eMedicine - Loeffler Endocarditis : Article by Sohail A Hassan, MD

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Loeffler Endocarditis

Last Updated: December 1, 2005

Synonyms and related keywords: endomyocardial fibrosis, Loeffler disease, Loeffler syndrome, restrictive cardiomyopathy, eosinophilia, eosinophilic myocarditis, thromboembolism, acute heart failure, hypereosinophilic syndrome, eosinophilic leukemia, eosinophilic endocardial disease, eosinophilic arteritis, myocarditis, eosinophilic endomyocardial disease, idiopathic eosinophilic endomyocarditis, hypereosinophilic syndrome, HES, posterior myocardial infarction, acute myocardial infarction, aortic valve regurgitation secondary to valve fibrosis and fibrotic vegetations on the aortic valve, amyloidosis, eosinophilic proliferation, peripheral eosinophilia, Loeffler endocarditis

AUTHOR INFORMATION Section 1 of 11

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Author: Sohail A Hassan, MD, Cardiovascular Disease Fellow, Department of Internal Medicine, Division of Cardiology, Eastside Cardiovascular Medicine PC
Coauthor(s): Viqar Maria, MD, Department of Internal Medicine, St John Hospital and Medical Center; Henry Kim, MD, MPH, Fellowship Director, Department of Cardiology, Henry Ford Hospital

Sohail A Hassan, MD, is a member of the following medical societies: American College of Cardiology

Editor(s): Hanumant Deshmukh, MD †, Former Chief of Cardiology, Veterans Affairs Medical Center; Former Associate Professor, Department of Medicine, Rosalind Franklin University of Medicine and Science; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Marschall S Runge, MD, PhD, Marion Covington Distinguished Professor of Medicine, Vice Dean for Clinical Affairs, School of Medicine, Chairman, Department of Medicine, University of North Carolina at Chapel Hill; Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital; and Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice

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INTRODUCTION

Section 2 of 11

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Background: Loeffler endocarditis and endomyocardial fibrosis are restrictive cardiomyopathies, defined as diseases of the heart muscle that result in impaired ventricular filling with normal or decreased diastolic volume of either or both ventricles. Systolic function and wall thickness may remain normal, especially early in the disease, as reported by Richardson and associates. Both conditions are associated with eosinophilia.

The associations among eosinophilia, active carditis, and multiorgan involvement were first described by Loeffler in 1936. Pathologic specimens in Loeffler endocarditis show eosinophilic myocarditis, a tendency toward endomyocardial fibrosis and clinical manifestations of thromboembolism, and acute heart failure.

Eosinophilic states that may occur in association with Loeffler endocarditis include hypereosinophilic syndrome, eosinophilic leukemia, carcinoma, lymphoma, drug reactions or parasites, as reported in multiple case series.

Although eosinophilic endocardial disease has been well described, myocardial and vascular damage due to eosinophilic infiltration and degranulation is rarely diagnosed during life, as reported by Oakley et al and others. Herzog et al and Tonnesen et al have proposed that the reason for this situation may be the rapidly fatal evolution of most cases of eosinophilic arteritis and myocarditis. These conditions are usually diagnosed based on postmortem examination and nonspecificity of clinical manifestations, as reported by Kim et al, Isaka et al, and Seshadri et al.

Pathophysiologically, the fibrotic stage of Loeffler endocarditis is very similar to the disease entity described as endomyocardial fibrosis, which is indolent in comparison to Loeffler endocarditis. The tropical form of endomyocardial fibrosis is associated with eosinophilia, a common finding in Loeffler endocarditis.

Pathophysiology: Endomyocardial damage is described in multiple histopathologic studies, including that by Solley and associates. Myocardial involvement is less well known and has been considered a manifestation of an acute necrotic stage of eosinophilic endomyocardial disease, as reported by Olsen and colleagues. More recently, cases of isolated eosinophilic myocarditis have been reported without signs of endomyocardial involvement, with or without vasculitis.

Additionally, idiopathic eosinophilic endomyocarditis, in the absence of peripheral eosinophilia, has been reported by Priglinger et al.

Morphologic abnormalities of eosinophils have been noted in patients with Loeffler endocarditis, suggesting that these eosinophils were mature or stimulated. The intracytoplasmic granular content of activated eosinophils is thought to be responsible for the toxic damage to the heart, as reported by Tai and associates. Spray et al reported eosinophilic degranulation of basic proteins causing myocardial damage in tissue cultures in vitro. Gliech et al reported a dose-dependent cytotoxic effect of the eosinophilic granular proteins, inhibiting multiple enzyme systems.

The cationic eosinophilic proteins bind to the anionic endothelial protein, thrombomodulin. This complex impairs anticoagulant activities, leading to enhanced endocardial thrombus formation, as reported by Slungaard and colleagues.

Toxins released by the eosinophils include eosinophil-derived neurotoxin, cationic protein, major basic protein, reactive oxygen species, and arachidonic acid derivatives. As described by Cunningham et al, these toxins may cause endothelial and myocyte damage, resulting in thrombosis, fibrosis, and infarction.

The intensity and timing of the active carditis is related closely to the severity of the circulating eosinophilia. Some have suggested that, particularly in the tropics, patients who present with later fibrotic stages of endomyocardial disease may have had either transient earlier bouts of moderate eosinophilia with spontaneous resolution, or only moderate levels of eosinophilia leading to a low-grade endomyocarditis with gradual progressive fibrosis, as reported by Olsen et al.

Molecular pathophysiology:

Cools et al reported a landmark finding by treating patients with hypereosinophilic syndrome (HES) with imatinib, a tyrosine kinase inhibitor.

The gene defect is localized to an interstitial chromosomal deletion on chromosome band 4q12, resulting in fusion of the Fip1-like1 (FIP1L1) gene to the platelet-derived growth factor gene alpha (PDGFRA). The protein product of this gene is a tyrosine kinase enzyme that transforms the hematopoietic stem cells. This FIP1L1-PDGFRA fusion gene defect was identified in 9 of 16 patients treated with imatinib.
This study also highlights the importance of reclassifying HES as a myeloproliferative disorder of a possible single clone based on genotyping, as the FIP1L1-PDGFRA gene rearrangement is a clonal abnormality.
Treatment with imatinib caused rapid regression of eosinophilic proliferation and endomyocardiopathy in subsequent cases reported by Vandenberghe et al and Rotoli et al.

The following list summarizes the initial clinical presentations of eosinophilic endomyocardial disease in relation to the predominant pathologic stage of the disease as reported by Alderman et al in the Textbook of Cardiovascular Medicine. Death is usually related to multiorgan dysfunction in the presence of congestive heart failure.

The initial clinical presentation and stages of eosinophilic endomyocardial disease are as follows*:

Necrotic stage (early stage)
- Hypereosinophilia with systemic illness (20-30%)
  - Fever
  - Sweating
  - Chest pain (as described by Bestetti et al)
  - Lymphadenopathy
  - Splenomegaly
- Acute carditis (20-50%)
  - Anorexia
  - Weight loss
  - Cough
  - Pulmonary infiltrates
  - Skin and retinal lesion
  - Atrioventricular valve (AV) valve regurgitation
  - Biventricular failure
Thrombotic stage
- Thrombotic emboli (10-20%)
  - Cerebral, splenic, renal, and coronary infarction
  - Splinter hemorrhages
Fibrotic stage (late stage)
- Restrictive myopathy (10%)
  - AV valvular regurgitation
  - Right and left heart failure

*Adapted from Alderman, 1999

Frequency:

In the US: The condition is rare and is seen mostly in immigrants from Africa, Asia, and South America.

Internationally: Loeffler endocarditis is primarily confined to the rain forest (tropical and temperate) belts of Africa, Asia, and South America.

Mortality/Morbidity: The literature reports a 35-50% 2-year mortality rate in patients with advanced myocardial fibrosis. Substantially better survival rates may be seen in less symptomatic patients who have milder forms of the disease. As noted, this rate may reflect underdiagnosis of clinically inapparent disease, as for other types of cardiomyopathy.

Race: The condition has a predilection for African and African American populations, notably the Rwanda tribe in Uganda, and for people of low socioeconomic status. Whether this is due to genetic factors or the epidemiology of underlying environmental factors is not known.

Sex: Loeffler endocarditis has a predilection for males. However, endomyocardial fibrosis, which has similar clinical manifestations, is found equally frequently in both sexes.

Age: The reported age range is 4-70 years. Loeffler endocarditis particularly affects young males, as does its close counterpart, endomyocardial fibrosis, which is more common in children and young adults.

CLINICAL

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History: Patients may present with weight loss, fever, cough, rash, and symptoms related to congestive heart failure. Initial cardiac involvement has been reported in about 20-50% of cases; however, cardiac involvement rarely presents with chest pain, as reported by Bestetti et al.

Physical: Signs of biventricular failure (eg, pedal edema, elevated jugulovenous pressure, pulmonary edema, third heart sound [S3] gallop) are commonly seen once congestive heart failure develops.

Cardiomegaly may be present without overt signs of congestive heart failure.

Murmur of mitral regurgitation may be present, as reported by multiple authors, including Weller et al.

Systemic embolism is frequent and may lead to neurologic and renal dysfunction.

The Kussmaul sign may be present.

S3 gallop may be present, but rarely fourth heart sound (S4).

Restrictive cardiomyopathy such as Loeffler endocarditis is sometimes difficult to differentiate from constrictive pericarditis. Physical signs in constrictive pericarditis that may help differentiate the two conditions include a nonpalpable apex (usually), presence of pericardial knock, and usually absent regurgitation murmurs.

Published case reports highlight presentations with unusual ECG changes mimicking posterior myocardial infarction as described by Maruyoshi et al, acute myocardial infarction as described by Mor et al, and aortic valve regurgitation secondary to valve fibrosis and fibrotic vegetations on the aortic valve as described by Gudmundsson et al.

Causes: See Background.

DIFFERENTIALS Section 4 of 11

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Cardiac Catheterization (Left Heart)
Cardiac Neoplasms, Primary
Cardiomyopathy, Hypertrophic
Cardiomyopathy, Restrictive

Other Problems to be Considered:

Restrictive cardiomyopathies, including cardiac amyloidosis, sarcoidosis, and multiple myeloma, should be ruled out. Other causes of diastolic dysfunction, including hypertensive cardiomyopathy, should be considered in the differential diagnosis. In children, a rare cause of fibrosis and space-occupying lesions is rhabdomyoma, a tumor that has a high degree of association with tuberous sclerosis.

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WORKUP

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Lab Studies:

CBC counts should be performed to look for the presence of eosinophils. Peripheral eosinophilia should not be considered mandatory for the diagnosis of Loeffler endocarditis, as described by Priglinger et al.

Cytogenetics, fluorescent in situ hybridization (FISH), and molecular analysis show the presence of the FIP1L1-PDGFRA fusion gene as demonstrated by Cools et al and Rotoli et al.

Imaging Studies:

ECG, echocardiography, Doppler studies using echocardiogram, cardiac catheterization, endomyocardial biopsy, and CT scan or MRI of the chest may all be useful in diagnosis, as for cardiomyopathy of any cause.

The echocardiographic hallmark of Loeffler endocarditis includes a restrictive pattern of filling with relatively preserved left ventricular systolic function, as reported by Parillo et al.

Localized thickening of the basal posterior wall of the left ventricular free wall and restricted motion of the posterior leaflet of the mitral valve are seen, as reported by Spyrou et al and Childs et al.

Apical thrombus in the left ventricle also has been reported.

Regurgitant AV valve lesions are often present.

Recurrent thrombosis of the prosthetic mitral valve in the setting of rising eosinophilia is reported by Watanabe et al.

Three echocardiographic features of amyloidosis, another cause of restrictive cardiomyopathy, include thickened interatrial septum; thickening of the cardiac valves; and granular, sparkling texture of the myocardium. All may be present in amyloidosis but not in Loeffler endocarditis.

Echocardiographic Doppler findings are of restrictive cardiomyopathy, including decreased right ventricular and left ventricular velocities with inspiration and inspiratory augmentation of hepatic-vein diastolic flow reversal.

Cardiac catheterization reveals markedly elevated ventricular filling pressures and the presence of mitral or tricuspid regurgitation.

On left ventriculography, a characteristic feature is preserved left ventricular systolic function with obliteration of the left ventricular apex, as reported by Weller and associates.

The hemodynamic picture on cardiac pressure tracings reveals a restrictive picture due to dense endocardial scarring and a reduction in left ventricular cavity caused by an organized thrombus, as reported by Weller et al and Parillo et al. The hemodynamic picture may include elevation of left ventricular end diastolic pressure, often greater than 5 mm Hg over right ventricular end diastolic pressure; however the pressures may be identical at times.

Other Tests:

ECG shows nonspecific ST-segment and T-wave abnormalities as reported by Spyrou et al and Arnold et al. Arrhythmia, especially atrial fibrillation, and conduction system defects, particularly right-bundle branch block, may be present, as reported by Fawzy et al. Nonspecific findings may include pseudo infarction patterns of left-axis deviation.

Procedures:

Percutaneous endomyocardial biopsy often confirms diagnosis. As endomyocardial involvement may be patchy, a false-negative biopsy result also is possible, as reported by Felice and colleagues.

Histologic Findings: Histologic specimens of the myocardial biopsy reveal thick and deep layers of loosely arranged collagen tissue, which, although localized primarily to the endocardium, may have strands extending into the underlying myocardium. Although peripheral eosinophilia is characteristic of Loeffler endocarditis, tissue eosinophilia is not seen, and the arteries initially show intimal thickening.

TREATMENT Section 6 of 11

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Medical Care: Symptomatic relief is achieved by routine cardiac therapy, including diuretics, digitalis, afterload reduction, and anticoagulation, as indicated by Weller et al and Parillo et al.

Treatment with low-dose imatinib causing rapid regression of both eosinophilic proliferation and endomyocardiopathy is described by Cools et al, Vandenberghe et al, and Rotoli et al.

Early phases of the disease have been treated with immune suppressant and cytotoxic medications with varying degrees of success.

Corticosteroids appear to be beneficial in acute myocarditis, as reported by Uetsuka et al, among others. Together with cytotoxic drugs, including hydroxyurea, corticosteroids may prolong survival substantially, as reported by Weller et al, Parillo et al, and Arnold et al.

Interferon therapy also has been reported by Butterfield et al as having some success.

Surgical Care: Once fibrosis ensues, surgical therapy may have a positive impact on palliation of symptoms.

Dubost et al performed the first endocardiectomy in endomyocardial fibrosis, which consists of decorticating the fibrosed endocardium in a manner similar to resection of constricting densely fibrotic pericardium.

In 150 published cases of surgical therapy of endomyocardial fibrosis and eosinophilic myocarditis, an operative mortality rate of 15-29% is reported, with AV block requiring a permanent pacemaker as a common complication.

Endocardiectomy is directed toward the predominant location of the restrictive process.

The mitral and tricuspid valves may be subject to replacement or repair, depending on the involvement of the subchordal apparatus.

Early surgery is assisted by the fact that fibrous septa may not have extended into the adjacent myocardium.

In advanced disease, surgical therapy is the only option.

The extent to which fibrosis recurs postoperatively is not known.

MEDICATION

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Symptomatic relief is achieved by routine cardiac therapy including diuretics, digitalis, afterload reduction, and anticoagulation.

Early phases of the disease have been treated, with varying degrees of success, with immune suppressants, including steroids and interferon therapy, and cytotoxic medications, particularly hydroxyurea.

Corticosteroids appear to be beneficial in acute myocarditis, together with cytotoxic drugs, including hydroxyurea, and may prolong survival substantially. Interferon therapy has also been reported as having some success.

Drug Category: Tyrosine kinase inhibitors -- These agents inhibit tyrosine kinase, which, in turn, inhibit activation of intracellular pathways that can promote deregulated cell proliferation.
Drug Name
Imatinib (Gleevec) -- Small molecule that selectively inhibits the tyrosine kinase activity of c-kit, bcr-abl, and PDGFR.
Adult Dose Starting dose: 100-400 mg PO qd as described in study by Cools et al, case series by Vandenberghe et al, and case report by Rotoli et al
Pediatric Dose Not established
Contraindications Documented hypersensitivity
Interactions CYP3A4 inhibitors (ketoconazole increases distribution of imatinib); CYP3A4 substrates (simvastin increases maximum concentration of imatinib by a 2- to 3.5-fold factor); CYP3A4 inducers (phenytoin decreases AUC by approximately one fifth of typical AUC); likely to increase blood levels of drugs that are substrates of CYP2C9, CYP2D6, and CYP3A4/5
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Dose must be reduced or interrupted if edema or anemia occurs, transaminases or bilirubin levels become elevated, or grade 3 or 4 neutropenia or thrombocytopenia develops; pediatric patients commonly experience musculoskeletal pain
Drug Category: Antineoplastic agents, antimetabolite -- These agents inhibit cell growth and proliferation.
Drug Name
Hydroxyurea (Hydrea) -- Inhibitor of deoxynucleotide synthesis and DOC for inducing hematologic remission in CML. Less leukemogenic than alkylating agents such as busulfan, melphalan, or chlorambucil.
Myelosuppressive effects last a few days to a week and are easier to control than those of alkylating agents. Hydroxyurea can be given as a single daily dose or divided bid or tid at higher dose ranges.
Adult Dose 30 mg/kg/d PO initially at average of 1000-1500 mg/d PO in 500 mg tab
Pediatric Dose Not established
Contraindications Documented hypersensitivity; severe anemia; bone marrow suppression
Interactions Decreases effects of indomethacin and probenecid; may increase lithium toxicity; fluorouracil can increase neurotoxicity
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Caution in renal impairment
Drug Category: Corticosteroids -- These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Drug Name
Methylprednisolone (Adlone, Solu-Medrol, Depo-Medrol, Medrol) -- Immune-modifying agents that can be used, with varying degrees of success, in early stage of Loeffler endocarditis. Monitoring of liver function tests and eosinophil count may help to observe long-term response.
Adult Dose 1 g/d IV for 3 d, followed by 60 mg/d PO
Pediatric Dose Not established
Contraindications Documented hypersensitivity; viral, fungal, or tubercular skin infections
Interactions May increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels; phenobarbital, phenytoin, and rifampin may decrease levels (adjust dose); monitor patients for hypokalemia when taking concurrently with diuretics
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections
Drug Category: Diuretics -- These agents provide relief of congestive heart failure symptoms.
Drug Name
Bumetanide (Bumex) -- Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle. Does not appear to act in distal renal tubule.
Adult Dose 0.5-2 mg/d PO 1-2 times/d; not to exceed 10 mg/d
Alternatively, 0.5-1 mg/dose IV/IM; not to exceed 10 mg/d
Pediatric Dose Not established
Contraindications Documented hypersensitivity; anuria; increasing azotemia
Interactions Decreases effects of indomethacin and probenecid; may increase lithium toxicity
Pregnancy D - Unsafe in pregnancy
Precautions Profound diuresis, with fluid and electrolyte loss, may occur; caution in hepatic failure
Drug Name
Furosemide (Lasix) -- Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. Dose must be individualized to patient. Depending on response, administer at increments of 20-40 mg, no sooner than 6-8 h after previous dose, until desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until satisfactory effect achieved.
Adult Dose 20-80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states
Pediatric Dose Not established; may administer 1-2 mg/kg/dose PO; not to exceed 6 mg/kg/dose; do not administer >q6h;
1 mg/kg IV/IM, slowly, under close supervision; not to exceed 6 mg/kg
Contraindications Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion
Interactions Interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; metformin decreases concentrations; aminoglycosides increase risk of auditory toxicity (hearing loss of varying degrees may occur); may enhance anticoagulant activity of warfarin; may increase plasma levels and toxicity of lithium
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Perform frequent determinations of serum electrolyte, CO₂, glucose, creatinine, uric acid, calcium, and BUN levels during first few months of therapy and periodically thereafter
Drug Category: Cardiac glycosides -- These agents are used for treatment of systolic dysfunction in congestive heart failure.
Drug Name
Digoxin (Lanoxin) -- Cardiac glycoside with direct inotropic effects in addition to indirect effects on cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult Dose 0.125-0.375 mg PO qd
Pediatric Dose 5-10 years: 20-35 mcg/kg PO
>10 years: 10-15 mcg/kg PO
Maintenance dose: 25-35% of PO loading dose
Contraindications Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid sinus syndrome
Interactions Alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil may increase serum levels; aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid may decrease serum levels
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Hypokalemia may reduce positive inotropic effect; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete AV block may progress to complete block; exercise caution in patients with hypothyroidism, hypoxia, and acute myocarditis
Drug Category: Angiotensin-converting enzyme inhibitors -- These agents are used to treat congestive heart failure and reduce afterload.
Drug Name
Enalapril (Vasotec) -- Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult Dose Dosing range: 10-40 mg/d PO in 1-2 divided doses
Alternatively, 1.25 mg/dose IV over 5 min q6h
Pediatric Dose Not established
Contraindications Documented hypersensitivity
Interactions NSAIDs may reduce hypotensive effects; may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; diuretics may exacerbate hypotensive effects
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Caution in renal impairment, valvular stenosis, or severe congestive heart failure
Drug Category: Interferons -- These agents are naturally produced proteins with antiviral, antitumor, and immunomodulatory actions. Alpha, beta, and gamma interferons may be given topically, systemically, and intralesionally.
Drug Name
Interferon alfa 2a (Roferon A) and 2b (Intron A) -- Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles. Butterfield et al reported use of interferon alpha in treatment of HES with some success.
Adult Dose 2 million U/m² SC 3 times/wk for 30 d
Pediatric Dose Not established
Contraindications Documented hypersensitivity
Interactions Theophylline may increase interferon alpha toxicity; cimetidine may increase antitumor effects; zidovudine and vinblastine may increase toxicity
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Caution in brain metastases, severe hepatic or renal insufficiencies, seizure disorders, MS, or compromised CNS

FOLLOW-UP Section 8 of 11

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Further Inpatient Care:

Further inpatient care includes serial CBC counts.

Further Outpatient Care:

Outpatient follow-up includes close follow-up observation for recurrence of symptoms of heart failure. Serial echocardiograms to evaluate ejection fraction are also helpful for titration of medications.

Prognosis:

The overall prognosis of patients with Loeffler endocarditis is poor and depends on the location of involvement in the heart.

Disease is usually slow in onset, with progression to increasing degrees of right and left heart failure.

Sudden death and syncope are not as common as in other causes of restrictive cardiomyopathy.

MISCELLANEOUS

Section 9 of 11

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Medical/Legal Pitfalls:

The disease can be missed on endomyocardial biopsy because of patchy infiltration of the myocardium.

PICTURES

Section 10 of 11

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Caption: Picture 1. Pathogenesis of Loffler (Loeffler) syndrome.
Click to see detail View Full Size Image

Picture Type: Photo

BIBLIOGRAPHY Section 11 of 11

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Loeffler Endocarditis excerpt