Put Down the ACE: Low Clinical Utility for Angiotensin-Converting Enzyme Levels in Sarcoidosis: A Single-Center Retrospective Cohort Study.

: ACE (angiotensin-converting enzyme) is considered a serological marker of sarcoidosis as elevated levels have been reported in 30-80% of patients. However, elevated ACE levels are also encountered in other medical conditions, and the clinical correlation between ACE levels and disease activity in sarcoidosis is disputable as well. To determine the significance of elevated ACE levels in the diagnosis and follow-up of sarcoidosis patients.

Early Methotrexate Treatment For Psoriatic Arthritis Prevention in Psoriasis Patients - a retrospective cohort study.

Objectives: Early initiation of biologic therapies for psoriasis has been explored to prevent or delay the onset of psoriatic arthritis (PsA). This has renewed interest in the potential role of methotrexate (MTX) in mitigating PsA risk in newly diagnosed psoriasis patients. The aim of this study was to evaluate the impact of early MTX initiation on PsA incidence in individuals with psoriasis.

Corticosteroids and other immunosuppressants for immune-related adverse events and checkpoint inhibitor effectiveness in melanoma.

Background: Recent studies indicate an association between immunosuppression for immune-related adverse events (irAEs) and impaired survival in patients who received immune checkpoint inhibitors. Whether this is related to corticosteroids or second-line immunosuppressants is unknown. In the largest cohort thus far, we assessed the association of immunosuppressant type and dose with survival in melanoma patients with irAEs.

The value of a repeat MRI examination of the sacroiliac joints following an inconclusive initial examination.

Objective: Assess the diagnostic utility of repeat sacroiliac joint (SIJ) magnetic resonance imaging (MRI) examinations following an inconclusive initial examination performed for suspected sacroiliitis.

Method: Subjects with > 1 SIJ MRI examinations, an inconclusive first scan and at least 6 months interval between scans, were included. All scans were evaluated for the presence of structural/active SIJ lesions as well as any other pathology.

Biologic Treatment Modification Efficacy in Concurrent Inflammatory Bowel Disease and Ankylosing Spondylitis: A Retrospective Cohort Study at a Single Tertiary Center.

Background: The link between ankylosing spondylitis (AS) and inflammatory bowel disease (IBD) is well-established, with concurrent prevalence estimates ranging from 5-10%. However, there are still significant gaps in our understanding, and a comprehensive treatment guideline for these co-diagnosed patients has yet to be established. Our objective was to explore patterns of treatment alterations following the diagnosis of AS in patients previously diagnosed with IBD, and vice versa.

Development and validation of Hebrew version of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0.

Aim: The aim of this study was to test the reliability of the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract (UCLA SCTC GIT) 2.0 questionnaire in Hebrew.

Methods: UCLA SCTC GIT 2.

Screening for Fabry's disease in a high-risk subpopulation of FMF.

Background: Familial Mediterranean fever (FMF) is an autosomal recessive disease associated with mutations in the Mediterranean fever gene (MEFV) that manifests with recurrent episodes of febrile serositis. Fabry's disease (FD) is an X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A gene and presents with a wide range of gastrointestinal, skin, vascular, renal and neurological manifestations. FMF and FD share similar manifestations, which may lead to misdiagnosis of one as the other; mostly FD is misdiagnosed as FMF.

The Autoinflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases.

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network.

Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform.

TNF inhibitors have a protective role in the risk of dementia in patients with ankylosing spondylitis: Results from a nationwide study.

Objectives: Ankylosing spondylitis (AS) is a chronic progressive and debilitating form of arthritis with associated extra-articular features including uveitis, intestinal and lung apical inflammation and psoriasis. Putative associations between AS and neurologic disorders has been relatively overlooked. The purpose of this study is to assess the link between AS and major neurologic disorders and whether treatment with Tumor-Necrosis-Factor inhibitors (TNFi) has an impact on that association.

The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease.

Familial Mediterranean fever (FMF), the most frequent monogenic autoinflammatory disease, is manifested with recurrent and chronic inflammation and amyloid A (AA) amyloidosis, driven by overproduction of interleukin 1 (IL-1) through an activated pyrin inflammasome. Consequently, non-responsiveness to colchicine, the cornerstone of FMF treatment, is nowadays addressed by IL-1- blockers. Each of the two IL-1 blockers currently used in FMF, anakinra and canakinumab, has its own merits for FMF care.

No correlation between diffuse idiopathic skeletal hyperostosis and coronary artery disease on computed tomography using two different scoring systems.

Background: An association between diffuse idiopathic skeletal hyperostosis (DISH) and a history of coronary artery disease (CAD) was previously reported.

Purpose: To investigate the association between DISH and CAD as assessed using the coronary artery calcification score (CACS) and the CAD-Reporting and Data System (CAD-RADS) score in patients with symptomatic chest pain.

Material And Methods: Consecutive cardiac CT scans performed before and after IV contrast administration were evaluated for CACS (Agatston method), CAD-RADS, and the presence of DISH.

Osteophytes' position in subjects with DISH and right-sided aorta: verification of the 'aortic pulsation protective effect' theory.

Objectives: To validate in a large cohort with right-sided aorta the theory that thoracic right-sided flowing osteophytes in DISH results from a 'protective' effect of the pulsating descending left-sided thoracic aorta.

Methods: Chest CTs of patients with DISH and right-sided aorta and controls with DISH and left-sided aorta were evaluated and compared on each intervertebral space (IS) for the location of the aorta (right, left, centre) and the location of the osteophyte relative to the aorta (contralateral, ipsilateral, bilateral).

Results: The study and control cohorts included 31 and 35 subjects, respectively (male 22/9 and female 27/8; median age 64.

Safety of the BNT162b2 mRNA COVID-19 vaccine in patients with familial Mediterranean fever.

Objectives: Evidence suggests a possible association between the COVID-19 vaccine and autoimmune disease flares or new onset of various autoinflammatory manifestations, such as pericarditis and myocarditis. The objective of this study was to assess the safety of an mRNA-based BNT162b2 anti-COVID-19 vaccine in individuals with FMF, a prototypic autoinflammatory disease.

Methods: Patients participating in this study fulfilled the criteria for diagnosis of FMF, were older than 18 years and received at least one dose of the vaccine.

The frequency of sacroiliitis on MRI in subjects over 55 years of age.

Objective: To evaluate the frequency of sacroiliitis in older subjects.

Materials And Methods: Consecutive MRI examinations of the sacroiliac joints (SIJs) performed for suspected sacroiliitis (2005-2019) in patients ≥ 18 years were retrospectively evaluated for the presence of active/structural lesions and were categorized for the presence/absence of sacroiliitis. Clinical and imaging parameters were compared between subjects with sacroiliitis according to age groups < 40 years, 40-55, and > 55 years.

Autonomic Nervous System Indices in Patients with Systemic Sclerosis without Overt Cardiac Disease.

Background: Systemic sclerosis (SSc) is a connective tissue disease that may affect the heart and the autonomic nervous system (ANS). There is little knowledge regarding the degree of ANS involvement in SSc patients with unknown cardiac disease.

Objectives: To evaluate cardiac and pupillary autonomic functions in patients before cardiac involvement has emerged.

Low Urine Secretion of Semaphorin3A in Lupus Patients with Proteinuria.

Immune semaphorins are important in controlling both innate and adaptive immune responses. The regulatory role of semaphorin3A (sema3A) in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and other autoimmune diseases is widely reported. Decreased levels of serum sema3A were shown to correlate with SLE disease activity.

Immune checkpoint inhibitor-associated sarcoidosis: A usually benign disease that does not require immunotherapy discontinuation.

Objective: To analyse the clinical patterns of sarcoidosis triggered by immune checkpoint inhibitors (ICIs) in patients with cancer.

Patients And Methods: The ImmunoCancer International Registry is a big data-sharing multidisciplinary network from 18 countries dedicated to evaluating the clinical research of immune-related adverse events related to cancer immunotherapies.

Results: We identified 32 patients with biopsy-proven sarcoidosis.

Effect of interleukin-1 inhibition in a cohort of patients with colchicine-resistant familial Mediterranean fever treated consecutively with anakinra and canakinumab.

Objectives: To evaluate the efficacy of IL-1 blockers in a cohort of patients with colchicine-resistant familial Mediterranean fever (crFMF) treated consecutively with anakinra and canakinumab.

Methods: Patients with crFMF treated with anakinra and canakinumab in any order were identified using the computerised database of Sheba Medical Centre. Background characteristics of the patients, reason for switching IL-1 inhibitor, and frequency of attacks under colchicine only, anakinra, and canakinumab were extracted from the computerised patient files.

Anakinra for colchicine refractory familial Mediterranean fever: a cohort of 44 patients.

Objective: FMF is an autoinflammatory disease of genetic origin. Colchicine is the mainstay of treatment for the prevention of attacks and long-term complications but 5-10% of FMF patients are resistant to colchicine therapy. The aim of our study was to investigate the real-life safety and efficacy of anakinra in a cohort of patients with colchicine-resistant FMF.

Bisphosphonates Related Ocular Side Effects: A Case Series and Review of Literature.

Objective: To describe the clinical features of patients with bisphosphonate related ocular side effects (BROSE).

Methods: The medical records of all patients with BROSE between January 2009 and December 2019 were reviewed.

Results: Nine cases with BROSE were identified.