Involvement In Sarcoidosis Research Articles

Localized basal left ventricular dyssynchrony induced by manifest accessory pathway: Successful differentiation from cardiac involvement of sarcoidosis with administration of flecainide acetate

We present a patient with non-cardiac sarcoidosis complicated with manifest ventricular preexcitation. Initially, cardiac involvement of sarcoidosis was suspected from the echocardiographic findings showing localized hypokinesia at the left ventricular basal inferior wall. We, however, considered that the hypokinesia was a preexcitation-induced mechanical dyssynchrony rather than cardiac sarcoidosis, because polarities of the delta-waves indicated a left ventricular inferior accessory pathway. Temporal administration of oral flecainide acetate eliminated the basal left ventricular motion abnormality. Accordingly, we could successfully differentiate the mechanism of hypokinesia. In this context, we could rule out cardiac sarcoidosis, and initiation of glucocorticoid therapy was reasonably withheld.

A Giant Enchondroma Mimicking Sarcoidosis: Report of Case and Review of the Literature

Sarcoidosis of the humerus is exceptionally rare and only a few cases have been reported. In this mini-review, a case of enchondroma in proximal humerus mimicking sarcoidosis and the features of bone involvement of sarcoidosis were reviewed. A 41-year-old female who was diagnosed with sarcoidosis in 2009 had used corticosteroids for 4 months. She had not any symptoms until 2014. She was admitted the clinic with a 2-month-history of erythema nodosum on her legs and right shoulder pain. Values of laboratory tests were all within normal limits except erythrocyte sedimentation rate and C-reactive protein levels. The mass in the proximal metaphyseal humerus has the characteristic as a medullary lesion which had calcifications in CT sections. MR images that the mass had low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. It had irregular nodular contrast and the mass did not cause the expansion of the bone. Increased activity was observed on scintigraphy. We suspected the mass which was realized incidentally in a patient with sarcoidosis, could be bone sarcoidosis. As a result of our biopsy, it was enchondroma. Bone neoplasms should be kept in mind in issues like our case report.

Ethnicity and association with ocular, systemic manifestations and prognosis in 194 patients with sarcoid uveitis.

To determine the ophthalmological and extra-ophthalmological clinical characteristics and visual prognosis of patients with sarcoid uveitis in different ethnic groups. We retrospectively analysed the data from patients with sarcoid uveitis seen at two departments of Ophthalmology between December 2003 and December 2017. Patients presented biopsy-proven sarcoidosis and/or presumed sarcoid uveitis based on the following criteria: compatible thoracic imaging, associated with elevated angiotensin-conversion enzyme (ACE) and/or lymphocytic alveolitis on bronchoalveolar lavage fluid analysis (> 15% lymphocytes and CD4/CD8 > 3.5). Ophthalmological and general characteristics, as well as visual and global prognoses, were compared in three pre-defined ethnic groups: White Europeans, North Africans and Afro-Caribbeans. A total of 194 patients were included: 145 with biopsy-proven and 49 with presumed sarcoid uveitis. Overall, 68% were White Europeans while 20.6% were North Africans and 11.3% were Afro-Caribbeans. Sixty-nine per cent were women and the median age at presentation was 52.1years. Median ages at first ocular manifestation of the disease in Afro-Caribbeans and North Africans were respectively 34.3 and 43.1years, while it was 57.8years in White Europeans (p < 0.001). Ocular involvement was bilateral in 77.8% (n = 151) of the cases and nearly half of the patients had panuveitis (48.5%). Anterior uveitis was more frequent in Afro-Caribbeans (59.1%; p < 0.0001), while White Europeans presented more frequently with intermediate uveitis. There was a significantly higher frequency of systemic involvement of sarcoidosis in North Africans while White Europeans showed a higher frequency of isolated ocular involvement at onset and during follow-up. Afro-Caribbeans, who had a complete visual recovery in 72.7% of the cases, had a better visual prognosis than other ethnic groups (p = 0.025). In this large European series of sarcoid uveitis, we observed ethnicity-related differences regarding uveitis clinical presentation and visual outcome. Although good overall, the visual prognosis seems to be better in Afro-Caribbeans than in other ethnic groups.

Advanced Imaging in Cardiac Sarcoidosis.

Sarcoidosis is a chronic disease of unknown etiology characterized by the presence of noncaseating granulomas. Cardiac involvement in sarcoidosis may lead to adverse outcomes such as advanced heart block, arrhythmias, cardiomyopathy, or death. Cardiac sarcoidosis can occur in patients with established sarcoidosis, or it can be the sole manifestation of the disease. Traditional diagnostic techniques, including echocardiography, have poor sensitivity for diagnosing cardiac sarcoidosis. The accumulating evidence supports the essential role of advanced cardiac imaging modalities such as MRI and PET in diagnosis, risk stratification, and management of patients with cardiac sarcoidosis. The current review highlights important theoretic and practical aspects of using cardiac imaging tools in the evaluation of patients with suspected or established cardiac sarcoidosis.

Diagnostic approach for cardiac involvement in sarcoidosis.

Cardiac sarcoidosis (CS) is a potentially life-threatening condition. Early detection of CS is therefore important. The aim of this study was to eludicate the usefulness of different investigations in a subgroup of patients with sarcoidosis regarded as having an increased risk for cardiac involvement. 42 sarcoidosis patients, who had an abnormal resting electrocardiogram (ECG) and/or symptoms indicating possible cardiac involvement (i.e. palpitations, pre-syncope or syncope), were included in the study. They were identified in a consecutive manner among patients followed-up at outpatient clinics for respiratory disorders. Holter monitoring, exercise test, transthoracic echocardiogram (TTE), cardiovascular magnetic resonance (CMR) and analysis of N-terminal pro B-type natriuretic peptide (NT-pro-BNP) in serum were performed. Note, that the role of FDG-PET was not investigated in this study. In the group with a pathologic ECG 11/25 (44%) were ultimately diagnosed with CS (all with pathologic CMR). However, in the group with only symptoms but a normal ECG just 1/17 got the diagnosis CS (p<0.05). This patient had a pathologic Holter monitoring. The risk for CS was increased if serum NT-pro-BNP was elevated (i.e. NT-pro-BNP>125 ng/L), sensitivity 78% (p<0.05), specificity 67%. By adding a pathologic ECG to an elevated NT-pro-BNP increased specificity to 93% and sensitivity remained at 78%. Our findings indicate that CMR should be performed at an early stage in sarcoidosis patients with an abnormal resting ECG. Holter monitoring and elevated levels of NT-pro-BNP may enhance the diagnostic accuracy whereas exercise testing and TTE in this study had less impact on the identification of CS.

Hepatic involvement in sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Liver is frequently involved in the pathological process. Wide range of clinical manifestations can be seen: from asymptomatic lesion with normal liver function tests to cirrhosis with portal hypertension. Biopsy plays the key role in diagnosis of the hepatic sarcoidosis. It is essential for morphological diagnosis to exclude other causes of granulomatous liver disease, most often - primary biliary cholangitis. Nowadays there are no standard treatment protocols for patients with hepatic sarcoidosis.

Renal involvement in sarcoidosis: Histologic findings and clinical course

Background: Renal failure in sarcoidosis is rare and data on its long-term outcome are scarce. Aim: To investigate the pattern of renal involvement in sarcoidosis, its clinical course and response to treatment in the long-term. Methods: A single-center retrospective study with review of renal biopsies and medical charts was performed. Results: Between January 2005 and December 2016, seven patients with sarcoidosis underwent a kidney biopsy. This is equivalent to a frequency of 1.6% in a total of 434 biopsies from native kidney performed in our institution. All patients presented with renal failure. Five patients had granulomatous interstitial nephritis (GIN) and one patient each interstitial nephritis without granuloma and nephrocalcinosis. Three patients had concomitant glomerular disease: IgA nephropathy (n = 2), membranous and focal proliferative glomerulonephritis (n = 1). Most patients (n = 5) presented with hypercalcemia. All patients initially received oral prednisolone 1 mg/kg/day (n = 3) or 0.5 mg/kg/day (n = 4), respectively, with subsequent tapering or suspension. One patient was started on azathioprine after 18 months to spare steroids. After a mean follow-up of 59 months mean estimated glomerular filtration rate (eGFR) had improved from 19 ± 7 at presentation to 49 ± 16 mL/min. No patient required dialysis. All patients started on prednisolone 1 mg/kg/day developed transient diabetes mellitus while patients on 0.5 mg/kg/day did not. Renal function improvement did not differ between both treatment groups. Conclusion: GIN was the most common diagnosis in sarcoidosis patients with renal failure. Initial hypercalcemia was observed in the majority. Early steroid treatment lead to sustained renal function improvement.

Diagnosis of isolated cardiac sarcoidosis

Heart involvement in sarcoidosis is diagnosed in vivo in 5-7%, at autopsy in 25% of cases as a manifestation of a systemic process and an isolated one. Difficulties in the diagnosis of isolated sarcoidosis are due to the absence of known causes of the disease and to the lack of specificity of clinical manifestations. The main symptoms include cardiac conduction and rhythm disturbances, cardiomyopathy with the development of heart failure, as well as pericardial involvement. Routine techniques (ECG, EchoCG, daily ECG monitoring) and imaging of the structures of the heart and its function evaluation (MRI, PET, and scintigraphy) are used in diagnosis. A set of clinical, instrumental, and histological data obtained at endomyocardial biopsy may suggest isolated cardiac sarcoidosis with the exception of other diseases.

Ocular involvement of sarcoidosis

Ocular involvement of sarcoidosis

The Role of Serum Angiotensin Converting Enzyme for Predicting Pulmonary Parenchymal and Extrapulmonary Involvement of Sarcoidosis

The Role of Serum Angiotensin Converting Enzyme for Predicting Pulmonary Parenchymal and Extrapulmonary Involvement of Sarcoidosis

Gastric damage in systemic sarcoidosis

Sarcoidosis is a multisystem disease of unknown etiology, characterized by specific granulomas without caseous necrosis in the affected organs and tissues. Along with respiratory organs, the gastrointestinal tract may be affected by sarcoidosis. Due to the rarity of gastric involvement of sarcoidosis, substantial difficulties arise in verifying the diagnosis, making a differential diagnosis, and examining gastric biopsy specimens. The paper describes in detail pathological changes in the gastric wall tissue specimen in sarcoidosis and comparatively analyzes the findings with the data available in the literature.

Poster 234: Progressive Neurologic Symptoms due to Leptomeningeal Involvement of Sarcoidosis in a Patient with Previous Stroke History

PM&RVolume 10, Issue 9S1 p. S78-S78 Poster 234: Progressive Neurologic Symptoms due to Leptomeningeal Involvement of Sarcoidosis in a Patient with Previous Stroke History Sheetal Shroff MBBS, Sheetal Shroff MBBS Houston MethodistSearch for more papers by this authorThomas Chai MD, Thomas Chai MDSearch for more papers by this authorAkhil Shivaprasad MBBS, Akhil Shivaprasad MBBSSearch for more papers by this authorGirish S. Shroff MD, Girish S. Shroff MDSearch for more papers by this author Sheetal Shroff MBBS, Sheetal Shroff MBBS Houston MethodistSearch for more papers by this authorThomas Chai MD, Thomas Chai MDSearch for more papers by this authorAkhil Shivaprasad MBBS, Akhil Shivaprasad MBBSSearch for more papers by this authorGirish S. Shroff MD, Girish S. Shroff MDSearch for more papers by this author First published: 26 September 2018 https://doi.org/10.1016/j.pmrj.2018.08.246Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume10, Issue9S12018 AAPM&R Annual Assembly AbstractsSeptember 2018Pages S78-S78 RelatedInformation

Updates on the Role of Imaging in Cardiac Sarcoidosis.

The non-specific symptom profile and subclinical nature of disease along with variable region of cardiac involvement in systemic sarcoidosis make the diagnosis particularly challenging. The yield of endomyocardial biopsy, a gold standard for diagnosis, is not high unless coupled with additional imaging modalities to detect regional involvement. This review is focused on highlighting the major recent advances in imaging modalities and diagnosis of cardiac sarcoidosis. There has been much interest and increasing research focused on developing newer and improved imaging modalities to establish diagnosis. CMR and 18F- FDG-PET are now considered imaging modalities of choice in most centers worldwide, but the data comparing both methodologies head-to-head is limited. Nevertheless, novel radiotracers (i.e. 68Ga-DOTANOC, 18F-Flurpiridaz, 13N-Ammonia) and hybrid combination PET/CMR imaging are coming to spotlight with improved sensitivity and specificity for earlier detection of myocardial sarcoid. As CMR and PET are showing increased utilization in cardiac sarcoidosis, 201Th-SPECT, 99mTc MDP SPECT, 67Ga Scintigraphy, and 82Rb PET are falling out of favor. Newer imaging modalities, radionuclide tracers, and hybrid PET/CMR combinations have been promising in better detecting cardiac sarcoidosis and are currently being evaluated in larger trials.

Specific Pro-Inflammatory Signaling Pathways are Induced in Patients with Cardiac Sarcoidosis; Potential Prognostic/Therapeutic Applications

Introduction While cardiac imaging has improved the diagnosis and risk assessment for cardiac sarcoidosis, treatment regimens have consisted of generalized heart failure (HF) therapies and non-specific anti-inflammatory regimens. Thus, improvements in identifying the relative progression of this cardiovascular condition as well as identifying more specific therapeutic targets are necessary. The overall goal of this study was to perform high sensitivity plasma profiling of specific inflammatory pathways in patients with suspected cardiac sarcoidosis. Hypothesis Using large format arrays, specific inflammatory signaling cascades will be upregulated in cardiac sarcoid patients when compared to age matched referent normal subjects. Methods Plasma samples were collected from patients with biopsy confirmed sarcoidosis undergoing F-18 fluorodeoxyglucose (FDG) positron emission tomography (FDG-PET) (n=32; age 32-75 yrs) and compared to referent normal subjects. Using a high sensitivity, automated multiplex array, soluble cytokine receptor profiles (an index of cytokine activation) were measured and then indexed to referent normal values (Table). As a composite, soluble receptor levels for interleukin (IL)-1, -2, and epidermal growth factor (EGFR) were increased in sarcoid patients. In those patients with cardiac inflammation (FDG uptake), IL-1RII and IL-2Ra subtype receptor levels were higher when compared to no cardiac inflammation as was the soluble tumor necrosis factor receptor-II. Conclusions The novel finding from this study was the identification of increased activation of a cluster of soluble receptors, which not only augment inflammatory cell maturation but also transmigration in patients with sarcoidosis and cardiac involvement. The specific change in a cluster of IL receptors may hold prognostic value in cardiac sarcoidosis as well as the potential for more specific therapeutic targeting.

Nationwide survey on the organ-specific prevalence and its interaction with sarcoidosis in Japan

Previous studies attempted to characterize the subjects with sarcoidosis according to differences in sex, age, and the presence of specific organ involvement. However, significant interactions among these factors precluded a clear conclusion based on simple comparison. This study aimed to clarify the age- and sex-stratified prevalence of specific organ involvement and the heterogenous nature of sarcoidosis. Using the data of 9,965 patients who were newly registered into a database at the Ministry of Health, Labour and Welfare, Japan between 2002 and 2011, we evaluated the age- and sex-specific prevalence of the eye, lung, and skin involvement of sarcoidosis. We also attempted corresponding analysis considering multiple factors. As compared with several decades ago, the monophasic age distribution in men became biphasic, and the biphasic distribution in women, monophasic. The prevalence of pulmonary and cutaneous lesions was significantly associated with age, whereas the prevalence of ocular involvement showed a biphasic pattern. The prevalence of bilateral hilar lymphadenopathy was significantly higher, whereas the prevalence of diffuse lung shadow was significantly lower, in subjects with ocular involvement than those without ocular involvement. Corresponding analysis visually clarified the complex interactions among factors. Our results contribute to a better understanding of the heterogeneous features of sarcoidosis.

Respiratory muscle involvement in sarcoidosis

ABSTRACTIntroduction: In sarcoidosis, muscle involvement is common, but mostly asymptomatic. Currently, little is known about respiratory muscle and diaphragm involvement and function in patients with sarcoidosis. Reduced inspiratory muscle strength and/or a reduced diaphragm function may contribute to exertional dyspnea, fatigue and reduced health-related quality of life. Previous studies using volitional and non-volitional tests demonstrated a reduced inspiratory muscle strength in sarcoidosis compared to control subjects, and also showed that respiratory muscle function may even be significantly impaired in a subset of patients.Areas covered: This review examines the evidence on respiratory muscle involvement and its implications in sarcoidosis with emphasis on pathogenesis, diagnosis and treatment of respiratory muscle dysfunction. The presented evidence was identified by a literature search performed in PubMed and Medline for articles about respiratory and skeletal muscle function in sarcoidosis through to January 2018.Expert commentary: Respiratory muscle involvement in sarcoidosis is an underdiagnosed condition, which may have an important impact on dyspnea and health-related quality of life. Further studies are needed to understand the etiology, pathogenesis and extent of respiratory muscle involvement in sarcoidosis.

Prognostic significance of ventricular late potentials in patients with pulmonary sarcoidosis

Early detection of cardiac involvement in sarcoidosis is difficult but essential to achieve optimal treatment. Signal-averaged electrocardiography (SAECG) can detect subtle cardiac electrical abnormalities termed late potentials (LPs) and would be useful for the early diagnosis of cardiac involvement. This study aims to investigate the prognostic significance of LP in patients with pulmonary sarcoidosis. We prospectively studied 74 patients with pulmonary sarcoidosis without overt electrocardiographic abnormalities. All participants underwent SAECG, cardiac echocardiography, and 24-hour ambulatory Holter monitoring. Serum angiotensin-converting enzyme and B-type natriuretic peptide levels were also evaluated. We followed these patients for the evaluation of incidence of cardiac events including cardiac death, arrhythmias, and heart failure requiring hospital admission. Of the studied population, 29 patients (39.2%) had detectable LP. During a mean follow-up period of 9.8 years, 8 patients with LPs had cardiovascular events, including development of complete atrioventricular block (n = 4), ventricular tachycardia (n = 2), and heart failure (n = 2). Meanwhile, only 1 of 45 patients without LP developed cardiac event (heart failure). Multivariate analyses revealed that LPs were associated with an increased risk of developing cardiac events (hazard ratio 9.66; 95% confidence interval 1.20-78.01; P = .033) whereas age, sex, serum angiotensin-converting enzyme and B-type natriuretic peptide levels, number of premature ventricular contractions on 24-hour Holter monitoring, and echocardiographic parameters were not associated with subsequent cardiac events. SAECG might possibly be useful for the early detection of cardiac sarcoidosis and, if independently validated, could eventually be considered as a screening test for further risk stratification.

Endocan Levels and Endothelial Dysfunction in Patients With Sarcoidosis.

A systemic inflammatory reaction is a common feature of both sarcoidosis and atherosclerosis. Endothelial-cell specific molecule 1 (endocan) is a marker of vascular pathology which also shows a correlation with inflammation, endothelial dysfunction, and atherosclerosis. The objective of this study was to evaluate the vascular involvement in sarcoidosis using serum endocan levels and brachial artery flow-mediated dilation (FMD), a marker of endothelial dysfunction. We included 53 patients with sarcoidosis without conventional cardiovascular risk factors and 40 healthy controls. Endothelial function was assessed using FMD. Endocan concentrations were measured using a commercially available enzyme-linked immunoassay. Patients with sarcoidosis had significantly higher endocan levels (306 [68] ng/mL vs 269 [73] ng/mL; P = .039) and lower FMD (2.7% [2.3%-3.2%] vs 8% [5%-13%]; P < .001) compared with the healthy group. A negative correlation was found between endocan levels and FMD in the sarcoidosis group ( r = -.325, P < .007). We conclude that sarcoidosis is associated with high levels of endocan and lower FMD values, which may indicate endothelial dysfunction and an early stage of atherosclerosis.

Clinical characteristics of biopsy-proven renal sarcoidosis in Japan.

Background: Although some articles have described renal sarcoidosis, the incidence among biopsy cases remains unclear. Here, we defined the incidence of renal sarcoidosis among renal biopsy cases and analyzed the clinical course. Methods: We performed an epidemiological study examining renal biopsy cases treated at 5 centers between January 2000 and September 2015 and identified 16 cases (7 men, 9 women; mean (±SD) age, 59.4±18.6 years) out of a total of 14191 renal biopsy cases. Renal involvement of sarcoidosis was defined as granulomatous tubulointerstitial nephritis, tubulointerstitial nephritis without granulomatous lesions, and renal calcinosis. Fifteen of the cases were treated with steroid therapy. One case initially received steroid pulse therapy. The outcome was evaluated based on the estimated glomerular filtration rate (eGFR), CKD stage, and the change in eGFR (ΔeGFR) after treatment. A favorable response was defined as ΔeGFR ≥25%. Results: The incidence of renal sarcoidosis was 0.11%. The mean eGFR was 28.2±16.1 mL/min/1.73 m2. At the last observation, the mean eGFR was 43.7±19.7 mL/min/1.73 m2. Although a favorable response to steroid therapy was found in the majority of cases (10/15, 67%), 12 of the 15 cases (80%) had residual renal dysfunction at the last observation and 8 cases (53%) had moderate to severe renal dysfunction. Conclusion: Renal sarcoidosis is extremely rare among renal biopsy cases. Among cases with an unfavorable response to steroid therapy, pathogenetic mechanisms other than sarcoidosis and severe nephron damage were observed. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 252-260).

Serial observation of electrocardiographic responses to corticosteroid therapy in a patient with right ventricular-predominant cardiac sarcoidosis

Predominant or isolated right ventricular involvement in cardiac sarcoidosis is uncommon, but should always be considered in a case of right ventricular hypertrophy combined with ventricular arrhythmia and/or conduction disturbance. Although improvement in right ventricular hypertrophy and atrioventricular conduction disturbance following corticosteroid therapy has been reported, the detailed serial electrocardiographic responses during corticosteroid therapy, as well as temporal changes in the electrocardiographic, biochemical, and morphological responses, have not been reported. We describe the clinical course and supportive imaging findings of reversible right ventricular hypertrophy and cardiac conduction disturbance in a case of right ventricular-predominant cardiac sarcoidosis.