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Study Objectives: Patients with sarcoidosis experience fatigue and excessive daytime sleepiness (EDS). However, the underlying pathomechanism is unclear. Studies suggested undiagnosed obstructive sleep apnea (OSA) to be an important contributor, but reliable data on prevalence and impact of OSA in sarcoidosis are scarce., Methods: 71 adult patients with sarcoidosis, 1-to-1 matched to 71 adult controls according to sex, age, and body mass index were included. Participants underwent structured interviews (including Epworth Sleepiness Scale [ESS], Fatigue Assessment Scale [FAS], and Functional Outcome of Sleep Questionnaire [FOSQ-30]) and level-3 respiratory polygraphy. OSA was defined as apnea-hypopnea index ≥ 5 events/h. Prevalence of OSA was assessed and possible risk factors for OSA in sarcoidosis were investigated., Results: Mild OSA (AHI ≥ 5 events/h) was prevalent in 32 (45%) sarcoidosis patients vs 22 (31%) controls ( P = .040). Sarcoidosis patients presented higher ESS compared with matched controls ( P = .037). FAS scores (median [quartile] of 21.5 [16, 27.5]) indicated fatigue in sarcoidosis patients. Patients with EDS (ESS ≥ 11) presented reduced FOSQ-30 results (median [quartile] of 16.7 [15.2, 17.8]). ESS, FAS, and FOSQ were not associated with AHI in sarcoidosis patients. Body mass index, sex, neck circumference, and NoSAS score were predictors for OSA in sarcoidosis., Conclusions: The risk for mild OSA is 2.5-fold higher in sarcoidosis patients compared with matched controls. OSA seems not to be the reason for increased sleepiness or fatigue in sarcoidosis. Risk factors such as body mass index, sex, neck circumference, and NoSAS score can be used to screen for OSA in sarcoidosis patients., Clinical Trial Registration: Registry: ClinicalTrials.gov; Name: Obstructive Sleep Apnoea in Sarcoidosis (OSASA); URL: https://clinicaltrials.gov/ct2/history/NCT04156789?V_2=View; Identifier: NCT04156789., Citation: Roeder M, Sievi NA, Schneider A, et al. The prevalence of obstructive sleep apnea in sarcoidosis and its impact on sleepiness, fatigue, and sleep-associated quality of life: a cross-sectional study with matched controls (the OSASA study). J Clin Sleep Med . 2022;18(10):2415-2422., (© 2022 American Academy of Sleep Medicine.)

Background: 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan has no recognized role in diagnosis, prognosis, and disease monitoring in patients with arrhythmic myocarditis., Objectives: The purpose of this study was to investigate the value of FDG-PET scan in arrhythmic myocarditis., Methods: The authors enrolled 75 consecutive patients (age 47 ± 14 years, 65% men) undergoing FDG-PET scan for arrhythmic myocarditis. Myocarditis was diagnosed by endomyocardial biopsy (EMB) and, whenever applicable, cardiac magnetic resonance (CMR)., Results: Indications for FDG-PET scan included either contraindication to CMR (n = 50) or mismatch between CMR and EMB (n = 25). Overall, 50 patients (67%) had positive FDG-PET. Sensitivity was 75% referring to EMB, and 73% to CMR. Specificity was 67% referring to EMB, and 59% to CMR. FDG-PET accuracy was lower in the presence of borderline myocarditis, and either late (>30 days) or on-immunosuppression FDG-PET scanning. Anteroseptal distribution pattern, found in 12 of 50 (24%) patients including 7 of 7 cardiac sarcoidosis cases, was associated with greater occurrence of ventricular arrhythmias and atrioventricular blocks in 4.2 ± 1.7 years of follow-up (10 of 12 vs 7 of 38, and 7 of 12 vs 0 of 38, respectively; both P < 0.001). In 39 patients (52%), FDG-PET was repeated by 13 ± 2 months, allowing immunosuppression withdrawal after FDG uptake normalization either by first (76%) or second reassessment (24%)., Conclusions: FDG-PET scan may be a clinically useful diagnostic technique in arrhythmic myocarditis, in particular when CMR is unsuitable because of irregular heartbeat or implantable cardioverter-defibrillator-related artifacts. Anteroseptal FDG distribution is associated with a worse arrhythmic outcome and should raise the suspicion of cardiac sarcoidosis. During follow-up, repeated FDG-PET allows myocarditis monitoring to guide immunosuppression withdrawal., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Facial nerve palsy is a cardinal manifestation of neurosarcoidosis, but dedicated studies of this disease feature have not been conducted. We sought to clarify the impact of facial palsy on the diagnosis of neurosarcoidosis, its subsequent clinicoradiographic evolution, and eventual treatment decisions., Methods: A single-center retrospective analysis of patients with neurosarcoidosis and facial palsy was conducted over the preceding 10 years (01/01/2011-08/12/2021)., Results: 23/218 (10.6%) patients with neurosarcoidosis developed facial neuropathy. It was the inaugural manifestation of neurosarcoidosis in 17/23 (73.9%) and presented in isolation of other neurologic deficits or extra-facial MRI abnormalities in 12/23 (52.2%). At onset, facial palsy was unilateral in 20/23 (87.0%), and multiple cranial neuropathies were seen in 8/23 (34.8%). Non-facial inflammatory MRI abnormalities were observed in 6/15 (40.0%) patients at onset with leptomeningitis being most common (5/15, 33.3%). 13/23 (56.5%) experienced a second attack of neurosarcoidosis at a median of 8 months, including 3/23 (13.0%) with recurrent facial palsies. In the 12 patients with isolated facial paresis at onset, 4/12 (33.3%) remained free of new deficits or neuroimaging abnormalities by last follow-up. 17/23 (73.9%) eventually required initiation of steroid-sparing immunosuppressants, almost all for development of non-facial disease. The final median House-Brackmann score was 1., Conclusion: Facial neuropathy occurred less commonly than historically reported, and it often acts as a forerunner to systemic sarcoidosis and more widespread neurologic disease. Recurrent attacks of neurosarcoidosis occur early at high frequency following facial palsy. Recovery of facial nerve function is typically excellent., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Objective: To review the current literature regarding cochlear implantation in patients with retrocochlear pathologies and extract speech perception scores between 6 months and 1 year after surgery., Databases Reviewed: PubMed/MEDLINE, Embase and Cochrane CENTRAL via Ovid, CINAHL Complete via Ebsco, and Web of Science., Methods: The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search strategies included keywords and subject headings to maximize retrieval and reflect cochlear implants and retrocochlear pathologies. Patients with previously resected vestibular schwannoma (VS) were excluded., Results: There were 2,524 abstracts screened against inclusion criteria, and 53 studies were included, with individual data available for 171 adult patients. Pathologies included were either observed or irradiated VS (previously operated tumors were excluded) (n = 99, 57.9%), superficial siderosis (n = 39, 22.8%), neurosarcoidosis (n = 11, 6.4%), and previous central nervous system or skull base radiation (n = 22, 12.9%). Mean (standard deviation) postoperative consonant-nucleus-consonant (CNC) word scores were 45.4% (24.2) for observed VS, 44.4% (20.8) for irradiated VS, 43.6% (21.0) for superficial siderosis, 89.5% (3.0) for neurosarcoidosis, and 30.0% (30.2) in patients with previous central nervous system or skull base irradiation. Irradiated compared with observed VS had similar postoperative CNC word scores (effect size, 0.06; p = 0.71). Age, sex, maximal tumor dimension, and neurofibromatosis type 2 status did not significantly impact cochlear implant performance in patients with VS. Eighty-two percent of patients with reported device usage were daily users, and overall, 82% of cases benefitted from cochlear implantation., Conclusion: Cochlear implantation in patients with concomitant retrocochlear pathology generally results in improved speech discrimination scores sustained over time., Competing Interests: J.T.R. is on the advisory board for Cochlear Pty Limited. S.O.M. is an advisor for Advanced Bionics. The remaining authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Background/objectives: To record the prevalence of ocular sarcoidosis (OS) cases present in Northern Ireland as diagnosed using the International Workshop on Ocular Sarcoidosis (IWOS) classification, 2019. There are currently no data regarding OS in this population., Subjects/methods: Retrospective case review of OS cases as identified by IWOS criteria 2019. Mid-year population estimates were used to calculate disease prevalence. Additional data collected included uveitis features, ocular complications and the presence of ocular only or multi-system disease., Results: A total of 86 patients were identified meeting the criteria for a diagnosis of OS, and the prevalence of OS in Northern Ireland was estimated to be 4.5 cases per 100,000. The most common type of uveitis was panuveitis in 36% of cases, and the most common ocular complication was ocular hypertension in 36% of cases and detectable glaucomatous changes in 10%. Overall, 80% of cases presenting with ocular only sarcoidosis subsequently developed second organ involvement at a rate of 14%/person-years. The most common extra-ocular site of sarcoidosis was pulmonary., Conclusions: The Northern Ireland population has a relatively high prevalence of OS compared with other European countries. OS presenting with only ocular involvement progressed to second organ involvement in 80% of patients at a rate of 14%/person-years. Raised intra-ocular pressure with or without glaucomatous damage was a frequent finding. Thoracic CT imaging should be requested if clinical suspicion of OS exists and the presence of lymphopenia has utility in diagnosis with concurrent use of systemic ACE inhibitors., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Cardiac sarcoidosis typically involves the myocardium. Pericardial effusion is uncommon, and symptomatic pericardial disease is even more infrequent. We report the case of a patient presenting with pericarditis as the first manifestation of sarcoidosis. A 50-year-old previously healthy man presented with chest pain and dyspnoea. The electrocardiogram confirmed the diagnosis of pericarditis. Computed tomography of the thorax showed pulmonary infiltrates with mediastinal and hilar adenopathies. Histological analysis of a lymph node biopsy was consistent with sarcoidosis. There was no evidence of myocardial involvement on Magnetic Resonance Imaging (MRI). We reviewed the available English literature and identified 31 cases with sarcoidosis and pericardial involvement. The majority of cases presented as pericardial effusion, which was often the first clinical manifestation of the disease. Pathological diagnosis usually occurs at extra-cardiac locations. Myocardial involvement, an important cause of morbidity and mortality, was found in 25.8% (8/31) of cases. Sarcoidosis should be considered in the differential diagnosis of patients presenting with pericardial disease. The optimal treatment regimen and long-term outcome remain largely unknown. Research in cardiac sarcoidosis should include pericardial disease as a separate manifestation in order to improve the management of this rare but likely underdiagnosed condition.

Background: Cardiac sarcoidosis (CS) predisposes to sudden cardiac death (SCD). Guidelines for implantable cardioverter defibrillators (ICDs) in CS have been issued by the Heart Rhythm Society in 2014 and the American College of Cardiology/American Heart Association/Heart Rhythm Society consortium in 2017. How well they discriminate high from low risk remains unknown., Methods: We analyzed the data of 398 patients with CS detected in Finland from 1988 through 2017. All had clinical cardiac manifestations. Histological diagnosis was myocardial in 193 patients (definite CS) and extracardiac in 205 (probable CS). Patients with and without Class I or IIa ICD indications at presentation were identified, and subsequent occurrences of SCD (fatal or aborted) and sustained ventricular tachycardia were recorded, as were ICD indications emerging first on follow-up., Results: Over a median of 4.8 years, 41 patients (10.3%) had fatal (n=8) or aborted (n=33) SCD, and 98 (24.6%) experienced SCD or sustained ventricular tachycardia as the first event. By the Heart Rhythm Society guideline, Class I or IIa ICD indications were present in 339 patients (85%) and absent in 59 (15%), of whom 264 (78%) and 30 (51%), respectively, received an ICD. Cumulative 5-year incidence of SCD was 10.7% (95% CI, 7.4%-15.4%) in patients with ICD indications versus 4.8% (95% CI, 1.2%-19.1%) in those without (χ 2 =1.834, P =0.176). The corresponding rates of SCD were 13.8% (95% CI, 9.1%-21.0%) versus 6.3% (95% CI, 0.7%-54.0%; χ 2 =0.814, P =0.367) in definite CS and 7.6% (95% CI, 3.8%-15.1%) versus 3.3% (95% CI, 0.5%-22.9%; χ 2 =0.680, P =0.410) in probable CS. In multivariable regression analysis, SCD was predicted by definite histological diagnosis ( P =0.033) but not by Class I or IIa ICD indications ( P =0.210). In patients without ICD indications at presentation, 5-year incidence of SCD, sustained ventricular tachycardia, and emerging Class I or IIa indications was 53% (95% CI, 40%-71%). By the American College of Cardiology/American Heart Association/Heart Rhythm Society guideline, all patients with complete data (n=245) had Class I or IIa indications for ICD implantation., Conclusions: Current ICD guidelines fail to distinguish a truly low-risk group of patients with clinically manifest CS, the 5-year risk of SCD approaching 5% despite absent ICD indications. Further research is needed on prognostic factors, including the role of diagnostic histology. Meanwhile, all patients with CS presenting with clinical cardiac manifestations should be considered for an ICD implantation.

BACKGROUND Sarcoidosis is a systemic granulomatous disease of unknown cause, often affecting the lungs and lymphatic system. Neurologic manifestations of sarcoidosis, called "neurosarcoidosis", can present as cranial neuropathies and occur in an isolated fashion or alongside other systemic findings. These findings occur in about 5% to 15% of individuals, and mainly in women between the ages of 30 and 40 years. Within those subsets of patients who develop neurologic manifestations, ocular manifestations occur 13% to 79% of the time. Less common presentations include secondary glaucoma, intermediate or posterior inflammation, or other neuro-ophthalmic findings. CASE REPORT A 63-year-old White man initially presented with blurry vision, acute glaucoma, and other symptoms closely simulating hypertensive retinopathy. He later developed diplopia and was not accurately diagnosed by general ophthalmologists and a retina specialist. Due to the unusual presentation, hypertensive retinopathy was the incorrect initial working diagnosis and the patient continued to develop more severe symptoms. A multidisciplinary approach to patient care through a nephrology referral led to the final diagnosis of neurosarcoidosis. Prompt treatment improved renal function and ocular disturbances. CONCLUSIONS Retinal cotton-wool spots, glaucoma, and optic nerve swelling are rare presentations of neurosarcoidosis. Unusual vascular symptoms warrant consideration of all vascular diseases and prompts for collaboration through a multidisciplinary team. This case serves to highlight the importance of sarcoidosis as a differential, even in patients with no previous signs of granulomatous disease, and how a team-based approach between multiple specialties improves accuracy, timeliness, and treatment regimen.

Aims: This study evaluated the prognosis and prognostic factors of patients with cardiac sarcoidosis (CS), an underdiagnosed disease., Methods and Results: Patients from a retrospective multicentre registry, diagnosed with CS between 2001 and 2017 based on the 2016 Japanese Circulation Society or 2014 Heart Rhythm Society criteria, were included. The primary endpoint was a composite of all-cause death, hospitalization for heart failure, and documented fatal ventricular arrhythmia events (FVAE), each constituting exploratory endpoints. Among 512 registered patients, 148 combined events (56 heart failure hospitalizations, 99 documented FVAE, and 49 all-cause deaths) were observed during a median follow-up of 1042 (interquartile range: 518-1917) days. The 10-year estimated event rates for the primary endpoint, all-cause death, heart failure hospitalizations, and FVAE were 48.1, 18.0, 21.1, and 31.9%, respectively. On multivariable Cox regression, a history of ventricular tachycardia (VT) or fibrillation [hazard ratio (HR) 2.53, 95% confidence interval (CI) 1.59-4.00, P < 0.001], log-transformed brain natriuretic peptide (BNP) levels (HR 1.28, 95% CI 1.07-1.53, P = 0.008), left ventricular ejection fraction (LVEF) (HR 0.94 per 5% increase, 95% CI 0.88-1.00, P = 0.046), and post-diagnosis radiofrequency ablation for VT (HR 2.65, 95% CI 1.02-6.86, P = 0.045) independently predicted the primary endpoint., Conclusion: Although mortality is relatively low in CS, adverse events are common, mainly due to FVAE. Patients with low LVEF, with high BNP levels, with VT/fibrillation history, and requiring ablation to treat VT are at high risk., Competing Interests: Conflict of interest: Y.M. received an honorarium from Otsuka Pharmaceutical Co. and Novartis Japan. Other authors have nothing to declare., (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Purpose: We retrospectively analyzed the frequency and clinical characteristics of organs rarely involved with sarcoidosis in a cohort of 1158 sarcoidosis patients cared for over nearly 11 years in a university outpatient sarcoidosis clinic., Methods: Data were analyzed from an institution-approved sarcoidosis clinical database containing demographic and clinical characteristics of sarcoidosis outpatients cared for between May 2011 and March 2021 at a university medical center sarcoidosis clinic. The diagnosis of sarcoidosis was established by standard international criteria. Rare organ involvement was defined as an organ not individually listed in the Word Association of Sarcoidosis and Other Granulomatous Disorders Sarcoidosis Organ Assessment Instrument., Results: Twenty-six of the 1158 patients (2.2%) had 27 instances of rare organ involvement. The most common rare organ involved was the GI tract, followed by the testicle and then the female reproductive tract. Only 1 of the 26 patients with rare sarcoidosis organ involvement required therapy for that organ involvement. In comparison with sarcoidosis patients without rare organ involvement, patients with rare organ involvement were more likely to be black, have more total organs involved with sarcoidosis, and more likely require anti-sarcoidosis therapy at some point during the course of their disease. There were no differences between these groups in terms of sex, age at diagnosis, and most recent percent predicted forced vital capacity., Conclusions: Rare sarcoidosis organ involvement occurred in 2.2 percent of this cohort. Although rare organ involvement rarely required treatment, it was associated with more extensive disease that was more likely to require therapy., Competing Interests: Declaration of competing interest No author has a conflict of interest in terms of publication of this manuscript., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: In cardiac sarcoidosis (CS), the risk and predictors of new-onset atrial fibrillation (AF) are poorly known., Objectives: The authors evaluated the incidence and characteristics of AF in newly diagnosed CS., Methods: The authors studied 118 patients (78 women, mean age 50 years) with AF-naive CS having undergone cardiac 18 F-fluorodexoyglucose positron emission tomography ( 18 F-FDG PET) at the time of diagnosis. Details of patient characteristics and medical or device therapy were collected from hospital charts. The PET scans were re-analyzed for presence of atrial and ventricular inflammation, and coincident cardiac magnetic resonance (CMR) studies and single-photon emission computed tomography (SPECT) perfusions were analyzed for cardiac structure and function, including the presence and extent of myocardial scarring. Detection of AF was based on interrogation of intracardiac devices and on ambulatory or 12-lead electrocardiograms., Results: Altogether 34 patients (29%) suffered paroxysms of AF during follow-up (median, 3 years) with persistent AF developing in 7 patients and permanent AF in 4. The estimated 5-year incidence of AF was 55% (95% CI: 34%-72%) in the 39 patients with atrial 18 F-FDG uptake at the time of diagnosis vs 18% (95% CI: 10%-28%) in the 79 patients without atrial uptake (P < 0.001). In cause-specific Cox regression analysis, atrial uptake was an independent predictor of AF (P < 0.001) with HR of 6.01 (95% CI: 2.64-13.66). Other independent predictors were an increased left atrial maximum volume (P < 0.01) and history of sleep apnea (P < 0.01). Ventricular involvement by PET, SPECT, or CMR was nonpredictive. Symptoms of AF prompted electrical cardioversion in 12 patients (35%). Three of the 34 patients (9%) experiencing AF suffered a stroke versus none of those remaining free of AF., Conclusions: In newly diagnosed CS, future AF is relatively common and associated with atrial inflammation and enlargement on multimodality cardiac imaging., Competing Interests: Funding Support and Author Disclosures This study was supported by a Finnish government grant for medical research, the Aarne Koskelo Foundation, and the Finnish Foundation for Cardiovascular Research. Dr Uusitalo has received scientific collaboration with and a lecture fee from GE Healthcare; and has received a lecture fee and has advisory board activity with Pfizer. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Renal sarcoidosis frequently causes granulomatous interstitial nephritis, but clinically relevant nephritis is uncommon. IgA nephropathy caused by sarcoidosis is usually associated with milder stages of renal dysfunction, and only one case of rapidly progressive IgAN has been reported to date. We present an interesting case of a patient with a rapidly progressive form of IgA nephropathy caused by sarcoidosis that was successfully treated.  DOI: 10.52547/ijkd.7027.

Manifestations of sarcoidosis are variable and non-specific. We are reporting on the case of a 23-year-old man who presented multiple bilateral pulmonary nodules spiculated with peripherical micronodules (galaxy sign). He had no clinical symptom. Investigations led to the diagnosis of sarcoidosis. The galaxy sign is a rare manifestation of sarcoidosis which can be useful for diagnosis., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: To clarify the proportion of ocular sarcoidosis with severe, refractory, and prolonged inflammation and their association with ocular complications and visual prognosis., Study Design: Multicenter, retrospective, longitudinal cohort study., Methods: Three hundred and twenty-three eyes of 164 patients (45 men; 119 women) with ocular sarcoidosis who visited Hokkaido University Hospital and Yokohama City University Hospital from 2010 to 2015. We newly defined severe, refractory, and prolonged inflammation in ocular sarcoidosis, and investigated their proportions, ocular complications and final visual acuity from medical records of our sarcoidosis patients., Results: The eyes with severe inflammation numbered 72/323 (22.3%), with refractory inflammation, 80/323 (24.8%), and with prolonged inflammation, 91/323 (28.2%). The number of eyes having neither severe, refractory, nor prolonged inflammation (defined as none) was 114/323 (35.3%). The numbers of eyes that reached irreversible visual dysfunction were 6/72 (8.3%) of those with severe inflammation, 10/80 (12.5%) with refractory inflammation, 12/91 (13.2%) with prolonged inflammation, and 4/114 (6.2%) with none. As complications, cataract (62.2%), glaucoma (28.5%), epiretinal membrane (24.1%), cystoid macular edema (22.6%), vitreous hemorrhage (2.8%), choroidal atrophy (2.5%), macular degeneration (1.2%), macular hole (0.9%) and retinal detachment (0.3%) were identified. Among them, secondary glaucoma (16 eyes) and macular degeneration (4 eyes) were major complications related to irreversible visual dysfunction., Conclusions: Although most of the patients with ocular sarcoidosis had a relatively good visual prognosis, some developed severe, refractory, and/or prolonged inflammation related to the development of ocular complications, that resulted in poor visual prognosis., (© 2022. Japanese Ophthalmological Society.)

Purpose of Review: In chronic pulmonary sarcoidosis, the transition from the inflammatory to the fibrotic stage of the lungs occurs in about 10-20% of cases, eventually causing end-stage fibrotic disease. To date, pathogenetic mechanisms and clinical management remain challenging; thus, we highlight the recent evidence in pulmonary fibrotic processes, clinical signs for an early detection and the potential role of the current investigated antifibrotic agents and promising targeted therapies., Recent Findings: Recent findings of relevant key cellular pathways can be considered as a glimmer of light in the complexity of sarcoidosis. In some patients, granulomas persist and serve as a nidus for fibrosis growth, sustained by several fibrosis-stimulating cytokines. Preclinical studies have detected profibrotic, antifibrotic and pleiotropic T cells as promoters of fibrosis. Epigenetics, genetics and transcriptomics research can lead to new target therapies. Antifibrotic drug nintedanib has shown a positive effect on non-idiopathic pulmonary fibrosis fibrotic lung diseases including fibrotic sarcoidosis; other antifibrotic drugs are under investigation., Summary: Pulmonary fibrosis strongly impacts the outcome of sarcoidosis, and a better understanding of the underlying pathogenic mechanisms can facilitate the development of novel treatments, improving clinical care and life expectancy of these patients. The greatest challenge is to investigate effective antifibrotic therapies once fibrosis develops. The role of these findings in fibrotic sarcoidosis can be translated into other interstitial lung diseases characterized by the coexistence of inflammatory and fibrotic processes., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Sarcoidosis is a multi-systemic disorder of unknown aetiology that primarily involves the pulmonary system with the histological hall mark of a non-caseating granuloma. Isolated hepatic involvement is a manifestation of sarcoidosis., Objective: We report an incidental finding of hepatic sarcoidosis in a hypertensive and diabetic patient who presented with abdominal pain and fatigue., Methods: A case report of sole diagnosis of hepatic sarcoidosis from a Gastroenterology service in Port Harcourt metropolis Nigeria. A literature search was made using the words 'sarcoidosis' and 'hepatic' in PubMed Central, Cochrane and Google search engines. From the search results, literature in English were extracted and reviewed., Results: The index case was referred on account of hepatomegaly with associated abdominal pain and fatigue. A diagnosis of isolated hepatic sarcoidosis was made from elevated liver enzymes, elevated angiotensin converting enzyme, sero-negative autoimmune studies, hepatomegaly with liver nodule on abdominal computerized tomography scan and non-caseating granuloma from liver biopsy. There was no evidence of other systems involvement., Conclusion: Hepatic sarcoidosis is rare in African literature more so as no specific laboratory or radiological test is diagnostic. Delayed diagnosis and underreporting of hepatic sarcoidosis are likely in our environment due to the elaborate evaluation needed., Competing Interests: The Authors declare that no competing interest exists., (Copyright © 2022 by West African Journal of Medicine.)

Competing Interests: Conflict of interest: None declared.

Background Sarcoidosis is an inflammatory, noncaseating, granulomatous disorder of unknown cause that can affect any body system and is associated with cardiovascular disease including sudden cardiac death (SCD). Cardiac involvement in sarcoidosis is associated with higher risk of SCD, but the SCD risk in the general sarcoidosis population is unknown. We aimed to determine the risk of SCD in people with sarcoidosis versus the matched general population. Methods and Results A population-based cohort of sarcoidosis and age- and sex-matched comparators from January 1, 1976 to December 31, 2013 was used; presence of other comorbidities in the comparator group was not an exclusion criterion. Mortality, including time, place, and cause of death were measured and manually adjudicated for SCD events. Incidence rates are reported per 100 000 person-years, and Cox models were used for group comparisons. Of the 345 incident cases of sarcoidosis (171 men; 50%) there were 58 reported deaths; 10 were definite/probable SCD versus 57 all-cause and 9 SCDs in comparators. Median follow-up was 12.9 years (interquartile range, 6.0-23.4 years) . Incidence rate of SCD in sarcoidosis was 192 (95% CI, 92-352) versus 155 (95% CI, 71-294) in comparators (hazard ratio [HR], 1.28 (95% CI, 0.52-3.17). Nocturnal deaths were more frequent in sarcoidosis 57 (95% CI, 12-168) versus 17 (95% CI, 0.4-95) (HR, 3.76 [95% CI, 0.39-36.47]). No significant differences were detected between the groups by sex, age, calendar year of diagnosis, or disease duration. Conclusions In a population-based cohort of patients with sarcoidosis, the risk for SCD compared with matched comparators was not increased. There were more nocturnal deaths among patients with sarcoidosis, yet this was statistically insignificant.

Cardiac sarcoidosis (CS) is an important cause of cardiomyopathy. The trajectory of left ventricular ejection fraction (LVEF) in patients with CS undergoing treatment remains unclear. Patients with CS who were treated with corticosteroids and who underwent transthoracic echocardiography were studied. Baseline characteristics, treatment, echocardiographic data (including baseline to follow-up change in LVEF), and outcomes were retrospectively evaluated. Among 100 patients, 55 had baseline reduced LVEF (<50%), and 45 had preserved LVEF (≥50%). At follow-up, 82% of patients demonstrated stable or improved LVEF. Change in LVEF was significantly higher in the baseline reduced than in the preserved LVEF group (5% [interquartile range 0 to 15] vs 0% [interquartile range -10% to 5%], p = 0.001). There was no difference in corticosteroid exposure or use of heart failure guideline-directed medical therapy between patients who did experience improvement in LVEF and those who did not experience improvement in LVEF. On multivariable analysis, baseline reduced LVEF (Odds ratio 54.89, 95% confidence interval 3.84 to 785.09, p = 0.003) and complete heart block (Odds ratio 28.88, 95% confidence interval 2.17 to 383.74, p = 0.011) at presentation were significantly associated with reduced LVEF after treatment. In conclusion, most patients with CS treated with corticosteroids maintain or improve LV systolic function. Cardiac characteristics at presentation impact prognosis in CS, despite treatment., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The presented clinical observation reflects the difficulties of differential diagnosis of progressive kidney damage in a patient with sarcoidosis who has undergone a new coronavirus infection. The differential circle included interstitial nephritis as an exacerbation of the underlying disease, acute drug-induced kidney injury, acute glomerulonephritis. Nephrobiopsy confirmed the diagnosis of acute sarcoid tubulointerstitial nephritis with acute tubular necrosis. Timely administration of corticosteroids led to the control of the sarcoidosis process, restoration of kidney function.

Purpose: Uveitis accounts for 10-15% of all cases of blindness in the developed world. Uveitic macular edema (UME) is a primary cause of permanent visual impairment in patients with uveitis. Because proinflammatory mediators elicit inflammation and lead to UME, we determined the profiles of proinflammatory mediators associated with complications, such as ME, in the vitreous humor of patients with panuveitis related to Behçet's disease (BD) and sarcoidosis., Methods: In this retrospective study, we enrolled 21 patients with uveitis, including 6 with BD and 15 with sarcoidosis, and 15 patients with idiopathic epiretinal membrane (iERM) at the Department of Ophthalmology, Kyushu University Hospital, between January 2008 and April 2016. Vitreous concentrations of 32 proinflammatory mediators, including cytokines and soluble receptors of tumor necrosis factor (TNF) and interleukin (IL)-6 families, were assessed using a bead-based multiplex assay and their association with clinical data was examined., Results: The levels of proinflammatory mediators, including a proliferation-inducing ligand (APRIL), B cell activating factor belonging to the TNF family (BAFF), soluble cluster of differentiation 30 (sCD30), soluble TNF receptor-1 (sTNFR1), sTNFR2, TNF-α, IL-6, and soluble IL-6 receptor-α (sIL-6Rα), were significantly higher in patients with uveitis. With regard to clinical parameters in patients with uveitis, vitreous levels of BAFF and sIL-6Rα were prominently elevated in patients with UME compared to in those without UME (P < 0.01, respectively)., Conclusions: Our results suggest that elevated vitreous levels of BAFF and sIL-6Rα are associated with the pathogenesis of UME in patients with panuveitis related to BD and sarcoidosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Sarcoidosis is a systemic granulomatous disease, characterized by the formation of non-necrotizing granulomas. Even though granulomas are frequently found in liver biopsy, related symptoms rarely occur. In the current article, a case report is pictured to increase the knowledge on portal hypertension in hepatic sarcoidosis., Clinical Situation: A 62-year-old female was diagnosed with variceal bleeding for which elastic banding was performed. The patient was admitted to the intensive care unit (ICU) as the bleeding persisted and she evolved in hemorrhagic shock. Liver ultrasound detected nodular hepatomegaly and partial portal thrombosis. Chest CT showed diffuse hilar adenopathies and interstitial micronodular lesion. Finally, PET-CT detected metabolic active liver, bone marrow, and upper and lower diaphragmatic adenopathies., Clinical Resolution: Multidisciplinary discussion brought major advantages in rapid diagnosis and prompt effective treatment. Cirrhosis was diagnosed by liver nodularity on imaging and liver biopsy. Sarcoidosis diagnosis was supported by the biopsies of liver and lymph node, which yielded non-caseating granulomas infiltration. Chest CT scan and PET-CT were also consistent with this diagnosis. The complementary analysis excluded differential diagnosis. The patient was treated with high-dose methylprednisolone with notable clinical improvements and discharge from the ICU., Conclusion: Hepatic sarcoidosis can present as life-threatening bleeding due to variceal bleeding caused by portal hypertension. Differential diagnosis is broad when hepatic sarcoidosis is suspected. Therefore, a multidisciplinary discussion is warranted. Anatomopathological examination of two potentially involved organs should be considered to make the appropriate diagnosis. Further studies are requested to investigate the pathophysiological mechanism of portal hypertension.

Objective and Design: A cross-sectional single-center study was conducted to assess cytokine levels in aqueous humor (AH) and plasma of three different uveitis entities: definite ocular sarcoidosis (OS), definite OS associated with QuantiFERON®-TB Gold test positivity (Q + OS) and presumed tubercular uveitis (TBU)., Subjects: Thirty-two patients (15 OS, 5 Q + OS, 12 TBU) were included., Methods: Quantification of selected cytokines was performed on blood and AH samples collected before starting any treatment. Statistical analysis was conducted using the Kruskal-Wallis test, the Mann-Whitney or Fisher test and the Principal Component Analysis (PCA)., Results: IL-6, IL-8 and IP-10 levels were higher in AH samples than in peripheral blood. In AH samples, BLC, IL-8 and IP-10 were significantly higher in definite OS than in presumptive TBU. There were no statistically significant differences in terms of cytokine levels between Q + OS and presumptive TBU. PCA showed a similar cytokine pattern in the latter two groups (IFNγ, IL-15, IL-2, IP-10, MIG), while the prevalent expression of BLC, IL-10 and MIP-3 α was seen in definite OS., Conclusions: The different AH and plasma cytokine profiles observed in OS compared to Q + OS and TBU may help to differentiate OS from TBU in overlapping clinical phenotypes of granulomatous uveitis (Q + OS)., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Purpose: To assess ocular involvement in sarcoidosis and to analyze the manifestations of ocular sarcoidosis (OS) in Taiwan., Methods: A retrospective review was performed in 364 patients diagnosed as sarcoidosis at National Taiwan University Hospital from 2015 to 2019. The OS diagnosis was based on the revised criteria of International Workshop on Ocular Sarcoidosis. Demographics, clinical manifestations, treatment and effects, complications, visual outcomes and risk factors of poor vision (<20/200) were analyzed., Results: A total of 122 eyes of 66 patients (13 males and 53 females) with OS were identified. Bilateral involvement accounted for 84.8%. Thirty-five patients were diagnosed with definite OS, 14 with presumed OS and 17 with probable OS. The average age was 51.9 ± 14.1 years, with no significant difference in sex. Most patients presented with panuveitis (61 eyes, 50.0%) and posterior uveitis (52 eyes, 42.6%). Systemic steroid was the mainstay treatment, and immunosuppressants or anti-metabolic agents were supplemented. Common complications included cataract (50.8%), ocular hypertension/glaucoma (25.4%) and posterior synechiae (20.5%). Forty-three eyes (36.1%) and 74 eyes (62.2%) had a final vision of 20/20 and 20/40 or better, respectively. The causes of poor visual outcome were diverse, and generalized estimating equations analysis indicated that female and poor initial vision were risk factors., Conclusion: Uveitis is an early sign of sarcoidosis and it might result in several complications. The overall visual outcomes were good if patients received proper treatment. Both poor initial vision and females that tended to have more severe complications were associated with poor outcome., Competing Interests: Declaration of competing interest The authors report no conflicts of interest in this work. No author has a financial or proprietary interest in any material or method mentioned., (Copyright © 2022 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

A 42-year-old man with no relevant medical history presented with a painless, red, disfiguring skin lesion located on the nose. He was diagnosed with lupus pernio, a rare, cutaneous subtype of sarcoidosis which is relatively resistant to therapy and is associated with systemic sarcoidosis.

Ventricular arrhythmias are a life-threatening factor in cardiac sarcoidosis (CS), posing a significant therapeutic challenge. Stellate ganglion phototherapy (SGP), a non-invasive procedure for modification of the sympathetic nervous system, is an effective treatment for refractory ventricular tachycardia (RVT). However, there are limited data on the efficacy of SGP for RVT in patients with CS. In our case report, we found that SGP was effective for treating RVT in a patient with CS.We present the case of a man in his 60s with multiple cardioversions of implantable cardioverter defibrillator for ventricular tachycardia. The patient was administered prednisolone for the management of CS, which subsequently led to an increase in anti-tachycardia pacing for ventricular tachycardias. We introduced SGP to suppress RVT and anti-tachycardia pacing decreased from 371 to 25 events. Thus, SGP could be a feasible option for the management of RVT in patients with CS., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Peripheral nerve disorders in sarcoidosis consist of granulomatous neuropathy and non-granulomatous small fiber neuropathy (SFN), which differ in their underlying pathology, diagnostic methods and treatment. While granulomatous nerve involvement is rare in sarcoidosis, SFN is reported in over 40% of systemic cases. Distal symmetric polyneuropathy and asymmetric polyradiculoneuropathy are the most common presentations of granulomatous neuropathy, which typically responds to corticosteroids. In contrast, SFN is often manifested as non-length dependent pain and paresthesias that may improve with intravenous immune globulin or infliximab. Early recognition and treatment of sarcoidosis neuropathy can lead to improved outcomes and patient quality of life., (Copyright © 2022 Elsevier B.V. All rights reserved.)

We report the case of a woman in her 30s who was referred to the ear, nose and throat department with sudden onset left-sided sensorineural hearing loss (SNHL), left anterior uveitis and erythematous lower limb lesions with bilateral pitting oedema. Based on her symptoms, an underlying inflammatory systemic disease was suspected. Autoantibodies were negative but an X-ray and high-resolution CT scan of the chest were suggestive of sarcoidosis, which was confirmed on endoscopic bronchial biopsy. Following treatment with a course of oral steroids, the patient's hearing has improved but she still suffers from episodes of uveitis. While immune-mediated inner ear disorders are a recognised cause of SNHL, sarcoidosis is a very rare cause. This case demonstrates the importance of screening for systemic autoimmune aetiology in SNHL and highlights the importance of an effective multidisciplinary team in the diagnosis and management of these patients., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

A woman in her mid-50s with IgA nephropathy, sarcoidosis and steroid-induced diabetes mellitus presented with generalised paraesthesia and spontaneous tetany. She had received denosumab 60 mg subcutaneously 8 weeks previously for parathyroid hormone independent hypercalcaemia.At admission, she had severe hypocalcaemia (5 mg/dL), hypophosphataemia (1.9 mg/dL), hypomagnesaemia (1.4 mg/dL) and elevated serum creatinine (1.48 mg/dL) with prolonged QTc (corrected QT interval) on electrocardiograph. She initially received intravenous calcium and magnesium followed by oral calcium carbonate and calcitriol. Her prednisolone dose was tapered to 5 mg/day. Evaluation showed secondary hyperparathyroidism (1474 pg/mL) and elevated 1,25-dihydroxy vitamin D (195 pg/mL). After 1 week of oral calcium carbonate (3000 mg/day) and calcitriol (1.5 µg/day), she achieved normocalcaemia (8.1 mg/dL).To conclude, denosumab for hypercalcaemia with renal insufficiency causes prolonged severe symptomatic hypocalcaemia and hypophosphataemia mimicking hungry bone syndrome. It is important to periodically monitor for hypocalcaemia after denosumab., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

The purpose of our work is to describe the actual knowledge concerning etiopathogenesis, clinical manifestations, diagnostic procedures, complications and therapy of ocular sarcoidosis (OS). The study is based on a recent literature review and on the experience of our tertiary referral center. Data were retrospectively analyzed from the electronic medical records of 235 patients (461 eyes) suffering from a biopsy-proven ocular sarcoidosis. Middle-aged females presenting bilateral ocular involvement are mainly affected; eye involvement at onset is present in one-third of subjects. Uveitis subtype presentation ranges widely among different studies: panuveitis and multiple chorioretinal granulomas, retinal segmental vasculitis, intermediate uveitis and vitreitis, anterior uveitis with granulomatous mutton-fat keratic precipitates, iris nodules, and synechiae are the main ocular features. The most important complications are cataract, glaucoma, cystoid macular edema (CME), and epiretinal membrane. Therapy is based on the disease localization and the severity of systemic or ocular involvement. Local, intravitreal, or systemic steroids are the mainstay of treatment; refractory or partially responsive disease has to be treated with conventional and biologic immunosuppressants. In conclusion, we summarize the current knowledge and assessment of ophthalmological inflammatory manifestations (mainly uveitis) of OS, which permit an early diagnostic assay and a prompt treatment.

Purpose: To identify peripheral blood transcriptome differences in uveitis patients with sarcoidosis compared to those with Vogt-Koyanagi-Harada (VKH) syndrome and controls., Methods: Ten patients with uveitis compatible with sarcoidosis (eight with pulmonary sarcoidosis, one with central nervous system sarcoidosis, and one with conjunctival sarcoidosis), nine patients with VKH, and nine healthy controls were prospectively enrolled., Results: Ten genes exhibited a four-fold difference in expression in sarcoidosis patients compared to controls, many being involved in regulating inflammatory processes or cellular responses to microbes., Conclusions: This research suggests that the transcriptome in sarcoidosis is robust enough to be detected in the peripheral blood and that sarcoidosis can be distinguished from healthy controls. Differentially expressed genes may serve as candidates warranting further investigation with respect to disease pathophysiology and may provide additional information, such as ability to stratify patients based on associated disease severity and anatomical location of inflammation within the eye.

Objective: We aimed to determine the prevalence and clinical characteristics of self-reported hyperthyroidism in patients with sarcoidosis., Methods: A national registry-based study investigating 3836 respondents to the Sarcoidosis Advanced Registry for Cures questionnaire in the period between June 2014 and August 2019 was conducted. This registry is generated from a web-based questionnaire that is self-reported by patients with sarcoidosis. We compared patients with sarcoidosis who had hyperthyroidism with those who did not. We used multivariate logistic regression analysis to study the association between hyperthyroidism and different cardiac manifestations in patients with sarcoidosis., Results: Three percent of the study respondents self-reported having hyperthyroidism and were generally middle-aged Caucasian women. Compared with patients without hyperthyroidism, patients with hyperthyroidism had more sarcoidosis-related comorbidities (59% vs 43%, P = .001) and more steroid-related comorbidities (56% vs 44%, P = .01), but there was no difference in the sarcoidosis-specific treatments they received, which included corticosteroids. Patients with hyperthyroidism reported sarcoidosis involvement of the heart (26.6% vs 14.9%, P = .005), kidneys (14.9% vs 8%, P = .033) and sinuses (17.7% vs 10.2%, P = .030) more frequently. Cardiac manifestations that were more frequently reported in patients with hyperthyroidism included atrial arrhythmias (11.3% vs 6.3%, P = .046), ventricular arrhythmias (17.2% vs 7.5%, P < .001), congestive heart failure (10.4% vs 5%, P = .017), and heart block (9.4% vs 4.7%, P = .036)., Conclusion: Hyperthyroidism is infrequent in patients with sarcoidosis but is potentially associated with different cardiac manifestations. We suggest considering routine screening for hyperthyroidism in patients with sarcoidosis, especially in those with cardiac involvement. Further studies are needed to investigate the impact of identifying and treating hyperthyroidism in patients with sarcoidosis., (Copyright © 2022 AACE. Published by Elsevier Inc. All rights reserved.)

Cardiac sarcoidosis (CS) frequently leads to ventricular tachycardia (VT), which is often refractory to antiarrhythmic and/or immunosuppressive medications and requires catheter ablation. We conducted a systematic review and meta-analysis to evaluate the role of catheter ablation in patients with refractory VT undergoing catheter ablation. We searched PubMed, Embase, and Scopus databases from their inception to December 31, 2021 with search terms "cardiac sarcoidosis" AND "electrophysiological studies OR ablation." Fifteen studies were ultimately included for evaluation. Patient demographics, VT mapping, and acute and long-term procedural outcomes were extracted. A total of 15 studies were included in our meta-analysis, with a total of 401 patients, of whom 66% were male, with ages ranging from 39 to 64 years. A total of 95% of patients were on antiarrhythmics and 79% of patients were on immunosuppressants. Left ventricular ejection fraction ranged from 35% to 49% and procedure duration ranged from 269 to 462 minutes. Ablation was reported using both irrigated and nonirrigated catheter tips. A total of 25% of patients (84/339) underwent repeat ablation. Acute procedural success was achieved in 57% (161/285). Procedure complications occurred in 5.7% (17/297) procedures. VT recurrence after first ablation was 55% (confidence interval 48% to 63%, 213/401); VT recurrence after multiple ablations was 37% (81/220). The composite end point of death, heart transplant, and left ventricular assist device implantation was 21% (confidence interval 14% to 30%, 55/297). In conclusion, catheter ablation is a useful modality in patients with CS with refractory VT. However, patients with CS presenting with refractory VT after undergoing VT ablation carry a poor prognosis., Competing Interests: Disclosures The authors have NO conflict of interests to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: The American College of Medical Genetics and Genomics (ACMG) and the Association for Molecular Pathology call for cautious interpretation of variants as causative of a monogenic disorder by stringent standards. We aimed to reclassify the pathogenicity of nucleotide binding oligomerization domain containing 2 (NOD2) variants according to the ACMG guidelines and to characterize clinical features in patients whose ocular disease might actually be explained by Blau syndrome., Design: Genetic analysis and descriptive study., Participants: A total of 1003 unrelated healthy individuals and 3921 sporadic patients who presented with uveitis., Methods: Whole-exome sequencing was performed on all healthy participants and 551 patients with uveitis, and targeted NOD2 resequencing was performed on the remaining 3370 patients with uveitis. Pathogenicity for Blau syndrome was classified for NOD2 variants identified by sequencing in study participants according to the ACMG guidelines. Clinical manifestations were compared among NOD2 variants of different levels of classification., Main Outcome Measures: Pathogenicity of variants., Results: Eight NOD2 gain-of-function mutations, p.R334W, p.R334Q, p.E383K, p.G481D, p.W490S, p.M513T, p.R587C, and p.N670K, were classified as pathogenic, and 66 patients (1.7%) with uveitis were diagnosed with Blau syndrome due to these mutations. Of 66 with Blau syndrome, anterior uveitis accounted for 39.4%, posterior uveitis for 9.1%, and panuveitis for 51.5%. A proportion of 21.2% of Blau syndrome presented as multifocal choroiditis, 48.5% had papillitis, and 74.2% showed retinal microvasculitis detected by fundus fluorescein angiography. Six NOD2 variants, p.P268S, p.R311W, p.R471C, p.A612T, p.R702W, and p.V955I, were considered nonpathogenic for Blau syndrome and were identified in 96 patients with uveitis. The incidence of bilateral uveitis (86.4%), secondary glaucoma (47.0%), epiretinal membrane (7.6%), choroidal neovascularization (4.6%), retinal atrophy (10.6%), arthritis (69.7%), joint deformity (51.5%), and skin rash (40.9%) was higher in Blau syndrome than in patients with uveitis carrying non-Blau-causing NOD2 variants. Patients with Blau syndrome permanently experienced overall poorer best-corrected visual acuity. Several rare NOD2 mutations, p.I722L (2 cases), p.T476P (1 case), p.T476del (1 case), and p.R439H (1 case), were newly identified., Conclusions: Pathogenic NOD2 variants for Blau syndrome were limited to those gain-of-function mutations and were associated with a high risk for arthritis, skin rash, permanent visual loss, and ocular complications in patients with uveitis., (Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Orbital sarcoid is a rare entity and may be the first manifestation of systemic sarcoidosis. We report a case of orbital sarcoidosis where diagnosis was complicated by a history of lower eyelid blepharoplasty. The patient presented with progressive swelling of the left lower eyelid, which was assumed to be a late complication of her surgery. After failing multiple treatments, MRI orbits was obtained and revealed an enhancing lesion in the left orbit inseparable from the lacrimal gland and inferior oblique muscle. Biopsy showed noncaseating granulomatous inflammation, and the patient was eventually diagnosed with sarcoidosis., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Background: Sarcoidosis is a systemic disease characterized by granulomatous inflammation. Cardiac involvement is associated with increased morbidity. However, differences in clinical characteristics and outcomes based on initial sarcoidosis organ manifestation in patients with cardiac sarcoidosis (CS) have not been described., Methods: A retrospective cohort of 252 patients with CS at an urban, quaternary medical center was studied. Presentation, treatment and outcomes of de novo CS and prior ECS groups were compared. Survival free of primary composite outcome (left ventricular assist device implantation, orthotopic heart transplantation (OHT), or death) was assessed., Results: There were 124 de novo CS patients and 128 with prior ECS at time of CS diagnosis. De novo CS patients were younger at CS diagnosis (p = 0.020). De novo CS patients had a more advanced cardiac presentation: lower left ventricular ejection fraction (LVEF) (p < 0.001), more frequent sustained ventricular arrhythmias (VA) (p = 0.001), and complete heart block (p = 0.001). During follow-up, new VA (p < 0.001), ventricular tachycardia ablation (p < 0.001), and OHT (p = 0.003) were more common in the de novo CS group. Outcome free survival was significantly shorter for de novo CS patients (p = 0.005), with increased hazard of primary composite outcome (p = 0.034) and development of new VA (p = 0.027) when compared to ECS patients. Overall mortality was similar between groups., Conclusion: Patients presenting with CS as their first recognized organ manifestation of sarcoidosis have an increased risk of adverse cardiac outcomes as compared to those with a prior history of ECS. Improved awareness and diagnosis of CS is warranted for earlier recognition., (Copyright © 2022 Elsevier Ltd. All rights reserved.)