High-dose Intravenous Steroids Research Articles

NEURORETINITIS ASSOCIATED WITH COVID-19 INFECTION AND VARICELLA-ZOSTER VIRUS REACTIVATION.

To report a case of neuroretinitis as the presenting manifestation of COVID-19 infection alongside VZV reactivation, in the absence of other systemic symptoms. A case report. A previously healthy 15-year-old male presented with a one-week history of acute unilateral visual loss and clinical findings consistent with neuroretinitis. Diagnostic work-up revealed a positive nasopharyngeal swab PCR for COVID-19 and serology showed the presence of IgM and IgG antibodies for VZV. Following a comprehensive treatment regimen involving intravenous acyclovir, high-dose intravenous steroids and oral doxycycline, rapid and remarkable improvements were observed. The macular star and optic disc swelling regressed, and visual acuity improved from 20/200 to 20/20. This case offers valuable insights into the neuroretinitis associated with COVID-19 infection and VZV reactivation.

Case report: Encephalitis with Guillain-Barré Syndrome secondary to extensively multi-drug resistant Salmonella infection

Abstract Background This case report presents a highly unusual and challenging medical case involving a 16-month-old previously healthy boy who developed encephalitis followed by Guillain-Barré Syndrome (GBS) as complications of multi-drug resistant Salmonella infection, despite an initial misdiagnosis of gastroenteritis and outpatient treatment with Cefixime. Methods The patient initially experienced an increase in stool frequency, accompanied by low-grade fevers, which was initially attributed to gastroenteritis. Outpatient treatment with oral Cefixime was started, but the child’s symptoms worsened, ultimately leading to high-grade fevers and an increase in vomiting and loose stools. In light of his clinical deterioration, he was admitted to our hospital with signs of mild metabolic acidosis and elevated inflammatory markers. During his hospitalization, the patient’s condition worsened, with drowsiness, irritability, and a generalized tonic-clonic seizure, prompting concern for brain involvement. Consequently, he underwent lumbar puncture, and neuroimaging, and received high-dose intravenous steroids. Further medical investigations including lumbar puncture and brain Magnetic Resonance Imaging (MRI), which revealed normal cerebrospinal fluid (CSF) analysis and non-enhancing white matter changes in the occipital region, respectively. In addition, a collected blood culture confirmed the presence of extensively multi-drug resistant Salmonella typhi and prompted an adjustment of antibiotics to intravenous Meropenem. Due to autonomic dysfunction, the patient was transferred to the Pediatric Intensive Care Unit (PICU) for close monitoring and specialized care. While in the PICU, he developed flaccid limb weakness and axial hypotonia. A repeat lumbar puncture was normal, but nerve conduction studies showed features of axonal polyneuropathy diagnosis, consistent with the diagnosis of GBS. To treat the encephalopathy and polyneuropathy, high-dose dexamethasone and Intravenous Immunoglobulin (IVIG) treatments were initiated, without necessitating respiratory or inotropic support. After receiving these treatments, the patient’s autonomic instability and motor strength improved, prompting his transfer back to the general pediatrics wards. He was treated with a 21-day course of IV Meropenem. Upon discharge, his motor power had returned to normal, and he displayed progress in movement transitions, including the ability to stand and walk independently, albeit with mild gait abnormalities. Results The co-occurrence of encephalitis and Guillain-Barré Syndrome due to Typhoid fever is exceedingly rare. The patient’s origin, with recent travel to Pakistan where extensively drug-resistant Salmonella typhi prevails, necessitates heightened awareness among healthcare providers. Initial misdiagnosis and inadequate antibiotics treatment may have contributed to encephalitis and polyneuropathy. High-dose dexamethasone and IVIG were pivotal in the patient’s treatment. While Typhoid fever-related neurological complications are infrequent, high-dose dexamethasone offers promise in reducing mortality and mitigating typhoid-induced encephalopathy. Importantly, IVIG is not conventionally employed in Salmonella encephalopalitis but was used due to polyneuropathy and its role in GBS management. Conclusion This exceptional pediatric case serves as a striking example of the possibility simultaneous encephalitis and Guillain-Barré Syndrome as rare complications of Typhoid fever. This case report underscores the importance of early diagnosis, prompt management with appropriate antibiotics and caution of possible neurological complications. Furthermore, it calls for further research into the role of high-dose glucocorticoids and IVIG in the management of extensively multi-drug resistant Salmonella infection and its neurological complications.

"White Cord Syndrome" as clinical manifestation of the spinal cord reperfusion syndrome: a systematic review of risk factors, treatments, and outcome.

Paralysis subsequent to spinal cord decompression in the cervical or thoracic region is infrequent, with White Cord Syndrome (WCS) being among its several causes. Due to WCS's infrequency, there exists a paucity of high-level evidence concerning its manifestations. Our primary objective is to systematically collate all documented WCS cases, discern prevalent risk and prognostic factors, appraise available treatment modalities, and evaluate patient outcomes. A systematic review was conducted following PRISMA guidelines. The search included PubMed, Scopus, Embase, and Web of Science databases. Inclusion criteria required studies to be written in English, be case reports, and contain data on clinical features, management, and treatment outcomes. Exclusion criteria excluded meta-analyses, reviews, editorials, letters, books, studies with insufficient clinical data, and studies not in English or with unavailable full texts. Grey literature was not actively pursued due to identification challenges, potentially introducing selection bias. Two authors independently evaluated papers based on criteria. Disagreements were resolved with a third author. Additionally, the included articles' references were screened for additional relevant articles. We found a total of 580 articles through our electronic search. After removing duplicates, 399 articles were screened. Out of the remaining 51 studies, 27 were included in the final quantitative analysis. The average age was 54 (3-79 years) with a male-to-female ratio of 2:1, 33% had OPLL, and Common medical histories were hypertension (30%), diabetes mellitus (20%), and previous ACDF surgery (8%). Of all Surgeries, 70% were done with a posterior approach and 30% with the anterior approach. 48% of cases used Intraoperative NeuroMonitoring(IONM), and Loss of Motor Evoked Potentials (MEP) occurred in 37% of cases. Patients received high-dose intravenous steroids. In 26% of cases, additional posterior cervical decompression was performed, and efforts were made to maintain mean arterial pressure above 85 mmHg in 37% of cases. Other medications were administered in 30% of cases. Over an average 26-week follow-up, 37% of patients had good recovery, 40% had partial recovery, and 23% showed no recovery. The average final Nurick grade was 3.2. WCS is a rare cause of postoperative neurological deficit following spinal cord decompression surgery. Risk factors for WCS include advanced age, extensive surgery, posterior approach for decompression, and the presence of OPLL. Treatment includes high-dose steroids, posterior cervical decompression, maintaining MAP over 85mmHg, rehabilitation, and sometimes neurotrophic drugs. Most patients can walk with or without assistance during follow-up, but around a quarter never regain neurological function. The only preoperative factor impacting outcomes is the preoperative neurological status (Nurick Grade).

Relapsing tumefactive demyelination lesions: A unique, distinct inflammatory brain pathology.

We report the case of a patient suffering from biopsy-proven relapsing tumefactive demyelinating lesions (TDLs) of the central nervous system who had five relapses in 16 years. No signs/symptoms suggestive of alternative pathologies emerged during the follow-up. A limited benefit was observed with intravenous (IV) high-dose steroids, while both plasma exchange and IV immunoglobulin G (IgG) administration were ineffective. A long-lasting (9 years) but transient clinical stabilization was obtained with cyclophosphamide. Our case supports the view that recurrent TDL is a relapsing brain inflammation not belonging to multiple sclerosis (MS) or myelin oligodendrocyte glycoprotein (MOG)-/AQP4-associated disorders. TDL concept and clinical features should be revised.

First reported case of Acute Disseminated Encephalomyelitis in pregnancy in Sri Lanka

Acute Disseminated Encephalomyelitis (ADEM) complicating pregnancy is a rare occurrence, posing challenges to the management due to maternal and fetal risks including fetal loss, fetal anomalies, intrauterine fetal growth restriction (FGR) and preterm labour. We report a case of a 21-year-old primiparous woman who was diagnosed with ADEM during the second trimester. She was treated with high dose intravenous (IV) steroids as well as IV immunoglobulin. She made complete neurological recovery within two months, but there were fetal complications including oligohydramnios, intrauterine growth restriction and low birth weight. The baby was delivered via elective caesarean section due to suspicious cardiotocograph and abnormal fetal doppler studies indicative of compromised fetal circulation. The baby was given special baby care unit (SBCU) care. However, both the mother and baby were safely discharged following six days of special neonatal care. This is the first reported case of ADEM in pregnancy in Sri Lanka, and highlights the challenges in management.

Unilateral Dacryoadenitis as the First Symptom of Systemic Lupus Erythematous.

This case report focuses on a patient with systemic lupus erythematous whose first symptom was dacryoadenitis. A 42-year-old Asian woman presented with a 3-day history of left periorbital swelling and pain. Gross examination revealed left periorbital swelling with no definite tenderness. Initial computed tomography with contrast showed diffuse enhancement and enlargement of the left lacrimal gland with adjacent fluid collection, indicating periorbital soft tissue swelling. She was diagnosed with left dacryoadenitis and treated with intravenous methylprednisolone (60mg/day) and oral prednisolone (10mg/day). Over the next 6 months, she experienced numerous instances of recurrence, which was shortly responsive to oral and intravenous high-dose steroids. Immunosuppressive therapy was selected, and upon conducting laboratory tests, she was diagnosed with systemic lupus erythematous. Since then, the patient has undergone treatment involving hydroxychloroquine (200mg/day) and azathioprine (50mg/day) with no recurrence.

Salvaging Vision: A Study of Non-Traumatic Optic Neuropathies

Background and Objectives: Various ear, nose, and throat (ENT) conditions can result in vision loss. The purpose of this study is to identify the etiologies, presentations, and radiological findings associated with impaired vision in the context of ENT. Additionally, this article discusses management protocols, including optic nerve decompression and orbital decompression.Methods: In a retrospective study, we examined the period from 2016 to 2022 at a tertiary care hospital in Mumbai, India. The analysis included 11 patients who presented with progressive diminution of vision. All patients received a regimen of broad-spectrum intravenous antibiotics and high-dose intravenous steroids. This was followed by either endoscopic optic nerve decompression or orbital decompression. Subsequent improvements in vision were documented, and any complications were evaluated.Results: A total of 11 patients were treated with medical management followed by successful surgery, with 10 patients demonstrating significant vision improvement.Conclusion: Identifying the etiology of vision loss and managing the condition can present challenges for otorhinolaryngologists. A thorough grasp of the underlying pathophysiology, combined with active surveillance of clinical and radiological indicators, can enable these clinicians to achieve effective and rewarding outcomes.

A Case of Thrombocytopenia with <italic>MYH9</italic> Gene Mutation Found in Siblings

Myosin heavy chain 9 (MYH9) related diseases are autosomal dominant diseases characterized by macrothrombocytopenia and inclusion bodies in leukocyte, which can be accompanied by extra-hematologic symptoms such as sensorineural hearing loss, renal dysfunction, and cataract. They are often diagnosed incidentally in adulthood or misdiagnosed as idiopathic thrombocytopenic purpura (ITP), leading to unnecessary treatment with intravenous high-dose gamma-globulin, steroids, or splenectomy. Here, we report the case of a brother and sister confirmed to have a MYH9 gene mutation during follow-up. An 8-year-old boy was confirmed to have thrombocytopenia at birth and treated with intravenous gamma-globulin under suspicion of ITP or sepsis. He was discharged after showing an increase in platelet count. Subsequently, during outpatient workup, he exhibited thrombocytopenia, large platelets, and neutrophilic inclusion bodies. His 10-year-old sister also presented with the same findings. In 2021, DNA analysis revealed that they share a mutation (c.4270G> A, p. Asp1424Asn), a pathogenic variant associated with MYH9-related disorder.

Clinical Changes and Autoantibody Level Changes in Thyroid Eye Disease Patients after Intravenous High-dose Steroid therapy

Purpose: To evaluate the effects of intravenous steroid therapy on the clinical activity score (CAS), exophthalmos, margin-reflex distance 1 (MRD1), and autoantibody levels in patients with thyroid eye disease.Methods: In this retrospective observational study, we enrolled 83 patients diagnosed with thyroid eye disease treated with intravenous steroid therapy. We assessed exophthalmos, MRD1, CAS, thyroid-stimulating antibody (TSAb), and thyroid-stimulating hormone receptor antibody (TSHR Ab) levels at baseline, 6 and 12 weeks after initiating therapy, and 2 months after therapy termination. Additionally, baseline serum selenium levels were examined to determine their impact on treatment efficacy.Results: TSAb, TSHRAb, and CAS levels showed a continuous decline from 443.22 to 341.03 specimen-to-reference control ratio (SRR%), 22.18 to 7.91 IU/L, and 3.37 to 1.88, respectively (<i>p</i> < 0.0001), up to 2 months following therapy termination. Exophthalmos did not show significant changes at any assessed time point up to 2 months after therapy termination. MRD1 decreased until 12 weeks after therapy initiation but showed no significant change 2 months later. No correlation was found between baseline serum selenium levels and changes in exophthalmos, MRD1, TSAb, TSHR Ab, or CAS.Conclusions: Intravenous steroid therapy was effective in reducing TSAb, TSHR Ab, and CAS levels up to 2 months after therapy termination. However, it had limited positive impact on exophthalmos or eyelid retraction. Baseline selenium levels did not correlate with the changes in any of the examined parameters.

#2600 Idiopathic full-house non-lupus nephropathy

Abstract Background and Aims A full-house pattern at immunofluorescence (IF) in kidney biopsies is usually associated with Lupus Nephritis (LN). However, it can be also secondary to other causes, such as infections, or it can idiopathic, without a defined origin. Idiopathic Full House non-lupus Nephropathy (iFH-N) represents a rare entity previously identified in small cohorts of patients, with different clinical features and outcomes. A limited number of the described cases developed overt systemic lupus erythematosus (SLE) during follow up, while renal outcome varied across different studies. A retrospective observational study aimed at evaluating clinical presentation and outcomes of patients with iFH-N in a Tertiary Center has been performed. Long-term renal function and development of overt SLE have been considered as main outcomes of the study. Method All native kidney biopsies performed from January 2012 to December 2022 in our Institution were considered. Full-house pattern was defined as simultaneous positivity for IgG, IgM, IgA, C3 and C1q staining at IF. Inclusion criteria were: age ≥18, full-house pattern at kidney biopsy, patients not meeting criteria for SLE diagnosis according to American College of Rheumatology (ACR) 1997 classification or Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria. Exclusion criteria: diagnosis of SLE, secondary causes of full-house pattern at kidney biopsy. We considered histological, clinical and laboratory features, performed therapies, renal function and SLE development during follow up. Results In the considered period, 2110 patients underwent a native kidney biopsy at our Institution. Of these, 91 patients had a histological finding of full-house nephropathy and were initially included for further evaluation. Of the 91 patients, 84 had received a diagnosis of SLE and were excluded, while 7 did not meet the criteria for a diagnosis of SLE. Two of these 7 were further excluded, having a full house pattern secondary to other conditions. Five patients were finally labelled as iFH-N. At kidney biopsy, all 5 included subjects had a Membranous Nephropathy, with extracapillary proliferation in 2 cases. They were all females, 4 Caucasians (80%), 1 Black (20%). At presentation, mean age was 28.2 years (range 21–40). All had impairment of kidney function, 2 of 5 had a full-blown nephrotic syndrome, 3 of 5 a nephrotic range proteinuria. Mean serum creatinine was 2.1 mg/dl (SD± 0.47), mean eGFR 35.2 ml/min/1.73 m2 (SD ±11), mean proteinuria 7.1 gr/24 h (SD ±3.3). Antinuclear antibodies (ANA) were negative in 4 of 5 patients; anti-dsDNA, anti-extractable nuclear antigens (ENAs) and antiphospholipid antibodies were negative in all subjects; slightly low C3 was present in 3 of 5 subjects. All patients received immunosuppressive therapy, including high dose intravenous (iv) steroids in association with cyclophosphamide (CYC) or mycophenolate mofetil, or Intensified B Cell Depletion Protocol (i.e. iv steroids + CYC and Rituximab). Mean follow-up from the time of kidney biopsy was 7.4 year (SD ± 2.4) (range 3–10 years). During follow up, 4 patients (80%) developed end stage renal disease (ESRD) needing renal replacement therapy, 3 of them within 24 months after presentation. The two patients with crescents had a severe prognosis, starting dialysis within 10 months after presentation. The fifth patient developed chronic kidney disease stage 4. One patient formally developed an ACR negative, SLICC positive SLE two years after presentation, with new positivity for ANA and anti-dsDNA antibodies at high titer and low C3, in addition to renal involvement. Conclusion All patients with iFH-N had similar presentation, being all young females, with a Membranous Nephropathy, nephrotic proteinuria and impairment of kidney function. They all had an aggressive form of kidney disease, refractory to different regimens of immunosuppression and with poor long-term renal outcome. Only one case developed SLE on the long term, although without systemic signs. The relationship between idiopathic full-house nephropathy and SLE remains controversial.

A Case of Asymmetrical Vogt-Koyanagi-Harada Disease in a Patient with Ota Nevus Following Laser Treatment

Purpose: To report a case of Vogt-Koyanagi-Harada (VKH) disease exhibiting asymmetric symptoms, predominantly in the affected eye, aside from the bilateral findings noted in indocyanine green angiography (ICG) in a patient who had undergone treatment for nevus of Ota.Case summary: A 33-year-old male presented with decreased visual acuity, ocular pain in the right eye, and a 3-week history of headache. The patient had undergone two laser treatments at 1-month intervals to address a nevus of Ota on the upper and lower eyelids and the maxillary area of the right side. He had no notable medical history. Fundus examination and optical coherence tomography indicated multiple serous retinal detachments. ICG displayed multiple hyperfluorescent spots in both eyes. Given these findings, the patient was diagnosed with VKH disease, predominantly affecting the right eye. Human leukocyte antigen (HLA) testing revealed the presence of the HLA-DRB1*09 and HLA-DQB1*03 (DQ8) alleles. The patient initially received high-dose intravenous steroid therapy, later transitioning to oral steroids. After a month of treatment, the serous retinal detachments had resolved and his vision improved. No signs of recurrence were evident during a 6-month follow-up.Conclusions: VKH disease can manifest asymmetrically, especially in the eye affected after laser treatment for nevus of Ota. Despite potential monocular presentation, it is crucial to assess for binocular involvement through comprehensive examinations.

Superior orbital fissure syndrome after deep lateral orbital wall decompression in Graves’ ophthalmopathy

ABSTRACT Purpose The superior orbital fissure contains cranial nerves III, IV, VI, and V1 with their three branches: frontal, lacrimal, and nasociliary. Superior orbital fissure syndrome (SOFS) is rare and can occur as a result of compression of these nerves due to trauma, bleeding, or inflammation in the retrobulbar space, but no cases of SOFS after deep lateral orbital wall decompression (DLOWD) have been reported. The aim of this paper is to describe this pathology, its possible causes, management, and outcome. Methods Retrospective study of 575 DLOWD in patients with disfiguring exophthalmos due to Graves’ ophthalmopathy performed in our hospital between 2010 and 2023. Three cases of postoperative SOFS were identified based on clinical presentation, history, physical examination, and radiological study. All patients were observed for a minimum of 12 months. Results SOFS was diagnosed with the presence of ophthalmoplegia, ptosis, fixed and dilated pupils, hypo/anesthesia of the upper eyelid and forehead, loss of corneal reflex, and no loss of vision after DLOWD. Fractures, edema, and hemorrhages were excluded. They were treated with high-dose intravenous steroids and the patients recovered completely. Conclusions DLOWD challenges orbital surgeons because it requires removing bones near the globe or neurovascular structures. SOFS may occur due to the proximity and increased pressure on these structures.

Searching The New Culprit of Optic Neuritis: The Role of Vitamin D

Abstract
 Introduction : Low vitamin D status has long been associated in multiple sclerosis patient with higher risk of disease progression and frequent relapses. Vitamin D plays an important role in modulating the immune process responsible for demyelination. To this date, the study on vitamin D deficiency in optic neuritis case is still rare.
 Case Illustration : Female, 20 years old, presenting with bilateral sudden visual loss since 1 week prior. The patient looked relatively pale and complained of accompanying headache and myalgia. Visual acuity in both eyes were light perception with dilated pupils and posterior segment showing optic disc swelling. CBC, infection and autoimmune screening came within normal limit and brain MRI further confirmed the presence of optic neuritis. The patient was given high dose intravenous steroid but showed no improvement. Later, she was screened for nutrient deficiency and found sign of low serum 25-hydroxyvitamin D. After one week of oral supplementation and neuroprotective agents, her vision drastically improved to 6/6 in both eyes.
 Discussion : Vitamin D deficiency nowadays is getting more prevalent in high risk individuals with low dietary intake and sunlight exposure. Recent study found that mean serum vitamin D level were significantly lower in autoimmune optic neuritis (ON) and were associated with ON attack severity. Vitamin D sufficiency is associated with better inflammatory outcome and long term neurodegenerative measures in demyelinating diseases, as shown by improvement in RNFL thickness after attack.
 Conclusion : Screening for vitamin D deficiency is essential to consider in managing patient with atypical optic neuritis.

A Case of Retinal Edema in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis

Purpose: To report a case of retinal edema and retinal hemorrhage in myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) positive optic neuritis.Case summary: A 49-year-old male visited the clinic due to decreased vision and eye pain in the left eye after 2 times of right eye and 1 time of left eye optic neuritis. The patient presented with eyelid swelling and retinal hemorrhage as well as optic disc swelling of the left eye. Optical coherence tomography (OCT) showed parafoveal intraretinal fluid. The patient received the intravenous high-dose steroids and the immune globulin therapy. He was then put on maintenance oral steroids and immunosuppressive agents. After the treatment, the best corrected visual acuity of left eye was improved to 0.2. Retinal hemorrhage and retinal edema of the left eye decreased, and optic nerve pale in both eyes was observed. OCT showed overall atrophy of the retinal nerve fiber layer in both eyes.Conclusions: Some patient with MOG-IgG positive optic neuritis will have eyelid swelling or retinal edema that is necessary to be differentiated from eyelid inflammatory disorder or macular disease such as diabetic retinopathy. Thus, clinician should consider MOG-IgG positive optic neuritis in patients with retinal edema when accompanied by eye pain with severe vision loss or repeated disc swelling. When diagnosed with MOG-IgG positive optic neuritis, a combined treatment of relatively long-term steroid treatment and immunosuppressants may be required.

A Rare Case of Overlapping Durvalumab-induced Myositis, Takotsubo-like Morphological Changes Caused by Myocarditis, and Myasthenia Gravis.

Immune checkpoint inhibitors can cause a range of immune-related adverse events, including myositis, Takotsubo cardiomyopathy, and myasthenia gravis. We herein report a rare case of a 78-year-old man with concurrent durvalumab-induced myositis, Takotsubo-like morphological changes caused by myocarditis, and myasthenia gravis. The patient initially required invasive ventilation and exhibited symptoms of myasthenia gravis after treatment with high-dose steroids. However, he subsequently achieved successful recovery after the administration of intravenous immunoglobulin, plasmapheresis, and high-dose steroids. We advocate vigilant neurological monitoring of patients with immune checkpoint inhibitor-induced myositis, including the assessment of ptosis and other relevant signs, so that prompt treatment can be initiated at the time of emergence or progression of immune checkpoint inhibitor-induced myasthenia gravis.

Cytokine release syndrome after bronchoalveolar lavage

BackgroundImmunosuppressed bone marrow transplant patients with pulmonary infiltrates routinely undergo bronchoscopy with bronchoalveolar lavage (BAL) to investigate potential etiologies. Cytokine release syndrome after BAL is unreported in the literature in general and in this patient population.Case presentationWe report on an allogeneic bone marrow transplant patient with non-infectious organizing pneumonia of the lungs who developed delayed and rapidly progressive shock and hypoxia post-procedure over the course of 12 h resulting in intensive care unit admission for supportive care. BAL was characterized by a marked lymphocytic, cytotoxic T cell infiltrate on pathology and flow cytometry without clear evidence of infection. The patient’s clinical status improved quickly only after the initiation of high dose intravenous steroids and returned to baseline as an outpatient.ConclusionThe patient’s clinical data and course suggest a cytotoxic T cell response from the lung and BAL as the etiology. With an increasing number of cellular therapies for cancer entering the clinic, the potential for unusual but morbid complications from routine bronchoscopy should be considered.

Chronic inflammatory demyelinating polyradiculoneuropathy in a patient with systemic lupus erythematosus without systemic activity.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon subtype of peripheral neuropathy, especially in systemic lupus erythematosus (SLE). We report a case of SLE presenting with CIDP successfully treated. The patient presented with bilateral, progressive, ascending, sensory, and motor neuropathy. Electrodiagnostic tests reported active motor and sensitive demyelinating polyneuropathy, and the diagnosis of CIDP was confirmed according to the European Federation of Neurological Societies/Peripheral Nerve Society criteria. Initial management with intravenous immunoglobulin and high-dose steroids was administered, then 6-month intravenous cyclophosphamide was initiated with improvement according to clinical scales. In conclusion, CIDP in SLE is rare, reported in just 0.2%. Immunosuppressive therapy should be considered whether initial improvement is not evidenced, as seen in our case requiring cyclophosphamide; interestingly, systemic activity was in remission as the peripheral nervous system is not part of neurological compromise, and we suggest evaluating this unusual presentation into rheumatological practice.

Widespread retinal hemorrhages in a patient with atypical optic neuritis

Retinal hemorrhages are rare in optic neuritis. We report a case of atypical optic neuritis with widespread retinal hemorrhages. A 30-year-old Indian woman presented with sudden painful and severe visual loss in the left eye for 3 days. Fundus examination showed minimal optic disk edema associated with intraretinal hemorrhages extending to the periphery in the left eye. On neuroimaging, long segment thickening of the intraorbital segment of the left optic nerve was appreciated with T2 hyperintensity. Serum myelin oligodendrocyte (MOG) antibodies and neuromyelitis optica (NMO) antibodies were negative. Visual acuity improved rapidly after the administration of intravenous high-dose steroids, and fundus abnormalities resolved rapidly. Retinal hemorrhages can also be present in atypical optic neuritis in the absence of severe optic disk edema. Clinicians should be mindful of the variable presentations of optic neuritis to initiate proper and timely treatment.

A209 ASSESSMENT OF OUTCOMES OF PATIENTS ADMITTED WITH ACUTE, SEVERE ULCERATIVE COLITIS ON ESTABLISHED BIOLOGIC THERAPY: A SINGLE CENTRE RETROSPECTIVE ANALYSIS

Abstract Background Acute, severe, ulcerative colitis (ASUC) is associated with a high morbidity and mortality. Current guidelines recommend the initiation of high dose intravenous steroids, followed by anti-TNF therapy if a satisfactory therapeutic response is not rapidly achieved. However, guidelines are agnostic on how to manage patients who are admitted for ASUC despite being on established biologic therapy. Furthermore, short-term clinical outcomes in this population are not well characterized. Purpose The aim of this study is to assess the differences in short-term clinical outcomes in patients admitted with ASUC who are on established biologic therapy compared to those not on established biologic therapy. Method We conducted a retrospective chart review of patients admitted with ASUC to Mount Sinai Hospital (MSH) in Toronto, Ontario from January 2018 until December 2021. Patients were included if they were deemed to have a severe flare, defined as having 6 or more loose bowel movements per day with at least one of the following features: temperature of 38.0 Celsius, tachycardia, anemia, or elevated inflammatory markers. Included subjects were considered to be on established biologic therapy if they had a biologic within 56 days prior to admission, all other admitted subjects were included as controls. Our primary outcome was the difference in hospital length of stay (HLOS). We also contrasted duration of intravenous steroids, rates of surgical consultation, rates of in-hospital colectomy, and readmission rates within 90 days of discharge. Result(s) 130 charts were included in our study, 53 of which were patients on established biologic therapy, and 77 of which were patients not on established biologic therapy. The HLOS between the two groups was not significantly different, (7.23 days [established biologic therapy] vs.7.47 days [not on biologic therapy], p value = 0.77). Patients on established biologic therapy were more likely to receive an inpatient surgical consultation (33.96% vs 7.79%, p-value <0.001). However, rates of colectomy prior to discharge were not statistically different (1.89% vs 0%, p-value = 0.23). Patients on established biologic therapy were significantly more likely to be readmitted within 90 days of discharge (30.19% vs 12.99%, p-value = 0.016). Image Conclusion(s) Although there were no differences in HLOS and colectomy rates between the 2 groups, patients with ASUC on established biologic therapy were more likely to be readmitted within 3 months of discharge. Further work is required to define optimal medical management of persons admitted with ASUC who are failing biologic therapies. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

High-Dose Intravenous Steroid Treatment Seems to Have No Long-Term Negative Effect on Bone Mineral Density of Young and Newly Diagnosed Multiple Sclerosis Patients: A Pilot Study.

High-dose intravenous steroid treatment (HDIST) represents the first choice of treatment for multiple sclerosis (MS) relapses. Chronic oral glucocorticoid (GC) administration correlates with bone loss whereas data regarding HDIST in MS are still conflicting. Twenty-five newly diagnosed MS patients (NDMSP) (median age: 37 years) were prospectively studied for the effects of HDIST on bone mineral density (BMD) and bone metabolism. Patients received 1000 mg methylprednisolone intravenously every day for 5 days followed by oral prednisolone tapering over 21 days. Bone metabolism indices were determined prior to GC, on days 2, 4, 6, and 90, and at months 6, 12, 18, and 24 post GC therapy. Femoral, lumbar-spine BMD, and whole-body measurement of adipose/lean tissue were assessed prior to GC-administration and then every six months. Ten patients completed the study. N-terminal-propeptide-procollagen-type-1 and bone-specific alkaline phosphatase showed a significant increase at day-90 (p < 0.05). A transient non-significant fall of BMD was observed at 6 months after GC-administration, which subsequently appeared to be restored. We conclude that HDIST seems not to have long-term negative effects on BMD, while the observed transient increase of bone formation markers probably indicates a high bone turnover phase to GC-administration. Additional prospective studies with larger sample size are needed.