Myocarditis classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Varun Kumar, M.B.B.S. Maliha Shakil, M.D. [2] Homa Najafi, M.D.[3]
Overview
Myocarditis can be classified based on the causative, histological, and clinicopathological criteria. Combination of the histologic data and clinical course of the disease resulted in clinicopathologic classification of myocarditis. Parameters such as onset of the disease, initial clinical and histological presentation, disease course and cardiac dysfunction define acute, fulminant, chronic active and chronic persistent subtypes of myocarditis. Acute myocarditis represents the most common type of myocarditis, in which symptoms last typically for days or weeks and the acute phase is followed by spontaneous improvement or development of stable DCM. In patients with fulminant myocarditis disease progresses rapidly resulting in severe heart failure and cardiogenic shock with mortality rate of 30–40% during the acute phase. Patients diagnosed with fulminant myocarditis surviving the acute phase have been instead suggested to have excellent long-term prognosis. In its chronic form, myocarditis is detected over a period of three or more months. Clinical and histologic relapses and development of ventricular dysfunction is characteristic for chronic active myocarditis, whereas chronic persistent myocarditis is characterized by persistent presence of inflammatory cells in the myocardium, but it is usually not associated with ventricular dysfunction.
Classification
- Myocarditis can be classified based on the causative, histological, and clinicopathological criteria.
- The causative criteria define infectious agents (virus, protozoa, or bacteria) or non-infectious condition (autoimmune diseases, medications etc.) associated with myocarditis.
- Identification of the infectious agent or potential non-infectious trigger may be indicative not only for disease etiology.
- In addition to identification of the causative agent, histological and immunohistological analyses are performed to categorize myocarditis based on the presence, morphology and type of inflammatory infiltrates in the myocardium.
- Lymphocytic myocarditis characterized by extensive infiltration of lymphocytes and monocytes with signs of cardiomyocyte necrosis (active lymphocytic myocarditis) represents the most frequent type of myocarditis.
- Lymphocytic myocarditis is often observed in myocardium tested positive for viral persistence.
- Less common forms of myocarditis represent giant cell myocarditis and eosinophilic myocarditis.
- Giant cell myocarditis is characterized by the presence of multinucleated giant cells and lymphocytes on heart biopsies.
- Presence of giant cells within non-caseating granulomas, usually associated with myocardial fibrosis is referred to as cardiac sarcoidosis.
- The characteristic feature of eosinophilic myocarditis is the presence of eosinophil-rich infiltrates in the myocardium and extensive myocyte necrosis, which is accompanied with elevated level of circulating eosinophils.
- Giant cell myocarditis and eosinophilic myocarditis are associated with particularly poor prognosis.
Clinicopathological criteria
- Fulminant myocarditis: Fulminant myocarditis occurs following a viral prodrome. Fulminant myocarditis presents as acute severe cardiovascular compromise with ventricular dysfunction.[1][2] On endomyocardial biopsy, there are multiple foci of inflammation.
- Acute myocarditis: Acute myocarditis presents with a less distinct onset of the illness. When the patient does present, there is already a decline in left ventricular dysfunction. Acute myocarditis may progress to dilated cardiomyopathy.
- Chronic active myocarditis: Chronic active myocarditis has a less distinct onset of the illness. There are clinical and histologic relapses and the development of ventricular dysfunction. Histologically, chronic inflammatory changes with mild to moderate fibrosis may be present.
- Chronic persistent myocarditis: Chronic persistent myocarditis has a less distinct onset of the illness. Histologically it is characterized by persistent infiltration and myocyte necrosis. Despite the presence of symptoms, ventricular dysfunction is absent.
Causative criteria
- Virus: coxsackievirus B3, adenoviruses or herpesviruses and other
- Protozoa: Trypanosoma cruzi (Chagas disease)
- Bacteria: Borrelia burgdorferi (Lyme disease) and other
- Immune checkpoint inhibitors: anti-CTLA-4, anti-PD-1 or anti-PD-L1 therapy
- Systemic autoimmune diseases: Systemic lupus erythematosus, myasthenia gravis and other
Histological criteria
- Active myocarditis: cardiac inflammation with apparent cardiomyocyte necrosis
- Borderline myocarditis: cardiac inflammation without evident cardiomyocyte necrosis
- Lymphocytic myocarditis: extensive infiltration of lymphocytes and monocytes
- Giant cell myocarditis: multinucleated giant cells and lymphocytes on heart biopsies
- Eosinophilic myocarditis: eosinophil-rich infiltrates with extensive myocyte necrosis
Overview
There is no established system for the classification of [disease name].
OR
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
OR
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
OR
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
OR
If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
OR
The staging of [malignancy name] is based on the [staging system].
OR
There is no established system for the staging of [malignancy name].
Classification
- Myocarditis can be classified based on the causative, histological, and clinicopathological criteria.
- Myocarditis may be classified according to causative criteria into three groups:
- Infectious myocarditis
- Immune-mediated myocarditis
- Toxic myocarditis
- Myocarditis may be classified according to histological criteria into five groups:
- Lymphatic myocarditis
- Eosinophilic myocarditis
- Polymorphic myocarditis
- Giant cell myocarditis
- Cardiac sarcoidosis
- Myocarditis may be classified according to clinicopathological four criteria into three groups:
- Fulminant myocarditis
- Acute myocarditis
- Chronic active myocarditis
- Chronic persistent myocarditis
Causative criteria
| B01 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| C01 | C02 | C03 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| D01 | D02 | D03 | D04 | D05 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||
| A01 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| B10 | B11 | B12 | B13 | B14 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Infectious myocarditis | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Bacterial | Spirochaetal | Fungal | Protozoal | Parasitic | Rickettsial | Viral | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| -Staphylococcus
-Streptococcus -Pneumococcus -Meningococcus -Gonococcus -Salmonella -Corynebacterium diphtheriae -Haemophilus influenzae -Mycobacterium tuberculosis -Mycoplasma pneumonia -Brucella | -Borrelia -Leptospira | - Aspergillus
-Actinomyces -Blastomyces -Candida -Coccidioides -Cryptococcus -Histoplasma -Mucormycosis -Nocardia -Sporothrix | -Trypanosoma cruzi
-Toxoplasma gondii -Entamoeba -Leishmania | -Trichinella spiralis
-Echinococcus granulosus -Taenia solium | -Coxiella burnetii
-R.rickettsii -R.tsutsugamushi | -Coxsackievirus
-Echoviruses -Polioviruses -Influenza A & B viruses -RSV -Mumps virus -Measles virus -Rubella virus -Hepatitis C virus -Dengue virus -Yellow fever virus -HIV-1 -Adenoviruses -Paravirus B19 -Cytomegalovirus -HSV-6 -EBV -VZV -HSV | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Histological criteria
Clinicopathological criteria
References
- ↑ Lieberman EB, Hutchins GM, Herskowitz A, Rose NR, Baughman KL (1991). "Clinicopathologic description of myocarditis". J Am Coll Cardiol. 18 (7): 1617–26. PMID 1960305.
- ↑ McCarthy RE, Boehmer JP, Hruban RH, Hutchins GM, Kasper EK, Hare JM; et al. (2000). "Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis". N Engl J Med. 342 (10): 690–5. doi:10.1056/NEJM200003093421003. PMID 10706898.