Signal Regulatory Protein α Expression in Systemic Vasculitis.

Objective: Signal regulatory protein α (SIRPα) is found primarily on myeloid cells, including macrophages and neutrophils; binds to CD47; and regulates phagocytosis, antigen presentation, cellular fusion, cell proliferation, and migration. Therefore, SIRPα may be involved in the pathogenesis of autoimmune diseases, including systemic vasculitis. This study aimed to assess SIRPα expression in tissue samples from patients with vasculitis.

Hypophysitis Secondary to Small Vessel ANCA Vasculitis Treated With Rituximab.

Background/objective: Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis is a rare small vessel vasculitis that can cause pituitary hypophysitis. Hypophysitis is difficult to treat, often requiring high doses of glucocorticoids with frequent flaring as glucocorticoids are tapered. We present a case of ANCA vasculitis involving the pituitary gland successfully treated with rituximab.

Patient-Reported Sinonasal Symptoms and Risk of Relapse in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Objective: Relapses are frequent and difficult to predict in antineutrophil cytoplasmic antibody-associated vasculitis (AAV), resulting in long-term use of immunosuppression. Although sinonasal disease is associated with relapse of AAV, detailed characterization of sinonasal symptoms is lacking. Using a patient-reported outcome, the 22-item SinoNasal Outcome Test (SNOT-22), we investigated the relationship between sinonasal symptoms and disease activity in AAV.

Engaging Multistakeholder Perspectives to Identify Patient-Centered Research Priorities Regarding Vaccine Uptake Among Adults With Autoimmune Conditions.

Objective: The study objective was to prioritize topics for future patient-centered research to increase uptake of common vaccines, such as for pneumococcal pneumonia, influenza, herpes zoster, human papillomavirus, and severe acute respiratory syndrome coronavirus 2, among adults living with autoimmune conditions.

Methods: A steering committee (SC) was formed that included clinicians, patients, patient advocates, and researchers associated with rheumatic diseases (psoriatic arthritis, rheumatoid arthritis, vasculitis), inflammatory bowel disease, and multiple sclerosis. Through a scoping review and discussions, SC members identified research topics regarding vaccine uptake and/or hesitancy for prioritization.

Patient Perceptions and Preferences Regarding Telemedicine for Autoimmune Rheumatic Diseases Care During the COVID-19 Pandemic.

Objective: To assess the perceptions and preferences of telemedicine among patients with autoimmune rheumatic diseases during the COVID-19 pandemic.

Methods: We conducted an online survey among patients with autoimmune rheumatic diseases. Attitudes about telemedicine (i.

Medication Interruptions and Subsequent Disease Flares During the COVID-19 Pandemic: A Longitudinal Online Study of Patients With Rheumatic Disease.

Objective: We aimed to assess trends in anxiety and interruptions in disease-modifying antirheumatic drug (DMARD) use among patients with rheumatic diseases during the COVID-19 pandemic and to evaluate whether DMARD interruptions were associated with disease flares.

Methods: ArthritisPower, the Vasculitis Patient-Powered Research Network, and other patient organizations invited members to join a 52-week longitudinal study, with baseline surveys completed March 29 to June 30, 2020, with follow-up through May 2021. Logistic regression incorporating generalized estimating equations evaluated associations between interruptions in DMARD use and self-reported disease flares at the next survey, adjusting for demographic characteristics, medications, disease, and calendar time.

The Cerebrospinal Fluid Presentations of Neuro-Bechet Disease, a Way to Know ‎the Etiopathogenesis and Improve Armamentarium‎.

Neuro-Behcet's disease (NBD) is a rare but potentially fatal manifestation of Behcet's disease. Common presentations of neuro-Behcet's disease are parenchymal (brainstem and hemispheric manifestations, meningoencephalitis, spinal cord lesions) and non-parenchymal (arterial occlusions, aneurysms, Dural sinus thrombosis). Cerebrospinal fluid (CSF) findings in parenchymal NBD usually show an inflammatory pattern with elevated cell count (usually high levels of polymorphonuclear leukocytes), high protein, and normal glucose levels, whereas the CSF findings in non-parenchymal NBD could be normal except for high opening pressure.

Social Distancing, Health Care Disruptions, Telemedicine Use, and Treatment Interruption During the COVID-19 Pandemic in Patients With or Without Autoimmune Rheumatic Disease.

Background: We aimed to compare concerns, social distancing, health care disruptions, and telemedicine use in patients with autoimmune rheumatic disease (ARD) and non-ARD and to evaluate factors associated with immunomodulatory medication interruptions.

Methods: Patients in a multistate community rheumatology practice network completed surveys from April 2020 to May 2020. Adults with common ARD (rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus) or non-ARD (gout, osteoarthritis, osteoporosis) were evaluated.

Concerns, Healthcare Use, and Treatment Interruptions in Patients With Common Autoimmune Rheumatic Diseases During the COVID-19 Pandemic.

Objective: To assess concerns and healthcare-related behaviors of patients with autoimmune rheumatic diseases during the coronavirus disease 2019 (COVID-19) pandemic.

Methods: Adults from the United States with rheumatoid arthritis (RA), psoriatic arthritis (PsA), ankylosing spondylitis (AS), and systemic lupus erythematosus (SLE) from the ArthritisPower Patient-Powered Research Network and CreakyJoints patient community completed surveys. Concerns and behaviors were compared among patients with different autoimmune conditions, disease-modifying antirheumatic drug (DMARD) use, and geographic measures of urban status, income, education, and COVID-19 activity.

Patient Perception of Cardiovascular Risk in Rheumatoid Arthritis.

Objective: Patients with rheumatoid arthritis (RA) have higher incidence of cardiovascular diseases (CVDs) compared with age- and sex-matched controls. The objective of our study was to measure the knowledge of patients with RA about the association between their disease and cardiovascular (CV) risk and to measure the frequency of counseling by physicians based on patient report.

Methods: A telephone survey was conducted among patients with RA enrolled in the Consortium of Rheumatology Researchers of North America RA registry to collect data on medical and social history and on knowledge about CVD risk in RA and how they learned about that risk.

Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment.

Neuro-Behcet's disease (NBD) is defined as a combination of neurologic symptoms and/or signs in a patient with Behcet's disease (BD). Relevant syndromes include brainstem syndrome, multiple-sclerosis like presentations, movement disorders, meningoencephalitic syndrome, myelopathic syndrome, cerebral venous sinus thrombosis (CVST), and intracranial hypertension. Central nervous involvement falls into parenchymal and non-parenchymal subtypes.

Vascular calcification in patients with large-vessel vasculitis compared to patients with hyperlipidemia.

Objective: Calcification of the coronary arteries, aorta, and branch vessels can occur in both large-vessel vasculitis (LVV) and atherosclerosis. The study objective was to determine the location and amount of vascular calcification in patients with LVV versus hyperlipidemia (HLD) and to identify risk factors associated with vascular calcification in LVV.

Methods: Patients with giant cell arteritis (GCA), Takayasu's arteritis (TAK), and HLD underwent non-contrast computed tomography of the aorta and branch vessels.

Low incidence of opportunistic Infections in Lupus Patients treated with Cyclophosphamide and Steroids in a Tertiary care setting.

Background: Infection is common cause of morbidity and mortality in systemic lupus erythematosus (SLE). Our objective was to determine incidence and types of infections, particularly opportunistic infections, in SLE patients receiving cyclophosphamide, and to identify contribution of variables like demographics, steroid, other immunosuppressives, white blood cell and absolute neutrophil count to infection risk.

Patients And Methods: We did retrospective chart review of SLE patients in our institute over last 10 years, who received minimum six cyclophosphamide infusions.

Patient Perception of Disease-Related Symptoms and Complications in Relapsing Polychondritis.

Objective: To assess patient-reported symptoms and burden of disease in relapsing polychondritis (RP).

Methods: Patients with RP completed a disease-specific online survey to identify symptoms attributed to illness. Patients were divided into subgroups based upon presence or absence of ear/nose, airway, or joint involvement.

JAK-STAT Signaling as a Target for Inflammatory and Autoimmune Diseases: Current and Future Prospects.

The Janus kinase/signal transduction and activator of transcription (JAK-STAT) signaling pathway is implicated in the pathogenesis of inflammatory and autoimmune diseases including rheumatoid arthritis, psoriasis, and inflammatory bowel disease. Many cytokines involved in the pathogenesis of autoimmune and inflammatory diseases use JAKs and STATs to transduce intracellular signals. Mutations in JAK and STAT genes cause a number of immunodeficiency syndromes, and polymorphisms in these genes are associated with autoimmune diseases.