History Of Systemic Lupus Erythematosus Research Articles

A rare case of lupus cerebritis presenting as ictal epileptic headache: A case report.

Ictal epileptic headache, characterized by headache as the sole symptom of a seizure attack, is a rare condition. In this case report, we present a 52-year-old female with a history of systemic lupus erythematosus who sought medical attention at the headache clinic due to a new type of headache. The headache was described as an intense painful wave followed by a dull headache, without autonomic symptoms or migrainous features. Magnetic resonance imaging revealed an enhancing lesion in the left hippocampus in addition to two other lesions in the corpus callosum and left parieto-occipital lobe. Electroencephalography during the headache episodes showed epileptic discharges originating from the left fronto-temporal region. The patient was initiated on levetiracetam, which resulted in the resolution of both the epileptic discharges and the headaches. This case underscores the significance of considering ictal epileptic headache as a potential secondary cause for headaches, particularly in patients with underlying conditions that may predispose them to epilepsy, such as systemic lupus erythematosus.

Comparison of Efficacy of 5% Tranexamic Acid vs 20% Azelaic Acid in Patients of Melasma

Background: Melasma is a significant cosmetic concern globally, affecting individuals across various ethnic backgrounds, particularly in South Asian populations. The condition's etiology is complex, involving genetic, hormonal, and environmental factors such as sun exposure. Despite numerous treatment options, melasma remains challenging to manage effectively. Recent studies have explored the efficacy of tranexamic acid and azelaic acid, both of which target the melanin synthesis pathway but through different mechanisms. Objective: The aim of this study was to compare the effectiveness of topical 5% tranexamic acid versus 20% azelaic acid in the treatment of melasma, with a focus on changes in the Melasma Area and Severity Index (MASI) scores. Methods: This prospective comparative study was conducted at the Dermatology department of PIMS Hospital, Pakistan, over six months from August 2023 to January 2024. A total of 200 patients with melasma were enrolled and randomly assigned to two groups: Group A received 5% tranexamic acid cream, and Group B received 20% azelaic acid cream, both applied twice daily. Inclusion criteria included men and women aged 18 to 50 years with a diagnosis of melasma for at least six months. Pregnant women, patients with a history of Systemic Lupus Erythematosus (SLE) or Discoid Lupus Erythematosus (DLE), and those on hormonal therapy were excluded. The primary outcome measure was the change in MASI scores from baseline to the end of the study period. Data were analyzed using SPSS Version 25. Results: The study found significant differences in treatment efficacy between the two groups. Group A (tranexamic acid) showed a reduction in MASI scores from 7.94 ± 1.88 at baseline to 5.50 ± 1.61 post-treatment. Group B (azelaic acid) demonstrated a decrease from 8.21 ± 1.96 to 5.89 ± 1.49. The proportion of patients experiencing excellent response was higher in Group A (36.0%) compared to Group B (19.0%), with a statistically significant difference in overall treatment efficacy (P<0.0001). Conclusion: Topical 5% tranexamic acid was more effective than 20% azelaic acid in improving MASI scores in patients with melasma. This study supports the inclusion of tranexamic acid as a preferable treatment option for melasma, offering a promising alternative for those not responding to conventional therapies.

Enigmatic CNS Lupus Vasculitis: A Heuristic Approach

Systemic lupus erythematosus is an autoimmune disease affecting all organs of the body. It has a higher incidence in women and can affect the central nervous system (CNS) in 10% to 20% of cases. Any structure in the CNS can be involved, resulting in a varied and often complex symptomatology. CNS lupus vasculitis is an even rarer manifestation characterized by small vessel inflammation that can result in vessel thrombosis and necrosis. We present the case of a 31-year-old woman with a history of systemic lupus erythematosus and Sjögren’s syndrome who was treated at our institution for an acute exacerbation of lupus complicated by CNS lupus vasculitis. This case was particularly challenging due to concurrent bacteremia and a travel history that broadened the differential diagnosis widely, thus requiring our input as infectious disease consultants. CNS lupus vasculitis is a diagnosis of exclusion that requires a high level of clinical suspicion to arrive at a prompt diagnosis paired with aggressive management to guarantee good outcomes. In this report, we share our stepwise approach to this challenging case in a didactic manner to benefit trainees and independent infectious diseases providers who may encounter similar clinical scenarios in their practice.

Moderate Mitral Regurgitation Secondary to Hammock Congenital Mitral Valve Disease

A 30-year-old woman with a history of systemic lupus erythematosus and hypothyroidism . She started with dyspnea on moderate exertion, orthopnea, and a sensation of palpitations an early systolic murmur of mitral regurgitation was found. Transthoracic and transesophageal echocardiography reported hammock congenital mitral valve disease as a cause of mitral regurgitation with impaired functional class, requiring surgical valve replacement. The mitral arcade, or hammock mitral valve, is a very rare entity, even more so in the adult population, with a reported prevalence of 0.5%. It is characterized by abnormal papillary muscles connected directly to the anterior mitral.

A rare case of Disseminated Histoplasmosis with Hemophagocytic Lymphohistiocytosis mimicking a flare of Systemic Lupus Erythematosus in a middle-aged man: A Case Report

Abstract Introduction/Objective Disseminated histoplasmosis is a progressive granulomatous disease caused by Histoplasmosis capsulatum which is an intracellular dimorphic fungus endemic to Ohio and Mississippi River valleys in the United States. The progressive spread of the infection to organs is thought to be due to the failure of the activation of the T-cell mediated immune response. Hemophagocytic lymphohistiocytosis is a rare but potentially fatal condition in which histiocytes and lymphocytes build up in and damage organs and other blood cells. Diagnosing Disseminated Histoplasmosis with Hemophagocytic Lymphohistiocytosis in a patient with Systemic Lupus Erythematosus is diagnostically challenging due to the rare presentation. Hence, we are reporting this case to alert physicians to keep a high index of suspicion in such presentations. Methods/Case Report We present a 37-year-old man with a past medical history of Systemic Lupus Erythematosus complicated by Lupus Nephritis on immunosuppressive therapy who presented to the emergency department with hypotension and was admitted for acute kidney injury. Prior to the presentation, he had persistent fever, myalgias, cough, mild shortness of breath, and back pain. CT Chest shows “eggshell” calcification and further parasitology evaluation revealed a diagnosis of histoplasmosis using a blood sample where intracellular Histoplasma organisms were identified. A diagnosis of Hemophagocytic lymphohistiocytosis was made clinically after “Clinical and Testing Criteria” was evaluated. Despite further management, he developed coagulopathy and sepsis which led to his death. Results (if a Case Study enter NA) At autopsy, we found organomegaly of the liver, spleen, and kidneys. Microscopically, these enlarged organs show old fibrotic granulomas and granulomatous inflammation with suspected fungal organisms. Periodic Acid Schiff (Fungus) and Gomori's Methenamine Silver special stain confirm these fungal organisms to be consistent with Histoplasma species (3-5 micron budding yeasts). Conclusion This case report highlights that physicians should be aware of the diagnostic challenge that Disseminated Histoplasmosis with Hemophagocytic Lymphohistiocytosis could pose in patients with Systemic Lupus Erythematosus, especially those on immunosuppression. The clinical presentation could easily mimic a flare of Systemic Lupus Erythematosus, leading to more aggressive immunosuppression. Failure to recognize the infection promptly could lead to grievous complications and sometimes death.

Bilateral central serous retinal detachment in a patient with nephrotic syndrome: a case report

BackgroundThe aim of this report is to highlight the importance of considering nephrotic syndrome as a potential underlying cause of bilateral central serous retinal detachment in a patient with systemic lupus erythematosus and to underscore the significance of a comprehensive systemic workup in these patients.Case presentationA 19-year-old Iranian female patient with history of systemic lupus erythematosus presented with progressive vision loss and bilateral macular elevation. Ophthalmic examination revealed periorbital edema, chemosis, and subretinal fluid at the macula of both eyes. Optical coherence tomography confirmed the existence of subretinal fluid and serous detachment located at the macula of both eyes. On fluorescein angiography, there were no signs of subretinal leakage such as smoke stack sign or expansile dot in late phases. Laboratory tests detected hypoalbuminemia and significant proteinuria, leading to the diagnosis of nephrotic syndrome. Treatment with prednisolone and albumin infusion resulted in improved visual acuity and resolution of subretinal fluid.ConclusionNephrotic syndrome can be a rare underlying cause of bilateral central serous retinal detachment, and its association with systemic lupus erythematosus should be considered. Hypoalbuminemia in nephrotic syndrome alters fluid dynamics in the retina, contributing to bilateral central serous retinal detachment. Early recognition and management of nephrotic syndrome are essential for vision recovery and preventing long-term complications.

Abstract 18975: First Reported Case of SLE Associated Recurrent Pericarditis Treated With Rilonacept

Description of case: A 42 year old female presented with a 1 month history of exertional dyspnea and intermittent pleuritic chest pain. She had a 12-year history of systemic lupus erythematosus (SLE) and non-specific interstitial pneumonitis being managed with mycophenolate mofetil. Labs showed an ESR of 76 mm/hr and CRP of 5.5 mg/dL. CT chest ruled out pulmonary embolism but showed a moderate pericardial effusion, also seen on an echocardiogram. A cardiac MRI (cMRI) showed moderate diffuse circumferential pericardial enhancement. She was diagnosed with lupus pericarditis and initially treated with ibuprofen, colchicine, prednisone, and hydroxychloroquine; however, continued to have persistent symptoms over the next few months. Repeat cMRI showed a transition to effusive constrictive physiology. She was offered pericardiectomy but following shared decision making and multi-disciplinary discussion, she was started on Rilonacept. She experienced significant and sustained relief in symptoms, improvement in inflammatory markers and reduction of pericardial enhancement on cMRI. Discussion To the best of our knowledge, this is the first case of Rilonacept used to successfully treat recurrent pericarditis from SLE. Recurrence in chronic idiopathic pericarditis is thought to be driven by the innate immune system and Interleukin-1 (IL-1), while in autoimmune diseases, it is mediated by the adaptive immune system and Type 1 Interferon, which is associated with a poorer prognosis. The RHAPSODY and AIRTRIP trials demonstrating efficacy of IL-1 blockers in recurrent pericarditis excluded patients with autoimmune diseases. Use of IL-1 blockers to treat Lupus pericarditis has only been reported in 2 cases using Anakinra. Our patient’s positive response to Rilonacept suggests the presence of additional target pathways in autoimmune diseases that may mediate pericarditis recurrence. Future prospective studies are needed to elucidate this further.

Adrenal Hemorrhage in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report and Literature Review.

Antiphospholipid syndrome (APS) is an autoimmune disorder while adrenal hemorrhage could be its rare complication. Herein, we report the case of a 32-year-old unmarried woman with a history of systemic lupus erythematosus (SLE) who was hospitalized after complaints of upper abdominal pain, limb weakness, and loss of appetite for 2 weeks. Laboratory examination revealed hyponatremia, low plasma cortisol levels, increased adrenocorticotropic hormone levels, and a positive anticardiolipin antibody status. Furthermore, computed tomography (CT) revealed the presence of bilateral adrenal masses. Ultimately, based on dynamic changes in CT images, these masses were diagnosed as adrenal hemorrhage owing to APS. A computer-assisted literature search was conducted to identify cases of primary adrenal insufficiency associated with APS and/or SLE. The clinical features, laboratory examination, treatments, and outcomes of these cases were summarized. Our findings emphasize the importance of screening for adrenal insufficiency in patients with SLE or APS who present with abdominal complaints, asthenia, and hyponatremia. It is also recommended to test for APS all patients with adrenal hemorrhage.

Relapse of lupus nephritis induced by nivolumab in an 84-year-old patient with oral cancer: a case report.

Various immunity-related adverse events have been reported to be associated with the inhibition of programmed cell death receptor 1. We report a rare case of a relapse of lupus nephritis (LN), involving rapidly progressive glomerulonephritis, which was induced by nivolumab treatment in a patient with oral cancer. The patient had a history of systemic lupus erythematosus and underwent treatment with steroids, rituximab, and plasmapheresis. However, her renal function did not improve, and she died of multiple organ failure. To our knowledge, this is the first description of severe LN induced by nivolumab.

Severe hypocalcemia after denosumab treatment leading to refractory ventricular tachycardia and veno-arterial extracorporeal membrane oxygenation support: a case report

BackgroundSevere hypocalcemia may lead to life-threatening arrhythmias. Denosumab is an effective treatment for osteoporosis that allows long intervals between doses. However, there is a risk of hypocalcemia in some patients. Due to the long half-life of denosumab, emergency physicians caring for patients presenting with symptoms of hypocalcemia may not be aware of the medication, and adverse effects may last longer.Case presentationA 55-year-old woman with a history of systemic lupus erythematosus (SLE) and anxiety disorder called for an ambulance for symptoms of hyperventilation and muscle cramps. After evaluation at the local hospital, she developed pulseless ventricular tachycardia and was resuscitated by defibrillation by the hospital staff. After conversion to sinus rhythm, she was transported to a tertiary center. Upon arrival, pulseless ventricular tachycardia occurred again, and veno-arterial extracorporeal membrane oxygenation (ECMO) and intra-aortic balloon pumping (IABP) were implemented. Laboratory results showed severe hypocalcemia (corrected calcium level of 5.3 mg/dL) whereupon intravenous calcium supplementation was started. She had received the first dose of denosumab (60 mg) by subcutaneous injection 24 days prior to hospitalization. She was eventually weaned from ECMO and IABP support.ConclusionCardiac arrest due to hypocalcemia is relatively rare but can be fatal. In the present case, hyperventilation may have acutely exacerbated pre-existing hypocalcemia, leading to ventricular tachycardia. The patient had a slightly decreased serum calcium level prior to denosumab. Close monitoring may be preferable after the primary dose of denosumab in selected patients. Emergency physicians caring for patients who may be suffering from symptoms/signs of hypocalcemia must be mindful of medications that have long half-lives and affect electrolyte balance when treating fatal arrhythmia due to hypocalcemia.

Risk Assessment Model for Postpartum Venous Thromboembolism Prevention in Patients with Systemic Lupus Erythematosus.

This article assesses the application of the Royal College of Obstetricians and Gynaecologists (RCOG) venous thromboembolism (VTE) risk model on a cohort of postpartum patients with a history of systemic lupus erythematosus (SLE). This is a secondary analysis of an ongoing patient registry of women with SLE from 2016 to 2022. There were 49 SLE patients with 55 pregnancies using the Definitions of Remission in SLE (DORIS) criteria to determine SLE disease activity. RCOG risk assessment model scoring was calculated for each patient prior to and after delivery. The primary outcome was the qualification of "active SLE" by standard rheumatologic criteria and assessment of recommendations for VTE prophylaxis based on RCOG VTE risk assessment scoring. Data were analyzed using Fisher's exact test, chi-square test, and Mann-Whitney U test with significance defined as p < 0.05. In the study cohort, 34 pregnancies (61.8%) were in DORIS remission at delivery. Twenty-one pregnancies (38.2%) were not and scored 3 points on the RCOG VTE risk model. Of these pregnancies, only 19% (n = 4) were recommended for VTE prophylaxis by the obstetrical provider despite RCOG score ≥3. Only 35.7% (n = 5) of pregnancies in DORIS remission, but with 3 points for non-SLE-related VTE risk factors (n = 14), were recommended for VTE prophylaxis. Of the 20 pregnancies in remission with an RCOG score < 3 after assessing all risk factors, 15% (n = 3) were nevertheless recommended for VTE prophylaxis. No patients had a postpartum VTE regardless of therapy. These data reveal a need to improve upon providing postpartum VTE prophylaxis to SLE patients not in remission while also recognizing a diagnosis of SLE alone should not equate with active disease. Moreover, SLE patients in remission may still warrant VTE prophylaxis if other non-SLE-related risk factors are present. · Those with SLE are at increased risk for VTE postpartum.. · VTE prophylaxis should be instituted when clinically appropriate.. · Caution should be exercised in broadly assigning disease activity for SLE diagnosis only.. · This study supports VTE prophylaxis use in postpartum patients with SLE..

Re-thinking uterine fibroids in immunocompromised patients: adenomatoid tumors

BackgroundAdenomatoid tumor (AT) is an ambiguous term used to describe benign mesothelial neoplasms, most often reported in organs of the reproductive system. Their neoplastic nature has been proven through the confirmation of pathogenic mutations in TRAF7. Several studies have proven an association between AToU and immunosuppression. Several groups have hypothesized immunosuppression causes an abnormal inflammatory state within mesothelial cells, increasing the likelihood of a selective activating mutations in TRAF7.Case presentationA 34 year old female patient with a history of systemic lupus erythematosus was seen in the Emergency Department due to abdominal pain. A diagnosis of uterine leiomyomatosis was made and she was surgically intervened. Macroscopically, several intramural and subserosal uterine nodules were identified. On microscopic examination, all of them except one corresponded to adenomatoid tumours. A somatic mutation in TRAF7 was identified.ConclusionsAdenomatoid tumors have been associated with immunosuppression. A decrease in immunosurveillance may explain the association between adenomatoid tumours and immunosuppression. Confirming their neoplastic nature is crucial. Further studies are required to characterize the biological significance TRAF7 has in adenomatoid tumours and their association to immunocompromised states.

Risk Assessment Model for Postpartum Venous Thromboembolism Prevention in Patients with Systemic Lupus Erythematosus

Background Patients with Systemic Lupus Erythematosus (SLE) may be at increased risk for developing a venous thromboembolism (VTE), particularly in the postpartum period. The Royal College of Obstetricians and Gynaecologists (RCOG) guideline for postpartum VTE prophylaxis is often used to triage which patients should get VTE prophylaxis. In this RCOG guideline, a score ≥ 3 drives a formal recommendation for postpartum anticoagulation. RCOG is unique in its inclusion of “active SLE” as an actionable VTE risk factor (adding 3 points). We sought to determine if a cohort of postpartum patients with a known history of SLE a) qualify as having “active” SLE by standard rheumatologic criteria b) have other risk factors for VTE c) received the recommended prophylaxis based on RCOG VTE risk assessment scoring and d) had a postpartum VTE. Objective To assess the application of the RCOG venous thromboembolism (VTE) risk model on a cohort of postpartum patients with a history of Systemic Lupus Erythematosus (SLE). Study Design This is a secondary analysis of an ongoing patient registry of women with SLE from 2016-2022. There were 49 SLE patients with 55 pregnancies using Definitions of Remission in SLE (DORIS) criteria to determine SLE disease activity. RCOG risk assessment model scoring was calculated for each patient prior to and after delivery. The primary outcome was the qualification of “active SLE” by standard rheumatologic criteria and assessment of recommendations for VTE prophylaxis based on RCOG VTE risk assessment scoring. Data were analyzed using Fisher’s Exact test, chi-square test, and Mann-Whitney U test with significance defined as p&lt;0.05. Results In the study cohort, 34 pregnancies (61.8%) were in DORIS remission at delivery. Twenty-one pregnancies (38.2%) were not and scored 3 points on the RCOG VTE risk model. Of these pregnancies, only 19% (n=4) were recommended for VTE prophylaxis by the obstetrical provider despite RCOG score ≥ 3. Only 35.7% (n=5) of pregnancies in DORIS remission, but with 3 points for non-SLE related VTE risk factors (n=14), were recommended for VTE prophylaxis. Of the 20 pregnancies in remission with an RCOG score &lt; 3 after assessing all risk factors, 15% (n=3) were nevertheless recommended for VTE prophylaxis. No patients had a postpartum VTE regardless of therapy. Conclusion These data reveal a need to improve upon providing postpartum VTE prophylaxis to SLE patients not in remission while also recognizing a diagnosis of SLE alone should not equate with active disease. Moreover, SLE patients in remission may still warrant VTE prophylaxis if other non-SLE-related risk factors are present.

Abstract #1406181: Complications in the Management of Hypercalcemia in a Patient with Systemic Lupus Erythematous and Lupus Nephritis During Pregnancy

Hypercalcemia can present with a multitude of symptoms including anorexia, nausea and vomiting, constipation and neuropsychiatric disturbances. There is an overlap in these symptoms with those experienced during both pregnancy and in systemic lupus erythematosus. This case report highlights the difficulties in the diagnostic evaluation and management of hypercalcemia during pregnancy. A 26-year-old female with history of Systemic lupus erythematosus (SLE) complicated by Class V lupus nephritis and antiphospholipid syndrome presented August 2022 with symptomatic hypercalcemia at 8-weeks gestation. The patient reported fatigue, nausea and vomiting and new onset severe constipation, polydipsia and polyuria. Lab work was notable for hypercalcemia with calcium 13.2 mg/dl (corrected 13.7 mg/dl for Albumin 3.4g/dl) and parathyroid hormone (PTH) 153pg/ml. No parathyroid nodules were seen on ultrasound and 1st trimester ultrasound demonstrated a single live intra-uterine pregnancy. The patient was diagnosed with primary hyperparathyroidism and started on 0.9% normal saline intravenous fluid at 200cc/hr and Furosemide 20mg/Q6H IV to promote diuresis. Despite this, calcium could not be lowered to less than 12.4 mg/dl (13.0 mg/dl albumin corrected). The patient was discharged with instructions to drink 3-4L water per day and take Furosemide 20mg daily. On follow up at 14 weeks, Calcium was still elevated at 11.9 mg/dl and reported intermittent vomiting and constipation. ENT was consulted to consider parathyroidectomy however opted for conservative management of hypercalcemia prior to delivery due to inability to localize an adenoma and exclude ectopic parathyroid on imaging. The patient was followed closely for the remainder of her pregnancy with improvement in her symptoms, however continued to have persistent hypercalcemia posing risk of materno-fetal complications. Treatment for hypercalcemia is based upon the severity and symptoms, but it is important to consider gestational age. Exceeding the upper normal limit of calcium during pregnancy of 9.5 mg/dL increases the risk of fetal complications including spontaneous abortion, still birth and neonatal tetany. There is added difficulty in determining true symptomatic hypercalcemia given the overlap in symptomatology with pregnancy. In pregnant patients undergoing parathyroid surgery, localization can be done with ultrasound rather than Sestamibi but risks missing ectopic parathyroid glands. If surgery is not recommended, there are limited therapeutic options during pregnancy for hypercalcemia. Notably, IV fluids and diuretics provide modest temporary benefit. Total serum calcium is reported to decline across gestation, likely due to plasma volume expansion, and this may temporize the harmful effects of hypercalcemia to defer surgery until after birth. There is inadequate data on the safe use of Cinacalcet during pregnancy with no known risk of teratogenicity reported in case reports. There is however an increased risk of neonatal hypocalcemia due to gestational suppression of the fetal parathyroid glands.

From (Cat) Scratch: A Unique Presentation of CNS Bartonella Infection (P2-10.003)

<h3>Objective:</h3> NA <h3>Background:</h3> Central Nervous System (CNS) Bartonella infection is rare. Diagnosis is usually performed with serology and confirmed with polymerase chain reaction (PCR). We describe a unique case of disseminated necrotizing brain abscess in an immunocompromised patient caused by Bartonella. <h3>Design/Methods:</h3> A 28-year-old woman with medical history of Systemic Lupus Erythematosus (SLE) on chronic immunosuppression presented with four months of progressive, bilateral, painless vision loss. Two months before presenting to ED, her ophthalmologist increased her prednisone regimen to 80 mg daily due to concern for lupus retinitis. On admission, her ophthalmologic exam was pertinent for decreased visual acuity (20/50 in the right eye and 20/400 in the left eye), chorio-retinal inflammatory lesions and macular edema. MRI of the brain revealed numerous contrast-enhancing lesions throughout the brainstem, cerebral and cerebellar hemispheres. She was started on empiric therapy with ceftriaxone, ganciclovir, voriconazole, pyrimethamine, and sulfadiazine. Infectious etiologies, including HSV, EBV, HIV, mycobacteria, Histoplasma, Blastomyces, Cryptococcus, Toxoplasma were ruled out. CSF showed 1 WBC, 35 Protein, 76 Glucose, flow cytometry and cytology were negative for CNS lymphoma. She was tested for Bartonella henslae and quintana her IgM was negative; IgG was 1:64 (equivocal) and 1:256 (positive), respectively. Due to the lack of clinical improvement, the patient underwent biopsy of the brain lesions with PCR confirming Bartonella infection. She was started on doxycycline and rifampin and discharged home. Brain MRI at 5 weeks post-discharge showed decreased number and size of lesions. <h3>Results:</h3> NA <h3>Conclusions:</h3> We emphasize the consideration of CNS Bartonella infection as an etiology of multiple brain lesions in immunocompromised patients. We encourage evaluation for Bartonella infection if standard infectious workup is negative and there is no improvement with empiric treatment. Prompt diagnosis is important as treatment can improve morbidity and mortality. <b>Disclosure:</b> Dr. Rodriguez has nothing to disclose. Ms. Fedorova has nothing to disclose. Dr. Rukmangadachar has nothing to disclose.

Neuromyelitis Optica Spectrum Disorder (NMOSD) Relapse Triggered by Secondary Syphilis: A Case Report (P10-5.021)

<h3>Objective:</h3> Not applicable <h3>Background:</h3> This report represents a case of post-infections NMOSD relapse due to syphilis. <h3>Design/Methods:</h3> Not applicable <h3>Results:</h3> A 25-year-old woman was admitted in the emergency room with an acute onset of paresthesia in the upper and lower limbs and an electric shock sensation irradiating to both arms during neck flexion (Lhermitte’s sign). She had round hyperchromic papular lesions in the palms and soles, which were painless, non-pruritic and appeared at the same time as the neurological picture. In the neurological examination, she presented sensory ataxic gait, positive Romberg maneuver, dysmetria in the finger-nose test with eyes closed, slight proximal and distal weakness in the left arm, hyperreflexia in all four limbs with bilateral Achilles clonus, apalesthesia with loss of proprioception in the hands, painful anesthesia in the right arm and painful hypoesthesia in the right foot and left leg. She had a previous history of systemic lupus erythematosus since the age of 14, and NMOSD with positive serum anti-AQP4 antibody diagnosed at the age of 23. In the current relapse, serum VDRL was 1:32, CSF was normal, and MRI showed an extensive area of increased signal in cervical cord suggesting longitudinally extensive myelitis. The diagnosis of an acute attack of NMOSD triggered by secondary syphilis (roseola syphilitic) was established. She received one dose of 2.4 million I.U. benzathine penicillin, and methylprednisolone treatment was initiated, with no positive results. Plasmapheresis was then performed, with improvement of the strength, sensibility, and coordination. <h3>Conclusions:</h3> The post-infectious acute attacks of NMOSD are represented in literature through few cases reported. The present case highlights the importance of seeking the specific etiology of an NMOSD relapse, allowing not only its treatment but also the identification and elimination of a possible underlying infectious agent. <b>Disclosure:</b> Dr. Lima has nothing to disclose. Dr. Schafascheck has nothing to disclose. Dr. Moura has nothing to disclose. Dr. Carvalho has nothing to disclose. Dr. Seide Cardoso has nothing to disclose. Dr. Colares Lessa has nothing to disclose. Miss Mageste has nothing to disclose. Dr. Gaudio has nothing to disclose. Dr. Braune has nothing to disclose.

E024 Optic neuritis with neuropsychiatric lupus: a rheumatological emergency

Abstract Background/Aims Optic neuritis (ON) is uncommon rheumatological emergency with prevalence of around 0.6-1% in SLE although neuropsychiatric manifestations are found in 20-40%. The mechanism can be vasculitic or inflammatory or vaso-occlusive, etc. Methods A 24-year-old female with history of systemic lupus erythematosus for four years presented with headache, typical skin rash, alopecia, frothy urine, and pedal oedema from 2-3 months. The patient attendant also complained of mood changes, increased irrelevant talking, and different behaviour. On second day of admission, she complained blurring of vision. Ophthalmological examination revealed blurred disc margins with bilateral papilledema, 6/6 vision in both eyes, and negative Schirmer's test. MRI of brain with orbit showed distension and hyperintensities of both optic nerves on T2W images s/o optic neuritis and small areas of blooming in right parietal lobes, left insula, and thalamus on T2W images and isointense on T1w images and chronic white matter changes. Then, right vision dropped to 6/36 and left to 6/24. 24-hour urinary protein was 2040 mg/day and CT-guided renal biopsy showed coexisting lesions of membranous lupus nephritis: ISN/RPS Class V and focal lupus nephritis: ISN/RPS Class III with a modified NIH disease activity index of 6/24 and chronicity 1/12. ANA (IFA) Reflex to ENA Profile showed nuclear, speckled 3+ ANA with primary titre of 1:100 and end titre of 1:1000, strongly-positive U1RNP, and Sm. Hemoglobin 6.5 gm%, TLC 3700 cells/cumm, Platelets: 1.35 lakhs/cumm, ESR 84 mm/hr, CRP 0.1 mg/L, Serum Ferritin 626 ng/ml. Urine complete, chest-X Ray, liver and thyroid function tests were within normal limits, and viral markers, urine, blood culture, cANCA, and pANCA were negative. Results She was given methylprednisolone pulse therapy for 5 days and on fifth day of pulse therapy, vision was again 6/6 in both eyes with resolving papilledema, normal colour vision and resolving ON on repeat MRI. She was then started on oral steroids and mycophenolate mofetil as induction agent along with supportive care considering presence of concomitant lupus nephritis. Cyclophosphamide shows better results in resistant or worsening ON or severe NPSLE or nephritis. As patient was improving, young, and unmarried mycophenolate was chosen with strict close follow-up. Lin, Y.C. (2009) reviewed 8 patients of SLE-associated-ON reported between 1986 and 2004 and found three had very poor outcomes and only four achieved 20/25 and one 20/80. Even though it is not considered rare, data are scarce and requires more research. Conclusion ON is a rheumatological emergency requiring early aggressive intervention which can be life/organ saving. Suspecting and diagnosing ON in patients with neuropsychiatric lupus is challenging, We need specific guidelines and a national registry for SLE-associated-ON. Mycophenolate is a good option for neuropsychiatric lupus, SLE-associated-ON, and neuromyelitis optic spectrum disorder. Disclosure H. Singh: None. G. Mohan: None. R. Kaur: None. S.H. Jagga: None. K. Kaur: None. C. Bansal: None.

Nonsteroidal Anti-Inflammatory Drug-Induced Jejunal Ulcer Identified by Capsule Endoscopy.

CASE REPORT A 59-year-old woman with a history of systemic lupus erythematosus and arthritis (chronic naproxen use) presented for evaluation of iron deficiency anemia (IDA). Her laboratory test results were consistent with IDA. Upper endoscopy and colonoscopy were nonrevealing. Evaluation with capsule endoscopy revealed a concentric ulceration in the jejunum. Other common causes of IDA including celiac disease, gastrinoma, inflammatory bowel disease, and malignancy were ruled out (Figure 1). This diaphragm-like structure is a rare, but pathognomonic, feature of nonsteroidal anti-inflammatory drug (NSAID)-induced enteropathy (Figure 1).1 Patients with NSAID-induced enteropathy present with IDA or symptoms of small bowel obstruction due to diaphragm lesions of the jejunum.2 Iron supplementation resulted in partial improvement of her IDA. This case report highlights the use of new diagnostic modalities, such as video capsule endoscopy, for diagnosis of small bowel injuries caused by NSAIDs. Video capsule endoscopy may assist in identifying these lesions and may be followed by double-balloon enteroscopy if pathologic diagnosis is required.Figure 1.: Concentric ulceration in the jejunum due to NSAID use.DISCLOSURES S.B. Syed and I. Vohra: substantial contributions to the conception or design of the work or the acquisition, analysis, or interpretation of data for the work. S.B. Syed: drafting the work or revising it critically for important intellectual content. I. Vohra, S. Dhillon, and O.S. Khokhar: final approval of the version to be published. S.B. Syed and I. Vohra: agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. S.B. Syed is the article guarantor. Financial disclosure: None to report. Informed consent was obtained for this case report.

Generalized Tonic-Clonic Seizures Cause Lumbar Spine Fracture

A 28-year-old man presented to the emergency department after a witnessed GTC seizure. The patient had a history of systemic lupus erythematosus, previous subarachnoid hemorrhage, and pulmonary embolism. He was on regular methylprednisolone 16 mg, enoxaparin 60 mg once daily, and levetiracetam (LEV) 500 mg twice daily. The patient had recently stopped taking LEV. There was no trauma in the history. Neurological examination was normal. He had severe lower back pain that failed to subside despite treatment with analgesics and opioids; therefore, this patient had an indication for radiological imaging. Urgent non-contrast computerized tomography (CT) scan of the lumbar spine revealed a burst fracture of L1. Non-contrast lumbar magnetic resonance imaging (MRI) revealed a bursal fracture in the L1 vertebral corpus that caused approximately 50% height loss, and there was compression of the posterior corpus of the vertebra on the thecal sac; however, no stenosis of the spinal canal was detected. At this level, there was bone marrow edema in the vertebral corpus (Figure 1,2). He was transferred to the neurosurgery department. Written informed consent was obtained from the patient for the publication of this case.

Brainstem and upper cervical cord involvement in a patient with posterior reversible encephalopathy syndrome: Neuro-images.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome with acute heterogeneous neurological symptoms. It is usually preceded by hypertension or chemotherapy. Brain magnetic resonance imaging (MRI) shows vasogenic edema over the cortex and subcortex of parieto-occipital lobes. Involvement of the brainstem, basal ganglia, and spinal cord is rare. Here we described an 18 years old woman with a history of systemic lupus erythematosus who was admitted due seizure and impaired consciousness. She had a mean arterial pressure of 160 and previously received cyclophosphamide. She had a unique involvement of diffuse bilateral asymmetric brain edema over the upper cervical cord, brainstem, cerebellum, thalami, basal ganglia, frontotemporal, and parieto-occipital lobes. She was diagnosed with PRES and after the treatment vasogenic edema vanished completely. When it is clinically applicable, physicians should consider PRES as the differential diagnosis of vasogenic edema over the upper cervical cord, cerebellum, and brainstem.