Severe Guillain–Barré syndrome in a patient receiving anti-TNF therapy. Consequence or coincidence. A case-based review

The adverse effects of anti-tumour necrosis factor alpha (TNFα) drugs include an increase in the risk of infections, congestive heart failure, lupus-like syndrome, and the onset or worsening of various demyelinating diseases such as, multiple sclerosis, optic neuritis, and Guillain–Barrè syndrome (GBS), among others. We describe the case of a patient who developed GBS while she was on treatment with adalimumab. A 50-year-old woman with rheumatoid arthritis (RA) was admitted to the hospital due to progressive severe bilateral symmetric weakness of the legs, which quickly extended to the upper limbs and to the respiratory muscles. Adalimumab was started 13 months before. GBS was diagnosed and the anti-TNFα therapy discontinued. The serological test for Campylobacter jejuni was positive. She required invasive mechanical ventilatory support for 9 months. Twelve months later, the patient was using a wheelchair following a rehabilitation programme, and at 24 months she was walking a few steps with assistive devices. The relevant literature on the relationship between GBS and anti-TNFα is reviewed. Twenty three cases of GBS occurring during anti-TNFα therapy have been reported so far in the literature. In several cases, there was no clear temporal association, more than half had a possible previous infection, and in two cases the drug was reintroduced without recurrence of GBS. Our case, which is best explained by C. jejuni infection, as well as some of the cases described are probably not a direct result of anti-TNFα treatment, but an accidental coincidence. We also discuss the potential therapeutic options after anti-TNFα discontinuation.     

A case of chronic active hepatitis C presenting severe myocarditis during interferon-β therapy

A 47-year-old woman was diagnosed as having chronic active hepatitis C in February, 1995. She was administered 6 MU of interferon (IFN) β daily, beginning on March 8. After 6 weeks of the treatment she complained of chest pain and she entered a state of shock 1 h after administration of IFN. On the electrocardiogram, negative conversion of the T wave at leads: II, III, AVF and V3-6 was seen. Since myocardial ischemia was suspected, catecholamine and nitroglycerin were administered; the symptoms abated, however the electrocardiogram on the fifth day indicated junctional rhythm. The patient was diagnosed as having myocarditis based on the findings of strongly positive C-reactive protein and elevated erythrocyte sedimentation rate together with the features seen on electrocardiograms, echocardiograms and myocardial scintigrams. After the IFN-β treatment was discontinued, the clinical symptoms, electrocardiographic and echocardiographic findings improved. The myocarditis in this case was interpreted as being due to the treatment of IFN-β, because the patient presented no evidence of previous heart disease.     

Fulminant myocarditis successfully treated with a left ventricular assist device: a case report

A previously healthy 28-year-old woman was admitted under a diagnosis of acute myocarditis. Six hours after admission, circulatory support using intraaortic balloon pumping and percutaneous cardiopulmonary support were introduced, because uncontrollable ventricular arrhythmia appeared unexpectedly. Subsequently, decreased peripheral platelet count appeared, in spite of improved hemodynamics. Therefore, a left ventricular assist device was implanted and she was weaned from the percutaneous cardiopulmonary support. On the fifth postoperative day, she was successfully weaned from the left ventricular assist device with full recovery of myocardial function. Myocardial biopsy demonstrated the appearance of acute viral myocarditis. This case suggests that the left ventricular assist device might offer effective circulatory support for acute fulminant myocarditis.     

A case of acute myocarditis associated with Chlamydia trachomatis infection: role of cardiac MRI in the clinical management

We report the case of a 32-year-old male with Chlamydia trachomatis infection and admitted with chest pain, signs of myocardial damage and coronary arteries free from significant atherosclerotic disease. Cardiac magnetic resonance imaging (MRI) documented imaging patterns of myocardial involvement suggestive of acute myocarditis, and repeated cardiac MRI examinations were used to define appropriate clinical management of the patient. In particular, the decision to submit the patient to an additional antibiotic course was based on evidence of persisting myocardial edema, while no further treatments were prescribed once these imaging findings disappeared. The case emphasizes the potential value of cardiac MRI as the only non-invasive modality currently available for evaluating the temporal evolution of myocardial involvement after acute myocarditis.     

Unusual Presentation of Listerial Myocarditis and the Diagnostic Value of Cardiac Magnetic Resonance

Listeria monocytogenes is an infrequent cause of bacterial myocarditis. Myocarditis without evidence of endocarditis is even rarer. Management in such cases involves early diagnosis, antibiotic therapy, and emergency treatment of arrhythmias.We report the case of a 47-year-old man who presented with features of acute ST-segment-elevation myocardial infarction complicated by ventricular tachycardia that necessitated urgent electrical cardioversion. Contrast-enhanced cardiac magnetic resonance images revealed hypertrophy, necrosis, and a mass that was determined to be an abscess caused by L. monocytogenes. Antibiotic treatment led to resolution of the listerial myocarditis.In addition to reporting our patient''s case, we discuss the comparative advantages of cardiac magnetic resonance versus transthoracic echocardiography in characterizing myocarditis, upon presentation and in follow-up evaluation.     

Acute myocarditis in fulminant systemic sclerosis.

Myositis and myocarditis have been reported in progressive systemic sclerosis, and these patients have had favorable therapeutic responses to intravenous pulse methylprednisolone. Thus far, premortem biopsy documentation of myocarditis and myocardial fibrosis has not been reported in such patients. We report the case of a patient with subacute congestive heart failure six months after she developed Raynaud's phenomenon. Clinical examination was typical of scleroderma but there was no proximal muscle weakness. She had elevated creatine kinase and MB-creatine kinase and laboratory evidence of hypothyroidism. Echocardiogram demonstrated four-chamber dilatation and severe left ventricular dysfunction. Cardiac catheterization revealed normal epicardial coronary arteries and severely decreased cardiac index. A skin biopsy specimen of the forearm was consistent with diffuse systemic sclerosis, and an endomyocardial biopsy specimen demonstrated mild fibrosis and lymphocytic infiltrate. Her heart failure initially improved with digoxin, furosemide, and enalapril. She also received L-thyroxine and intravenous methylprednisolone. The heart failure progressed over the next six weeks and she died. Patients with scleroderma and new-onset heart failure may have acute myocarditis.     

TandemHeart as a Bridge to Recovery in Legionella Myocarditis

Legionnaires'' disease is the designation for pneumonia caused by the Legionella species. Among the rare extrapulmonary manifestations, cardiac involvement is most prevalent, in the forms of myocarditis, pericarditis, postcardiotomy syndrome, and prosthetic valve endocarditis. Mechanical circulatory support has proved to be a safe and effective bridge to myocardial recovery in patients with acute fulminant myocarditis; however, to our knowledge, this support has not been used in infectious myocarditis specifically related to Legionellosis.We describe a case of Legionella myocarditis associated with acute left ventricular dysfunction and repolarization abnormalities in a 48-year-old man. The patient fully recovered after left ventricular unloading with use of a TandemHeart percutaneous ventricular assist device. In addition, we review the English-language medical literature on Legionella myocarditis and focus on cardiac outcomes.     

MRSA-associated bacterial myocarditis causing ruptured ventricle and tamponade

We report a case of an 81-year-old man with bacterial myocarditis presenting with elevated troponins and sepsis, who succumbed due to a ruptured ventricle. The infecting organism was found to be methicillin-resistant Staphylococcus aureus. Bacterial myocarditis is a rare occurrence when independent of infective endocarditis. Generally, this is a complication of bacteremia that is discovered post-mortem. Rarely, as in our patient, it causes significant necrosis of the myocardium leading to rupture of a ventricle. As with viral myocarditis, this disease can present with signs and symptoms of acute myocardial infarction, complicating the diagnosis. Much of the available data on bacterial myocarditis was collected before the development of many modern diagnostic tests and before antibiotics. Accordingly, the appropriate workup, diagnosis and treatment remain unclear. Our patient represents the first reported case of ventricular rupture due to methicillin-resistant S. aureus-associated bacterial myocarditis.     

Coxsackie B4 viral myocarditis causing ventricular aneurysm

This report describes a patient with viral myocarditis who developed acute dilated cardiomyopathy. The patient recovered well but a left ventricular aneurysm was detected angiographically. There was no history of myocardial infarction and the coronary arteries were normal. This is the first reported case of left ventricular aneurysm due to viral myocarditis.     

Cardiovascular syphilis-associated acute myocardial infarction: A case report

Rationale:In recent decades, the incidence of advanced syphilis has declined due to early recognition and the application of effective antibiotics. Advanced syphilis often manifests in the cardiovascular system as simple aortitis, aortic valve insufficiency, coronary artery stenosis or obstruction, Aortic aneurysm and mucinous myocarditis. In most case reports on the subject, acute myocardial infarction caused by syphilis was reported to be due to aortic valve insufficiency and coronary stenosis as a result of the involvement of the aorta.Patient concerns:The patient was a 48-year-old woman. She was admitted to our hospital because of intermittent upper abdominal pain with chest tightness for 3 hours. The patient reported a past syphilis infection, when she was hospitalized for hysteromyoma surgery four years ago, and had no related treatment.Diagnosis:According to the characteristics of coronary angiography and results of lab tests and echocardiography, she was finally diagnosed with myocardial infarction associated with syphilis.Interventions:At the first diagnosis of syphilis, the patient did not received antibiotics treatment. After the diagnosis of myocardial infarction, she received the percutaneous coronary intervention (PCI) operation assisted by extracorporeal membrane oxygenation (ECMO) technology, successfully got drug -eluted stents in right coronary artery ostium and left main ostium. Then the patient received penicillin to treat the syphilis infection.Outcomes:After coronary revascularization, the cardiac function of the patients was gradually improved, and the left ventricular ejection fraction was gradually improved after combined with optimized drug therapy.Lessons:The cardiovascular system is often involved in the stages of advanced syphilis with severe complications like myocardial infarction. Standard treatment should be given as soon as syphilis is diagnosis. For stenosis of coronary ostium, the PCI assisted by ECMO technology did not only ensure the effectiveness of the treatment, but also reduce the surgical risk of the patient. This case indicated the effectiveness of ECMO-assisted PCI, and thus may provide a reference for future patient treatment.     

Cytomegalovirus myocarditis in an immunocompetent patient

Viral infections can lead to myocardial inflammation, resulting in acute myocarditis. Acute myocarditis is mostly self-limiting, but it can lead to severe dilated cardiomyopathy and rarely to acute cardiac tamponade. We report a rare case of myocarditis in a young immunocompentent male patient due to a recent cytomegalovirus (CMV) infection. The clinical presentation was an influenza-like syndrome, classical for a CMV infection, in combination with mild chest pain. Further exploration showed inflammation-compatible perimyocardial images on magnetic resonance imaging (MRI). Following the symptomatic perimyocardial inflammation, the patient developed an acute asymptomatic self-limiting CMV hepatitis, a rare combination. Moreover, there was a remarkable evolution of both clinical signs with first increasing cardiac enzymes due to myocarditis and later on development of acute hepatitis. In this case report, we present the typical MRI images of myocarditis and discuss the CMV cardiac effects as well as the combination of CMV myocarditis and hepatitis.     

Unexpected eosinophilic myocarditis in a young woman with rapidly progressive dilated cardiomyopathy

We present the case of 23-year-old woman with good living conditions, one year history of ventricular arrhythmia and 6 months history of decreased exercise tolerance, who was found to have dilated cardiomyopathy after aborted sudden death. Endomyocardial biopsy did not show specific findings. Within 3 months she developed profound bradycardia requiring pacemaker implantation and refractory heart failure, treated with heart transplantation. Intense eosinophilic myocarditis was found in the explanted heart. Retrospective analysis of the patient's blood count revealed mild eosinophilia (eosinophil count: 0.86 x 109/l) on one examination only. Following heart transplantation the patient had persistent eisinophilia (eosinophil count: 0.62 x 109/l). Although there was no proven parasitic infestation, based on positive family history of Enterobius vermicularis infestation she was treated with broad-spectrum antiparasitic agent: albendazole and her eosinophil count returned to normal values. This case shows that active eosinophilic myocarditis may present clinically as progressive dilated cardiomyopathy with severe involvement of conduction system. Massive myocardial tissue eosinophilia occurred in the setting of mild and transient blood eosinophilia. Favourable outcome following antiparasitic treatment suggests a potential parasitic infestation as a cause of the disease.     

Isolated Streptococcus agalactiae tricuspid endocarditis in elderly patient without known predisposing factors: Case report and review of the literature

Group B streptococcal (GBS) tricuspid infective endocarditis is a very rare clinical entity. It affects intravenous drug users, pregnant, postpartum women, and the elderly. We report the case of a 68-year-old patient without known predisposing factors who presented a GBS tricuspid endocarditis treated by penicillin and aminoglycosides with no response. The patient was operated with a good evolution. Our case is the 25th reported in the literature. GBS disease is increasing in the elderly and is mainly associated to comorbid conditions. Tricuspid infective endocarditis with Group B streptococcus predominantly presents as a persistent fever with respiratory symptoms due to pulmonary embolism. Therefore, it requires a medicosurgical treatment and close follow-up.     

A loud third heart sound and asymptomatic myocarditis during Mycoplasma pneumoniae infection

A 20-year-old man had a fever and cough due to Mycoplasma pneumoniae pneumonia. He had no heart symptoms, but auscultation revealed an exceptionally loud third heart sound, suggesting cardiac involvement. Marked myocardial enzyme release, serial electrocardiographic ST-T changes, and transient increase in interventricular thickness and inferior wall hypokinesis at echocardiography supported the diagnosis of acute infectious myocarditis. Recovery was quick. This case shows that acute myocarditis with significant myocardial injury may pass without any subjective heart symptoms.     

A case of fulminant type 1 diabetes mellitus accompanied by myocarditis

This report presents the case of a 47-year-old female patient with fulminant type 1 diabetes mellitus and myocarditis. Following a high fever, nausea, vomiting and diarrhea, diabetic ketoacidosis occurred and she was transferred to the hospital. The plasma glucose level was 63.6 mmol/L and HbA1c was 7.0%. C-peptide was undetectable in her plasma. Blood gas analysis showed a pH of 6.99. Antibodies to glutamic acid decarboxylase nor insulinoma associated antigen-2 were not detected. She was diagnosed to have fulminant type 1 diabetes mellitus. Her electrocardiogram showed diffuse ST-segment elevations on the second day of admission, along with a positive troponin test. However coronary angiography revealed neither occlusion nor stenosis of the cardiac arteries. An endomyocardial biopsy revealed hypertrophic cardiomyocytes with a disarrangement of myofibers and the focal accumulation of mononuclear cells in the stroma, thus suggesting myocarditis or mild myocarditic change. Viruses are an important cause of myocarditis and the preceding flu-like symptoms indicate the association of viral infection with myocarditis in this case. The mechanisms by which fulminant type 1 diabetes mellitus occurs is still uncertain, but the presence of islet injury accompanied by myocardial inflammation in the current case suggested that a viral infection accounted for the onset of this type of diabetes.     

Listerial myocarditis as a complication of Listerial meningoencephalitis

Here, we present a case report of a 53-year-old patient who was admitted for an acute ischaemic stroke. During the hospitalization period, the patient developed meningoencephalitis and acute myocarditis with recurrent episodes of ventricular fibrillation that required defibrillation repeatedly. Listeria monocytogenes was isolated from blood culture samples. The patient was treated with antibiotic therapy and showed a good clinical response. The diagnosis of myocarditis was based on the clinical and ECG presentation (ventricular arrhythmia) and elevation of troponin T level, echocardiography and MRI showed myocardial hypertrophy and pericardial effusion. In the context of this case, it is important to note that the patient suffered from scleroderma and was ambulatory treated with long-term corticosteroid therapy (Medrol 32 mg per day).     

Myocarditis caused by Toxoplasma gondii and Aspergillus fumigatus after orthotopic heart transplantation

This report describes the case of a 56-year-old patient, who died 53 days after orthotopic heart transplantation due to myocarditis caused by infection with Aspergillus fumigatus and Toxoplasma gondii. Aspergillosis was diagnosed by transthoracic needle aspiration of a pulmonary infiltration. Endomyocardial biopsy showed toxoplasma pseudocysts. Autopsy revealed myocardial infection with both infectious agents. Despite specific therapy, myocarditis determined the fatal outcome in this case. The value of invasive techniques for specific diagnosis of infectious diseases in immunocompromised patients is discussed.     

Myocarditis mimicking acute myocardial infarction: role of endomyocardial biopsy in the differential diagnosis

OBJECTIVE—To test the hypothesis, using endomyocardial biopsies, that unexplained cases of apparent acute myocardial infarction were caused by myocarditis.
MATERIAL—Between 1992 and 1998, 12 patients were admitted to the coronary care unit with severe chest pain, ST segment elevation, increased serum creatine kinase and MB isoenzyme, and with wall motion abnormalities on echocardiogram highly suggestive of acute myocardial infarction. These patients were further investigated by endomyocardial biopsy, as their coronary angiograms were normal. A diagnosis of myocarditis was made according to the Dallas criteria. A panel of antibodies was used for immunohistochemical characterisation of inflammatory cell infiltrate. Polymerase chain reaction (PCR) was used to detect viral genomes in seven cases.
RESULTS—Haematoxylin and eosin staining of the endomyocardial biopsy showed active myocarditis in six patients and borderline myocarditis in one. Immunohistochemistry was positive for inflammatory cell infiltrates in 11 patients, including all the seven who were positive on haematoxylin and eosin staining according to the Dallas criteria. Only one patient had no evidence of inflammation. PCR was positive in two patients, both for Epstein-Barr virus. Follow up showed complete resolution of echocardiographic abnormalities in all patients except one.
CONCLUSIONS—Myocarditis can mimic acute myocardial infarction in patients with angiographically normal coronary arteries, leading to errors of treatment. In patients with apparent myocardial infarction and a normal coronary angiogram, endomyocardial biopsy may help in the diagnosis of myocarditis. The sensitivity of endomyocardial biopsy was enhanced by using immunohistochemical and molecular biological techniques.


Keywords: acute myocardial infarction; endomyocardial biopsy; myocarditis     

Sex Differences in Translocator Protein 18 kDa (TSPO) in the Heart: Implications for Imaging Myocardial Inflammation

Myocarditis is more severe in men than in women and difficult to diagnose due to a lack of imaging modalities that directly detect myocardial inflammation. Translocator protein 18 kDa (TSPO) is used extensively to image brain inflammation due to its presence in CD11b⁺ brain microglia. In this study, we examined expression of TSPO and CD11b in mice with coxsackievirus B3 (CVB3) myocarditis and biopsy sections from myocarditis patients in order to determine if it could be used to image myocarditis. We found that male mice with CVB3 myocarditis upregulated more genes associated with TSPO activation than female mice. TSPO expression was increased in the heart of male mice and men with myocarditis compared with female subjects due to testosterone, where it was expressed predominantly in CD11b⁺ immune cells. We show that TSPO ligands detect myocardial inflammation using microSPECT, with increased uptake of [¹²⁵I]-IodoDPA-713 in male mice with CVB3 myocarditis compared with undiseased controls.