Cases reported "Sarcoidosis"

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1/25. The spectrum of dilated cardiomyopathy. The Johns Hopkins experience with 1,278 patients.

    This report describes the evaluation of 1,278 patients referred to The Johns Hopkins Hospital with dilated cardiomyopathy. After a careful history and physical examination, selected laboratory tests, and endomyocardial biopsy, a specific diagnosis was made in 49% of cases. In 16% of cases the biopsy demonstrated a specific histologic diagnosis. myocarditis and coronary artery disease were the most frequent specific diagnoses; 51% of patients were classified as idiopathic. Thus a rigorous and systematic search can demonstrate an underlying cause for approximately one-half of patients with unexplained cardiomyopathy. Endomyocardial biopsy plays a crucial role in this evaluation. Six cases are presented which demonstrate the utility of endomyocardial biopsy in specific clinical situations. In addition to its routine use in monitoring rejection in heart transplant recipients, endomyocardial biopsy is indicated in the evaluation of possible infiltrative cardiomyopathy, in differentiating restrictive cardiomyopathy from constrictive pericarditis, and in diagnosing and monitoring doxorubicin cardiotoxicity. The importance of diagnosing myocarditis remains controversial, and disagreement persists about the utility of immunosuppressive therapy in these patients. A combination of clinical and histologic features can divide patients with myocarditis into 4 subgroups--acute, fulminant, chronic active, and chronic persistent. This classification provides prognostic information and may identify those patients who may respond to immunosuppression, as well as those likely to have adverse outcomes from such treatment. The continued development of novel molecular techniques may allow endomyocardial biopsy to provide greater prognostic and therapeutic information in the future.
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ranking = 1
keywords = cardiomyopathy
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2/25. sarcoidosis presenting as spiculated breast masses.

    A 67-year-old woman sought medical treatment of cardiomyopathy, which had been diagnosed 2 years earlier; the causative factor was sarcoidosis. A screening mammogram revealed multiple spiculated masses in both breasts. A review of previous films obtained elsewhere showed that these masses had been increasing in prominence during the past 3 years. The patient had no visible axillary nodal abnormalities. sarcoidosis was considered a diagnostic possibility, and a large-core needle biopsy was done with stereotactic guidance. The histological diagnosis was non-necrotizing granulomatous inflammation, consistent with sarcoidosis.
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ranking = 0.125
keywords = cardiomyopathy
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3/25. sarcoidosis in a patient with 5q-myelodysplasia. A possible pathogenetic link between the two diseases.

    This study describes the occurrence of sarcoidosis with lung and skin involvements in a 56-yr-old woman who suffered from 5q-myelodysplastic syndrome since the age of 50. The 5q-myelodysplastic syndrome is marked by deletion of the long arm of chromosome 5, which carries the genes coding for T-helper cell 2 cytokines, such as interleukins-3, -4 and -5, and granulocyte-macrophage colony-stimulating factor. Although the aetiology of sarcoidosis remains unclear, sarcoid granulomatous inflammation is marked by predominant expression of T-helper cell 1 cytokines, with reduced expression of T-helper cell 2 cytokines. The authors suggest that 5q-abnormality may have predisposed to sarcoidosis through an imbalance in the cytokine network, caused by the deletion of genes coding for T-helper cell 2 cytokines.
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ranking = 0.059789601941455
keywords = dysplasia
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4/25. Cardiac sarcoidosis mimicking right ventricular dysplasia.

    A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.
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ranking = 816.56067879019
keywords = ventricular dysplasia, right ventricular dysplasia, dysplasia
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5/25. Cardiac sarcoidosis masquerading as right ventricular dysplasia.

    patients with cardiac sarcoidosis may present with clinical and morphological features similar to arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC). Three cases of cardiac sarcoidosis are presented that clinically mimicked ARVD or ARVC until a pathology diagnosis of sarcoidosis was made at biopsy or autopsy. A diagnostic distinction, while often difficult to make, is important since treatment with corticosteroids may benefit those with sarcoidosis but is not expected to be useful in cases with ARVD or ARVC.
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ranking = 729.53465350173
keywords = ventricular dysplasia, right ventricular dysplasia, arrhythmogenic, arrhythmogenic right, cardiomyopathy, dysplasia
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6/25. Cushing's syndrome due to primary adrenocortical nodular dysplasia, cardiac myxomas, and spotty pigmentation, complicated by sarcoidosis.

    A 23-year-old male patient revealed hypercortisolism with stigmata of Cushing's syndrome, and post-operative pathological examination demonstrated primary adrenal nodular dysplasia. Because of the presence of cardiac myxomas and skin pigmentation, the diagnosis of Carney's complex was given. After the control of hypercortisolism by adrenalectomy, the patient experienced iridocyclitis and bilateral hilar lymphadenopathy with elevated levels of serum angiotensin-converting enzyme and lysozyme, all of which indicated the presence of sarcoidosis. Despite the numerous recent descriptions concerning Carney's complex, an association with sarcoidosis has not yet been documented. Moreover, the sequential occurrence of sarcoidosis after adrenalectomy suggests an etiological link between these two rare disorders.
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ranking = 0.074737002426819
keywords = dysplasia
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7/25. Electroanatomic mapping of the right ventricle in a patient with a giant epsilon wave, ventricular tachycardia, and cardiac sarcoidosis.

    A 47-year-old man presented with sustained monomorphic ventricular tachycardia of right ventricular origin. Surface ECG recorded during sinus rhythm showed a bizarre "double QRS" pattern. Biventricular cardiomyopathy was found with predominant right ventricular involvement, due to cardiac sarcoidosis. Electroanatomic mapping was used to characterize the right ventricular substrate abnormalities and to decipher the specific activation abnormalities responsible for the ECG findings.
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ranking = 0.125
keywords = cardiomyopathy
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8/25. sarcoidosis and immunoglobulin lambda II light-chain amyloidosis diagnosed after orthotopic heart transplantation: a case report and review of the literature.

    Cardiac involvement by sarcoidosis and concomitant deposition of AL amyloid is an uncommon association. We describe the case of a 53-year-old African-American man with a 7-year history of dilated nonischemic cardiomyopathy and severe cardiac failure who underwent orthotopic heart transplantation. His prior cardiac biopsies had only mild myocyte hypertrophy and minimal interstitial fibrosis. After surgery, numerous sarcoid granulomas and amyloid deposition were identified in the native heart. Six days after the transplant the patient died due to aspiration bronchopneumonia and acute renal failure. At autopsy, both sarcoidosis and immunoglobulin (Ig) lambda light-chain amyloidosis were present in the native atria, lungs, thyroid, liver, spleen, and kidneys. Sarcoid granulomas alone were present in the parathyroids, lymph nodes, and bone marrow. Amyloid deposition alone was present in the aorta, stomach, large bowel, and urinary bladder. There was no evidence of plasma cell dyscrasia, or underlying gammopathy. This unusual association was described in only two other cases in the medical literature. However, this is the first case of sarcoidosis and AL amyloidosis with successful sequencing and identification of Ig lambda light-chain amyloid, and in which there was no evidence of plasma cell dyscrasia.
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ranking = 0.125
keywords = cardiomyopathy
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9/25. Cardiac sarcoidosis: two cases with autopsy findings.

    Cardiac sarcoidosis is a disease of young adults. In most cases it presents with sudden death, arrhythmias, conduction disorders, heart failure or cardiomyopathy. The authors describe two cases of myocardial involvement by sarcoidosis that lead to death of the patients. Case one was a 26-year-old Indian man who was previously well and presented with sudden death. autopsy showed nodules of sarcoid granuloma involving the heart, lungs and lymph nodes. Case two was a 47-year-old Indian lady who complained of reduced effort tolerance. echocardiography showed that she had restrictive hypertrophic cardiomyopathy with heart failure. Seven months after initial presentation, she developed worsening of heart failure and died. autopsy revealed involvement of the heart, lungs and liver by sarcoidosis.
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ranking = 0.25
keywords = cardiomyopathy
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10/25. recurrence of cardiac sarcoidosis in a heart transplant recipient.

    We present the case of a young man who received a left ventricular assist device (LVAD) as a bridge to transplantation for end-stage heart failure, in whom the diagnosis of sarcoid cardiomyopathy was made at the time of LVAD implantation. He subsequently underwent uncomplicated heart transplantation. Nineteen months later, a routine surveillance endomyocardial biopsy specimen demonstrated recurrence of sarcoidosis in the transplanted heart.
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ranking = 0.125
keywords = cardiomyopathy
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