosis[20][21]
  • Early cardiac amyloidosis is a major challenge to diagnose
  • Advance amylodosis presents as right sided congestive heart failure
      • Fatigue
      • Dyspnea
      • Dizziness
      • Orthopnea
      • Peripheral edema
      • Weight loss due to cardiac cachexia
      • Ascites
      • Syncope on exertion
      • Transthyretin (ATTRwt) associated more common in African-Americans during sixth to seventh decade of life
  • Elevated jugular pressure

Periorbital purpura: Often occurs with sneezing, coughing or with minor trauma. Indicates capillary involvement of AL type amyloidosis.

  • Macroglossia
  • Abnormal phonation
  • Hepatomegaly
  • Ascites may be present in the setting of heart failure
  • Valvular involvement murmurs of mitral and tricuspid regurgitation (systolic).
Lab findings:
  • Normocytic mormochromic anemia
  • Serum free-light-chain assay positive
  • Increased BNP, ANP and β2 microglobulin
  • Voltage-to-mass ratio is more sensitive than EKG, 2D Echo and nuclear scanning alone
  • Biopsy:
    • Diffuse deposition of amorphous hyaline material (nodular pattern - 8 to15 nm in diameter), in mesangium (weakly staining with periodic acid-Schiff (PAS)
    • Gold standard test:
      • Biopsy:
      • Diffuse deposition of amorphous hyaline material (nodular pattern - 8 to15 nm in diameter), in mesangium (weakly staining with periodic acid-Schiff (PAS)

Imaging findings:


  • Supportive care
  • Tafamidis
  • Melphalan-prednisone/dexamethasone
  • Dexamethasone plus Cyclophosphamide-thalidomide

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

Natural History, Complications, and Prognosis

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:


Treatment


References

  1. Braunwald's Heart Disease 8th Ed, 2007, Libby P
  2. "Sarcoidosis: eMedicine Pediatrics: General Medicine".
  3. Mayo Clinic Cardiology, Concise Textbook, 3rd edition, 2007
  4. Hurst's The Heart, Fuster V, 11th (printed) and 12th (online) editions, 2004-2008
  5. Harris: Kelley's Textbook of Rheumatology, 7th ed. 2005
  6. Robbins and Cotran PATHOLOGIC BASIS OF DISEASE, 7th Edition, 2005
  7. Washington Manual of Medical Therapeutics, The, 32nd Edition, 2007
  8. Cecil Textbook of Medicine, 23rd Edition, 2007
  9. Harrison's Principals of Internal Medicine, 16th Edition, 2005
  10. 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. 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. 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.
  13. 13.0 13.1 13.2 13.3 Roberts WC, McAllister HA, Ferrans VJ (1977). "Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 11)". Am J Med. 63 (1): 86–108. doi:10.1016/0002-9343(77)90121-8. PMID 327806.
  14. 14.0 14.1 Schoppet M, Pankuweit S, Maisch B (2003). "Cardiac sarcoidosis: cytokine patterns in the course of the disease". Arch Pathol Lab Med. 127 (9): 1207–10. doi:10.1043/1543-2165(2003)127<1207:CSCPIT>2.0.CO;2. PMID 12946220.
  15. 15.0 15.1 FERRANS VJ, HIBBS RG, BLACK WC, WALSH JJ, BURCH GE (1965). "MYOCARDIAL DEGENERATION IN CARDIAC SARCOIDOSIS: HISTOCHEMICAL AND ELECTRON MICROSCOPIC STUDIES". Am Heart J. 69: 159–72. doi:10.1016/0002-8703(65)90033-5. PMID 14256691.
  16. Sekiguchi M, Numao Y, Imai M, Furuie T, Mikami R (1980). "Clinical and histopathological profile of sarcoidosis of the heart and acute idiopathic myocarditis. Concepts through a study employing endomyocardial biopsy. I. Sarcoidosis". Jpn Circ J. 44 (4): 249–63. doi:10.1253/jcj.44.249. PMID 7373823.
  17. Yazaki Y, Isobe M, Hiroe M, Morimoto S, Hiramitsu S, Nakano T; et al. (2001). "Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone". Am J Cardiol. 88 (9): 1006–10. doi:10.1016/s0002-9149(01)01978-6. PMID 11703997.
  18. Okayama K, Kurata C, Tawarahara K, Wakabayashi Y, Chida K, Sato A (1995). "Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis". Chest. 107 (2): 330–4. doi:10.1378/chest.107.2.330. PMID 7842756.
  19. Smedema JP, Snoep G, van Kroonenburgh MP, van Geuns RJ, Dassen WR, Gorgels AP; et al. (2005). "Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis". J Am Coll Cardiol. 45 (10): 1683–90. doi:10.1016/j.jacc.2005.01.047. PMID 15893188.
  20. Fontana M, Banypersad SM, Treibel TA, Abdel-Gadir A, Maestrini V, Lane T; et al. (2015). "Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study". Radiology. 277 (2): 388–97. doi:10.1148/radiol.2015141744. PMID 25997029.
  21. Martinez-Naharro A, Hawkins PN, Fontana M (2018). "Cardiac amyloidosis". Clin Med (Lond). 18 (Suppl 2): s30–s35. doi:10.7861/clinmedicine.18-2-s30. PMC 6334035. PMID 29700090.
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  24. Kandolin R, Lehtonen J, Airaksinen J, Vihinen T, Miettinen H, Ylitalo K; et al. (2015). "Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study". Circulation. 131 (7): 624–32. doi:10.1161/CIRCULATIONAHA.114.011522. PMID 25527698.
  25. Nunes H, Freynet O, Naggara N, Soussan M, Weinman P, Diebold B; et al. (2010). "Cardiac sarcoidosis". Semin Respir Crit Care Med. 31 (4): 428–41. doi:10.1055/s-0030-1262211. PMID 20665393.
  26. Matsui Y, Iwai K, Tachibana T, Fruie T, Shigematsu N, Izumi T; et al. (1976). "Clinicopathological study of fatal myocardial sarcoidosis". Ann N Y Acad Sci. 278: 455–69. doi:10.1111/j.1749-6632.1976.tb47058.x. PMID 1067031.
  27. Perry A, Vuitch F (1995). "Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations". Arch Pathol Lab Med. 119 (2): 167–72. PMID 7848065.
  28. Yazaki Y, Isobe M, Hiramitsu S, Morimoto S, Hiroe M, Omichi C; et al. (1998). "Comparison of clinical features and prognosis of cardiac sarcoidosis and idiopathic dilated cardiomyopathy". Am J Cardiol. 82 (4): 537–40. doi:10.1016/s0002-9149(98)00377-4. PMID 9723651.
  29. Tachibana T, Ohmori F, Ueda E (1986). "Clinical study on cardiac sarcoidosis". Ann N Y Acad Sci. 465: 530–42. doi:10.1111/j.1749-6632.1986.tb18530.x. PMID 3014961.
  30. Gideon NM, Mannino DM (1996). "Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data". Am J Med. 100 (4): 423–7. doi:10.1016/S0002-9343(97)89518-6. PMID 8610729.
  31. Yazaki Y, Isobe M, Hiroe M, Morimoto S, Hiramitsu S, Nakano T; et al. (2001). "Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone". Am J Cardiol. 88 (9): 1006–10. doi:10.1016/s0002-9149(01)01978-6. PMID 11703997.
  32. Youssef G, Leung E, Mylonas I, Nery P, Williams K, Wisenberg G; et al. (2012). "The use of 18F-FDG PET in the diagnosis of cardiac sarcoidosis: a systematic review and metaanalysis including the Ontario experience". J Nucl Med. 53 (2): 241–8. doi:10.2967/jnumed.111.090662. PMID 22228794.

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