scholarly journals Don’t Forget That Chorea!

2021 ◽  
Author(s):  
Lokesh Krishnaji Kolhe ◽  
Yumin Audrey Tham
Keyword(s):  
Lupus Erythematosus ◽  
Brain Mri ◽  
Normal Serum ◽  
Adverse Outcomes ◽  
The Body ◽  
Limb Weakness ◽  
Metabolic Derangement ◽  
Specific Finding ◽  
Ct Brain ◽  
T1 Hyperintensity

Abstract Background: Hemichorea-hemiballismus is a spectrum of involuntary, continuous non-patterned movement involving one side of the body. Possible causes of hemichorea-hemiballismus include haemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, NHH (non-ketotic hyperglycaemic hemichorea), Wilson’s disease, and thyrotoxicosis. Amongst the metabolic causes, chorea associated with NHH is noteworthy and is mainly reported in elderly Asian women. The pathophysiology of this syndrome remains controversial. It is likely that a combination of hyperglycaemia induced basal ganglia metabolic derangement and failure of cerebral blood flow autoregulation contribute to the syndrome.Case presentation: A 45-year-old Malay gentleman presented to our Emergency Department with right upper and lower limb weakness associated with hemichorea for 3-4 days. His initial blood glucose level was 22 mg/dl with normal serum ketone and bicarbonate levels. CT brain showed a hyperdensity in the left caudate nucleus and globus pallidus region. Subsequent brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycaemia-induced hemichorea-hemiballismus syndrome. The hemiballismus/hemichorea improved rapidly within the next day. Conclusions: This unusual clinical presentation is often accompanied by severe hyperglycaemia. Appropriate blood glycaemic control is important because it is reversible with correction of hyperglycaemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.

scholarly journals A young female of systemic lupus erythematosus with hepatic necrosis

2016 ◽  
Vol 44 (1) ◽  
pp. 43-45
Author(s):  
Md Abu Shahin ◽  
Mohammad Imtiaz Sultan ◽  
Nadia Sultana ◽  
Atia Saeed ◽  
Sabrina Yeasmin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
High Titer ◽  
Chronic Inflammatory Disease ◽  
Normal Serum ◽  
The Body ◽  
Unknown Etiology ◽  
High Dose ◽  
Systemic Lupus ◽  
Serum Lipase

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown etiology which can affect the skin, joints, kidneys, lungs, nervous system and other organs of the body. Clinical and radiological liver diseases are uncommon in patients with SLE. We report a 40-year-old female with known SLE presented with fever, severe abdominal pain, progressive abdominal distension, vomiting & absence of bowel movement for 4 days. Laboratory tests revealed thrombocytopenia, elevated ALT, AST, Alkaline phosphatase and high titer anti ds-DNA. USG revealed mild hepatosplenomegaly. serum bilirubin, serum electrolyte, serum amylase, 24 hour urinary amylase and serum lipase were normal. Serum anti phospholipid antibody was negative. Computed tomography (CT) of abdomen showed hypodense lesions in the liver that mimicked multiple liver abscesses and CT abdominal angiography showed hepatic infarction. She was treated with pulse methylprednisolone followed by high dose oral prednisolone and intravenous pulse cyclophosphamide. Patient improved clinically; platelet count & liver enzymes returned to the normal range. This patient represents a rare case of SLE who had hepatic vasculitis mimicking multiple liver abscesses.Bangladesh Med J. 2015 Jan; 44 (1): 43-45

Biocompatible Nanovesicular Drug Delivery Systems with Targeting Potential for Autoimmune Diseases

2020 ◽  
Vol 26 (42) ◽  
pp. 5488-5502 ◽  
Author(s):  
Yub Raj Neupane ◽  
Asiya Mahtab ◽  
Lubna Siddiqui ◽  
Archu Singh ◽  
Namrata Gautam ◽  
...  
Keyword(s):  
Drug Delivery ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Drug Delivery Systems ◽  
Drug Carriers ◽  
Immunological Tolerance ◽  
Delivery Systems ◽  
The Body ◽  
Autoimmune Response ◽  
Treatment Modalities

Autoimmune diseases are collectively addressed as chronic conditions initiated by the loss of one’s immunological tolerance, where the body treats its own cells as foreigners or self-antigens. These hay-wired antibodies or immunologically capable cells lead to a variety of disorders like rheumatoid arthritis, psoriatic arthritis, systemic lupus erythematosus, multiple sclerosis and recently included neurodegenerative diseases like Alzheimer’s, Parkinsonism and testicular cancer triggered T-cells induced autoimmune response in testes and brain. Conventional treatments for autoimmune diseases possess several downsides due to unfavourable pharmacokinetic behaviour of drug, reflected by low bioavailability, rapid clearance, offsite toxicity, restricted targeting ability and poor therapeutic outcomes. Novel nanovesicular drug delivery systems including liposomes, niosomes, proniosomes, ethosomes, transferosomes, pharmacosomes, ufasomes and biologically originated exosomes have proved to possess alluring prospects in supporting the combat against autoimmune diseases. These nanovesicles have revitalized available treatment modalities as they are biocompatible, biodegradable, less immunogenic and capable of carrying high drug payloads to deliver both hydrophilic as well as lipophilic drugs to specific sites via passive or active targeting. Due to their unique surface chemistry, they can be decorated with physiological or synthetic ligands to target specific receptors overexpressed in different autoimmune diseases and can even cross the blood-brain barrier. This review presents exhaustive yet concise information on the potential of various nanovesicular systems as drug carriers in improving the overall therapeutic efficiency of the dosage regimen for various autoimmune diseases. The role of endogenous exosomes as biomarkers in the diagnosis and prognosis of autoimmune diseases along with monitoring progress of treatment will also be highlighted.

scholarly journals Electroencephalography in systemic lupus erythematosus patients with neuropsychiatric manifestations

2020 ◽  
Vol 32 (1) ◽  
Author(s):  
Howaida E. Mansour ◽  
Reem A. Habeeb ◽  
Noran O. El-Azizi ◽  
Heba H. Afeefy ◽  
Marwa A. Nassef ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Epileptiform Activity ◽  
Brain Mri ◽  
Periventricular White Matter ◽  
Normal Brain ◽  
Systemic Lupus ◽  
Specific Test ◽  
Eeg Abnormalities ◽  
Neuropsychiatric Manifestations

Abstract Background Neuropsychiatric manifestations are frequently reported in systemic lupus erythematosus (SLE) patients. This study was done to describe electroencephalographic (EEG) findings in SLE patients with neuropsychiatric manifestation (NPSLE). Results Among 60 SLE patients, there were 50 females (83.3%) and 10 males (16.7%). EEG abnormalities were reported in 12 patients out of 30 (40%) with NPSLE, while all patients with non-NPSLE (n = 30) had no EEG abnormalities; diffuse slowing (20%) was the most common abnormalities, followed by generalized epileptiform activity (13.3%), and lastly temporal epileptiform activity (6.7%). Seizure was the most reported neuropsychiatric disorder in 13 patients (43.3%); 8 of them had abnormal EEG (61.5%). Periventricular white matter lesion (23.3%) followed by infarction (13.3%) were the most common MRI brain findings among 53.3% of NPSLE group. Half of the cases with EEG abnormality had normal brain MRI. SLEDAI score and ACL IgM positivity were higher in the NPSLE group than the non-NPSLE group. EEG is not a sensitive or specific test for detecting NPSLE with sensitivity (37.5%) and specificity (57.1%). Conclusion Not all patients with NPSLE must have abnormal brain MRI or EEG. EEG is a useful assistant tool in the assessment of different manifestations of NPSLE, but it cannot be used as a screening test alone and must be supplemented by neuroimaging studies.

scholarly journals POS0719 ADVERSE OUTCOMES AND REHOSPITALIZATION AFTER DELIVERY AMONG WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS OR RHEUMATOID ARTHRITIS AND THEIR INFANTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 609.1-609
Author(s):  
J. Sabo ◽  
N. Singh ◽  
D. A. Crane ◽  
D. R. Doody ◽  
M. A. Schiff ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Hospital Discharge ◽  
Birth Outcomes ◽  
Gestational Age ◽  
Small For Gestational Age ◽  
Lupus Erythematosus ◽  
Adverse Outcomes ◽  
Systemic Lupus ◽  
Cesarean Deliveries

Background:Women with rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) have greater risk of adverse obstetric and birth outcomes than women without these conditions. Infant outcomes are less well-studied. It is unknown whether re-hospitalization after delivery occurs more often for affected mothers and their infants.Objectives:We compared obstetric outcomes among women with and without RA or SLE, and birth outcomes among their infants. Maternal and infant rehospitalizations <2 years of delivery were also compared.Methods:This population-based cohort study used linked birth-hospital discharge data from Washington State for 1987-2014. International Classification of Disease 9th revision (ICD9) codes identified all women with RA (ICD9 714.X, 725.X) and SLE (ICD9 710, 710.0, 710.1) in the hospital discharge record at delivery, and a 10:1 comparison group of women without these codes. Analyses were restricted to singleton live births (1,223 RA; 1,354 SLE). Poisson regression with robust standard errors estimated relative risks (RR) and 95% confidence intervals (CI) for selected outcomes, accounting for delivery year, maternal age, and parity.Results:Many adverse outcomes were more common among RA and SLE cases than among comparison women. Preeclampsia occurred more often during pregnancies of women with RA (RR 1.42, 95% CI 1.17-1.71) or SLE (RR 2.33, 95% CI 2.01-2.70), as did preterm rupture of membranes (PROM, RR 2.85, 95% CI 2.20-3.72 for RA; RR 3.28, 95% CI 2.54-4.23 for SLE). Cesarean deliveries were more common among nulliparous women in both groups (RR 1.32, 95% CI 1.18-1.48 for both conditions). Infants of women with RA or SLE were more likely to weigh <2500 g (RR 2.08, 95% CI 1.72-2.52 for RA; RR 4.88, 95% CI 4.27-5.58 for SLE), be small for gestational age (RR 1.25, 95% CI 1.07-2.50; RR 2.30; 2.04-2.59, respectively), delivered at <32 weeks gestation (RR 1.83, 95% CI 1.13-2.97; RR 5.13, 95% CI 3.75-7.01, respectively), and require neonatal intensive care unit admission (NICU, RR 1.89, 95% CI 1.56-2.30; RR 2.71, 95% CI 2.25-3.28, respectively). Infants of women with SLE were more likely to have a malformation (RR 1.46, 95% CI 1.21-1.75) or die within 2 years (RR 2.11, 95% CI 1.21-3.67). Rehospitalization levels among both women with RA (RR 2.22; 1.62-3.04) and SLE (RR 2.78, 95% CI 2.15-3.59) were greatest <6 months of delivery and declined over time. Infants of women with SLE had increased rehospitalization <6 months (RR 1.64, 95% CI 1.36-1.98).Conclusion:Consistent with prior literature, we found women with RA or SLE experienced many adverse outcomes. In our data, these included preeclampsia, PROM, and cesarean deliveries, with increased risks more notable among women with SLE. Infants of women with either condition were more likely to weigh <2500g, be <32 weeks gestation, small for gestational age, and require NICU admission than infants of comparison women. Only infants of women with SLE had increased malformations. Maternal rehospitalization after delivery was more common in both groups; most marked at <6 months. Infant rehospitalizations were increased in both cohorts to a lesser extent. Close follow-up during this time period is crucial to minimize adverse outcomes.Disclosure of Interests:Julianna Sabo: None declared, Namrata Singh: None declared, Deborah A. Crane: None declared, David R. Doody: None declared, Melissa A. Schiff: None declared, Beth A. Mueller Shareholder of: Household owns shares in AstraZeneca

scholarly journals AB0300 LUPUS DISEASE ACTIVITY CORRELATES WITH QUALITY OF LIFE BUT NOT WITH HEALTH LITERACY STATUS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1176.2-1176
Author(s):  
E. Eraslan ◽  
R. Bilici Salman ◽  
H. Satiş ◽  
A. Avanoglu Guler ◽  
H. Karadeniz ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Health Literacy ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Patient Reported Outcome ◽  
The Body ◽  
Limited Health Literacy ◽  
Patient Reported ◽  
Limited Health

Background:Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown etiology that can affect any organ of the body. SLE is associated with adverse effects on both health and non-health-related quality of life (HRQOL and non-HRQOL). Lupus PRO is a patient reported outcome measure that has been validated in many languages. It has 44 items that cover both HRQOL and non-HRQOL (1). Health literacy is defined as the degree to which individuals have the capacity to obtain, process and understand basic health information and services needed to make appropriate health decisions. Multiple studies indicate that people with limited health literacy have worse health status and higher rates of hospitalization (2).Objectives:We aimed to evaluate the relationship between the LLDAS (Lupus Low Disease Activity State) criteria and the Lupus PRO test, as well as the health literacy status of lupus patients.Methods:83 SLE patients (94% women) were included in the study. We performed Lupus PRO and the European Health Literacy Survey tests during the routine follow-up visits of lupus patients to our rheumatology outpatient clinic and admissions to rheumatology inpatient clinic. Available clinical data on medical records were obtained, physician global assessments (PGA) were recorded by the attending physician.Results:LLDAS criteria strongly and inversely correlated with the total score, as well as the mood subunit of the Lupus PRO. Similarly, it also significantly inversely correlated with the body appearence and goals subunits. Health literacy status of the patients did not correlate with their LLDAS scores, ie their disease activities.Conclusion:Our results suggest that lupus disease activity, assessed by LLDAS criteria, significantly correlates with measures of quality of life, spesicifically Lupus PRO test, but not with health literacy status. Further studies are needed to evaluate if health literacy is related with damage, hospitalization or mortality associated with lupus.References:[1]Jolly M, Pickard AS, Block JA, Kumar RB, Mikolaitis RA, Wilke CT, et al., editors. Disease-specific patient reported outcome tools for systemic lupus erythematosus. Seminars in arthritis and rheumatism; 2012: Elsevier.[2]Paasche-Orlow MK, Parker RM, Gazmararian JA, Nielsen-Bohlman LT, Rudd RR. The prevalence of limited health literacy. Journal of general internal medicine. 2005;20(2):175-84.Disclosure of Interests:None declared

scholarly journals Provider perceptions on the management of lupus during pregnancy: barriers to improved care

Lupus ◽  
2018 ◽  
Vol 28 (1) ◽  
pp. 86-93 ◽  
Author(s):  
M E B Clowse ◽  
A M Eudy ◽  
J Revels ◽  
L Neil ◽  
G D Sanders
Keyword(s):  
Lupus Erythematosus ◽  
Adverse Outcomes ◽  
Optimal Management ◽  
Pregnancy Planning ◽  
Provider Perceptions ◽  
Medically Ill ◽  
Treatment Plans ◽  
Poor Outcomes ◽  
Physician Patient ◽  
Patient Confidence

Background More than half of pregnancies in women with systemic lupus erythematosus (lupus) result in adverse outcomes for the mother or the fetus. We sought to identify aspects of current rheumatologic care that could be improved to decrease the frequency of poor outcomes. Methods Focus groups with clinical rheumatologists, based on the PRECEDE/PROCEED framework, identified factors that influenced care. A group of women with lupus on their reproductive journey contributed to our understanding of the dilemmas and care provided. Results Medically ill-timed pregnancies and medication non-adherence during pregnancy were identified by rheumatologists as the two key dilemmas in care. We identified several communication gaps as key modifiable barriers to optimal management. The approach to physician–patient communication was often unsuitable to sensitive discussions about pregnancy planning. The communication of treatment plans was frequently hampered by gaps in knowledge and both physician and patient confidence in the data, encouraging non-adherence among nervous patients. Finally, local rheumatologists and obstetricians/gynecologists providers frequently did not communicate, leading to varying treatment plans and confusion for patients. Conclusions To decrease the frequency of ill-timed pregnancy and medication non-adherence it will be essential to empower rheumatologists, and women with lupus to have open and accurate conversations about pregnancy planning and management.

scholarly journals Diffuse Leukoencephalopathy and Subacute Parkinsonism as an Early Manifestation of Systemic Lupus Erythematosus

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Gary G. Tse ◽  
Alberto S. Santos-Ocampo ◽  
Dominic C. Chow ◽  
Aaron M. McMurtray ◽  
Beau K. Nakamoto
Keyword(s):  
Lupus Erythematosus ◽  
Poor Prognosis ◽  
Single Case ◽  
Brain Mri ◽  
Central Nervous System Involvement ◽  
Good Recovery ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Early Manifestation

Parkinsonism in SLE is rare. Diffuse leukoencephalopathy is equally uncommon and is associated with a poor prognosis. We present a single case of a 50-year-old Filipino man who presented with a generalized discoid rash after starting lisinopril. The rash persisted despite discontinuation of lisinopril, and over the next three months, he developed rapidly progressive parkinsonism. Brain MRI showed symmetric confluent T2-hyperintensities involving the white matter and basal ganglia. Four of the 11 American College of Rheumatology criteria for the classification of SLE were met. A rheumatologist made a diagnosis of SLE with cutaneous and central nervous system involvement. Significant neurologic and radiologic improvement occurred following treatment with IV steroids followed by a prolonged taper. This report highlights a case of subacute parkinsonism with a diffuse leukoencephalopathy as an early manifestation of SLE which resulted in a good recovery following treatment with only immunosuppressive therapy.

scholarly journals Systemic Lupus Erythematosus Disease: An Overview of the Clinical Approach to Pathogenesis, Diagnosis, and Treatment

2021 ◽  
Vol 4 (2) ◽  
pp. 91-98
Author(s):  
Saurabh Nimesh ◽  
Md. Iftekhar Ahmad ◽  
Shikhka Dhama ◽  
Pradeep Kumar ◽  
Muhammad Akram ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Chronic Disease ◽  
Lupus Erythematosus ◽  
The Body ◽  
Clinical Approach ◽  
Systemic Lupus Erythematosus Disease ◽  
Systemic Lupus ◽  
Organ Systems ◽  
Novel Approaches

The systemic lupus erythematosus (SLE), commonly known as Lupus, is a rare and complex multisystem autoimmune disease where one’s immune system is overactive, and the body attacks its organ systems. SLE is a historically old disease described already in antiquity; it is an example of a chronic disease with physical, psychological, financial, and social implications for individuals diagnosed. It has inspired medical and basic biological scientists that focus on molecular biology, basic immunology, immunopathology, clinical science, genetics, and epidemiology. The syndrome is real in its existence-although hidden behind obstacles, cumbersome for patients and clinicians, and rebellious for scientists. There is currently no cure for SLE. The goal of treatment is to ease symptoms. This article will review information on the general approach to SLE therapy, focusing on currently approved therapies and novel approaches that might be used in the future.

scholarly journals A case report of a severe neonatal systemic vasculitis on the first day of life

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Stephanie Wong ◽  
Erkan Demirkaya ◽  
Roberta Berard
Keyword(s):  
Lupus Erythematosus ◽  
Reactive Arthritis ◽  
Index Finger ◽  
Systemic Vasculitis ◽  
Clinical Signs ◽  
The Body ◽  
Oral Steroid ◽  
Left Index Finger ◽  
High Dose ◽  
New Onset

Abstract Background Neonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides. Here we report a novel association of a case of new-onset maternal seronegative inflammatory arthritis associated with a transient systemic vasculitis in a neonate. Case presentation In the first 24 h of life, a preterm Caucasian baby boy was noted to have blue discoloration to all four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, the discoloration remained, particularly in the left index finger. This was associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial echocardiogram showed enlarged coronary arteries with normalization on repeat 1 week later. Clinical signs and symptoms responded to high dose oral steroid administration. MRI angiography (MRA) of the body and heart showed tortuosity of arteries in the upper and lower extremities with gadolinium uptake, suggestive of vasculitis. Autoantibody profile negative. Genetic panel for hereditary autoinflammatory diseases was negative as was whole exome sequencing performed on the trio. The baby was weaned off steroids by 5 months of age. A small distal autoamputation of the left index finger occurred. He was born to a 28-year-old woman who developed new onset severe symmetrical polyarthritis at 8 weeks gestation. This was presumed a reactive arthritis secondary to a dental infection. Infectious work up and autoantibodies were negative. She was treated with high dose prednisone for the remainder of her pregnancy. The mother was weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum and remains in remission. A repeat MRA done at 1 year old showed mild residual tortuosities of the arteries in the forearms. The remainder of the medium and large vessels were within normal limits with no gadolinium enhancement to suggest active disease. The child is now 4 years old with normal growth and development. Conclusion This is a unique case of new-onset seronegative presumed reactive arthritis in a mother with the rare development of a successfully treated medium vessel vasculitis in an infant.

Cephalexin-Induced Stevens-Johnson Syndrome

1992 ◽  
Vol 26 (10) ◽  
pp. 1230-1233 ◽  
Author(s):  
Kim M. Murray ◽  
Michael S. Camp
Keyword(s):  
Gastrointestinal Tract ◽  
Lupus Erythematosus ◽  
Skin Surface ◽  
Skin Lesions ◽  
Infectious Complications ◽  
The Body ◽  
Stevens Johnson Syndrome ◽  
Medline Search ◽  
Johnson Syndrome ◽  
Macular Rash

OBJECTIVE: To report a case of cephalexin-induced Stevens-Johnson syndrome (SJS), a devastating adverse drug reaction that involves the entire skin surface and mucosal areas of the body. DATA SOURCES: MEDLINE search (key terms cephalosporins, Stevens-Johnson syndrome, erythema multiforme, and systemic lupus erythematosus) and references identified from bibliographies of pertinent articles. DATA SYNTHESIS: Clinical presentation and manifestations of SJS include the skin, eyes, gastrointestinal tract, and pulmonary system. Infectious complications are the leading cause of mortality. Early intervention is important to prevent progression of SJS. The case described is consistent with the features of this syndrome. The patient presented with fever, arthralgias, and malaise. Skin lesions included a diffuse violet macular rash with erythema and multiple bullous lesions on her neck and abdomen. The skin biopsy was consistent with SJS. Multiple mucocutaneous ulcers were found in her mouth, but no evidence of lower gastrointestinal tract involvement was documented. She remained relatively free of pulmonary complaints except for the presenting bronchitis. CONCLUSIONS: Cephalexin should be added to the list of agents to consider as iatrogenic causes of SJS.

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