Oral Prednisone Taper Research Articles

Outcomes of older Black renal transplant recipients receiving basiliximab induction and early steroid withdrawal

AbstractThe efficacy and safety of early steroid withdrawal (ESW) in Black renal transplant recipients (RTR) remains uncertain, especially when combined with non‐lymphocyte depleting induction. This study compared patient and graft outcomes of older Black and Non‐Black RTR who received basiliximab induction followed by ESW.Included subjects in this single center, retrospective study were first‐time deceased and living donor RTR aged over 65 years who received basiliximab induction therapy and ESW. Black RTR and Non‐Black deceased donor RTR received an oral prednisone taper starting POD3 which was discontinued by POD21 per protocol.Of 139 patients (55 Black and 84 Non‐Black), the Black patient group included a higher percentage of deceased donors and hepatitis C positive donors, and more frequently underwent renal replacement therapy prior to transplant. One Black and zero Non‐Black RTR experienced graft loss within 3 years (p = 1.0). Patient survival was similar at 1 year (93% vs. 97%, p = .16) and 3 years (91% vs. 96%, p = .17). Rejection‐free survival and biopsy‐proven rejection did not differ at 1 or 3 years. Median eGFR and change in eGFR also did not differ between groups. Black patients were maintained on significantly higher median mycophenolate doses from 6 months post‐transplant to the end of the study period (p = .03 and .048).In older Black RTR, basiliximab induction followed by ESW produced comparable patient, graft, and rejection‐free survival compared to Non‐Black RTR over 3 years post‐transplant.

Effectiveness of oral prednisone tapering following intravenous methylprednisolone for acute optic neuritis in multiple sclerosis

Acute optic neuritis treatment lacks standardized protocols. The value of oral prednisone taper (OPT) following intravenous methylprednisolone (IVMP) on visual outcome parameters in optic neuritis (ON) has never been explored. In the present retrospective study, we investigated whether OPT after IVMP affects the structural and functional visual outcomes of inaugural clinically isolated syndrome (CIS)- or multiple sclerosis (MS)-ON. Adult patients with acute, inaugural, unilateral CIS- or MS-ON, treated with IVMP in Germany and Israel were stratified into patients treated with IVMP alone—versus IVMP and OPT. Inclusion criteria were age ≥18, CIS or MS diagnosis according to McDonald criteria 2017, available visual acuity (VA) at nadir before treatment initiation and at follow-up ≥5 months, as well as a spectral domain optic coherence tomography (OCT) data scan at follow-up. Exclusion criteria included recurrent ON, concomitant ophthalmological comorbidities, optical coherence tomography (OCT) of insufficient quality and ON-related escalation therapy after IVMP. The structural outcome was defined as the average retinal nerve fiber layer (RNFL) difference between the ON-affected and the unaffected eye, while the functional outcome was defined as the final high-contrast best-corrected VA (HC-BCVA) at follow-up compared to nadir. The comparative analysis was performed using linear regression analysis, adjusted for sex, age, and days-to-treatment. Fifty-one patients met the inclusion criteria (25% male). The mean age was 33.9 (±10.23) years. Twenty-six patients (51%) received OPT following IVMP. There was no difference in nadir HC-BCVA between the groups (0.39 No OPT; 0.49 With OPT, P = 0.36). Adjusted linear regression analysis did not indicate an influence of OPT on RNFL thickness or on HC-BCVA (beta coefficient for RNFL difference in percentages: 0.51, 95%-CI: [-4.58, 5.59], beta coefficient for logMAR: 0.11, 95%; CI [-0.12, 0.35] at follow-up. In conclusion, the addition of OPT to IVMP did not affect RNFL thickness or the final VA in a retrospective cohort of 51 patients with inaugural acute CIS- or MS-ON. The results of this exploratory study are currently being re-examined in a large-scale, demographically diverse, prospective study.

FRI572 Teprotumumab In Unexplored Presentation Of Thyroid Eye Disease

Abstract Disclosure: M.T. Torres Torres: None. A.N. Gonzalez Bossolo: None. M.M. Mangual Garcia: None. Thyroid eye disease (TED) is an autoimmune disease of the orbital soft tissue associated with facial disfigurement, diminished quality of life, and potentially vision-threatening consequences that can occur in 40% of patients with Graves’ disease (GD). All the therapies for this condition were off-label with questionable long-term effects until 2020 when the Federal Drug Administration approved teprotumumab, a human monoclonal antibody against insulin-like growth factor-1 receptor, for the treatment of TED. Randomized placebo-controlled trials have demonstrated that teprotumumab reduces proptosis, diplopia, and Clinical Activity Score (CAS) in patients with active TED of less than 1.3 years of duration who were treatment-naïve or previously treated with < 1g of methylprednisolone equivalent therapy. Data on the efficacy of teprotumumab on cases of longer TED duration or previously treated with eye surgery or > 1g of methylprednisolone equivalent therapy for TED is limited as these patients were excluded from the trials. A 76-year-old nonsmoker male with long-standing hyperthyroidism due to GD, glaucoma, and type 1 diabetes mellitus was referred by ophthalmologist due to TED. He received radioactive iodine (RAI) for treatment of GD after failure to Methimazole therapy. After RAI, he reported progressively worsening bilateral eye edema, redness, pain, vision loss, and diplopia requiring hospitalization for an emergent orbital decompressive surgery and intravenous steroid therapy due to sight-threatening Grave’s orbitopathy. He was discharged home with oral prednisone taper down until it was discontinued 6 months later with mild improvement of symptoms. Physical exam was remarkable for bilateral strabismus, orbital chemosis, conjunctival injection and redness, eyelid swelling, limited eye motion, severely impaired visual acuity and color vision, no proptosis, and a CAS of 5 suggesting active TED. Laboratories showed biochemical euthyroidism and controlled glycemia. Teprotumumab was started 2 years and 8 months after symptom onset with marked improvement of inflammatory signs and symptoms, eye motion, visual acuity, diplopia, intraocular pressure, and CAS score decrease to 1 point suggesting inactive disease within 4 weeks of therapy without adverse side effects. This case describes the positive effects of teprotumumab in a patient with characteristics not previously studied such as active TED of longer duration than in clinical trials, previous treatment with orbital surgery or > 1g of methylprednisolone equivalent therapy, and increased IOP. Rather than drawing conclusions, our goal is to create awareness and promote further research of teprotumumab and novel agents in patients with TED with previously uncharacterized presentations as it may have important implications for their management including significant improvement in their quality of life. Presentation: Friday, June 16, 2023

Severe case of Pembrolizumab-induced triad of myasthenic crisis, myocarditis, and anti-SSA myositis

A man in his late 70s presented with one week of progressive chest and muscle pain, generalized weakness, and fatigue. He received a single infusion of pembrolizumab three weeks prior as adjuvant immunotherapy for stage IIb malignant melanoma after undergoing wide local surgical excision. He was started on steroids for pembrolizumab-induced myositis and myocarditis. Symptoms progressed to include severe dyspnea, dysphagia and eyelid ptosis requiring tracheostomy and PEG tube placement. Serologic studies ultimately confirmed myasthenia gravis and anti-SSA myositis. 
 He was emergently treated with plasma exchange, pyridostigmine, Intravenous Immunoglobulin (IVIG) and is making gradual improvement with physical therapy and oral prednisone taper.
 We report a severe case of Pembrolizumab-induced triad of myasthenic crisis, myocarditis with myositis specific anti-SSA 52kD Ab IgG autoantibody for the first time. More studies are needed to assess the clinical significance and prognostic value of this autoantibody in patients presenting with Pembrolizumab-induced triad.

NEIM-15 AN EXCEPTION TO THE METASTATIC RULE: A NON-ENHANCING MIDBRAIN LESION DUE TO METASTATIC FIBROMYXOID SARCOMA WITH EXTENSIVEINFLAMMATORY INFILTRATE

Abstract Metastases from extra-CNS sites typically enhance on MR after the administration of gadolinium-based contrast agents due to disruption of the blood-brain barrier. Fibromyxoid sarcoma involving the brain is extremely rare, with only a few reported cases manifesting as well-circumscribed enhancing masses. Here, we report an extremely rare non-enhancing brain metastasis secondary to an already rare sarcoma. A 44-year-old woman with gluteal fibromyxoid sarcoma metastatic to cervical paraspinal muscles and adrenal gland treated with ipilimumab/nivolumab presented with diplopia and was found to have an 8 mm T2-hyperintense midbrain lesion without enhancement or mass effect. An inflammatory etiology associated with checkpoint inhibitor therapy was suspected, and she was treated with IV methylprednisolone 1 g daily for 3 days followed by an oral prednisone taper, IV immunoglobulin, and plasma exchange without improvement. The midbrain lesion steadily grew over 15 months of follow up, eventually involving the bilateral medial thalami and demonstrating diffusion restriction in the midbrain, but never showing gadolinium enhancement. The imaging was reminiscent of peripheral brainstem non-enhancing band-like lesions reported in leptomeningeal lung cancer, however CSF cytology was repeatedly normal, as was extensive infectious and inflammatory testing. Her neurologic disability continued to progress, with development of bilateral oculomotor nerve palsies, dysarthria, and depressed level of alertness, devolving into non-convulsive status epilepticus requiring intubation. She died six months after onset of neurologic symptoms. At autopsy, histologic and immunohistochemical evaluation revealed fibromyxoid sarcoma (MUC-4 positive, TAOK1-FUS fusion) centered on the periventricular aqueduct with accompanying disseminated single-cell infiltration throughout the midbrain. The background midbrain and periventricular parenchyma were notable for voluminous microglial activation and histiocytic infiltrate. In sum, this case represents a radiographically unique sarcoma brain metastasis which on pathologic exam showed robust inflammatory changes far out of proportion to the tumor present.

Missing Piece of Puzzle: Electro-Convulsive Therapy (ECT) in Patients with anti-NMDA Receptor Encephalitis (P5-5.012)

<h3>Objective:</h3> To emphasize the importance of early clinical recognition of catatonia and implementation of ECT as adjuvant therapy in patients with anti-NMDAR encephalitis. <h3>Background:</h3> Catatonia is a symptom of anti-NMDAR encephalitis which can evolve rapidly into malignant catatonia with autonomic instability and increased morbidity. Although early immunomodulatory therapy is important to treat the underlying autoimmune disorder, resistant catatonia may require adjuvant management with ECT. <h3>Design/Methods:</h3> Not applicable. <h3>Results:</h3> We describe the cases of three females aged 23.7 ± 2.5, who were diagnosed with anti-NMDAR encephalitis. They presented with neuropsychiatric symptoms and were promptly and empirically treated with IV solumedrol with oral prednisone taper, IVIG, +/− plasma exchange (case 3), preceding diagnostic confirmation via CSF NMDA antibody positivity. Ovarian teratomas were detected in all cases and resected shortly after hospitalization. Additionally, cases 1 and 2 received Rituximab while hospitalized, and cases 2 and 3 received anti-seizure medications for temporal onset seizures. In spite of this, all required escalating large doses of benzodiazepines, in conjunction with amantadine and bromocriptine, for refractory malignant catatonia with dysautonomia. ECT treatments were thus initiated (once to twice daily for first week, followed by three times weekly) with bilateral electrode placement. All cases tolerated ECT treatments well and were gradually noted to have marked clinical improvement as was measured by the Bush-Francis Catatonia Rating Scale (BFCRS) (initial assessment: 27.6 ± 8.3; last assessment 1.3 ± 1.5). <h3>Conclusions:</h3> This case series supports the growing body of evidence emphasizing the importance of clinical recognition of catatonia among patients with suspected or confirmed anti-NMDAR encephalitis, and the early implementation of ECT into treatment algorithms. The use of standardized assessment tools such as the BFCRS and cognitive impairment screening scales should be considered for monitoring of symptom response and tolerability. <b>Disclosure:</b> Dr. Wadi has nothing to disclose. Dr. Mandge has nothing to disclose.

Pruritic crusted lesions in an 89‐year‐old woman

Pruritic crusted lesions in an 89‐year‐old woman

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies.

Relentless placoid chorioretinitis (RPC) is a rare, bilateral disease of the retinal pigment epithelium. The clinical course is prolonged and relapsing. No standard treatment has been established to date. The purpose of this case series is to report four cases of RPC in pediatric and young adult patients in which varying treatments were used, comparing them to previously published cases. A literature review was conducted to investigate currently published presentations and treatment options for RPC. A multicenter retrospective chart review was also performed on four consecutive patients. These patients were diagnosed with RPC because of new chorioretinitis lesions continuing to appear without or despite therapy for 5-36 months (2 patients), with a clinical course prolonged and relapsing, or because of the atypical location of the multiple lesions (>50) extending from the posterior pole to the equator and mid-peripheral retina (all four patients), which were not consistent with other entities like acute posterior multifocal placoid pigment epitheliopathy and serpiginous choroiditis. All four cases of RPC received oral or IV steroids acutely, and three of these patients were transitioned to a steroid-sparing agent and biologic therapy: anti-TNF alpha or anti-IL-6. Quiescence of the chorioretinitis lesions was obtained after 7 months, 1 month, and 36 months; however, the latter had issues with treatment adherence. Mycophenolate mofetil was insufficient to control the disease in one patient, but tocilizumab and infliximab thereafter were effective after cessation of adalimumab due to side effects. Adalimumab when started the first month after the presentation was effective in controlling the disease in one patient. After the failure of interferon-alpha-2a, one patient displayed long-term control with infliximab. One patient did not require a steroid-sparing agent after oral prednisone taper as there was no evidence of progression or recurrence. This case series adds to the current knowledge regarding potential treatments for RPC, specifically the use of anti-TNF-alpha treatment and anti-IL-6 tocilizumab. In this case study, relapses of RPC were found among patients on mycophenolate mofetil and interferon-alpha-2a, and one case did not relapse on oral steroids without a steroid-sparing agent. Our findings suggest that adalimumab, infliximab, and tocilizumab may be useful medications to obtain quiescence of RPC.

Radiation-Induced Localized Bullous Pemphigoid.

Radiation-Induced Localized Bullous Pemphigoid.

A Case of Recurrent Cerebral Cortical Encephalitis in MOG Antibody-Associated Disease

Objective NA. Background Cerebral cortical encephalitis is a recently recognized syndrome of myelin-oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD), yet most descriptions report single episodes without recurrence. Design/Methods Case report of recurrent cerebral cortical encephalitis in MOGAD. Results This is a case report of a 32-year-old patient who initially developed meningoencephalitis when he was three. He was stable until age 31, when he developed headaches followed by status epilepticus secondary to cerebral cortical encephalitis with accompanying unilateral right temporal cerebral cortical T2-hyperintensity with leptomeningeal enhancement. Cerebrospinal fluid analysis revealed 505 white cells/µl (normal, 0-5) but oligoclonal bands were negative. Over the subsequent two weeks he developed bilateral severe vision loss (20/400 bilaterally) accompanied by bilateral optic disc edema, and MRI orbits showed bilateral anterior segment optic nerve enhancement extending &gt;50% of the length of each nerve, confirming bilateral optic neuritis. MOG-IgG was tested in serum and was positive at a titer of 1:100. He was treated with intravenous methylprednisolone 1 gram daily for 5 days and a subsequent slow oral prednisone taper along with anti-epileptic treatment with levetiracetam, later transitioned to oxcarbazepine, although he later self-discontinued all anti-epileptics. He received maintenance intravenous immunoglobulins (IVIg) 0.4 g/Kg weekly, that was later weaned to 1 g/Kg every 4 weeks. He then developed recurrent cerebral cortical encephalitis 18 months after his prior episode manifesting with seizures and left occipito-temporal T2-hyperintensity and swelling with leptomeningeal enhancement. He was treated with high dose IV steroids, an oral prednisone taper and his IVIg dose was changed back to 0.4 g/Kg once weekly and anti-seizure medications reinitiated. Conclusions Cerebral cortical encephalitis in MOGAD can be recurrent. Close scrutiny of the MRI in patients with MOGAD who develop seizures after prior cerebral cortical encephalitis is important as it can discriminate a recurrent cerebral cortical encephalitis attack from a breakthrough seizure related to prior damage.

Frosted branch angiitis presenting after a SARS-CoV-2 infection

PurposeTo report a case of frosted branch angiitis presenting in a pediatric patient with unremarkable laboratory work-up apart from SARS-CoV-2 IgG antibodies.ObservationsLess than four weeks after a SARS-CoV-2 infection, a 10 year-old female presented to the emergency department with severe headache and intermittent fevers. During her hospital admission, the ophthalmology service was consulted for blurry vision. Subsequent eye examination revealed frosted branch angiitis. The patient initially received intravenous corticosteroids but was escalated to plasmapheresis to achieve resolution of her symptoms. Outpatient maintenance therapy consisted of an oral Prednisone taper and Infliximab infusion.Conclusion and importanceThis case represents a unique ocular manifestation of COVID-19, as recent SARS-CoV-2 was the sole identifiable cause of the patient’s frosted branch angiitis. Additionally, this patient required plasmapheresis to control disease progression.

S2722 Cytomegalovirus Colitis in an Immunocompetent Patient Presenting With New Onset Ulcerative Colitis: A Clinical Vignette

Introduction: Cytomegalovirus (CMV) colitis is a rare disease among immunocompetent patients presenting with inflammatory bowel disease (IBD). CMV can cause steroid-refractory IBD, which may require antiviral and infliximab (IFX) combination therapy. Case Description/Methods: A 23-year-old woman was admitted for dehydration and poor oral intake in the setting of eight bloody bowel movements per day, tenesmus, and weight loss. She presented to an outside hospital 5-weeks prior with abdominal cramps and bright red blood per rectum. The CT scan showed bowel wall thickening and mucosal enhancement in the rectosigmoid and descending colon. A colonoscopy 2-weeks prior to admission was suggestive of severe ulcerative colitis (UC) and she was started on mesalamine/budesonide. Due to poor clinical response, she was switched to prednisone 40 mg for 5 days. Outpatient medical management failed and, upon admission, she was converted to methylprednisolone 20 mg every eight hours. Serum CMV PCR was positive at 1,758 IU/mL and colonic biopsies showed scattered CMV inclusions by immunohistochemical staining. She was started on ganciclovir 5mg/kg/dose IV in addition to the steroid regimen and IFX infusions were deferred. By day 2 of ganciclovir IV and day 7 of methylprednisolone IV, IBD symptoms began to resolve. On discharge at hospital day 12, patient was transitioned to twice daily oral valganciclovir 900 mg for a 3-week course and an oral prednisone taper. CMV PCR was 1,185 IU/mL on discharge and < 100 IU/mL at one week follow up. IFX therapy was initiated 45 days post discharge with improved symptoms, as characterized by a 17-lb weight gain at 3-month follow-up. (Figure) Discussion: CMV colitis is a rare diagnosis in immunocompetent patients with early, steroid naïve IBD. Serum PCR positive infection has been reported more commonly after 2-3 weeks of steroid therapy. CMV infection is a recognized complication and marker of poor prognosis in moderate to severe UC, especially those who present with steroid refractory disease. It is believed that the inflammatory mediators associated with UC play a synergistic role in CMV reactivation and subsequent disease. In addition, local corticosteroid induced immunosuppression may further facilitate CMV gene transcription, resulting in disease progression and poor steroid response. There remains no consensus for management of UC with concomitant CMV colitis however, in our patient the use of antiviral and biologic therapy (IFX) promoted disease regression and clinical improvement.Figure 1.: H&E Stain (20x) demonstrating active chronic colitis (left). Immunohistochemical staining (40x) showing CMV immunoreactivity (right).

S2738 Isolated Celiac Artery Vasculitis Presenting as Ileus in a Patient With Ulcerative Colitis

Introduction: Small case series have reported an association between IBD and large vessel vasculitides, ANCA-associated vasculitis, and cutaneous vasculitis. Gastrointestinal (GI) involvement of vasculitis can be a severe complication, resulting in mesenteric ischemia, ileus, or GI bleeding, and often requires aggressive medical or surgical interventions. We report a case of celiac artery vasculitis presenting as abdominal pain and ileus in a patient with ulcerative colitis (UC). Case Description/Methods: A 69 year old male with an 11 year history of UC in remission on infliximab and azathioprine (AZA) presented to the emergency room with abdominal pain and constipation for five days. He had decreased AZA from 200 mg to 100 mg one month prior. Initial CT abdomen and pelvis showed stranding surrounding the celiac artery, fluid-filled small bowel loops, and segmental areas of distal colon collapse with proximal colon distention. He developed progressive small bowel and colonic distention with cecum diameter of 10 cm. A colonoscopy was performed for decompression. He had no evidence of active UC. EGD demonstrated dusky appearing gastroesophageal junction and inflammation with cratered ulcers in the gastric cardia and body. Biopsies confirmed ischemic necrosis. CTA demonstrated perivascular inflammation of a patent celiac artery without evidence for dissection, suggestive of acute vasculitis. ESR and CRP were 50 mm/hr and 7.8 mg/dL respectively. Myeloperoxidase, serine-proteinase 3, rheumatoid factor, ANA, were negative. He was started on methylprednisolone IV with oral prednisone taper at discharge, and AZA was increased to 200 mg daily. A CT one month later showed minimal residual inflammation. ESR and CRP had normalized. (Figure) Discussion: We present the first case of isolated celiac artery vasculitis in a patient with IBD. A diagnosis of IBD often precedes vasculitis, and patients are typically on immunosuppressive therapy at the time of onset. Anti-TNF agents have been implicated as a potential inciting cause. Although it is unclear whether long-term anti-TNF therapy precipitated vasculitis in our patient, the dose reduction in AZA prior to presentation may have unmasked vasculitis. This was supported by his rapid clinical and radiographic improvement with combination of steroids and increased AZA dose despite continuation of infliximab. This case highlights the importance of maintaining a high degree of suspicion for vasculitis in patients with IBD on immunosuppressive therapy presenting with ischemia or ileus.Figure 1.: A 69 year old male underwent EGD for evaluation of abdominal pain and NG tube placement; he was found to have inflammation with a dusky appearing mucosa at the gastroesophageal junction (GEJ) (A). Non-bleeding gastric ulcers with overlying pigment (Forrest Class IIc) were identified (B). H&E stain image shows the GEJ biopsy with extensive ischemic necrosis and hemorrhage (C). Initial CTA abdomen/pelvis shows fat stranding surrounding the celiac artery (D).

35423 ASIA syndrome following deoxycholic acid injection

A 50-year-old woman with a history of hypertension presented for progressive and debilitating cutaneous fibrosis. Onset occurred after injection of an Argentinian deoxycholic acid product (46% “Desoxicolato”) meant to induce cosmetic lipolysis. The examination revealed sharply demarcated, indurated, depressed plaques with an inflammatory border on the left arm, hips and buttocks. Laboratory values were notable for thrombocytosis, but otherwise unremarkable complete blood cell count and comprehensive metabolic panel. ANA, anti-scleroderma, anti-centromere, SM, and SM/RNP antibodies were negative. Histopathology had features suggestive of scleroderma or scleredema. MRI revealed diffuse subcutaneous edema in the left arm and pelvis. Given acute worsening, she was hospitalized for intravenous methylprednisolone and sirolimus, before being discharged on an oral prednisone taper and sirolimus. She continues to follow longitudinally and was recently initiated on mycophenolate mofetil. Gas spectrometry analysis was considered but the there was no additional product available. ASIA syndrome (autoimmune/inflammatory syndrome induced by adjuvants) is an umbrella term encompassing syndromes united by the underlying hypothesis that genetically susceptible individuals exposed to an adjuvant may develop granulomatous or systemic autoimmune disorders. There remains controversy over the diagnosis, as the criteria regarding adjuvant dose or temporal association evolve. Historically management of ASIA syndrome was guided by explantation of extraneous substances from the body; however, recent data suggest a role for immunomodulatory and immunosuppressive therapy, including corticosteroids and calcineurin inhibitors. In light of the epidemic of illicit cosmetic procedures, sometimes using counterfeit or contaminated products, dermatologists should educate patients about how to identify appropriate product, technique, and practitioners.

FC057: Incidence of Infections in the Avacopan Group Versus Prednisone Group in Anca-Associated Vasculitis, Results from the Phase 3 Advocate Study

Abstract BACKGROUND AND AIMS Standard therapy for ANCA-associated vasculitis (AAV) includes glucocorticoids (GC), which are associated with increased infections. Avacopan is an orally administered selective inhibitor of the C5a receptor recently approved for treatment of AAV. The phase 3 ADVOCATE study tested daily avacopan as a substitute for a standard, scheduled, daily oral GC tapered regimen (all with standard background therapy), resulting in a 63% mean reduction in overall GC load for the avacopan group. At week 26, the avacopan group achieved a comparable remission rate to prednisone (72.3% versus 70.1%) and superior sustained remission at Week 52 (65.7% versus 54.9%; one-sided P = 0.0066) (1). The present analysis compared infections in avacopan and prednisone groups in the ADVOCATE trial. METHOD ADVOCATE, a phase 3, randomized, double-blind, controlled trial in 330 patients, evaluated the efficacy and safety of avacopan. AAV patients (new onset or relapsing disease) were randomized to receive avacopan 30 mg twice daily, or a standard 20-week oral prednisone taper, plus matching placebos. Both groups had background therapy of either cyclophosphamide (followed by azathioprine) or rituximab, and additional GC exposure from premedication for rituximab, from taper of pre-randomization GC, and as required per investigator judgment. Patients were on blinded study drug for 52 weeks. Infections occurring from first dose of blinded study drug through day 56 after last dose were analyzed. RESULTS Total mean GC use during the first 26 weeks of the study was 1073 mg with avacopan and 3193 mg with prednisone groups, and in the last 26 weeks, 296 and 489 mg, respectively. Infections were higher in the prednisone group than with avacopan: 291 events (prednisone) versus 233 (avacopan), with a 75.6% incidence (124/164) with prednisone versus 68.1% (113/166) with avacopan (Table 1). During the prednisone taper period (first 20 weeks), infection incidence was 54.9% in prednisone group versus 49.4% for avacopan, and after the first 20 weeks 52.4% in prednisone group versus 44.0% avacopan. Fewer patients in the avacopan group experienced serious AEs of infection (13.3%/25 events) versus prednisone group (15.2%/31 events); also serious opportunistic infections (3.6% versus 6.7%), life-threatening AEs of infection (0.6% versus 1.2%), and infections resulting in death (0.6% versus 1.2%) were fewer with avacopan (Table 1). No Neisseria meningitidis infections occurred in any patient in the trial. CONCLUSION In the ADVOCATE trial, the mean dose of GCs from all sources was 63% lower in the avacopan group than the prednisone group (1349 versus 3655 total mg, respectively). The avacopan group had a numerically lower incidence of infections, including serious, life-threatening, fatal and serious opportunistic infections compared with the prednisone group.

Severe IgA vasculitis with features of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome in a 60‐year‐old male treated with plasmapheresis

AbstractObjective: To explore a possible association between systemic immunoglobulin A (IgA) vasculitis and RS3PE syndrome and to investigate specific treatment regimens for adults who present with IgA vasculitis with renal involvement.Methods: The patient was treated with plasmapheresis and fresh frozen plasma (FFP) every other day with 1g methylprednisolone daily for three days followed by oral prednisone taper. Mycophenolate mofetil twice daily and trimethoprim‐sulfamethoxazole for Pneumocystis jirovecii prophylaxis was started. In total the patient received two cycles of plasmapheresis and fresh frozen plasma.Results: The patient's renal function drastically improved with resolution of both abdominal pain and nausea.Conclusion: We illustrate a possible association between systemic IgA vasculitis and RS3PE syndrome, and this case demonstrates IgA vasculitis with renal involvement that acutely resolved with high‐dose glucocorticoids and plasmapheresis. Additionally, our specific treatment regimen can be a potential standard of care for adults who present with IgA vasculitis with renal involvement.

Abstract 10819: Eosinophilic Myocarditis Due to Idiopathic Hypereosinophilic Syndrome

Background: Idiopathic Hypereosinophilic Syndrome (iHES) is a rare disease with varied clinical presentations including acute heart failure. Case: A 44-year-old man who had been treated for asthma 9 months ago was admitted with worsening exertional dyspnea. EKG demonstrated sinus tachycardia with anterolateral ST depressions. Labs revealed troponin-I 44.95 ng/ml, BNP 405 pg/mL, and white blood cell count 21.2k with 50% eosinophils. Transthoracic echocardiogram showed normal left ventricular size with globally reduced ejection fraction (30-35%). Coronary angiography found normal coronary arteries. An extensive infectious and auto-immune workup was unrevealing, and bone marrow biopsy was negative for monoclonal proliferation (Fig 1A). He received 1 gram of IV methylprednisolone for 2 days lowering eosinophil count to zero and was discharged on heart failure GDMT. Two weeks later, he was readmitted from clinic for ventricular tachycardia (VT) near 120bpm (Fig 1B). Cardiac MRI and endomyocardial biopsy confirmed active eosinophilic myocarditis (Fig 1C/D). Patient was diagnosed with iHES and treated with IV methylprednisolone (500mg BID x 3 days) with an oral prednisone taper. Amiodarone and lidocaine infusions were temporarily needed to suppress his VT and an ICD was placed. After clinical stabilization and immunology consultation, an interleukin-5 inhibitor was used for further treatment of iHES. Discussion: Only 6% of patients with iHES present with cardiac symptoms, however one in five eventually develop cardiac manifestations that are often rapidly progressive. Prompt treatment with glucocorticoids is indicated for severe presentations, and subsequent use of targeted therapies can dramatically reduce morbidity and mortality. Recognition of severe peripheral eosinophilia should prompt a broad hematologic, infectious, and auto-immune workup to guide treatment and prevent end-organ damage.

S2431 Ulcerative Colitis Flare-Ups Following mRNA COVID-19 Vaccination

Introduction: Ulcerative colitis (UC) is an autoimmune disease commonly managed with immunosuppressants. However, its put UC patients at a higher risk for infections. Therefore, vaccination is an important part of management. Messenger RNA (mRNA) vaccine is a new type of vaccine only widely used against COVID-19. The effect of mRNA vaccines on UC patients on immunomodulatory treatement is relatively unknown. Here, we present the first case of UC flare-up after receiving the SARS-CoV-2 mRNA vaccine. Case description/methods: A 36-year-old female with past medical history of ulcerative colitis, autoimmune pancreatitis presented with intermittent lower abdominal pain and diarrhea for 2 weeks with fever for 1 day. Her last flare-up was 3 years ago before she was put on Tofacitinib. She never required hospitalization. Her symptoms started the second day after she received the second dose of Pfizer COVID-19 vaccine. She had daily 5-10 times of non-bloody diarrhea and abdominal cramps. Her symptoms subsided after 7 days of initial onset. On day 11, she started to have daily up to 15 times bloody bowel movements, along with nausea, vomiting, unable to maintain oral intake. Day 17 after vaccination, patient went to ED after fever of 100.5F. On presentation, she is afebrile, WBC 11.9, CRP 61.8, calprotectin 1840, Clostridioides difficile negative, GI Panel negative. CT showed severe colitis involving the left colon from splenic flexure to rectum. Colonoscopy showed erythematous, granular, hemorrhagic, and ulcerated mucosa in the entire examined colon. Terminal ileum, cecum, ascending, transverse, descending, sigmoid and rectum biopsies were negative for CMV, showed chronic active colitis. Patient received intravenous Methylprednisolone, then transited to oral Prednisone taper and resumed her Tofacitinib. Her symptoms gradually improved. Discussion: To our best knowledge, no report has shown the association of mRNA vaccine and UC flare-up. In general, non-live vaccines are safe for UC patients regardless of immunosuppressants usage. Live vaccines are also contraindicated Tofacitinib users like our patient. In the Pfizer COVID-19 vaccine trial, patients on immunosuppressive therapy were excluded. Given the temporal relationship between the vaccine and her flare-up in the setting of long-term remission history, we are concerned that the stress induced by the mRNA vaccine may increase the risk of flare-up. More studies need to be done to evaluate the safety profile of mRNA vaccine for UC patients on immunosuppressants.Figure 1.: A: Nodular mucosa in second portion of duodenum. B: Multiple ulcers in terminal ileum. C and D: 1.7 cm segment of terminal ileum circumferential bowel wall thickening with mucosal hyperenhancement and submucosal edema.

Reduction of Chronic Steroid Use in Patients Discharged for Inflammatory Bowel Disease Flares: A 28-Month Quality Improvement Study

Patients hospitalized with inflammatory bowel disease (IBD) flares are at risk of chronic steroid use when they are discharged without a clear prednisone tapering plan (and steroid-sparing treatment strategy) or gastroenterology follow-up. Our quality improvement (QI) project examines the impact of a new hospital-wide 6-week standardized oral prednisone taper electronic order set.

MOG-IgG Associated Disease (MOG-AD) in Adults

This review offers a look into available treatment strategies for patients with MOG-IgG associated disease (MOG-AD). We will focus on evidence-based management options in the acute setting. We will also present recommendations for initiation of long-term immunotherapy for patients with a relapsing course and discuss our approach for monitoring disease progression and escalation of therapy. As commercial testing for MOG-IgG did not become widely available until 2016, most of the evidence concerning management is from retrospective observational studies. Acutely, 1 g daily of intravenous methylprednisolone (IVMP) for 3–5 days has been associated with better outcomes for optic neuritis. This is typically followed by a prolonged oral prednisone taper. Long-term immunotherapy is reserved for patients with relapsing or severely disabling disease. There are yet to be any prospective trials for selection of the most appropriate agent. Rituximab, azathioprine, mycophenolate mofetil, and IVIG have all been used. Disease-modifying therapies (DMTs) typically used for multiple sclerosis (MS) are avoided. IVMP followed by a prolonged prednisone taper is the mainstay of acute management. However, there is limited consensus on the best long-term immunotherapy and prospective studies are needed to compare the efficacy of different agents. The development of targeted therapies is expected in the future as the pathogenesis of MOG-IgG becomes better understood.