Methylprednisolone Pulse Therapy Research Articles

The benefit of intravenous immune globulin in the treatment of delayed radiation myelopathy.

Delayed radiation myelopathy (DRM) is arare yet severe complication of radiotherapy. This condition has aprogressive pattern that is often irreversible. Several therapeutic strategies have been introduced to alleviate disease complications, including corticosteroids, hyperbaric oxygen, anticoagulants, and antivascular endothelial growth factor (VEGF) agents. However, despite their beneficial effect, they have not been the definitive treatments for DRM. Here we present the case of a55-year-old woman with ahistory of multiple myeloma who developed neurological complications 11months after radiation therapy. As her radiologic findings demonstrated transverse myelitis, based on the DRM diagnostic criteria, the diagnosis of delayed radiation myelitis was reached. Therefore, methylprednisolone pulse therapy was initiated, resulting in the complete resolution of her neurological symptoms. However, on her follow-up examination, although she did not have new neurological complications, magnetic resonance imaging (MRI) demonstrated aresidual enhancement in the thoracic spinal cord area. Hence, due to the possibility of myelitis progression and spinal cord atrophy, intravenous immune globulin (IVIG) was administered, resulting in the resolution of lesion enhancement. Considering this outcome and the immunomodulatory properties of IVIG, it could be regarded as apotential therapeutic option in the case of DRM activity.

Lymphopenia is a risk factor for severe infections in older patients with microscopic polyangiitis: a retrospective cohort study in Japan.

Previous studies have identified the predictors of severe infections in ANCA-associated vasculitis. However, lymphopenia has not been fully evaluated as a predictor of subsequent severe infections in patients with microscopic polyangiitis (MPA). The aim of this study was to assess the association between lymphopenia and severe infections requiring hospitalization after receiving immunosuppressive therapy for MPA. This single-centre retrospective cohort study included 130 consecutive patients with newly diagnosed MPA from Aichi Medical University Hospital, Japan, who received immunosuppressive therapy between March 2004 and December 2020. The relationship between lymphopenia and subsequent severe infections was assessed using time-dependent multivariate Cox proportional hazard models adjusted for clinically relevant factors. During the follow-up period (median: 38 months; interquartile range: 15-63 months), 56 severe infectious episodes occurred in 51 patients (39.2%). Time-dependent multivariate Cox proportional hazard analyses identified older age [adjusted hazard ratio (HR) = 1.74 per 10 years, 95% CI: 1.13, 2.67], methylprednisolone pulse therapy (adjusted HR = 2.04, 95% CI: 1.03, 4.02), moderate lymphopenia (vs normal, adjusted HR = 7.17, 95% CI: 3.10, 16.6) and severe lymphopenia (vs normal, adjusted HR = 36.1, 95% CI: 11.8, 110.9) as significant predictors of severe infection. Lymphopenia is a predictor of subsequent severe infections in patients with MPA who receive immunosuppressive therapy. These results suggest the importance of sustained infection surveillance, particularly in older patients who develop lymphopenia during strong immunosuppressive therapy.

E32 Intra cranial hypertension in juvenile neurolupus

Abstract Introduction Neurolupus is one of the two key prognostic parameters of lupus. Its manifestation can be central or peripheral, revealing the disease or occurring during the course of evolution. It’s important to not consider all neurological manifestations during SLE as related to lupus, ruling out Central nervous system (CNS) infection and anti-phospholipid syndrome (APS) is primordial. Objectives To share 3 rare observations of neurolupus with intracranial hypertension syndrome and discuss their therapeutic modalities. To highlight the lack of evidence in the treatment of such cases. Method A retrospective analysis of data from medical records. Results We report three observations of Intracranial hypertension syndrome involving three girls, ranging from 9 to 14 years. Clinical symptomatology was dominated by headache, vomiting and visual disturbances. Examination found papillary oedema stage 3 in all the three patients, convergent strabismus in two patients and elevated intracranial pressure measurement in all the three girls ranged from 95 mm to 57 mm of H2O. Brain MRI performed in all three-patients was normal. Data from laboratory tests showed, thrombocytopenia in one patient, pancytopenia in one patient, positive antinuclear antibodies in all patients, positive anti-DNA antibody in one patient, C3, C4 consumption in two patients, positive proteinuria in two patients and positive anti phospholipid antibodies in one patient. All patients had active SLE at the time of admission with a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) at least at 17. Symptomatic treatment with carbonic anhydrase inhibitor was initiated, followed by methylprednisolone pulse therapy then oral corticosteroids and hydroxychloroquine in all the three patients. Immunosuppressive therapy with Rituximab 1 g at two-week intervals was administrated in two patients, the third one did not receive any suppressive treatment in addition to corticosteroids due to the favorable outcome. The course was favorable in all patients with regression of clinical symptoms of intracranial hypertension and papillary oedema immediately after cerebro spinal fluid (CSF) subtraction. Discussion and Conclusion Neurolupus presents with heterogeneous symptoms that make its diagnosis difficult. Intracranial hypertension syndrome is a rare manifestation resulting from a disorder of CSF resorption, its pathogenesis is poorly understood with an inflammatory hypothesis based on immune deposits. There are no therapeutic recommendations to date. This isolated syndrome without other neurological signs of severity responds very well to symptomatic treatment with carbonic anhydrase inhibitors and to CSF withdrawal, as was the case in our patients. The use of immunosuppressive therapy (rituximab in our patients) was indicated due to the severity of the clinical picture with severe renal involvement. In addition, other immunosuppressive drugs can be an alternative option therapy. Ethics The study was performed according to the principles of the Declaration of Helsinki.

Neuromyelitis Optica Spectrum Disorder (Device’s Disease): - A Case Report Of Full Recovery With Corticosteroids, Therapeutic Plasmapheresis And Immunotherapy

Neuromyelitis optica is a severely disabling inflammatory disorder of autoimmune etiology of the central nervous system, mainly affecting the optic nerves and spinal cord. Here, we present a case report of neuromyelitis optica in a 28-year-old Indian woman who presented with sudden painless loss of vision with tingling followed by weakness of all four limbs with bowel involvement that developed over a period of one week, with a significant history of recent vaccination with COVISHIELD (ChAdOx1 nCoV- 19) vaccine . Clinical evaluation showed diminished vision in both eyes, which progressed to loss of power across all major muscle groups in all limbs and bowel involvement. Magnetic resonance imaging of brain with contrast was done which showed T2W/FLAIR hyperintense non enhancing signal in bilateral thalami extending caudally in mid brain on left side and in midline anterior medulla with subtle T2 hyperintense signal in body of corpus callosum, similar foci are seen in bilateral frontoparietal white matter suggestive of ADEM or demyelination. Also T2 hyperintense signal in both optic nerves [left> right] in posterior part of orbit suggestive of optic neuritis. MRI whole spine showed multiple patchy hyperintense signals on T2 and hypointensity on T1 all over cervical and dorsal vertebrae through whole segment in contiguous manner. The patient was immediately started on intravenous methylprednisolone pulse therapy followed by plasmapheresis and immunomodulator therapy. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. Since, treatment was started early patient responded very well and made full recovery. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.

A CASE OF RELAPSING MULTIPLE SCLEROSIS MANAGEMENT WITH DISEASE MODIFYING THERAPY

Introduction. Multiple sclerosis (MS) is a chronic inflammatory disease characterized by central nervous system lesions that can lead to severe physical or cognitive disability as well as neurological defects. The pathologic hallmark of MS is focal demyelination within the brain and spinal cord. Currently, there is no definite cure for MS, immunomodulating and antiinflammatory agents can diminish its progression and decrease some of the pathological symptoms. Case report. We report a case of 18 years female patient. The patient had first approached to neurology hospital on May 2019 after upper respiratory tract infection with neurology symptoms, such as numbness on the right side and problems with balance as well as coordination. On neurological examination, she had involuntary laughing attacks. Muscle power: lower extremities proximal part – 5/5, distal part – 3/5, upper extremities 4/5. Reflexes are increased in all extremities, D=S. Babinski sign was positive bilaterally. There are bilateral dysmetria and adiadochokinesia. Gait is ataxic. Brain MRI: multiple white matter demyelinating lesions in the mesencephalon, pons and bulbus with contrast enhancement. Spine MRI: multiple white matter demyelinating lesions within C2-C4, C7-T4 , T5-T7 level, without contrast enhancement. She started on treatment methylprednisolone pulse therapy afterwards continued with oral steroid. After she had clinical and radiological improvement. 3 month later, she admitted to hospital with progressive walking disturbance and imbalance. She did MRI again and 6 months after and it revealed new contrast enhanced plaques in brain and spine. The progressive white matter lesions and clinical symptoms were judged as the MS relapse, the patient was given methylprednisolone for five consecutive days. Fingolimod was started at this time because the patient had no neurological improvement. She lost ability to walk without support. She has been taking Fingolimod for 1 year. (Lymphocyte count 0.59). In response to this therapy, her walking ability slightly improved and there were no new neurological symptoms. Since clinically and radiologically positive dynamics is observed in the patient, the treatment is continued with Fingolimod and patient is being monitored.

Caution to Poor Adherence With Immunosuppressant Medication That Causes Coma-Onset Autoimmune Encephalitis: A Case Report and Literature Review

Autoimmune encephalitis after liver transplantation (LT) is a rare disorder. This is because patients are usually in an immunosuppressed state after LT. Here, we report a rare case of autoantibody-negative autoimmune-encephalitis-induced coma after living-donor (LD) LT. A 45-year-old woman who underwent LDLT for primary biliary cholangitis (PBC) was brought to our hospital with the chief complaint of cognitive deficiency and an episode of memory loss. Physical examination, laboratory tests, and cerebrospinal fluid analysis revealed no significant findings. However, diffusion-weighted magnetic resonance imaging showed hyperintensity in the bilateral hippocampus. No autoantibodies associated with autoimmune encephalitis were detected. The diagnosis of antibody-negative autoimmune encephalitis was made on the basis of low immunosuppressive drug levels in the blood (indicative of poor adherence) and the presence of PBC as the autoimmune disease. The patient regained consciousness after intravenous methylprednisolone pulse therapy and plasma exchange. This case highlights that when examining patients with impaired consciousness after LDLT, it is important to consider autoimmune encephalitis as a potential diagnosis.

Hypertrophic Pachymeningitis Associated with Myeloperoxidase-anti-neutrophil Cytoplasmic Antibodies Induced by Propylthiouracil: A Case Report.

A 71-year-old man with hyperthyroidism complained of headache lasting two months. He had been using propylthiouracil (PTU) for 14 years. Treatment intensification did not improve the symptoms. Blood tests detected a positive myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA). Diffuse dural thickening was identified by magnetic resonance imaging. The patient was diagnosed with hypertrophic pachymeningitis (HP) due to ANCA-associated vasculitis (AAV). He received methylprednisolone pulse therapy followed by prednisolone and methotrexate, which improved his headache. PTU-induced AAV-related HP is a rare and indiscernible disease. Therefore, the possibility of the disease should be proactively considered when a PTU user experiences refractory headaches.

Duplication Cysts and Autoimmune Hemolytic Anemia: An Association or a Coincidence?

Duplication cysts are rare congenital anomalies of the gastrointestinal tract and the occurrence of multiple such cysts is an extremely rare occurrence. Autoimmune hemolytic anemia is an extremely unusual manifestation of these duplication cysts. Herein, we report a case with two duplication cysts (one cyst in the thorax; another in the abdomen) complicated with autoimmune hemolytic anemia. This patient was a five year-old-male child who presented with congestive cardiac failure due to severe anemia (which, on evaluation, was diagnosed as autoimmune hemolytic anemia). Imaging studies (chest radiograph and computed tomography) revealed duplication cysts in the chest and abdomen. Anemia flare-up was noted in the patient despite two months of prednisolone therapy (2 mg/kg/day) and two high dose intravenous methylprednisolone pulse therapy courses (30 mg/kg/day for 5 days each). The steroid refractory autoimmune hemolytic anemia responded to rituximab (375 mg/ m2 body surface area/week for 4 weeks). Four months after tapering off the steroid therapy, the patient did not experience flare-up of the anemia. To the best of our knowledge, this is the youngest and first pediatric age group patient to be reported, with a possible association between gastrointestinal duplication cysts with autoimmune hemolytic anemia.

Efficacy and safety of mizoribine in comparison with cyclophosphamide for treatment of lupus nephritis: Protocol for a multi-center, open-label, randomized controlled trial

Background: Monthly intravenous cyclophosphamide (IVCY) is a widely accepted induction therapy for lupus nephritis (LN) because this regimen balances the time needed for renal remission with the risk of adverse events. However, IVCY is associated with numerous severe toxicities. Mizoribine (MZR) was originally used as an antibiotic against Candida albicans, but researchers found it had strong immunosuppressive activity in various animal models. Previous clinical trials also examined the efficacy of MZR as an immunosuppressant, and it has been used for treatment of LN in Japan since 1990. We will conduct a phase 3 study in China to evaluate the efficacy and safety of oral MZR in comparison with standard IVCY in patients with LN. Methods: This study will be a multi-center, randomized, controlled, open-label clinical trial that consists of a screening period (seven days) followed by a treatment period (52 weeks). After screening, all eligible subjects will be randomized to an MZR or IVCY group in a 1:1 ratio. Then, subjects will initially receive methylprednisolone pulse therapy (0.5 g/day) for three days, followed by the study drug (MZR or CY) with oral corticosteroid therapy from visit two (day four). The efficacy and safety of oral MZR in comparison with standard IVCY will be determined. Discussion: This paper describes the protocol of a multi-center, open-label, randomized controlled trial that compares the efficacy and safety of MZR with IVCY for treatment of LN. The results may help determine whether LN should be considered an indication for this drug in China. Trial registration: ClinicalTrials.gov register, NCT02256150. Registered 2014-10-01. The protocol version number is 1.3 (2016-08-30).

Corrigendum to "Methylprednisolone pulse therapy for critically ill patients with COVID-19: a cohort study".

Corrigendum to "Methylprednisolone pulse therapy for critically ill patients with COVID-19: a cohort study".

Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report.

Sjögren's syndrome is a chronic autoimmune disorder that predominantly affects exocrine organs. It is characterized by an organ-specific infiltration of lymphocytes. The involvement of the major cerebral arteries in Sjögren's syndrome has rarely been reported. A recent study reported a case of successful extracranial-intracranial (EC-IC) bypass without complications, even in the active inflammatory state, although the optimal timing of such a bypass remains unclear. We here report the case of a 43-year-old woman presenting with acute ischemic stroke due to progressive middle cerebral artery (MCA) occlusion and signs of primary Sjögren's syndrome. During intensive immunosuppressive therapy for active Sjögren's syndrome, the patient was monitored using contrast-enhanced magnetic resonance vessel wall imaging (MR-VWI). A couple of intravenous cyclophosphamide injections combined with a methylprednisolone pulse and antiplatelet therapy resulted in clear resolution of vessel wall enhancement, which suggested remission of inflammatory vasculitis. Nevertheless, she still experienced a transient ischemic attack (TIA) due to decreased regional cerebral blood flow by MCA occlusion, as demonstrated by the conventional time-of-flight MR angiography and single-photon emission computed tomography. Considering the increased risk of further stroke, the decision was made to perform an EC-IC bypass as a treatment for medically uncontrollable hemodynamic impairment. Her postoperative course was uneventful without further repeated TIAs, and continued immunosuppressive therapy for Sjögren's syndrome provided effective management. Our findings emphasize the diagnostic value of contrast-enhanced MR-VWI in monitoring the effect of immunosuppressive therapy for the major cerebral artery vasculitis and in determining the timing of EC-IC bypass as a "rescue" treatment for moyamoya syndrome associated with active Sjögren's syndrome.

MRI of fatal course of acute hemorrhagic leukoencephalitis in a child with SARS-CoV-2 omicron BA 2.0 infection.

We present a pediatric case of acute hemorrhagic leukoencephalitis associated with SARS-CoV-2 Omicron BA 2.0 infection. A previously healthy girl presented with ataxia and diplopia three weeks after the COVID-19 confirmation from a nasopharyngeal swab. Acute and symmetrical motor weakness and drowsiness ensued within the following 3days. She then became spastic tetraplegic. MRI revealed multifocal lesions in the cerebral white matter, basal ganglia, and brainstem, with hemorrhagic changes confirmed with T1-hyperintensity and hypointensity on susceptibility-weighted images. Peripheral areas of decreased diffusion, increased blood flow, and rim contrast enhancement were noted in the majority of lesions. She was treated with a combination of intravenous immunoglobulin and methylprednisolone pulse therapy. Neurological deterioration ensued with coma, ataxic respiratory pattern and decerebrate posture. Repeated MRI performed on day 31 revealed progression of abnormalities, hemorrhages and brain herniation. Despite the administration of plasma exchange, she died two months after admission.

Combined use of pulse therapy and molecular hydrogen in rats with experimental rheumatoid arthritis: Clinical and histological evaluation of therapeutic effectiveness

Introduction: Glucocorticosteroid therapy is the basic therapeutic option for rheumatoid arthritis; however, long-term treatment with low and medium doses is associated with the development of negative side effects. As reported, oxidative stress is crucial in the pathogenesis of rheumatoid arthritis. Therefore, pharmacotherapy that combines application of methylprednisolone pulse therapy and an aqueous molecular hydrogen solution seems to be a reasonable treatment option in these cases.
 Materials and Methods: The study of the effectiveness of combined pharmacotherapy for rheumatoid arthritis was carried out on mature male rats. Rheumatoid arthritis was simulated by introducing bovine type II collagen into the right knee joint. Animals of the 1st – control – group received placebo; animals of the 2nd group received molecular hydrogen-enriched water intragastrically; animals of the 3rd group received methylprednisolone solution intravenously by catheterization of the tail vein, and animals of the 4th group received water enriched with molecular hydrogenintragastrically and methylprednisolone solution intravenously. After withdrawal of animals from the experiment, microsections of their joint tissues were analysed histologically and biomarkers of the joint inflammation were detected immunohistochemically.
 Results and Discussion: Morphological analysis of microsections taken from animals of the 4th group evidenced effectiveness of the combined therapy based on quantitative estimation of the inflammatory biomarker expression. Dynamic polarization of M1/M2 macrophage was manifested with high quality in animals of this group.
 Conclusion: The search for new therapeutic options for rheumatoid arthritis is expanded due to major antioxidant substances that can reduce duration of treatment, while ensuring positive dynamics of the course of the disease.
 Graphical abstract:

Systematic surveillance of immune-related adverse events in clinical practice and impact of subsequent steroid medication on survival outcomes.

Recent advances in immune-checkpoint inhibitors (ICIs) have highlighted the need for effective management of immune-related adverse events (irAEs). This study aimed to conduct a systematic surveillance of real-world development of irAEs for understanding their characteristics and examine the prognostic impact of steroid use for these events. We retrospectively investigated cancer patients treated with ICIs between 2014 and 2021 and collected information about irAEs throughout their development, management, and clinical outcomes. Overall, 458 patients (45.4%) developed 670 irAEs. The prevalence of irAEs varied by cancer type, but it was increased in regimens with longer treatment durations. Severe irAEs were more common in the nivolumab + ipilimumab and pembrolizumab + axitinib regimens. Patients who received steroids for irAEs at a dosage of < 2mg/kg had comparable prognosis to those who did not receive steroids; however, patients who received methylprednisolone pulse therapy, primarily for severe pneumonitis and hepatitis, had shorter overall survival than those who did not receive steroids (7.8 versus 23.4months, p = 0.016). Furthermore, methylprednisolone pulse therapy for irAEs was a poor prognostic factor in multivariate analysis (hazard ratio: 2.19, 95% confidence interval: 1.34-2.86, p < 0.001). Steroid treatment for irAE does not affect prognosis and should thus be used promptly to control inflammation. However, pulse therapy for severe cases is a poor prognostic factor, and early detection remains the key to managing such irAEs. The irAE characteristics in each regimen should be clarified to establish and provide more sophisticated irAE management, and the current findings will be beneficial to this goal.

Aggressive Behavior in a Critically Ill COVID-19 Patient: Possible Role of Medication Discrepancies

Coronavirus disease 2019 (COVID-19) has different clinical manifestations that besides its iatrogenic intervention could affect brain cognitive function. Medication omission has serious effects on a patient’s clinical course changing the disease’s mortality and morbidity; herein possible role of the iatrogenic intervention that increased the risk of psychological disturbance including medication omission was reported.&#x0D; A 40-years-old man with a history of gout treated with allopurinol was admitted due to productive cough and hemoptysis. His physical exam and lung spiral chest CT scan revealed moderate to severe lung infiltrations in favor of COVID-19 which was confirmed with COVID-19 RT-PCR. Due to his clinical course tocilizumab, methylprednisolone pulse, and other conservative therapies were started while allopurinol was omitted. During his hospitalization anxiety and irritability appeared and progressed gradually making him refuse to get oxygen supplementation. Through immediate intervention and controlling his behavioral symptoms with psychotherapy, selective serotonin reuptake inhibitors, benzodiazepines, and initiation of allopurinol, the patient’s psychological disturbance were relieved. In the end, he died due to acute respiratory distress syndrome.&#x0D; COVID-19 and its iatrogenic interventions could trigger a psychiatric disturbance in patients with a variety of pathways. Corticosteroid therapy, non-hypnotic antihistamines, quarantine stress conditions, hypoxia, and medication omission are underlying factors for it. Reporting this medication omission would help physicians become familiar with this pharmacological phenomenon, its prevention, and the way to respond to it. On the other hand, researchers can study the etiology of this phenomenon to understand the mood-stabilizing role of allopurinol in additional studies.

A case of bullous pemphigoid developing under treatment with benralizumab for bronchial asthma.

Bullous pemphigoid (BP) is an autoimmune disease characterized by itchy erythema and tense blisters on the whole body. Recent reports have unveiled the pathogenic roles of eosinophils in BP (e.g., dermal-epidermal separation, generation of pruritus). Thus, eosinophils are considered a therapeutic target. Benralizumab is an anti-IL-5 receptor alpha (IL-5Rα) monoclonal antibody (mAb) that is widely used to treat severe eosinophilic asthma. By affecting IL-5Rα, benralizumab depletes eosinophils and basophils due to apoptosis through antibody-dependent cell-mediated cytotoxicity. The efficacies of benralizumab and other biologics, including bertilimumab (anti-eotaxin-1 mAb) and mepolizumab (anti-IL-5 mAb), were evaluated in several clinical trials. Also, reslizumab, an anti-IL-5 mAb, was reported as a successful treatment option in a case of BP. We present a case of severe asthma treated with benralizumab at 8-week intervals for 3 years before BP developed. Histologically, subepidermal blisters without eosinophilic infiltration were observed. Methylprednisolone pulse therapy followed by 40 mg/day (1 mg/kg/day) of oral prednisolone (PSL) was initiated, but the skin lesions worsened. Additional intravenous immunoglobulin and oral azathioprine enabled the oral PSL to be tapered. The benralizumab was discontinued after the onset of BP because the asthma did not worsen. To the best of our knowledge, there have been no reports of BP developing during anti-eosinophil therapy. BP may occur paradoxically via various pathways during treatment with drugs that are typically effective against BP.

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.

504 A single-center retrospective study evaluating the efficacy of intravenous methylprednisolone pulse therapy for 113 patients with alopecia areata

504 A single-center retrospective study evaluating the efficacy of intravenous methylprednisolone pulse therapy for 113 patients with alopecia areata

METHYLPREDNISOLONE - THE SAVIOR IN CHILDREN WITH MULTISYSTEM INFLAMMATORY SYNDROME (MIS-C)

Background: Multisystem inammatory syndrome in children (MIS-C), also known as pediatric inammatory multisystem syndrome is a new entity and is fatal disease that is temporally associated with SARS-COV 2 (PIMS-TS) [1]. We aimed at studying varied clinical presentation, management and outcome in children with MIS-C associated with covid-19 treated with IvIg and methylprednisolone at tertiary care centre, Davangere. This is a retrospective observational Methods: study conducted at two tertiary care hospitals at Davangere from October 2020 to August 2021 (11 months.). All cases who fullled WHO criteria for MIS-C were included in the study. Institutional ethics committee approval was taken. Data was obtained from hospital records .Parameters studied were demographics, symptomatology, laboratory markers, medications and immediate outcome. All children were then grouped into 2. Group 1- those who presented with shock and group 2- those who presented without shock . Data were entered and analyzed using SPSS software version 22.0. For interpretation of results signicance was adopted at p-value less than 0.005 and at 95% condence interval. A total of 182 patients w Results: ith MIS-C were treated during the study period. The median age of presentation was 6.8±4.5 years. We had 14 cases of infants. M:F ratio 1.35:1 showing no sex predilection. Children who presented with shock had signicant symptoms of pain abdomen, tachypnoea, Lower GCS and lower saturation levels and had signicantly higher CRP, LDH, ferritin, D-dimer levels and had lower albumin levels which is statistically signicant . Also requirement of IVIg was signicantly more in children with shock (TABLE 2). Out of 182 children with MIS-C, only 21 received IVIg and rest all received only steroids - methylprednisolone ( due to nancial constraints ). However the outcome did not vary much with IVIg or steroids. IV methylprednisolone pulse therapy was associated with favorable immediate outcome which is comparable with IVIg in our study. Mortality in our series was 6.04% which is comparable to international studies [11,12]. In our Conclusion study most of our children with MIS-C recovered with timely use of pulse methylprednisolone therapy alone with favourable short term outcomes. Thus in resource limited settings methylprednisolone pulse therapy can help in saving lot of young children with MISC.

CLINICAL PROFILE AND SHORT TERM OUTCOME OF CHILDREN WITH COVID-19 RELATED MULTISYSTEM INFLAMMATORY SYNDROME (MISC) TREATED WITH PULSE METHYLPREDNISOLONE AND/OR IV IMMUNOGLOBULIN : A CASE SERIES FROM TERTIARY CARE PAEDIATRIC HOSPITAL IN BANGALORE

Introduction: MIS-C in children is a rare, but life threatening complication of covid 19 infection. The complications of MIS-C can be severe, including cardiogenic shock or distributive shock with poor vasomotor tone. Aim: To study the clinical prole and outcome of children treated with methylprednisolone pulse therapy and/or IV immunoglobulin. Materials and methods: Case series of children diagnosed with COVID 19 related MISC admitted to Vydehi Institute of Medical Sciences and Research Centre, Bangalore from march to august 2021 , who met the WHO and CDC criteria were reviewed. Results: The case series describes 11 cases of MISC in our hospital. 3 children were treated with IV immunoglobulin and 8 children were received IV pulse methylprednisolone. All children had fever upon arrival to hospital lasting for atleast 5 days. Children with shock also had higher D-dimer , lower albumin , higher CRP, high lactate and lower ejection fraction compared to other children. Conclusion: We have concluded a favourable role of IV immunoglobulin or pulse methylprednisolone in treatment of MISC.