scholarly journals Cardiac sarcoidosis: Case presentation and Review of the literature

2019 ◽  
Vol 57 (1) ◽  
pp. 7-13 ◽  
Author(s):  
Natalia Chamorro-Pareja ◽  
Julian A. Marin-Acevedo ◽  
Răzvan M. Chirilă

Abstract Cardiac sarcoidosis usually occurs in the context of systemic disease; however, isolated cardiac involvement can occur in up to 25% of cases and tends to be clinically silent. When symptoms are present, they are often nonspecific and occasionally fatal, representing a diagnostic challenge. A high index of clinical suspicion and the integration of appropriate imaging, laboratory, and pathologic findings is always required. Treatment aims to control the systemic inflammatory condition while preventing further cardiac damage. However, even with adequate diagnosis and treatment strategies, prognosis remains poor. We describe the case of a patient who presented with cardiac symptoms, whose initial examination was unrevealing. Diagnosis was made retrospectively based on later systemic manifestations that revealed characteristic sarcoidosis findings.

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Mohankumar Kurukumbi ◽  
Lauren Gardiner ◽  
Shevani Sahai ◽  
John W. Cochran

Sarcoidosis is a systemic disease with cardiac involvement occurring in 20-50% of cases. Cardiogenic stroke caused by cardiac sarcoidosis, especially PCA infarction, is a rare clinical presentation that necessitates timely diagnosis and may warrant treatment prophylaxis against CVA. In this case report, we describe a 54-year-old Caucasian male presenting with left PCA stroke in the setting of cardiac and pulmonary sarcoidosis, and hypertension. His presenting symptoms included right partial hemianopia, difficulty with naming, memory, and recall, and alexia without agraphia. Cardiogenic stroke is an uncommon manifestation of cardiac sarcoidosis, and given the disabling nature of these sequelae, the importance of early diagnosis and prevention with anticoagulation is crucial to prevent morbidity and mortality.


2012 ◽  
Vol 5 ◽  
pp. CCRep.S10138 ◽  
Author(s):  
Okosa Michael Chuka ◽  
Anyiam Daniel Chukwuemeka Darlinton

Introduction Orbital masses in adults are often caused by systemic diseases or are associated with systemic manifestations. Juvenile xanthogranuloma as a cause is rare and unreported in Africa. We present clinical features, management, and outcomes of bilateral orbital adult onset juvenile xanthogranuloma. Case Presentation A 27 year old Nigerian woman presented with bilateral upper-lid lumps having lasted 5 months. These increased in size for about 1 month and stopped. Lid swelling was preceded by itchy eyes, redness of conjunctiva, and occasional mild pain. There were no visual or systemic symptoms. The lumps were firm, slightly mobile, not tender, and not attached to skin but rather to deeper structures. There was restriction on up-gaze but no proptosis or diplopia. Hematological, biochemical, and X-ray investigations were normal. Prednisolone tablets 10 mg daily for two weeks were not useful. Tissue biopsy was invaluable in diagnosis of this rare condition and disclosed juvenile xanthogranuloma. Partial surgical excision was done under lidocaine infiltration. No recurrence has occurred in 40 months of follow-up. No systemic disease has manifested. Conclusion Juvenile xanthogranuloma can present as bilateral superior orbital tumor in adults; functional and cosmetic aims were achieved by sub-total excision.


2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
R Caldeira Da Rocha ◽  
B Picarra ◽  
AR Santos ◽  
J Pais ◽  
M Carrington ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Sarcoidosis is a multisystemic inflammatory disease, which accounts for substantial morbimortality. Cardiac involvement portends a worse prognosis. A major limitation in the evaluation of cardiac sarcoidosis is that no gold standard clinical diagnostic criteria exists. Cardiac magnetic resonance (CMR) may be indicated in patients with suspected cardiac involvement as a diagnostic and prognostic tool. Purpose The aim of this study was to characterize the features of cardiac involvement of sarcoidosis in patients with stablished diagnosis of the systemic disease, as well as to describe changes observed. Methods A multicenter, 6-years prospective study of all patients with sarcoidosis who performed CMR to evaluate possible cardiac involvement. We followed a protocol to evaluate the left and right ventricles (VE; RV) both anatomically and functionally, T2- weighted STIR sequences to evaluate myocardial edema and presence of late gadolinium enhancement(LGE). Results A total of 20 patients were included. Female patients accounted for 75% of the cases, and the mean age was 53 ± 15 years old. A majority of the patients (90%) had preserved LV (mean LV ejection fraction(EF)63 ± 6%) and RV ejection fraction (mean RV EF 62 ± 7%). Patients observed presented with mean LV end diastolic indexed volume(EDIV) of 72 ± 19 mL/m2 and mean RV EDIV of 63 ± 18mL/m2, with only one patient presenting with LV dilation(LV EDIV 138mL/m2) and two with RV dilation (mean RV EDIV 107 ± 6mL/m2). Possible features of cardiac sarcoidosis were present in 10% (n = 2) of patients. One of them presented with biventricular dilation and severe ejection fraction depression(LV EF 22% and RV EF 28%). LGE was observed in these two patients, with one presenting with an intramyocardial lesion with nodular appearance on the apical inferior segment and the other patient having its distribution characterized with two different patterns: linear appearance on the septal intramyocardium and subepicardial on the basal and mid segments of the inferior wall. On STIR sequences none of the patients presented with hypersignal suggestive of edema. None of them presented with left atrium dilation. Conclusion CMR provides a noninvasive and multidimensional assessment of the heart for evaluation of cardiac sarcoidosis. In our population of patients with sarcoidosis but without established cardiac involvement diagnosis, CMR allowed a 10% increasement on the diagnosis of cardiac sarcoidosis. CMR myocardial fibrosis detection allowed a better stratification of patients with sarcoidosis.


2020 ◽  
Vol 1 (1) ◽  
pp. 42-48
Author(s):  
Olga Karpova ◽  
◽  
Leonid Dvoretsky ◽  

Patient with pulmonary sarcoidosis and signs of cardiac involvement under the lung symptoms improvement: case report. The possible cause of cardiac symptoms (granulomatous inflammatory response, myocardial fibrosis) is discussed. The data on morbidity, clinical manifestations and cardiac lesion diagnosis in patients with sarcoidosis is reported. The need for the heart lesion signs thorough search for the purpose of early diagnosis and adequate therapy is emphasized.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Van Der Velde ◽  
A Poleij ◽  
H.C Hassing ◽  
M.J Lenzen ◽  
R.P.J Budde ◽  
...  

Abstract Background Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. The aim of this study is to evaluate our diagnostic results and follow-up for the diagnosis of CS in a tertiary center. Methods We studied 188 patients with proven extra-cardiac sarcoidosis referred to our outpatient clinic for evaluation of cardiac involvement. Eight patients were excluded because electrocardiogram (ECG) and/or transthoracic echocardiography (TTE) was missing. Cardiac magnetic resonance (CMR) and/or positron emission tomography (PET) was performed in 66% and 37% of the patients, respectively. Median follow-up duration was 2.9 [1.2–5.3] years. The diagnosis of CS was based on the Heart Rhythm Society criteria. Results Cardiac symptoms defined as palpitations, angina, dyspnea and (near)-syncope were present in 156 of 180 (87%) patients. Any abnormality on ECG (bundle branch blocks, atrioventricular blocks, sinus tachycardia or atrial fibrillation) and/or TTE (left ventricular ejection fraction <55%, presence of regional wall abnormalities or myocardial hypertrophy) was found in 92/180 (51%) patients. CS was diagnosed in 42 of 180 (23%) patients, of whom 31 (74%) had any ECG and/or TTE abnormalities. However, ECG and/or TTE abnormalities were also present in 44% of the patients without cardiac involvement. Patients with CS showed a second type II or third degree AV-blocks in 3/42 (7%), a left ventricular ejection fraction <35% on TTE in 9/42 (21%), late gadolinium enhancement by CMR consistent with CS in 28/34 (82%), and myocardial FDG uptake by PET in 19/31 (61%). In 84 of the 138 patients without cardiac involvement, CMR was performed. In 15 patients an alternative diagnosis was found (i.e. myocardial infarction or other non-ischemic cardiomyopathy). The estimated 8-year cumulative event rate composite endpoint of sustained ventricular tachycardia, ventricular fibrillation, aborted sudden cardiac death, heart transplantation and all-cause mortality was 41% in the CS patients and 12% in the patients without CS (Figure 1, p<0.001). Conclusions In our study, 23% of the patients with proven extra-cardiac sarcoidosis was diagnosed with CS. Cardiac symptoms, ECG and TTE were of limited diagnostic value for screening for CS. CMR provided a good diagnostic yield and identified other cardiac diseases in a substantial number of patients. Figure 1. KMCurve_CompositeEndpoint Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 10 (11) ◽  
pp. 2476
Author(s):  
Alida L. P. Caforio ◽  
Anna Baritussio ◽  
Renzo Marcolongo ◽  
Chun-Yan Cheng ◽  
Elena Pontara ◽  
...  

Background: Sarcoidosis is an immune-mediated disease. Cardiac involvement, a granulomatous form of myocarditis, is under-recognized and prognostically relevant. Anti-heart autoantibodies (AHAs) and anti-intercalated disk autoantibodies (AIDAs) are autoimmune markers in nonsarcoidosis myocarditis forms. Objective: The aim was to assess serum AHAs and AIDAs as autoimmune markers in cardiac sarcoidosis. Methods: This is a cross-sectional study on AHA and AIDA frequency in: 29 patients (aged 46 ± 12, 20 male) with biopsy-proven extracardiac sarcoidosis and biopsy-proven or clinically suspected and confirmed by 18-fluorodeoxyglucose positron emission tomography and/or cardiovascular magnetic resonance (CMR) cardiac involvement; 30 patients (aged 44 ± 11, 12 male) with biopsy-proven extracardiac sarcoidosis without cardiac involvement (no cardiac symptoms, normal 12-lead electrocardiogram, echocardiography and CMR), and control patients with noninflammatory cardiac disease (NICD) (n = 160), ischemic heart failure (IHF) (n = 141) and normal blood donors (NBDs) (n = 270). Sarcoidosis patients were recruited in two recruiting tertiary centers in the USA and Italy. AHAs and AIDAs were detected by indirect immunofluorescence on the human myocardium and skeletal muscle. Results: AHA and AIDA frequencies were higher in sarcoidosis with cardiac involvement (86%; 62%) than in sarcoidosis without cardiac involvement (0%; 0%), NICD (8%; 4%), IHF (7%; 2%) and NBD (9%; 0%) (p = 0.0001; p = 0.0001, respectively). Sensitivity and specificity for cardiac sarcoidosis were 86% and 92% for positive AHAs and 62% and 98% for positive AIDAs, respectively. AIDAs in cardiac sarcoidosis were associated with a higher number of involved organs (p = 0.04). Conclusions: Serum AHAs and AIDAs provide novel noninvasive diagnostic autoimmune markers for cardiac sarcoidosis.


Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 364
Author(s):  
Keisuke Miki

In chronic obstructive pulmonary disease (COPD), exertional dyspnea, which increases with the disease’s progression, reduces exercise tolerance and limits physical activity, leading to a worsening prognosis. It is necessary to understand the diverse mechanisms of dyspnea and take appropriate measures to reduce exertional dyspnea, as COPD is a systemic disease with various comorbidities. A treatment focusing on the motor pathophysiology related to dyspnea may lead to improvements such as reducing dynamic lung hyperinflation, respiratory and metabolic acidosis, and eventually exertional dyspnea. However, without cardiopulmonary exercise testing (CPET), it may be difficult to understand the pathophysiological conditions during exercise. CPET facilitates understanding of the gas exchange and transport associated with respiration-circulation and even crosstalk with muscles, which is sometimes challenging, and provides information on COPD treatment strategies. For respiratory medicine department staff, CPET can play a significant role when treating patients with diseases that cause exertional dyspnea. This article outlines the advantages of using CPET to evaluate exertional dyspnea in patients with COPD.


2021 ◽  
Vol 22 (Supplement_3) ◽  
Author(s):  
J Borges-Rosa ◽  
M Oliveira-Santos ◽  
R Silva ◽  
J Lopes De Almeida ◽  
L Goncalves ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Overt cardiac involvement is reported in 5% of patients with sarcoidosis, although autopsy and imaging studies suggest higher prevalence, worldwide variation. The role of 18F-fluorodeoxyglucose positron emission tomography ([18F]FDG-PET) in non-invasive diagnosis and follow-up has increased in the last decade. Purpose Our goal is to describe the prevalence, clinical manifestations and outcomes of cardiac sarcoidosis (CS), diagnosed through [18F]FDG-PET, in a southern European population. Methods We included all patients with histological diagnosis of extracardiac sarcoidosis screened with [18F]FDG-PET between 2009 and 2020. We collected data on clinical manifestations, cardiac magnetic resonance (CMR) results, and mortality outcomes and compared those with and without cardiac involvement. We applied the criteria for the diagnosis of CS from Heart Rhythm Society. Results Of the 400 patients screened with [18F]FDG-PET, 128 had a histological diagnosis of extracardiac sarcoidosis (54.7% females, mean age 51.0 ± 14.2 years). None underwent endomyocardial biopsy. Ten patients had a pattern of [18F]FDG uptake consistent with CS defined as diffuse (n = 5), focal (n = 3), and focal on diffuse (n = 2). Of the 128 patients, 14 also underwent CMR, which identified 2 subjects with positive findings in both modalities and 3 additional patients: focal (n = 1), multifocal mid-wall (n = 2), focal mid-wall (n = 2), and multifocal subepicardial (n = 1) delayed gadolinium enhancement. Overall, 13 patients (10.2%) fulfilled the criteria for probable CS (53.8% female, mean age 56.2 ± 12.6 years), all with multiorgan involvement, mostly lung and lymph nodes (each 92%), followed by skin and central nervous system (each 15%). Median left ventricle ejection fraction was 62% [55-65] and there were cardiac manifestations of CS in 6 patients (46%): sick sinus syndrome (n = 2), complete heart block (n = 1), frequent premature ventricular complexes (n = 1), ventricular tachycardia plus heart failure (n = 1), and bifascicular block plus heart failure (n = 1). Eleven patients (85%) with probable CS were medicated with immunosuppressant drugs: corticosteroids (n = 9), methotrexate (n = 4), and azathioprine (n = 2). Four patients with previous [18F]FDG screening were revaluated after treatment, each showing no cardiac uptake.  After a mean follow-up of 4.0 ± 1.0 years, mortality was three-fold higher in patients with cardiac involvement, despite the absence of statistical significance (15% vs. 5%, P = 0.151). Conclusions In a southern European population with histological extracardiac sarcoidosis, the prevalence of cardiac involvement was 10.2%, most asymptomatic. [18F]FDG-PET improves the diagnostic yield and plays an important role in monitoring response to therapy. The higher mortality trend in those with CS needs to be ascertained in longer follow-up.


2019 ◽  
Vol 2 (1) ◽  
pp. e4-e11
Author(s):  
John E. Conto

Limbal stem cell deficiency (LSCD) can be secondary to multiple etiologies including contact lens wear, chemical or thermal trauma, and systemic disease, any of which can result in the reduction of the number of stem cells or their decreased functionality. Primary LSCD is seen with a variety of congenital anterior segment disorders. Often LSCD can be stabilized and timely diagnosis is the key. The use of topical corticosteroids and artificial tear lubricants, along with treatment of any underlying conditions, and discontinuation of contact lenses are important initial treatment strategies. Advance treatments include amniotic membranes, limbal stem cell transplantation and keratoprostheses.


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