Recurrent Vogt-Koyanagi-Harada disease presenting with diffuse orbital inflammation.

Purpose: To report diffuse orbital inflammation as a manifestation of recurrent inflammation in a patient with Vogt-Koyanagi-Harada (VKH) disease. Observations: 20-year-old African American male, who was previously diagnosed with VKH, presented with right eye pain, swelling, and binocular double vision. He had run out of methotrexate while on steroid taper. Neuroimaging was consistent with diffuse orbital inflammation with myositis. He was started on intravenous steroids and then transitioned to oral steroids, with complete resolution of his symptoms. Conclusions and importance: Central nervous system involvement as a manifestation of VKH has been previously reported, however, there have been no reports of orbital inflammatory syndrome resulting from VKH. Thus, in the appropriate clinical context, orbital signs may be recognized as features of recurrent VKH.

Tackling Health Care Disparities: How to Build a Sarcoidosis Center.

Sarcoidosis is a multi-organ system inflammatory disease of unknown etiology that disproportionately affects women and black patients in the United States. In addition, woman and minority patients have worse outcomes. In 2015, sarcoidosis physicians in cardiology, pulmonary medicine and rheumatology joined forces to create a multidisciplinary sarcoidosis at Virginia Commonwealth University. In 2019, the clinic was recognized as a World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) Center of Excellence. We identify four pillars of a patient-centered sarcoidosis clinic: clinical care, research, teaching, and community outreach. We detail how each of these facets plays a critical role in improving the health of individual patients, creating a strong infrastructure to improve the future of sarcoidosis treatment, and developing community-based resources that can empower patients. Most importantly, we highlight how a multidisciplinary clinic can help identify and combat healthcare disparities.

Interleukin-1 blockade in cardiac sarcoidosis: study design of the multimodality assessment of granulomas in cardiac sarcoidosis: Anakinra Randomized Trial (MAGiC-ART).

BACKGROUND: Sarcoidosis is an inflammatory disease characterized by the formation of granulomas, which involve the heart in up to 25% of patients. Cardiac sarcoidosis can lead to life threatening arrhythmias and heart failure. While corticosteroids have been used as a treatment for over 50 years, they are associated with hypertension, diabetes, and weight gain, further increasing cardiovascular risk. Interleukin-1 (IL-1) is the prototypical proinflammatory cytokine that works to activate the nuclear transcription factor NF-kB, one of the targets of glucocorticoids. IL-1 also plays an important role also in the pathophysiology of heart disease including atherosclerosis, myocardial infarction, and myocarditis. METHODS: Building on a network of research collaborators developed in the Cardiac Sarcoidosis Consortium, we will investigate the feasibility and tolerability of treatment of CS with anakinra at two National Institute of Health Clinical and Translational Science Award (CTSA) hubs with expertise in cardiac sarcoidosis. In this pilot study, up to 28 patients with cardiac sarcoidosis will be recruited to compare the administration of an IL-1 blocker, anakinra, 100 mg daily on top of standard of care versus standard of care only for 28 days and followed for 180 days. Utilizing surrogate endpoints of changes in systemic inflammatory biomarkers and cardiac imaging, we aim to determine whether IL-1 blockade with anakinra can combat systemic and cardiac inflammation in patients with cardiac sarcoidosis. DISCUSSION: The current trial demonstrates an innovative collaborative approach to clinical trial development in a rare, understudied disease that disproportionately affects females and minorities. Trial Registration The trial was registered prospectively with ClinicalTrials.gov on July 12, 2019, identifier NCT04017936.

Complete heart block in cardiac sarcoidosis reversed by corticosteroid therapy: time course of resolution.

A 53-year-old man was admitted for recurrent syncope and found to have complete heart block (CHB). Cardiac magnetic resonance imaging MRI) showed extensive patchy late gadolinium enhancement in the apical and lateral walls, consistent with cardiac sarcoidosis (CS) but no scar in the septum. A fluorodeoxyglucose (FDG)-positron emission tomography showed FDG uptake in the septum and basal lateral walls. Imaging suggested active inflammation in the septum affecting atrioventricular (AV) conduction but no irreversible fibrosis. Diagnosis of isolated CS requires a high level of suspicion and multidisciplinary teamwork involving heart failure specialists, electrophysiologists and rheumatologists. After specialist and patient discussion, treatment of the disease was initiated with prednisone 40 mg daily, 11 months after presenting with CHB. Three weeks later, ECG with pacing inhibited showed second-degree AV block Mobitz type II and 4 weeks later, AV conduction recovery. This highlights the importance of immediate therapy in reversing AV conduction abnormalities in CS.

Infection prevention in sarcoidosis: proposal for vaccination and prophylactic therapy.

Sarcoidosis is a systemic inflammatory disease characterized by granuloma formation in affected organs and caused by dysregulated immune response to an unknown antigen. Sarcoidosis patients receiving immunosuppressive medications are at increased risk of infection. Lymphopenia is also commonly seen among patient with sarcoidosis. In this review, risk of infections, including opportunistic infections, will be outlined. Recommendations for vaccinations and prophylactic therapy based on literature review will also be summarized. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 87-98).

Atrial Fibrillation and Ventricular Tachycardia in a Patient with Cardiac Sarcoidosis.

Cardiac sarcoidosis (CS) can cause atrial and ventricular arrhythmias, conduction system disease, and congestive heart failure. The use of advanced imaging modalities including cardiac magnetic resonance and positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose can be helpful in evaluating the extent of disease and response to treatment. The management of CS patients can be challenging, requiring immunosuppression medications, antiarrhythmic drugs, implantable cardiac devices, and cardiac ablation procedures. We report a patient with CS initially presenting with paroxysmal atrial fibrillation who later developed polymorphic ventricular tachycardia, highlighting the complexity of diagnosis and management in patients with multisystem sarcoidosis.