Systemic Rheumatologic Disease Research Articles

Efficiency of therapeutic plasma exchange in critically ill systemic rheumatologic diseases: A single-center 9-year experience.

Therapeutic plasma exchange (TPE), an effective method to eliminate harmful soluble mediators associated with tissue injury, serves as a crucial intervention for systemic rheumatologic diseases (SRDs). However, its value in critically ill SRDs remains uncertain. This retrospective study aims to evaluate the efficacy of TPE in SRDs. Critically ill SRD patients admitted to the department of intensive care unit of a large tertiary hospital receiving TPE from January 2011 to December 2019 were included. A total of 91 critically ill SRD patients received TPE were enrolled. Their mean age was 47.67 ± 16.35 years with a female predominance (n = 68). Significant decrease in SOFA score post-TPE treatment was observed (p < 0.05). There were no TPE-related fatalities. Improvement was observed in 64 (70.32%) patients. This study shows favorable clinical outcomes. TPE may be an acceptable treatment option for critically ill SRD patients.

The development of anticyclic citrullinated peptide (anti-CCP) antibody following severe COVID-19.

The dysregulated immune response is one of the cardinal features of severe coronavirus disease 2019 (COVID-19). This study was conducted to clarify the occurrence of autoantibodies (AABs) associated with systemic autoimmune rheumatic diseases (SARDs) in hospitalized patients with a moderate, severe, and critical form of COVID-19. The serum samples obtained from 176 hospitalized COVID-19 patients were investigated in this study, including patients with moderate (N = 90), severe (N = 50), and critical (N = 36) forms of COVID-19. Also, the serum samples collected from healthy subjects before the COVID-19 pandemic were used as controls (N = 176). The antinuclear antibodies (ANAs), antidouble-stranded DNA (anti-dsDNA), cytoplasmic-anti neutrophil cytoplasmic antibody (c-ANCA), perinuclear ANCA (p-ANCA), antiphospholipid antibodies (aPLs), and anticyclic citrullinated peptide (anti-CCP) occurrence was evaluated using a solid-phase enzyme-linked immunosorbent assay (ELISA). The results showed that the occurrence of ANAs, anti-dsDNA, anti-CCP, c-ANCA, and p-ANCA was significantly higher in the COVID-19 patients compared to serum obtained from healthy subjects (p < .0001, p < .0001, p < .0001, p < .05, and p < .001, respectively). The positive number of anti-CCP tests increased significantly in severe COVID-19 compared to the moderate group (p < .01). Our study further supports the development of autoantibodies related to systemic autoimmune rheumatologic diseases. To the best of our knowledge, this is the first study with a large sample size that reported the occurrence of anti-CCP in a severe form of COVID-19.

Neuropsychiatric symptoms in Systemic Lupus Erythematosus: mixed methods analysis of patient-derived attributional evidence in the international INSPIRE project.

Attribution of neuropsychiatric symptoms in systemic lupus erythematosus (SLE) relies heavily on clinician assessment. Limited clinic time, variable knowledge, and symptom under-reporting contributes to discordance between clinician assessments and patient symptoms. We obtained attributional data directly from patients and clinicians in order to estimate and compare potential levels of direct attribution to SLE of multiple neuropsychiatric symptoms using different patient-derived measures. Quantitative and qualitative data analysed included: prevalence and frequency of neuropsychiatric symptoms, response to corticosteroids, and concurrence of neuropsychiatric symptoms with non-neuropsychiatric SLE disease activity. SLE patients were also compared with controls and inflammatory arthritis (IA) patients to explore attributability of neuropsychiatric symptoms to the direct disease effects on the brain/nervous system. We recruited 2,817 participants, including 400 clinicians. SLE patients (n = 609) reported significantly higher prevalences of neuropsychiatric symptoms than controls (n = 463) and IA patients (n = 489). SLE and IA patients' quantitative data demonstrated multiple neuropsychiatric symptoms relapsing/remitting with other disease symptoms such as joint pain. Over 45% of SLE patients reported resolution/improvement of fatigue, positive sensory symptoms, severe headache, and cognitive dysfunction with corticosteroids. Evidence of direct attributability in SLE was highest for hallucinations and severe headache. SLE patients had greater reported improvement from corticosteroids (p= 0.008), and greater relapsing-remitting with disease activity (p< 0.001) in the comparisons with IA patients for severe headache. Clinician and patients reported insufficient time to discuss patient-reported attributional evidence. Symptoms viewed as indirectly related/non-attributable were often less prioritised for discussion and treatment. We found evidence indicating varying levels of direct attributability of both common and previously unexplored neuropsychiatric symptoms in SLE patients, with hallucinations and severe headache assessed as the most directly attributable. There may also be-currently under-estimated-direct effects on the nervous system in IA and other systemic rheumatological diseases.

Виявлення антитіл до вірусу SARS-COV-2 у хворих на ішемічну хворобу серця та системні ревматичні захворювання до розвитку пандемії COVID-19

Seasonal coronaviruses may serve as an informative model for determining the duration of protection against reinfection during future waves of coronavirus disease. Data from the literature suggest that ELISA kits designed to detect antibodies to SARS-CoV-2 may also be sensitive to seasonal coronaviruses. The aim of our work was to perform a retrospective study to determine the presence of IgG and IgM to coronaviruses in Ukraine before the COVID-19 pandemic. The study was conducted on the serum of 321 patients with various cardiorheumatologic profiles who were treated in a hospital during 2013-2018. The obtained data indicate the possibility of cross-immunity among coronaviruses since antibodies to this virus were present in 3.3% of patients with acute myocardial infarction, 5.9% with systemic rheumatological diseases, and 27.3% with chronic coronary heart disease.

P18 Bullous leukocytoclastic vasculitis in a patient with known undifferentiated autoimmune rheumatic disease

P18 Bullous leukocytoclastic vasculitis in a patient with known undifferentiated autoimmune rheumatic disease

Beyond Systemic Lupus Erythematosus and Anti-Phospholipid Syndrome: The Relevance of Complement From Pathogenesis to Pregnancy Outcome in Other Systemic Rheumatologic Diseases.

Evidence about the relevance of the complement system, a highly conserved constituent of the innate immunity response that orchestrates the elimination of pathogens and the inflammatory processes, has been recently accumulated in many different rheumatologic conditions. In rheumatoid arthritis, complement, mainly the classical pathway, contributes to tissue damage especially in seropositive subjects, with complement activation occurring in the joint. Data about complement pathways in psoriatic arthritis are dated and poorly consistent; among patients with Sjögren syndrome, hypocomplementemia exerts a prognostic role, identifying patients at risk of extra-glandular manifestations. Hints about complement involvement in systemic sclerosis have been recently raised, following the evidence of complement deposition in affected skin and in renal samples from patients with scleroderma renal crisis. In vasculitides, complement plays a dual role: on one hand, stimulation of neutrophils with anti-neutrophil cytoplasmic antibodies (ANCA) results in the activation of the alternative pathway, on the other, C5a induces translocation of ANCA antigens, favouring the detrimental role of antibodies. Complement deposition in the kidneys identifies patients with more aggressive renal disease; patients with active disease display low serum levels of C3 and C4. Even though in dermatomyositis sC5b-9 deposits are invariably present in affected muscles, data on C3 and C4 fluctuation during disease course are scarce. C3 and C1q serum levels have been explored as potential markers of disease activity in Takayasu arteritis, whereas data in Behçet disease are limited to in vitro observations. Pregnancies in women with rheumatologic conditions are still burdened by a higher rate of pregnancy complications, thus the early identification of women at risk would be invaluable. A fine-tuning of complement activation is required from a physiological progression of pregnancy, from pre-implantation stages, through placentation to labour. Complement deregulation has been implicated in several pregnancy complications, such as recurrent abortion, eclampsia and premature birth; low complement levels have been shown to reliably identify women at risk of complications. Given its physiologic role in orchestrating pregnancy progression and its involvement as pathogenic effector in several rheumatologic conditions, complement system is an attractive candidate biomarker to stratify the obstetric risk among women with rheumatologic conditions.

Prevalence of autoantibodies in pediatric patients with idiopathic pulmonary hemosiderosis: a scoping review of the literature in the period 1980–2021

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology. Due to the frequent findings of autoimmune antibodies - autoantibodies, immunologic causation of the diffuse alveolar hemorrhage in IPH has been proposed, to assess the prevalence/frequency and type of autoantibodies in pediatric patients with IPH. In addition, the patient demographics, diagnostic modalities used to diagnose IPH, treatment, and outcomes were also evaluated. Scoping review: The PubMed, Medline, and Embase databases were searched with appropriate MeSH terms to identify relevant papers consistent with the defined inclusion criteria. Thirteen observational studies comprising a total of 352 pediatric patients were included in this review. The majority of subjects were girls 217 out of 352 (61.6%). The mean and median ages of patients ranged from 3.1-6.5years to 2.3-7years, respectively. In the 10 studies that specified the number of patients in their cohorts with either at least one positive autoantibody or no antibody, the overall prevalence of autoantibodies was 76 out of 288 patients (26.4%). The prevalence of specific antibodies was as follows: ANA, 20.3%; ANCA, 17%; anti-dsDNA, 9.1%; RF, 12%; anti-SMA, 23.2%; and celiac antibodies, 25.9%. Cow's milk protein allergy was present in 16.2% of the children. The significance of an association between IPH and the presence of autoantibodies has not been clarified. The autoantibodies could be suggestive of an overall immune dysregulation rather than causation. However, limited evidence based on a single study suggests that the presence of ANA may be associated with a higher risk of recurrence and worse outcomes. Further research, including prospective studies, will be crucial to explore a possible genetic linkage between vasculitides, systemic rheumatologic diseases, and IPH.

Polymyalgia rheumatica.

Polymyalgia rheumatica is the second most prevalent systemic rheumatological disease in adults. Lifetime risk is 2.4% for women and 1.7% for men, with incidence rising from age 50 to 80 years.[1][1] Polymyalgia rheumatica is characterized by acute-onset bilateral shoulder pain with morning

Systemic, environmental and lifestyle risk factors for dry eye disease in a mediterranean caucasian population

ObjectivesTo assess systemic, environmental and lifestyle risk factors for dry eye disease (DED) in a Mediterranean Caucasian population. MethodsA cross-sectional study was performed on 120 Caucasian participants aged between 18 and 89 years (47.0 ± 22.8 years). Medical history, information regarding environmental conditions and lifestyle, Ocular Surface Disease Index, Dry Eye Questionnaire-5, non-Invasive (Oculus Keratograph 5 M) breakup time, tear film osmolarity and ocular surface staining parameters were assessed in a single clinical session to allow DED diagnosis based on the guidelines of the Tear Film and Ocular Surface Society Dry Eye Workshop II Diagnostic Methodology Report. A multivariate logistic regression model was constructed including those variables with a p-value less than 0.15 in the univariate analysis. ResultsA prevalence of 57.7 % for DED was found. No age differences were found between those with and without DED (U = 1886.5, p = 0.243). Nevertheless, the DED group had more females (X2 = 7.033, p = 0.008). The univariate logistic regression identified as potential risk factors for DED the following: female sex, sleep hours per day, menopause, anxiety, systemic rheumatologic disease, use of anxiolytics, daily medication, ocular surgery, poor diet quality, more ultra-processed food in diet, not drinking caffeine and hours of exposure to air conditioning per day. Multivariate logistic regression revealed that hours of sleep per day, menopause and use of anxiolytics were independently associated with DED (p ≤ 0.026 for all). ConclusionsDED is associated with systemic, environmental and lifestyle risk factors. These findings are useful to identify potentially modifiable risk factors, in addition to conventional treatments for DED.

Endocrine Disorders in Autoimmune Rheumatological Diseases: A Focus on Thyroid Autoimmune Diseases and on the Effects of Chronic Glucocorticoid Treatment

Autoimmune rheumatological diseases’ incidence and prevalence have risen over the last decades and they are becoming increasingly important worldwide. Thyroid autoimmune diseases share with them an imbalance in the immune system that lead to a pro-inflammatory environment. Usually this is the result of a multi-factorial process. In fact, it includes not only a possible genetic predisposition, but also environmental causes like microbiota dysbiosis, diet rich in processed foods, exposure to toxicants and infections. However, many aspects are currently under study. This paper aims to examine the factors that participate in the developing of rheumatological and thyroid autoimmune diseases. Moreover, as glucocorticoids still represent a leading treatment for systemic autoimmune rheumatological diseases, our secondary aim is to summarize the main effects of glucocorticoids treatment focusing on iatrogenic Cushing’s syndrome and glucocorticoids’ withdrawal syndrome.

Ocular Manifestations in Colombian Patients with Systemic Rheumatologic Diseases.

PurposeTo establish the prevalence of ocular involvement in a Colombian population with rheumatologic diseases.DesignObservational cross-sectional study.MethodsWe included a probabilistic sample size of 797 patients who attended a rheumatologic disease center in Bogotá, Colombia. Statistical analysis with descriptive measures and Chi-square independence test between rheumatologic diseases and ophthalmological symptoms and diseases was performed.ResultsEighty-four percent of the population were women, and the mean age was 54.61± 15.64 years. The most common condition was rheumatoid arthritis (33.37%), followed by fibromyalgia (22.71%), Sjögren Syndrome (19.72%), and systemic lupus erythematosus (9.91%). Almost 7% of the patients presented polyautoimmunity. Thirty-five percent of the patients reported one or more ophthalmological symptoms, being dry eye sensation the most common (30.86%), followed by ocular pain (2.76%), red-eye, and decreased visual acuity (both 2.63%). Similarly, 21.45% of the patients presented one or more ophthalmological diagnoses, being keratoconjunctivitis sicca the most common (15.93%), followed by cataract, uveitis (1.38% each), and scleritis (1.25%).ConclusionAlmost a third of the patients reported any ocular involvement. It is crucial to be aware of the most common ophthalmic manifestations among the different rheumatologic diseases in our population, to offer early specialist referral and timely treatment.

Systemic risk factors of dry eye disease subtypes: A New Zealand cross-sectional study

PurposeTo evaluate systemic risk factors of dry eye disease, aqueous tear deficiency, and meibomian gland dysfunction. MethodsThree hundred and seventy-two community residents (222 females, 150 males; mean ± SD age, 39 ± 22 years) were recruited in a cross-sectional study. Past medical history, dry eye symptomology, ocular surface characteristics, and tear film quality were evaluated for each participant within a single clinical session. The diagnosis of dry eye disease, aqueous tear deficiency, and meibomian gland dysfunction were based on the global consensus recommendations of the Tear Film and Ocular Surface Society's Dry Eye Workshop II (TFOS DEWS II) and International Workshop on Meibomian Gland Dysfunction. ResultsOverall, 109 (29%) participants fulfilled the TFOS DEWS II criteria for dry eye disease, 42 (11%) had aqueous tear deficiency, and 95 (26%) had meibomian gland dysfunction. Multivariate logistic regression analysis demonstrated that systemic rheumatologic disease and antidepressant medication were independently associated with aqueous tear deficiency (both p < 0.05). Significant risk factors for meibomian gland dysfunction included age, East Asian ethnicity, migraine headaches, thyroid disease, and oral contraceptive therapy (all p ≤ 0.01). ConclusionsBoth etiological subtypes of dry eye disease were associated with a number of systemic risk factors. These findings would support routine systemic inquiry of dry eye disease and associated systemic conditions and medications, in order to facilitate opportunistic screening and timely inter-disciplinary referral where necessary.

P16 Is there an association between paediatric rheumatological disease and autism?

Abstract Background The prevalence of autistic spectrum disorder (ASD) in children in UK is ∼1 in 100. There is emerging evidence that dysregulation of the immune system may be an underlying cause of ASD. The CARRA registry from the USA reported a frequency of 0.3% co-morbidity of autism in children with JIA. There is a paucity of data from the UK reporting this association. Methods The department’s database of all patients that attended the local paediatric rheumatology clinic was reviewed over a 12-month period from April 2018 to April 2019. Diagnoses were grouped broadly into inflammatory (JIA, systemic disease, non-JIA uveitis) and non-inflammatory (mechanical, bone and collagen disorder and other). Those with a co-morbidity of autism were also identified within inflammatory and non-inflammatory groups. Results There were 196 individual patients within the cohort of paediatric rheumatology patients seen in Bradford in April 2018 to April 2019. These were subcategorised into JIA (N = 79,40%), mechanical (N = 56, 29%), systemic rheumatological disease (N = 31, 16%), bone and collagen disorders (N = 12, 6%), other non-inflammatory diagnoses (N = 11, 6%), non-JIA associated uveitis (N = 7, 3%). This cohort was divided into 117 patients with inflammatory paediatric rheumatological conditions and 79 patients with non-inflammatory causes for joint pain. Nine children (see Table 1) had a co-morbidity of autism, of which 6 children had an inflammatory diagnosis and 3 a non-inflammatory cause for joint pain. The prevalence of autism in our cohort of children with paediatric rheumatological disease was 5%, which is higher than expected from a UK prevalence of autism of 1%. P16 Table 1: Patients with Autism in Paediatric Rheumatology cohort. Diagnosis Age Age at presentation to paediatric rheumatology Gender Inflammatory/ Non-inflammatory Oligo JIA 5y 4y Male Inflammatory Oligo JIA 11y 10y Female Inflammatory Oligo JIA 13y 7y Female Inflammatory Ext- oligo JIA 18y 8y Male Inflammatory Ext- oligo JIA 14y 7y Male Inflammatory CTD 15y 2y Female Inflammatory Hypermobility 16y 16y Male Non-inflammatory Mechanical 14y 14y Female Non-inflammatory Mechanical 11y 11y Female Non-inflammatory Conclusion In this cohort of paediatric rheumatology patients there was a higher prevalence of autism than the UK average. Two of these patients were diagnosed with autism after presenting to our service. This association is compatible with the emerging recognition of dysregulation of the immune system as a potential underlying cause of ASD. It is important to consider the co-morbidity of autism in children presenting to paediatric rheumatology clinic and potentially paediatric rheumatological conditions in children already diagnosed with autism, where there may be a delay in their presentation due to communication difficulties. There is the need for a larger UK study to review this association further. Conflicts of Interest The authors declare no conflicts of interest.

Imaging for screening cardiovascular involvement in patients with systemic rheumatologic diseases: more questions than answers.

Cardiovascular involvement due to systemic rheumatologic diseases (SRDs) remains largely underdiagnosed despite causing excess mortality and limiting the favourable effect of therapeutic developments on survival. Traditional risk scoring systems are poorly calibrated for SRD patients. There is an unmet need to develop a cardiovascular (CV) risk stratification tool and screening algorithm for CV involvement dedicated to asymptomatic patients with SRDs. Even though accelerated atherosclerosis is the most prominent cause of major CV events, a more comprehensive approach is crucial to detect different pathological processes associated with SRDs that are leading to CV complications. In that regard, incorporation of imaging parameters obtained from echocardiography and carotid ultrasound (CUS) might help to improve risk models, to detect and monitor subclinical CV involvement. These two imaging modalities should be an integral part of screening SRD patients with suspicion of CV involvement on top of electrocardiogram (ECG). Cardiac magnetic resonance and multi-slice computerized tomography angiography and nuclear imaging modalities seem very important to complement echocardiography and CUS for further evaluation. However, to answer the question 'Should asymptomatic patients with SRDs undergo screening with echocardiography and CUS on top of ECG?' necessitates large studies performing cardiac screening with a standard approach by using these imaging methods to obtain longitudinal data with hard CV outcomes.

Evaluation of autoantibody positive in multiple sclerosis patients

Objective: Multiple sclerosis (MS) is the most common autoimmune disease of the central nervous system in which impaired immune activation is involved. It is known that other autoimmune diseases are seen more frequently in MS patients. Many rheumatologic diseases could cause neurologic disorders that mimic MS. Our aim in this study is to determine the rates of autoantibody positivity and the clinical significance of these positivity rates in patients with MS. Method: 110 patients who were followed up with MS diagnosis in our clinic between 2008 and 2018 were retrospectively evaluated for autoimmune disease biomarkers. The diagnosis of MS was confirmed using both 2005 and 2010 Revised Mc Donald Criteria. Results: The ANA (antinuclear antibody) positivity rate in our patients was 10.9%, the ANA profile positivity was 9.1%. Anti-ds DNA (double stranded deoxyribonucleic acid) positivity rate was 1.8%, anti-cardiolipin Ig (immunglobulin) G and Ig M were 0.9% positive. Anti-microsomal antibody positivity was 11.8% while anti-thyroglobulin antibody was positively 13.6%. The value of complement 3 (C3) was found to be 92.7% normal and 4.5% higher and 2.7% lower, respectively. The C4 values ​​were 98.2% normal, while they were 11.8% higher than normal . Six patients had six different diseases in which ethiopathogenesis the role of autoimmunity was also revealed ( ankylosing spondylitis, psoriasis, systemic lupus erythematosus, morfea, ptriyazis, Sjögren's syndrome). Conclusions: A lthough some autoantibody positivities in MS patients are more frequent than healthy controls, these positivities are not usually associated with systemic rheumatologic disease and haven’t clinical significance. Therefore, the evaluation of autoantibodies in each MS patient does not seem cost effective. So, the MS patients whose autoantibody levels are to be measured should be well selected before the evaluation the patients' medical history and complaints should be taken into consideration.

Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.

This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing. The Chicago Classification version 3.0 has redefined major and minor esophageal motility disorders using high-resolution esophageal manometry. There is a dearth of publications based on research on absent contractility, which historically has been associated with myopathic processes such as systemic sclerosis (SSc). We conducted a retrospective, multicenter study. Data of patients diagnosed with absent contractility were pooled from Cleveland Clinic, Cleveland, OH (January 2006 to July 2016) and Metrohealth Medical Center, Cleveland, OH (July 2014 to July 2016) and included: age, gender, associated medical conditions, surgical history, medications, and specific antibody testing. A total of 207 patients, including 57 male individuals and 150 female individuals, with mean age of 56.1 and 60.0 years, respectively, were included. Disease distribution was as follows: SSc (diffuse or limited cutaneous) 132, overlap syndromes 7, systemic lupus erythematosus17, Sjögren syndrome 4, polymyositis 3, and dermatomyositis 3. Various other etiologies including gastroesophageal reflux disease, postradiation esophagitis, neuromuscular disorders, and surgical complications were seen in the remaining cohort. Most practitioners use the term "absent contractility" interchangeably with "scleroderma esophagus"; however, only 63% of patients with absent contractility had SSc. Overall, 20% had another systemic autoimmune rheumatologic disease and 16% had a nonrheumatologic etiology for absent contractility. Therefore, alternate diagnosis must be sought in these patients. We propose an algorithm for their etiologic evaluation.

Joint Hypermobility Syndrome Affects Response to a Low Fermentable Oligosaccharide, Disaccharide, Monosaccharide and Polyol Diet in Irritable Bowel Syndrome Patients: A Retrospective Study.

BackgroundThe low fermentable oligosaccharide, disaccharide, monosaccharide and polyol (FODMAP) diet causes significant clinical improvement in patients with irritable bowel syndrome (IBS). Joint hypermobility syndrome (JHS), defined as musculoskeletal symptoms in a hypermobile individual in the absence of systemic rheumatological disease, may be associated with functional gastrointestinal symptoms, including IBS. The aim of this study is to examine whether JHS can affect the response to the low FODMAP diet in patients with IBS.MethodsIn this retrospective study, we included patients with IBS according to Rome III criteria who had followed a low FODMAP diet. Symptoms scores were measured before and after the low FODMAP diet.ResultsA total of 165 patients (130 females, age 44 ± 14 years) were included. Diarrhea predominant IBS (IBS-D) was present in 40.6% of our patients while JHS was present in 21.2%. The score for abdominal pain was higher for JHS compared to non-JHS prior to intervention (P = 0.011). Symptoms improved in both groups of patients after a low FODMAP diet (P < 0.0001). The largest effects were shown with significant decreases of the average score and bloating. When broken down by JHS and IBS type, a low FODMAP diet significantly improved pain, bloating, diarrhea, constipation, and the average score with the largest effect in JHS/constipation predominant IBS (IBS-C), JHS/mixed IBS and unclassified IBS (IBS-M), JHS/IBS-D, non-JHS/IBS-C and JHS/IBS-M, respectively.ConclusionsOur study suggests that a low FODMAP diet has a greater effect on IBS symptoms in JHS than non-JHS patients.

Noninfectious uveitis in the Asia-Pacific region.

Uveitis is a sight-threatening disease. Up to 35% of patients may have impaired vision. Inflammation of the uvea tissue has more than 60 etiologies. Previous reports have shown that 20-40% of uveitis cases were noninfectious. Some of them may be associated with systemic rheumatological and autoimmune diseases but some may affect the eyes only. The epidemiology and clinical situations of some specific uveitis entities vary worldwide because they are influenced by genetic, ethnic, environmental, and socioeconomic factors. The Asia-Pacific region comprises more than 30 countries. Epidemiology and patterns of uveitis vary greatly in this region. However, some uveitis entities, such as Behcet's disease, sarcoidosis, and Vogt-Koyanagi-Harada disease, are more common in this region. Many studies on the epidemiology, risk factors, and immune pathogenesis of this disease have been conducted. In this article, we review the epidemiology of noninfectious uveitis and special situations of these three uveitis entities in the Asia-Pacific region.

Is this a worrisome red eye? Episcleritis in the primary care setting

ABSTRACTEpiscleritis is the inflammation of the thin, loose, highly vascular connective tissue layer that lies between the conjunctiva and sclera. Incidence is less than 1/1000. It is more common in women and those between 40 and 50 years of age. Most cases are idiopathic. It is classified into simple and nodular. Most attacks resolve within 1–3 months. The nodular type tends to be more recurrent and painful. It presents with acute onset of redness, lacrimation, and photophobia. The diagnosis of is essentially clinical, and eye pain or tenderness should raise the concern for scleritis. Ophthalmological referral is recommended to rule out scleritis. Bloodwork to diagnose associated systemic rheumatological disease may be helpful. Cold compresses and artificial tears provide symptomatic relief. Topical nonsteroidal anti-inflammatory drugs (NSAIDs) and steroids are used for persistent symptoms. Rarely, systemic steroids may be necessary. Immunosuppressive treatment to control an underlying autoimmune disorder is the last resort for resistant cases.

Clinically isolated aortitis: pitfalls, progress, and possibilities.

Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Some systemic disorders may initially present as CIA including giant cell arteritis (GCA), IgG4-related disease, infectious aortitis, and granulomatosis with polyangiitis. CIA most commonly occurs in women of European descent over the age of 50 and, thus, mirrors the gender, age, and geographic distribution of GCA. CIA most often demonstrates a granulomatous/giant cell pattern of inflammation (GPI), and CIA-GPI is pathologically indistinguishable from aortitis due to GCA. In many cases, CIA may be a manifestation of extracranial GCA. CIA is being identified both pathologically in resected aortic tissue and radiologically by computed tomography scanning, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography. However, there appears to be significant differences between pathologically defined CIA and radiologically defined CIA. Multiple studies have shown that patients with CIA are at increased risk for subsequent aortic events (new aneurysms or dissections) and thus it is recommended to monitor these patients with periodic aortic imaging. While the data is currently limited, there is increasing evidence that at least some patients with CIA may benefit from immunosuppressive therapy.