Giant cell myocarditis: Difference between revisions

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[[image:GCM1.jpg|Diffuse geographic myocardial necrosis at low-power magnification. Numerous giant cells (arrows) can be identified within the inflammatory infiltrate (hematoxylin and eosin, x100)|thumb|200px]] [[image:GCM2.jpg|Multinucleated giant cells (long arrows) are seen adjacent to degenerating myocytes (short arrows). The cellular infiltrate contains lymphocytes, histiocytes, and collections of eosinophils (arrowheads) (hematoxylin and eosin, x400)|thumb|200px]]
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{{SI}}
{{Myocarditis}}
{{CMG}}; '''Associate Editor:''' {{CZ}}


{{CMG}}; '''Associate Editor:''' {{CZ}}
{{SK}} GCM; idiopathic giant cell myocarditis; IGCM.


==Overview==
==Overview==
Giant cell myocarditis (GCM) or Idiopathic Giant cell myocarditis (IGCM) is a rare but often fatal inflammatory process involving the myocardium. Other than cardiac transplantation, there is no known effective treatment for giant cell mycoarditis.
Giant cell myocarditis is a rare but often fatal inflammatory process involving the myocardium. Other than cardiac transplantation, there is no known effective treatment for giant cell mycoarditis.


==Pathophysiology==
==Pathophysiology==
because of the unknown nature of the disorder. GCM is usually characterized by progressive [[congestive heart failure]], and is frequently associated with refractory ventricular [[arrhythmias]].  The majority of patients die secondary to congestive heart failure, although some have survived for long periods, often after immunosuppressive treatment. <ref>Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol 1992;8:788-92</ref> <ref>Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol 1993;6:402-7.</ref>
Data from a Lewis Rat model and from observational human studies suggest that giant cell myocarditis is mediated by [[T lymphocytes]].  The disease may therefore respond to treatment aimed at attenuating T cell function.  
 
This disease often affects young otherwise healthy individuals. The rate of death or heart transplantation is approximately 70% at one year. Data from a Lewis Rat model and from observational human studies suggest that GCM is mediated by [[T lymphocytes]] and may respond to treatment aimed at attenuating T cell function.  


Numerous autoimmune disorders have been associated with giant-cell myocarditis in case reports, but
Numerous autoimmune disorders have been associated with giant cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.
no data have been available on the incidence of these disorders in a study population with giant-cell myocarditis.


The Giant Cell Myocarditis Registry is a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers. Findings from the registry include the following: the sensitivity of endomyocardial biopsy for GCM for patients who undergo transplantation or autopsy is approximately 80%. Registry subjects who received [[cyclosporine]] and/or [[azathioprine]], with steroid and sometimes muromonab-CD3 had prolonged transplant-free survival (12.6 months vs 3.0 months for no immunosuppression.<ref>Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6</ref>.
===Gross Pathology===
 
===Gross Pathological Findings===
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]


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</div>


===Microscopic Pathological Findings===
===Microscopic Pathology===
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]


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Image:2356.jpg|Boecks Sarcoid Giant Cell Myocarditis
Image:2356.jpg|Boecks Sarcoid Giant Cell Myocarditis
Image:410214.jpg|22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma.
Image:410214.jpg|22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma.
Image:GCM1.jpg|Diffuse geographic myocardial necrosis at low-power magnification. Numerous giant cells (arrows) can be identified within the inflammatory infiltrate (hematoxylin and eosin, x100)
Image:GCM2.jpg|Multinucleated giant cells (long arrows) are seen adjacent to degenerating myocytes (short arrows). The cellular infiltrate contains lymphocytes, histiocytes, and collections of eosinophils (arrowheads) (hematoxylin and eosin, x400)
</gallery>
</gallery>
</div>
</div>


==Epidemiology and Demographics==
Giant cell myocarditis often affects young otherwise healthy individuals.
==Natural History, Complications and Prognosis==
===Complications===
Giant cell myocarditis is generally characterized by progressive [[congestive heart failure]], and is frequently associated with refractory ventricular [[arrhythmias]].  The majority of patients die secondary to [[congestive heart failure]], although some have survived for long periods, often after [[immunosuppressive]] treatment.<ref>Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol 1992;8:788-92</ref><ref>Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol 1993;6:402-7.</ref>
===Prognosis===
The rate of death or [[heart transplantation]] is approximately 70% at one year.  Survival following [[cardiac transplantation]] is approximately 71% at five years despite a 25% rate of giant cell infiltration in the donor heart.
==Diagnosis==
=== Endomyocardial Biopsy===
The Giant Cell Myocarditis Registry is a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers.  Using pathologic examination at transplantation or autopsy as  the gold standard, the sensitivity of [[endomyocardial biopsy]] in detecting giant cell myocarditis was 80% in the GCM registry. <ref>Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6</ref>


==Natural History, Complications, and Prognosis==
===Electrocardiogram===
Post-transplantation survival is approximately 71% at five years despite a 25% rate of giant cell infiltration in the donor heart.  
[[Heart block]] is frequently observed in giant cell myocarditis.


These findings should be confirmed with a randomized trial of immunosuppression including [[muromonab-CD3]], [[cyclosporine]], and steroids.  
==Treatment==
=== Immunosuppressive Therapy===
Immunosuppressive therapy including [[muromonab-CD3]], [[cyclosporine]] and/or [[azathioprine]], and steroids has been used to treat giant cell myocarditis.  Giant Cell Myocarditis Registry subjects who received [[cyclosporine]] and/or [[azathioprine]], with steroids and sometimes [[muromonab-CD3]] had prolonged transplant-free survival: 12.6 months vs 3.0 months for those patients treated with no immunosuppression.<ref>Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6</ref> While this data is encouraging, it should be noted that it is drawn from a modest registry experience and randomized placebo-controlled trials are lacking.


Giant cell myocarditis may recur after transplantation but may respond to augmented immunosuppression.
=== Cardiac Transplantation===
Giant cell myocarditis may recur after [[cardiac transplantation]] but may respond to augmented immunosuppression.


==References==
==References==
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[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Cardiovascular diseases]]
[[Category:Inflammations]]
[[Category:Emergency medicine]]
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[[Category:Up-To-Date cardiology]]


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Latest revision as of 15:08, 8 October 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and keywords: GCM; idiopathic giant cell myocarditis; IGCM.

Overview

Giant cell myocarditis is a rare but often fatal inflammatory process involving the myocardium. Other than cardiac transplantation, there is no known effective treatment for giant cell mycoarditis.

Pathophysiology

Data from a Lewis Rat model and from observational human studies suggest that giant cell myocarditis is mediated by T lymphocytes. The disease may therefore respond to treatment aimed at attenuating T cell function.

Numerous autoimmune disorders have been associated with giant cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.

Gross Pathology

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

  • Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

    Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

  • Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

    Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

  • Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

    Giant Cell Myocarditis: Gross dilated LV with focal fibrosis

Microscopic Pathology

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

  • Boecks Sarcoid Giant Cell Myocarditis

    Boecks Sarcoid Giant Cell Myocarditis

  • Boecks Sarcoid Giant Cell Myocarditis

    Boecks Sarcoid Giant Cell Myocarditis

  • 22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma.

    22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma.

  • Diffuse geographic myocardial necrosis at low-power magnification. Numerous giant cells (arrows) can be identified within the inflammatory infiltrate (hematoxylin and eosin, x100)

    Diffuse geographic myocardial necrosis at low-power magnification. Numerous giant cells (arrows) can be identified within the inflammatory infiltrate (hematoxylin and eosin, x100)

  • Multinucleated giant cells (long arrows) are seen adjacent to degenerating myocytes (short arrows). The cellular infiltrate contains lymphocytes, histiocytes, and collections of eosinophils (arrowheads) (hematoxylin and eosin, x400)

    Multinucleated giant cells (long arrows) are seen adjacent to degenerating myocytes (short arrows). The cellular infiltrate contains lymphocytes, histiocytes, and collections of eosinophils (arrowheads) (hematoxylin and eosin, x400)

Epidemiology and Demographics

Giant cell myocarditis often affects young otherwise healthy individuals.

Natural History, Complications and Prognosis

Complications

Giant cell myocarditis is generally characterized by progressive congestive heart failure, and is frequently associated with refractory ventricular arrhythmias. The majority of patients die secondary to congestive heart failure, although some have survived for long periods, often after immunosuppressive treatment.[1][2]

Prognosis

The rate of death or heart transplantation is approximately 70% at one year. Survival following cardiac transplantation is approximately 71% at five years despite a 25% rate of giant cell infiltration in the donor heart.

Diagnosis

Endomyocardial Biopsy

The Giant Cell Myocarditis Registry is a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers. Using pathologic examination at transplantation or autopsy as the gold standard, the sensitivity of endomyocardial biopsy in detecting giant cell myocarditis was 80% in the GCM registry. [3]

Electrocardiogram

Heart block is frequently observed in giant cell myocarditis.

Treatment

Immunosuppressive Therapy

Immunosuppressive therapy including muromonab-CD3, cyclosporine and/or azathioprine, and steroids has been used to treat giant cell myocarditis. Giant Cell Myocarditis Registry subjects who received cyclosporine and/or azathioprine, with steroids and sometimes muromonab-CD3 had prolonged transplant-free survival: 12.6 months vs 3.0 months for those patients treated with no immunosuppression.[4] While this data is encouraging, it should be noted that it is drawn from a modest registry experience and randomized placebo-controlled trials are lacking.

Cardiac Transplantation

Giant cell myocarditis may recur after cardiac transplantation but may respond to augmented immunosuppression.

References

  1. ↑ Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol 1992;8:788-92
  2. ↑ Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol 1993;6:402-7.
  3. ↑ Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6
  4. ↑ Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6


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