Autoimmune Inflammatory Conditions Research Articles

Systemic Lupus Erythematosus Patient with Bicytopenia and Motor Aphasia

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune condition with unclear cause that shows a broad ranging clinical symptoms, course and prognosis. Cerebrovascular illness, a severe symptom SLE in the central nervous system by neuropsychiatric SLE (NPSLE). Aphasia is considered to be one of the rarer signs, produced by secondary antiphospolipid syndrome (APS), of a cerebrovascular condition of NPSLE. Case: A 37 years-old married woman that has background of miscarriage in SLE with speech blocking, motor aphasia, and had nasal voice was diagnosed with NPSLE with APS. After initiating therapy with cyclophospamide and aspilet the patient’s clinical condition improved significantly. Conclusion: APS needs to bear in mind in SLE patients that has background of miscarriage or thrombosis. The diagnosis was based on history, clinical, and laboratory examinations which include phospholipid antibodies, There was dramatic improvement after 12 days of 500 mg of cyclophospamide and oral aspilet 100 mg daily therapy.

Juvenile Dermatomyositis: Advances in Pathogenesis, Assessment, and Management.

Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes. The purpose of this article is to provide readers with an update on the evaluation, diagnosis, and the treatment of juvenile dermatomyositis. A PubMed search was performed in Clinical Queries using the key term "juvenile dermatomyositis" in the search engine. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article. Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); the presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is a high-dose corticosteroid (oral or intravenous) in combination with methotrexate. For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited, and their use in the treatment of juvenile dermatomyositis is considered investigational.

Pain Management in Painful Psoriasis and Psoriatic Arthropathy: Challenging and Intricately Intertwined Issues Involving Several Systems.

Psoriasis and psoriatic arthropathy are inflammatory autoimmune conditions that can lead to profound emotional distress, social stigmatization, isolation, disfigurement, pain, disability, unemployment, and decreased quality of life. Thus, this disease has immense psychological, social, and economic implications as the pain experienced is closely associated with the primary disease burden. This review focuses on discussing the primary disease burden of psoriasis and psoriatic arthropathy, as well as management of different types of pain in these patients. Pain affects over 40% of patients with psoriasis, ranging from neuropathic to nociceptive. Treatment of pain largely focuses on treating the underlying disease with mild topical steroids and non-steroidal medications including vitamin D analogs followed by systemic immunomodulatory agents for more severe disease. Interventional options such as corticosteroid injections are available for select cases (conditional recommendation). Psoriasis and psoriatic arthropathy have been associated with underreporting and resultant undertreatment of pain. Pain control in these conditions is complex and requires a multidisciplinary approach. More research and guidelines are needed in the areas of reporting of psoriatic disease, associated pain, psoriatic nociception, and optimal clinical management.

Antibody glycosylation in autoimmune diseases.

The glycosylation of the fragment crystallizable (Fc) region of immunoglobulins (Ig) is critical for the modulation of antibody effects on inflammation. Moreover, antibody glycosylation may induce pathologic modifications and ultimately contribute to the development of autoimmune diseases. Thanks to progress in the analysis of glycosylation, more data are available on IgG and its subclass structures in the context of autoimmune diseases. In this review, we focused on the impact of Ig glycosylation in autoimmunity, describing how it modulates the immune response and how glycome profiles can be used as biomarkers of disease activity. The analysis of antibody glycosylation demonstrated specific features in human autoimmune and chronic inflammatory conditions, including rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel disease and autoimmune liver diseases, among others. Within the same disease, different patterns are associated with disease severity and treatment options. Future research may increase the information available on the distinct glycome profiles and expand their potential role as biomarkers and as targets for treatment, ultimately favoring an individualized approach.

Assessment of cerebral hemodynamics in multiple sclerosis patients

BackgroundMultiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. Autonomic dysfunction is present in a wide range of patients (15–80%) that might present with abnormalities in the regulation of the cardiovascular system, sleep disturbance, urinary symptoms, sexual problems, and temperature regulation problem. Although the predominant view is that of an autoimmune inflammatory condition, changes in brain vasculature can occur and contribute to pathophysiology.ObjectivesThe aim of this study was to evaluate cerebral hemodynamics and assess autonomic dysfunction in patients with MS and explore its relationship with disease status.MethodsEighty patients diagnosed with MS according to McDonald criteria 2017 were recruited to this comparative cross-sectional study. All participants underwent assessment of cerebral hemodynamics through transcranial Doppler ultrasonography. Cerebral vasomotor reactivity (CVR) to hypercapnia was measured by the breath-holding index (BHI). They were also assessed for autonomic dysfunction by autonomic questionnaire and blood pressure measurements in both erect and supine positions.ResultsBHI values showed decreased CVR and were significantly lower in SPMS than in RRMS patients. Autonomic functions were more affected in SPMS than RRMS with total score 2.58 ± 1.13 for the RRMS group and 4.05 ± 1.04 for the SPMS group.ConclusionsCerebrovascular hemodynamic insufficiency in MS may be secondary to the downstream effects of neuro-inflammatory cascades.

Pain, depression, and quality of life in adults with MOG‐antibody–associated disease

Myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD) is an inflammatory autoimmune condition of the central nervous system. However, data on pain and depression have remained scarce. The aim of this study was to assess features of chronic pain and depression as well as their impact on health-related quality of life (hr-QoL) in MOGAD. Patients with MOGAD were identified in the Neuromyelitis Optica Study Group registry. Data were acquired by a questionnaire, including clinical, demographic, pain (PainDetect, Brief Pain Inventory-Short Form, McGill Pain Questionnaire-Short Form), depression (Beck Depression Inventory-II), and hr-QoL (Short Form-36 Health Survey) items. Twenty-two of 43 patients suffered from MOGAD-related pain (11 nociceptive, eight definite neuropathic, three possible neuropathic) and 18 from depression. Patients with neuropathic pain had the highest pain intensity and most profound activities of daily living (ADL) impairment. Fifteen patients reported spasticity-associated pain, including four with short-lasting painful tonic spasms. Later disease onset, profound physical impairment, and depression were associated with chronic pain. Physical QoL was more affected in pain sufferers (p<0.001) than in pain-free patients, being most severely reduced by neuropathic pain (p=0.016). Pain severity, visual impairment, and gait impairment independently predicted lower physical QoL. Depression was the only factor reducing mental QoL. Twelve patients still suffering from moderate pain (pain severity 4.6±2.3) received pain medication. Only four out of 10 patients with moderate to severe depression took antidepressants. Being highly prevalent, pain and depression strongly affect QoL and ADL in MOGAD. Both conditions remain insufficiently controlled in real-life clinical practice.

Multiple Sclerosis flare-ups Diagnostic and Management Approach in Emergency Department, Review Article

Multiple sclerosis is a chronic inflammatory autoimmune condition that causes axonal demyelination and neural dysfunction. It is the most prevalent non-traumatic disabling condition in young adults, with a prevalence of over 2 million cases worldwide. Patients are classified into different subtypes based on their clinical course. The majority of cases have the Relapsing-remitting form that is characterized by acute exacerbation also known as flare-ups. Which could have an immense toll on patient

Does ARFI elastography complement B-mode ultrasonography in the radiological diagnosis of idiopathic granulomatous mastitis and invasive ductal carcinoma?

Idiopathic granulomatous mastitis (IGM) is a chronic, unpleasant autoimmune inflammatory condition and is clinically and radiologically often confused with breast malignancy. To investigate the contributions of qualitative and quantitative aspects of acoustic radiation force impulse (ARFI) elastography to the differential diagnosis between IGM and invasive ductal carcinoma (IDC) in the breast. Ninety-four women with IDC and 39 with IGM were included in the study. Shear wave velocity (SWV) was calculated for all lesions using quantitative elastography. Next, each lesion's correspondence on qualitative elastographic images to those on the B-mode images was evaluated: pattern 1, no findings on elastography images; pattern 2, lesions that were bright inside; pattern 3, lesions that contained both bright and dark areas; and pattern 4, lesions that were dark inside. Pattern 4 was subdivided into 4a (dark area same size as lesion) and 4b (dark area larger than lesion size). Sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy were calculated. The mean SWV based on ARFI elastography was 3.78 ± 1.26 m/s for IGM and 5.34 ± 1.43 m/s for IDC lesions (P < 0.05). Based on qualitative ARFI elastography, IDC lesions were mostly classified as pattern 4b, while IGM lesions were mostly classified as pattern 1 or 2 (P = 0.01). Evaluation of both the qualitative and quantitative aspects of ARFI elastography yielded a sensitivity of 89% and specificity of 84%. ARFI elastography may facilitate the differential diagnosis between IGM and IDC.

Consultation préconceptionnelle dans les maladies auto-immunes systémiques

Many systemic autoimmune diseases and chronic inflammatory rheumatic conditions affect women of childbearing age. Pregnancy is most often possible, with generally acceptable fetal-maternal risks. So, pregnancy wish must be regularly sought and the optimal timing of pregnancy is essential. Women should be reassured that a good pregnancy outcome is generally possible if conception occurs in a stable remission state. The planification is ideally carried out during a preconception counseling appointment in order to look for possible contraindications (infrequent) and risk factors for obstetrical complications (previous obstetrical complication, antiphospholipid biology or syndrome, activity of the disease at conception, and definite organ failure) in order to optimize the management. The most frequent obstetrical complications are fetal losses and/or consequences of placental insufficiency. Anti-SSA and/or anti-SSB antibodies exposes to a low risk of fetal atrioventricular block. Adequate drugs adjustment should be done before conception to avoid an early relapse and to replace teratogenic drugs. Finally, organization of the follow up of this future pregnancy need to be defined in a multidisciplinary approach and explained to the patient at this time.

ANMCO/SIC Consensus document on the management of myocarditis

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.

Rheumatoid arthritis significantly increases incidence of heart failure: examination of an underestimated co-morbidity

Abstract Background The association between coronary heart disease and autoimmune inflammatory conditions such as rheumatoid arthritis has been well established and studied. We aim to elucidate another very important, but overlooked co-morbidity: heart failure. Purpose To evaluate the increased incidence of both systolic and diastolic heart failure in patients who have co-morbid rheumatoid arthritis, compared to the general population. We hypothesize that patients with inflammatory diseases such as rheumatoid arthritis will have significantly increased incidence of heart failure which will require earlier screening and prevention in this patient population. Methods This study is based on a systematic review of published literature utilizing MEDLINE, EMBASE and PUBMED that investigated the association between rheumatoid arthritis and congestive heart failure. Our study included 5,220,960 patients. We also performed a meta-regression analysis to evaluate the effect of age on incidence of heart failure. Summary relative risk (RR) and 95% confidence intervals (CI) were estimated using a random-effects model. Results In 5,220,960 patients, the incidence of heart failure was 2-fold higher in patients with rheumatoid arthritis compared to a matched control population (OR 1.835, 95% CI 1.77–1.90, p&amp;lt;0.00001). On meta-regression, when plotting log odds ratio of incidence of heart failure (y-axis) against age (x-axis), incidence increased further with older age (coefficient = 0.344, p=0.0001). Women were also found to have 5-fold higher incidence of heart failure with rheumatoid arthritis. Conclusion For patients with rheumatoid arthritis, there was a markedly higher incidence of heart failure compared to a matched patient population without rheumatoid arthritis. Meta-regression showed a trend towards increased incidence of heart failure with increased age that reached statistical significance. Women were also at a drastically higher risk of heart failure if they had rheumatoid arthritis. Funding Acknowledgement Type of funding source: None

Coronary vasomotor dysfunction is associated with worse outcomes in patients with inflammatory disease

Abstract Background Rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and psoriasis (PsO) are common inflammatory conditions with excess cardiovascular (CV) risk compared to the general population. This excess CV risk is associated with traditional risk factors, glucocorticoid treatment, and systemic inflammation. Systemic inflammation perturbs endothelial function and has been linked to coronary vasomotor dysfunction. It is not clear if coronary vasomotor dysfunction would be associated with worse clinical outcomes in systemic autoimmune inflammatory conditions. Purpose We tested the hypothesis that impaired coronary flow reserve (CFR), which in the absence of flow-limiting obstructive coronary artery disease (CAD) reflects vasomotor dysfunction, among patients with SLE, RA, and PsO is associated with worse clinical outcomes. Methods We included patients with RA, SLE, and PsO who underwent clinically indicated rest/stress myocardial perfusion positron emission tomography (PET) at a large academic medical center from 2006 to 2019. Patients with an abnormal myocardial perfusion study (summed stress score &amp;gt;3) or left ventricular ejection fraction &amp;lt;40% were excluded. CFR was calculated as the ratio of myocardial blood flow (MBF, ml/min/g) at peak stress compared to the MBF at rest and adjusted for baseline heart rate and blood pressure. Results Among the 175 patients (median age 65.1 years, 80% female) in the cohort, 24% had SLE, 35% PsO, and 41% RA. There was no difference in mean CFR between patients with RA, SLE, or PsO. Over a median follow-up of 8.5 years after PET, there were 47 deaths. Patients in the lowest and middle tertile (CFR &amp;lt;2.18) had a higher all-cause mortality when compared with the highest (Figure 1), and this association remained significant after adjusting for age and a composite clinical score incorporating sex, symptoms, and CV risk factors (lowest vs. highest tertile: HR 2.8; 95% confidence interval 1.2–6.5; p=0.01). CV risk factors such as diabetes, hypertension, obesity, tobacco use, and a family history of CAD were not significantly different across CFR tertiles, suggesting that inflammatory-disease specific risk factors may contribute to coronary vasomotor dysfunction. Conclusions In patients with systemic inflammatory disease, coronary vasomotor dysfunction was associated with worse outcomes independent of traditional CV risk factors and may have utility as a marker of CV risk among patients with inflammatory disease. Figure 1 Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): 1. 5T32HL094301-02 NIH T32 Training Grant, “Noninvasive Cardiovascular Imaging Research Training Program”

CLINICAL CHARACTERISTICS OF PATIENTS WITH SUSPECTED AND CONFIRMED CARDIAC SARCOIDOSIS REFERRED TO A SPECIALIZED CLINIC

Sarcoidosis is an autoimmune chronic inflammatory condition with symptomatic cardiac involvement in 5% of patients. Cardiac Sarcoidosis (CS) can present with heart failure, heart block, and ventricular arrhythmias. Diagnosis is made using endomyocardial biopsy, cardiac MRI and FDG-PET imaging. At our center, patients are treated in a specialized CS clinic allowing us to amalgamate data from our EMR to better understand the types of symptoms CS patients present with, what the most effective ways to diagnose the condition are, and how patients respond to treatments and inform guidelines with the results.

EP34 Ovarian cancer presenting as primary Sjögren’s syndrome: a rare but important mimic

EP34 Ovarian cancer presenting as primary Sjögren’s syndrome: a rare but important mimic

EP33 Sjögren’s and lymphoproliferative disorders

Case report - IntroductionSjögren’s syndrome (SS) is a chronic autoimmune inflammatory condition characterised by lymphocytic infiltration of predominantly exocrine glands as well as other organs involving more commonly in skin, lungs, and neurological system. It is associated with increased risk of both benign and malignant lymphoproliferative disorders.Case report - Case descriptionA 29-year-old woman with 5-year history of Primary SS (ANA, Ro/La positive) was reviewed in a routine follow up. She has mild dry eyes, intermittent vasculitic rash around the ankles, occasional pleuritic chest pain but no cough or dyspnoea initially. She used artificial tears but took no regular mediations. Oxygen saturation at rest was 99% and BP 127/70. Heart sounds were normal, and chest was clear.Blood results showed alanine transaminase (ALT) had risen from 50 (normal 5-45) to 145 over 6 months. Viral screen (hepatitis, HIV, CMV, EBV) and liver ANA panel were negative. C4 was low 0.09 (n 0.15-0.55), C3 normal and DsDNA antibodies 1 (n 0-15.1). Ig G was high, fluctuating between 27 - 33 (n 7.67-15.9). ESR had been mildly elevated at 26 (n 0-19). CRP had always been within normal limits. Ultrasound abdomen showed enlarged spleen (15 cm). CT chest abdomen and pelvis showed multifocal groundless opacification affecting mid to upper zones, bilateral cyst formation, solid nodularity, and mild septal thickening with no pleural effusion. There were multiple prominent bilateral axillary and mediastinal lymph nodes measuring upto15 mm. The spleen measured 15.4 cm. Six months later, she developed mild dyspnoea on exertion. Examination revealed fine basal crackles. Oxygen saturation remained 99% at rest. TLco was mildly reduced and Kco in lower limit of normal. Echocardiogram was normal. Ultrasound guided core biopsy of axillary lymph node showed benign reactive features with follicular hyperplasia and plasmacytosis.The diagnosis of lymphocytic interstitial pneumonitis was made. She was started on a reducing dose of Prednisolone 30 mg and Mycophenolate stepwise increment to 1 gm BD. Chest pain resolved shortly after starting glucocorticoid therapy and exercise tolerance improved.Liver enzyme normalised. Case report - DiscussionThe diagnosis of lymphocytic interstitial pneumonia (LIP) in this patient was incidental from the retrospective investigation of the persistently abnormal unexplained liver function test. Splenomegaly is a recognised manifestation of active systemic SS. The main differential diagnoses are lymphoma and other types of interstitial lung diseases (ILD). Her presentation was insidious and there were no constitutional symptoms. Her ANA, Ro, La and Rheumatoid factor were strongly positive. She has hypergammaglobulinemia which is commonly associated with LIP. Histological findings were typical reactive changes with characteristic polymorphic plasmacytosis. In the absence of malignant lymphoid cells and radiological features of ground glass changes and bilateral cysts formation suggested that the most likely diagnosis was LIP secondary to primary SS.The management strategies vary for different types of ILD based on their etiological conditions. There are no specific guidelines for the treatment of LIP. For initial acute stage, glucocorticoid therapy is usually prescribed for symptomatic improvement. For later stages and cysts, immunosuppressants are used and depending on the severity, azathioprine, cyclosporin, rituximab and cyclophosphamide have been used.Median survival for LIP is 11.2 years. Increased risk of secondary infection because of immunosuppressive therapy is common and there are recommendations for atypical chest infection prophylaxis.Case report - Key learning pointsInterstitial lung disease is commonly associated with SS and predicts a higher mortality rate.Glucocorticoid is the main stay of treatment for ILD acute stage with active inflammatory changes.For refractory cases and chronic cystic stages on radiological findings, further immunosuppression therapy may be required.Standardised treatment guidelines are not available.Secondary chest infections may be seen in those with cystic disease and on immunosuppression.Close monitoring and MDT approach is required in the management of LIP.LIP may transform into lymphoma or co-present with lymphoma associated with SS.

'More extraordinary than mundane … ' A phenomenological analysis of the experiences of individuals living with CLE and their taking care in the sun.

IntroductionCLE is a chronic inflammatory autoimmune condition of which photosensitivity is a major symptom. Individuals living with CLE are advised to practice photoprotection. Despite the benefits for disease control, many individuals living with CLE do not practice optimal photoprotection. The aim of this study was to gain a deep insight into the lived experiences of individuals with CLE and their photoprotective practices.MethodsA qualitative study approach was conducted, using Hermeneutic phenomenology. Individuals living with CLE were recruited and interviewed. Rich pictures were used to enrich the interviews. Interviews were transcribed and analysed using Template Analysis.ResultsAnalysis revealed four themes: ‘Much more than just a photosensitive skin condition’, ‘The impact of sun on CLE and social dynamics’, ‘Drifting to the sun: personal transitions and social norms’ and ‘Taking care in the sun: easier said than done’.Discussion and conclusionThis study provides a nuanced insight into the lived experiences of individuals with CLE and their photoprotective practices. Taking care in the sun is not a simplistic process. Beyond the biomedical model of illness, the social impact that CLE has on individuals has a dominant influence on their photoprotective behaviours. Such insights could help healthcare professionals tailor photoprotective advice.

Neuromyelitis optica spectrum disorder after presumed coronavirus (COVID-19) infection: A case report

Neuromyelitis optica spectrum disorder is an inflammatory autoimmune condition, predominantly affecting the optic nerves and spinal cord. It has been stated that viral infections play a role in the development of neuromyelitis optica. Several murine coronaviruses can cause inflammatory demyelinating diseases, including optic neuritis. Here we report, to the best of our knowledge, the first human case linking a presumed SARS-CoV-2 infection to the development of NMOSD.

Artritis Reumatoide y embarazo. Actualización

Rheumatoid Arthritis (RA) is a frequent chronic inflammatory autoimmune condition, characterized by persistent inflammation of synovial joints. Managing RA during pregnancy is complex, considering the need for an appropriate balance between monitoring disease activity and mitigating symptoms with non-toxic but effective therapies. During pregnancy, a decrease in disease activity can be observed in 48 to 86% of patients. Some RA patients may develop flares, as well as pregnancy complications such as preterm labor, low birth weight and miscarriage. NSAIDs may increase the risk of subfertility in patients with RA. Hydroxychloroquine and sulfasalazine can be safely administered throughout pregnancy. Glucocorticoids at doses greater than 7.5mg/day prednisone or equivalent were associated with preterm delivery. During the first trimester of pregnancy, continuation of anti-TNF therapy is recommended. Both Leflunomide and Methotrexate should be discontinued when pregnancy is being planned.

Cerebral hemodynamics in patients with multiple sclerosis

Background and PurposeMultiple sclerosis (MS) is a demyelinating disease of the central nervous system. Although the currently predominant view is that of an autoimmune inflammatory condition, changes in brain vasculature can occur and contribute to pathophysiology. The aim of this study was to evaluate cerebral hemodynamics in patients with MS and explore its relationship with disease status. MethodsPatients with MS and age- and sex- matched healthy controls were recruited. All participants underwent assessment of cerebral hemodynamics through transcranial Doppler ultrasonography. Cerebral vasomotor reactivity (CVR) to hypercapnia was measured by the breath-holding index (BHI). ResultsA total of 80 patients with MS and 80 healthy controls were recruited. BHI values obtained in healthy controls, relapsing-remitting (RR)-MS and secondary progressive (SP)-MS patients were 1.15±0.11, 0.87±0.18 and 0.51±0.20, respectively (p<0.001). Group-wise, patients showed decreased CVR in comparison to controls and BHI values were significantly lower in SP-MS than in RR-MS patients. At linear regression analysis, the disease form was significantly associated with cerebral hemodynamics being the SP phenotype an independent predictor of lower BHI values [β=-0.36, 95% confidence interval (CI): -0.44 to -0.27, p<0.001; adjusted β=-0.37, 95% CI: -0.50 to -0.23, p<0.001). ConclusionsCerebrovascular hemodynamic insufficiency in MS may be secondary to the downstream effects of neuro-inflammatory cascades.

Early Life Exposure to the Great Chinese Famine and Risk of Rheumatoid Arthritis in Adulthood

Abstract Objectives Rheumatoid arthritis (RA) is a chronic, inflammatory autoimmune condition. Because the immune system develops early in life, it is possible that exposure to adversity like famine in utero or during early childhood may have lifelong impacts on risk of autoimmune disease. We thus investigated whether exposure (in utero or in early life) to the Great Chinese Famine of 1959–1961 was associated with risk of RA in adulthood. Methods Included were 101,510 participants of the Kailuan Study that joined the study at baseline (2006). RA cases were confirmed by medical record review. We used logistic regression to calculate the odds ratio (OR) and 95% confidence interval (95% CI) for RA, according to famine exposure status (exposed in utero, between 0 and 3 years, between 3 and 6 years, or at 6 years or older), in comparison to participants born after 1961 (not exposed to famine). Potential confounders (e.g., sex, body mass index, smoking status, and plasma concentrations of c-reactive protein, low-density lipoprotein and high-density lipoprotein) were adjusted in the model. Results During 12 years of follow-up (2006–2018), we identified 187 RA cases. Individuals exposed to the Famine in utero or in ages 0–3 years, had a higher prevalence of RA, relative to other groups (0.24%–0.35% vs 0.08–0.20%). After adjustment for potential confounders, the OR of RA was 2.70 (95% CI: 1.40 to 5.21) for in utero famine exposed individuals, 4.44 (95% CI: 2.66 to 7.39) for those exposed in age 0–3 y, 2.50 (95% CRI: 1.40 to 4.47) for those exposed in age 3–6 y, and 2.61 (95% CI: 1.63 to 4.18) for those exposed after at least 6 years old versus individuals who were born after 1961. A similar association was observed for men and women (P-interaction = 0.86). Conclusions Individuals with exposure to famine in utero or early childhood (0–3 years), were more likely to suffer from RA in adulthood. This study reflects the importance of early life as a key developmental period for the immune system, and demonstrates that exposure to famine during this time results in increased risk of RA in adulthood. Funding Sources Study funded by start-up grant from the College of Health and Human Development and the Department of Nutritional Sciences, The Pennsylvania State University, and Natural Science Foundation of Hebei Province (H2018209318).