Loeffler endocarditis

Last updated
Loeffler endocarditis
Other namesEosinophilic endocarditis
Specialty Cardiology   OOjs UI icon edit-ltr-progressive.svg

Loeffler endocarditis is a form of heart disease characterized by a stiffened, poorly-functioning heart caused by infiltration of the heart by white blood cells known as eosinophils. [1] Restrictive cardiomyopathy is a disease of the heart muscle which results in impaired diastolic filling of the heart ventricles, i.e. the large heart chambers which pump blood into the pulmonary or systemic circulation. Diastole is the part of the cardiac contraction-relaxation cycle in which the heart fills with venous blood after the emptying done during its previous systole (i.e. contraction). [2]

Contents

An eosinophil-based specific type of heart damage was first described by the Swiss doctor, Wilhelm Loeffler, in 1936. [3] [4] As initially described, the disorder manifests as a restrictive cardiomyopathy, i.e. a poorly expanding and contracting rigid heart that was infiltrated with eosinophils and showed replacement of heart cells by stiff fibrotic connective tissue. [5]

Loeffler endocarditis is now regarded as a manifestation of eosinophilic myocarditis, a disorder that involves the infiltration of the heart's muscular layer by eosinophils that leads to three progressive clinical stages. The first stage involves acute inflammation and subsequent death of heart muscle cells. This stage is dominated by signs and symptoms of the acute coronary syndrome such as angina, heart attack, and congestive heart failure. In the second stage, the endocardium (i.e. interior wall) of the heart forms blood clots which break off and then travel through and block various arteries; this clotting stage may dominate the initial presentation in some individuals. The third stage is a fibrotic stage, i.e. Loeffler endocarditis, wherein scarring replaces damaged heart muscle tissue to cause a poorly contracting heart and/or heart valve disease. Recent publications commonly refer to Loeffler endocarditis as a historical term for the third stage of eosinophilic myocarditis. [5] [6] [7]

Signs and symptoms

The signs and symptoms of Loeffler endocarditis tend to reflect the many underlying disorders causing eosinophil dysfunction as well as the widely differing progression rates of cardiac damage. Before cardiac symptoms are detected, individuals may suffer symptoms of a common cold, asthma, rhinitis, urticarial, or other allergic disorder. Cardiac manifestations include life-threatening conditions such as cardiogenic shock or sudden death due to abnormal heart rhythms. More commonly, however, the presenting cardiac signs and symptoms of the disorder are the same as those seen in other forms of cardiomyopathy: the heart arrhythmia of ventricular fibrillation seen as an irregular pulse and heart rate, other cardiac arrhythmias, symptoms of these arrhythmias such as chest palpitations, dizziness, light headedness, and fainting; and symptoms of a heart failure such as fatigue, edema, i.e. swelling, of the lower extremities, and shortness of breath. [8] [9]

Hypereosinophilia (i.e. blood eosinophil counts at or above 1,500 per microliter) or, less commonly, eosinophilia (counts above 500 but below 1,500 per microliter) are found in the vast majority of cases and are valuable clues pointing to this rather than other types of cardiomyopathies. However, elevated blood eosinophil counts may not occur during the early phase of the disorder. Other, less specific laboratory findings implicate a cardiac disorder but not necessarily eosinophilic myocarditis. These include elevations in blood markers for systemic inflammation (e.g. C reactive protein, erythrocyte sedimentation rate) and cardiac injury (e.g. creatine kinase, troponins); and abnormal electrocardiograms ( mostly ST segment-T wave abnormalities). [10]

Pathogenesis

Eosinophilic states that may occur in and underlie Loeffler endocarditis (as well as the other stages of eosinophilic myocarditis) include primary and secondary eosinophilias or hypereosinophilias. Primary eosinophilias or hypereosinophilias (i.e. disorders in which the eosinophil appears to be intrinsically diseased) that lead to Loeffler endocarditis are clonal hypereosinophilia, chronic eosinophilic leukemia and the hypereosinophilic syndrome. [5] [8] [10] [11] Secondary causes (i.e. disorders in which other diseases cause the eosinophil to become dysfunctional) include allergic and autoimmune diseases; infections due to certain parasitic worms, protozoa, and viruses; malignant and premalignant hematologic disorders commonly associated with eosinophilia or hypereosinophilia; and adverse reactions to various drugs. [1] [12]

The disorder develops because of eosinophilic penetration into the cardiac tissues. This leads to a fibrotic thickening of portions of the heart (similar to that of endomyocardial fibrosis) and heart valves. In consequence, the heart becomes rigid and poorly contractile while the heart valves may become stenotic or insufficient, i.e. reduced in ability to open or close, respectively. The damaged heart may also develop mural thrombi, i.e. clots which lay against ventricle walls, tend to break off, and flow through and block arteries; this condition often precedes the fibrotic stage of eosinophilic myocarditis and is termed the thrombotic stage. [5]

Diagnosis

The diagnosis of Loeffler endocarditis should be considered in individuals exhibiting signs and symptoms of poor heart contractility and/or valve disease in the presence of significant increases in blood eosinophil counts. Ancillary tests may help in the diagnosis. Echocardiography typically gives non-specific and only occasional findings of endocardium thickening, left ventricular hypertrophy, left ventricle dilation, and involvement of the mitral and/or tricuspid valves. Gadolinium-based cardiac magnetic resonance imaging is the most useful non-invasive procedure for diagnosing eosinophilic myocarditis. It supports this diagnosis if it shows at least two of the following abnormalities: a) an increased signal in T2-weighted images; b) an increased global myocardial early enhancement ratio between myocardial and skeletal muscle in enhanced T1 images and c) one or more focal enhancements distributed in a non-vascular pattern in late enhanced T1-weighted images. Additionally, and unlike in other forms of myocarditis, eosinophilic myocarditis may also show enhanced gadolinium uptake in the sub-endocardium. [8] [10] However, the only definitive test for Loeffler endocarditis is cardiac muscle biopsy showing the presence of eosinophilic infiltrates. Since the disorder may be patchy, multiple tissue samples taken during the procedure improve the chances of uncovering the pathology but in any case, negative results do not exclude the diagnosis. [7] [10]

Treatment

Small studies and case reports have directed efforts towards: a) supporting cardiac function by relieving heart failure and suppressing life-threatening abnormal heart rhythms; b) suppressing eosinophil-based cardiac inflammation; and c) treating the underlying disorder. In all cases of Loeffler endocarditis that have no specific treatment regimens for the underlying disorder, available studies recommend treating the inflammatory component of this disorder with non-specific immunosuppressive drugs, principally high-dosage followed by slowly-tapering to low-dosage maintenance corticosteroid regimens. Afflicted individuals who fail this regimen or present with cardiogenic shock may benefit from treatment with other non-specific immunosuppressive drugs such as azathioprine or cyclophosphamide, as adjuncts to, or replacements for, corticosteroids. However, individuals with an underlying therapeutically accessible disease should be treated for this disease; in seriously symptomatic cases, such individuals may be treated concurrently with a corticosteroid regimen. Examples of diseases underlying Loefflers myocarditis that are recommended for treatments directed at the underlying disease include: [10] [13] [14] [15]

See also

Related Research Articles

<span class="mw-page-title-main">Cardiomyopathy</span> Disease of the heart muscle

Cardiomyopathy is a group of diseases that affect the heart muscle. Early on there may be few or no symptoms. As the disease worsens, shortness of breath, feeling tired, and swelling of the legs may occur, due to the onset of heart failure. An irregular heart beat and fainting may occur. Those affected are at an increased risk of sudden cardiac death.

<span class="mw-page-title-main">Eosinophil</span> Variety of white blood cells

Eosinophils, sometimes called eosinophiles or, less commonly, acidophils, are a variety of white blood cells and one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates. Along with mast cells and basophils, they also control mechanisms associated with allergy and asthma. They are granulocytes that develop during hematopoiesis in the bone marrow before migrating into blood, after which they are terminally differentiated and do not multiply. They form about 2 to 3% of white blood cells in the body.

<span class="mw-page-title-main">Eosinophilia</span> Blood condition

Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5×108/L (500/μL). Hypereosinophilia is an elevation in an individual's circulating blood eosinophil count above 1.5 × 109/L (i.e. 1,500/μL). The hypereosinophilic syndrome is a sustained elevation in this count above 1.5 × 109/L (i.e. 1,500/μL) that is also associated with evidence of eosinophil-based tissue injury.

<span class="mw-page-title-main">Myocarditis</span> Inflammation of the heart muscle

Myocarditis, also known as inflammatory cardiomyopathy, is an acquired cardiomyopathy due to inflammation of the heart muscle. Symptoms can include shortness of breath, chest pain, decreased ability to exercise, and an irregular heartbeat. The duration of problems can vary from hours to months. Complications may include heart failure due to dilated cardiomyopathy or cardiac arrest.

Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.

<span class="mw-page-title-main">Eosinophilic granulomatosis with polyangiitis</span> Medical condition

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).

In medicine, myopathy is a disease of the muscle in which the muscle fibers do not function properly. This results in muscular weakness. Myopathy means muscle disease. This meaning implies that the primary defect is within the muscle, as opposed to the nerves or elsewhere. Muscle cramps, stiffness, and spasm can also be associated with myopathy.

Eosinophilic pneumonia is a disease in which an eosinophil, a type of white blood cell, accumulates in the lungs. These cells cause disruption of the normal air spaces (alveoli) where oxygen is extracted from the atmosphere. Several different kinds of eosinophilic pneumonia exist and can occur in any age group. The most common symptoms include cough, fever, difficulty breathing, and sweating at night. Eosinophilic pneumonia is diagnosed by a combination of characteristic symptoms, findings on a physical examination by a health provider, and the results of blood tests and X-rays. Prognosis is excellent once most eosinophilic pneumonia is recognized and treatment with corticosteroids is begun.

<span class="mw-page-title-main">Löffler's syndrome</span> Medical condition

Löffler's syndrome is a disease in which eosinophils accumulate in the lung in response to a parasitic infection. The parasite can be Ascaris, Strongyloides stercoralis, or Dirofilaria immitis which can enter the body through contact with the soil. The symptoms of Löffler's syndrome include those of a parasitic infection such as abdominal pain and cramping, skin rashes and fatigue. Löffler's syndrome itself will cause difficulty breathing, wheeze, coughing as well as a fever.

<span class="mw-page-title-main">Eosinophilic esophagitis</span> Allergic inflammatory condition of the esophagus

Eosinophilic esophagitis (EoE) is an allergic inflammatory condition of the esophagus that involves eosinophils, a type of white blood cell. In healthy individuals, the esophagus is typically devoid of eosinophils. In EoE, eosinophils migrate to the esophagus in large numbers. When a trigger food is eaten, the eosinophils contribute to tissue damage and inflammation. Symptoms include swallowing difficulty, food impaction, vomiting, and heartburn.

<span class="mw-page-title-main">Eosinophilic gastroenteritis</span> Medical condition

Eosinophilic gastroenteritis is a rare and heterogeneous condition characterized by patchy or diffuse eosinophilic infiltration of gastrointestinal (GI) tissue, first described by Kaijser in 1937. Presentation may vary depending on location as well as depth and extent of bowel wall involvement and usually runs a chronic relapsing course. It can be classified into mucosal, muscular and serosal types based on the depth of involvement. Any part of the GI tract can be affected, and isolated biliary tract involvement has also been reported. The stomach is the organ most commonly affected, followed by the small intestine and the colon.

Drug rash with eosinophilia and systemic symptoms or drug reaction with eosinophilia and systemic symptoms (DRESS), also termed drug-induced hypersensitivity syndrome (DIHS), is a rare reaction to certain medications. It involves primarily a widespread skin rash, fever, swollen lymph nodes, and characteristic blood abnormalities such as an abnormally high level of eosinophils, low number of platelets, and increased number of atypical white blood cells (lymphocytes). However, DRESS is often complicated by potentially life-threatening inflammation of internal organs and the syndrome has about a 10% mortality rate. Treatment consists of stopping the offending medication and providing supportive care. Systemic corticosteroids are commonly used as well but no controlled clinical trials have assessed the efficacy of this treatment.

<span class="mw-page-title-main">FIP1L1</span> Protein-coding gene in the species Homo sapiens

Factor interacting with PAPOLA and CPSF1 is a protein that in humans is encoded by the FIP1L1 gene. A medically important aspect of the FIP1L1 gene is its fusion with other genes to form fusion genes which cause clonal hypereosinophilia and leukemic diseases in humans.

Acute eosinophilic leukemia (AEL) is a rare subtype of acute myeloid leukemia with 50 to 80 percent of eosinophilic cells in the blood and marrow. It can arise de novo or may develop in patients having the chronic form of a hypereosinophilic syndrome. Patients with acute eosinophilic leukemia have a propensity for developing bronchospasm as well as symptoms of the acute coronary syndrome and/or heart failure due to eosinophilic myocarditis and eosinophil-based endomyocardial fibrosis. Hepatomegaly and splenomegaly are more common than in other variants of AML.

Coxsackieviruses-induced cardiomyopathy are positive-stranded RNA viruses in picornavirus family and the genus enterovirus, acute enterovirus infections such as Coxsackievirus B3 have been identified as the cause of virally induced acute myocarditis, resulting in dilated cardiomyopathy. Dilated cardiomyopathy in humans can be caused by multiple factors including hereditary defects in the cytoskeletal protein dystrophin in Duchenne muscular dystrophy (DMD) patients). A heart that undergoes dilated cardiomyopathy shows unique enlargement of ventricles, and thinning of the ventricular wall that may lead to heart failure. In addition to the genetic defects in dystrophin or other cytoskeletal proteins, a subset of dilated cardiomyopathy is linked to enteroviral infection in the heart, especially coxsackievirus B. Enterovirus infections are responsible for about 30% of the cases of acquired dilated cardiomyopathy in humans.

Kounis syndrome is defined as acute coronary syndrome caused by an allergic reaction or a strong immune reaction to a drug or other substance. It is a rare syndrome with authentic cases reported in 130 males and 45 females, as reviewed in 2017; however, the disorder is suspected of being commonly overlooked and therefore much more prevalent. Mast cell activation and release of inflammatory cytokines as well as other inflammatory agents from the reaction leads to spasm of the arteries leading to the heart muscle or a plaque breaking free and blocking one or more of those arteries.

Eosinophilic myocarditis is inflammation in the heart muscle that is caused by the infiltration and destructive activity of a type of white blood cell, the eosinophil. Typically, the disorder is associated with hypereosinophilia, i.e. an eosinophil blood cell count greater than 1,500 per microliter. It is distinguished from non-eosinophilic myocarditis, which is heart inflammation caused by other types of white blood cells, i.e. lymphocytes and monocytes, as well as the respective descendants of these cells, NK cells and macrophages. This distinction is important because the eosinophil-based disorder is due to a particular set of underlying diseases and its preferred treatments differ from those for non-eosinophilic myocarditis.

Clonal hypereosinophilia, also termed primary hypereosinophilia or clonal eosinophilia, is a grouping of hematological disorders all of which are characterized by the development and growth of a pre-malignant or malignant population of eosinophils, a type of white blood cell that occupies the bone marrow, blood, and other tissues. This population consists of a clone of eosinophils, i.e. a group of genetically identical eosinophils derived from a sufficiently mutated ancestor cell.

Lymphocyte-variant hypereosinophilia is a rare disorder in which eosinophilia or hypereosinophilia is caused by an aberrant population of lymphocytes. These aberrant lymphocytes function abnormally by stimulating the proliferation and maturation of bone marrow eosinophil-precursor cells termed colony forming unit-Eosinophils or CFU-Eos.

Familial eosinophilia is a rare congenital disorder characterized by the presence of sustained elevations in blood eosinophil levels that reach ranges diagnostic of eosinophilia or, far more commonly, hypereosinophilia. Although high eosinophil levels are associated with certain diseases and thought to contribute to the tissue destruction found in many other eosinophilia-related diseases, clinical manifestations and tissue destruction related to the eosinophilia in familial eosinophilia is uncommon: this genetic disease typically has a benign phenotype and course compared to other congenital and acquired eosinophilic diseases.

References

  1. 1 2 Loeffler Endocarditis at eMedicine
  2. ↑ "Cardiomyopathy | National Heart, Lung, and Blood Institute (NHLBI)".
  3. ↑ synd/582 at Who Named It?
  4. ↑ Löffler, W. (1936). "Endocarditis parietalis fibroplastica mit Bluteosinophilie. Ein eigenartiges Krankheitsbild" [Endocarditis parietal fibroplastica with eosinophilia. A strange disease]. Schweizerische medizinische Wochenschrift (in German). 66: 817–20.
  5. 1 2 3 4 Cheung CC, Constantine M, Ahmadi A, Shiau C, Chen LY (November 2017). "Eosinophilic Myocarditis". The American Journal of the Medical Sciences. 354 (5): 486–492. doi:10.1016/j.amjms.2017.04.002. PMID   29173361. S2CID   205476783.
  6. ↑ Diny NL, Rose NR, Čiháková D (2017). "Eosinophils in Autoimmune Diseases". Frontiers in Immunology. 8: 484. doi: 10.3389/fimmu.2017.00484 . PMC   5406413 . PMID   28496445.
  7. 1 2 Li H, Dai Z, Wang B, Huang W (2015). "A case report of eosinophilic myocarditis and a review of the relevant literature". BMC Cardiovascular Disorders. 15: 15. doi: 10.1186/s12872-015-0003-7 . PMC   4359588 . PMID   25887327.
  8. 1 2 3 Séguéla PE, Iriart X, Acar P, Montaudon M, Roudaut R, Thambo JB (April 2015). "Eosinophilic cardiac disease: Molecular, clinical and imaging aspects". Archives of Cardiovascular Diseases. 108 (4): 258–68. doi: 10.1016/j.acvd.2015.01.006 . PMID   25858537.
  9. ↑ Janík M, Krajčovič J, Straka Ľ, Hejna P, Novomeský F (2017). "Extensive myocardial infarction in a 20-year-old associated with diabetic ketoacidosis". Kardiologia Polska. 75 (10): 1051. doi:10.5603/KP.2017.0190. PMID   29057448.
  10. 1 2 3 4 5 Kuchynka P, Palecek T, Masek M, Cerny V, Lambert L, Vitkova I, Linhart A (2016). "Current Diagnostic and Therapeutic Aspects of Eosinophilic Myocarditis". BioMed Research International. 2016: 2829583. doi: 10.1155/2016/2829583 . PMC   4738989 . PMID   26885504.
  11. ↑ Gotlib J (November 2017). "World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management". American Journal of Hematology. 92 (11): 1243–1259. doi: 10.1002/ajh.24880 . PMID   29044676.
  12. ↑ Kleinfeldt, Tilo; Ince, Hueseyin; Nienaber, Christoph A. (2011). "Hypereosinophilic Syndrome: A rare case of Loeffler's endocarditis documented in cardiac MRI". International Journal of Cardiology. 149 (1): e30–2. doi:10.1016/j.ijcard.2009.03.059. PMID   19375183.
  13. ↑ Sohn KH, Song WJ, Kim BK, Kang MK, Lee SY, Suh JW, Yoon YE, Kim SH, Youn TJ, Cho SH, Chang YS (April 2015). "Eosinophilic myocarditis: case series and literature review". Asia Pacific Allergy. 5 (2): 123–7. doi:10.5415/apallergy.2015.5.2.123. PMC   4415178 . PMID   25938077.
  14. ↑ Nunes MC, Guimarães Júnior MH, Diamantino AC, Gelape CL, Ferrari TC (May 2017). "Cardiac manifestations of parasitic diseases". Heart. 103 (9): 651–658. doi:10.1136/heartjnl-2016-309870. PMID   28285268. S2CID   206974794.
  15. ↑ Roufosse F (2015). "Management of Hypereosinophilic Syndromes". Immunology and Allergy Clinics of North America. 35 (3): 561–75. doi:10.1016/j.iac.2015.05.006. PMID   26209900.
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.