Course Of Prednisone Research Articles

TRACHEAL AND BRONCHIAL STENOSIS CAUSED BY CHRONIC UNCONTROLLED GASTROESOPHAGEAL REFLUX DISEASE

TRACHEAL AND BRONCHIAL STENOSIS CAUSED BY CHRONIC UNCONTROLLED GASTROESOPHAGEAL REFLUX DISEASE

S1787 Steroid-refractory IgG4-Related Disease Presenting as Sclerosing Cholangitis

Introduction: IgG4-related disease (IgG4-RD) is characterized by tumefactive infiltration of IgG4-bearing plasmablasts and other lymphocytes, along with storiform fibrosis, into one or multiple organs, with resultant organ enlargement, fibrosis, and dysfunction. Although initial reports of IgG4-RD concerned involvement of the pancreas, other organs such as the biliary tract can be involved. We present a case of steroid refractory IgG4- RD sclerosing cholangitis (IgG4-SC). Case Description/Methods: A 67-year-old man with hypertension presented with a 2-week history of unintentional weight loss, painless jaundice, and pruritus. Exam showed jaundice and scleral icterus. Laboratory revealed elevated liver tests with an obstructive pattern, with ALP, GGT, & Bilirubin of 431 U/L, 438 IU/L, 17.6mg/dL, respectively. MRCP, ERCP, and subsequent EUS demonstrated diffuse pancreatic parenchymal hypo-echogenicity, long segment strictures at the distal common bile duct with proximal duct dilation, dilated common hepatic duct, and multiple segmental intrahepatic biliary duct strictures. Biliary sphincterotomy and stent placement resolved symptoms. Biopsy of the Ampulla of Vater revealed chronic inflammation with ulceration. Further work-up uncovered an elevated serum IgG4 of 266 mg/dL with a negative ANA, SMA, AMA, and tumor markers. He was then diagnosed with IgG4-SC and completed a course of prednisone. Relapse of symptoms prompted a repeat ERCP which uncovered biliary tree pruning and irregularity, necessitating biliary stent replacement. Due to poor tolerance with rituximab infusion, he was treated with Mycophenolate Mofetil which accomplished a year of disease quiescence. Discussion: The prime gastrointestinal manifestations of IgG4-RD include type 1 Autoimmune Pancreatitis and less commonly, IgG4-SC. Management involves high dose steroids for 4 weeks with a slow taper over years to prevent permanent fibrosis. Patients are at highest risk of relapse during steroid taper or within 6 months of completion, with a relapse rate of 15-60%. Although our patient did not have the classic predictive factors of relapse, such as proximal strictures or serum IgG4 >280 mg/dL, he had a suboptimal response to steroids which prolonged his clinical course and exposed him to repeated procedures. There is still a need for further studies regarding the role for possible combination therapy (steroids & immunosuppressive therapy) to prevent relapse in high-risk individuals.

S3026 Hepatic Sarcoidosis Hiding Beneath Mesenteric Panniculitis

Introduction: Mesenteric panniculitis is a rare inflammatory disease that affects the adipose tissue of intestinal mesentery. The incidence of mesenteric panniculitis ranges from 0.16%-3.4%. Though its etiology is unknown, it has been linked to a range of conditions including vasculitides, malignancies, abdominal trauma, and autoimmune diseases. We present a case of a 32-year-old male with a history of chronic abdominal pain and unintentional weight loss who presented with worsening transaminitis eventually found to have mesenteric panniculitis and hepatic sarcoidosis. Case Description/Methods: A 32-year-old male with a history of sarcoidosis and pulmonary embolism, not compliant with medication, presented to the emergency department with a six-month history of sharp, intermittent, and diffuse abdominal pain. He denied any fevers, nausea, vomiting, diarrhea, changes in medications or diet, recent travel, or sick contacts. He also endorsed a 20-pound weight loss. On presentation, he was tachycardic to 121 and febrile to 100F. Labs were significant for a hemoglobin of 8.2, AST 105 U/L, ALT: 81U/L, and ALP 51 U/L. On physical exam, the abdomen was diffusely tender to palpation in the epigastric region and right upper quadrant. He was started on IV Ceftriaxone. Computed Tomography (CT) of the abdomen and pelvis revealed a hazy appearance of mesentery with numerous mesenteric lymph nodes suspicious of mesenteric panniculitis, hepatomegaly, multistational upper abdominal lymphadenopathy, and a 2.0 cm ill-defined hepatic lesion (Figure 1). Percutaneous liver biopsy revealed hepatic sarcoidosis and mesenteric panniculitis. He was initiated on a two-week tapered course of prednisone. Azathioprine was later added with marked improvement in his symptoms. Discussion: Mesenteric panniculitis is a rare fibroinflammatory condition of unknown etiology. There is limited understanding of the pathogenic mechanism, but it has been linked to autoimmune processes. Presenting symptoms are vague and highly variable, but abdominal pain is reported to be the most common. CT scan is the imaging modality preferred, however, diagnosis is established by histological confirmation. No specific treatment exists, but steroids have demonstrated promising results. Our case illustrates the need for maintaining a high index of suspicion for mesenteric panniculitis when evaluating patients with a history of autoimmune disease presenting with vague abdominal pain in addition to transaminitis.Figure 1.: CT scan (axial view) showing focal lesion in the liver and mesenteric lymphadenopathy.

S3191 TNF Inhibitor-Induced Liver Injury: Clinical Presentations and Outcomes

Introduction: Inflammatory bowel disease (IBD) is commonly treated with a sub-set of medications that inhibit tumor necrosis factor (TNF), an integral component during the pro-inflammatory phase. However, like all medications, TNF inhibitors are not without adverse side effects, one of them being drug induced liver injury (DILI). Although commonly described in literature, clinical presentation is often not encountered or recognized. We describe the cases of two individuals with IBD and the development of DILI secondary to TNF inhibition. Case Description/Methods: Our first case describes a 40-year-old female with ulcerative colitis who was started on adalimumab. Within 8 months of starting, aminotransferases were found to be elevated. Abdominal ultrasound showed hepatic steatosis, Fibroscan CAP 100, E Score 12kPa, and liver biopsy was consistent with autoimmune pattern liver injury with focal periportal fibrosis. Her only symptom was constipation. Adalimumab was discontinued and a short course of prednisone was started with improvement in aminotransferase elevations. Our second case is a 25-year-old female with Crohn’s Disease who was started on infliximab. Within 6 months, aminotransferases were found to be elevated. Liver biopsy showed portal-based chronic hepatitis with mild activity suspicious for DILI. Autoimmune hepatitis workup including ANA (640), A-SMA (negative), AMA (negative), Anti-dsDNA (60-borderline), IgG (1713) was not convincing but autoimmune hepatitis due to anti-TNF activity could not be excluded. At this time, infliximab was discontinued, and the patient began to have an improvement in clinical presentation. Discussion: All the TNF inhibitors currently marketed have been associated with DILI. However, the optimal management of liver injury related to TNF inhibitors is still a matter of debate. Some practitioners recommend the discontinuation of treatment in the case of elevated aminotransferase levels or the occurrence of jaundice. Others have recommended the continuation TNF inhibitor in the setting of similar clinical and laboratory findings with hopeful resolution of liver injury. Too often, the clinical signs or evidence of DILI is occult and can go unnoticed. Further research into best practice outcomes when IBD patients are taking such medications is needed. Additionally, better guidelines for the use and management of TNF inhibitors in IBD patients is imperative to minimize the risk of unchecked drug induced liver injury.

S3137 Post-COVID-19 Cholestatic Hepatitis

Introduction: Coronavirus Disease 2019 (COVID-19) caused by SARS-COV-2 has led to a global crisis of unprecedented reach and proportion. In addition to the pulmonary consequences, the incidence of liver injury is increasingly being reported. Understanding the hepatotropism of SARS-COV-2 is of paramount importance as it can lead to a severe COVID-19 course. This case details a unique presentation of cholestatic hepatitis likely a sequela of a severe SARS-COV-2 infection in our patient. Case Description/Methods: A 49-year-old lady with a history of seizure disorder, migraines, hypertension, and recent six-week ICU admission for COVID illness requiring mechanical ventilation (ECMO), presented to our gastroenterology clinic for a post-discharge follow-up. During admission, she consistently had an abnormal elevation of liver enzymes (Peak ALP 2000, AST, ALT 300, Bilirubin 2.5). MRCP was normal and a liver biopsy showed marked intralobular bile duct injury showing severe cytologic atypia, periductal edema, periductal and intraductal neutrophilic, and lymphocytic inflammation and marked ductular reaction, focal mild hepatocellular cholestasis, Kupffer cell hyperplasia. She had no prior history of chronic liver disease and LFT was normal at baseline. Post discharge LFT showed alkaline phosphatase of 1514 with total bilirubin 1.4. Infective (Hepatitis A, B, C panel), Inflammatory/Autoimmune (ANA, ASMA, AMA, P-ANC, Anti-SLA/LP, Anti ds-DNA, ALKM-1, and ALKM-3) workup was negative. Repeat imaging with USG and MRI was unremarkable. She received a course of prednisone after discharge with no improvement in LFT, it was tapered off. She is currently asymptomatic 6 months after acute illness but continues to have persistently elevated liver enzymes with a gradual downtrend with the latest being ALP 490, AST 86, ALT 95, and T. Bilirubin 0.8. Discussion: We describe a unique case of post-COVID-19 cholestatic hepatitis with persistently elevated liver biochemistry with unclear etiology despite extensive workup. It could be a confluence of Secondary Cholangitis in Critically Ill Patients (SSC-CIP) and direct hepatic injury from COVID-19. Further data is needed to understand the patterns of liver injury due to COVID and the development of possible treatment for this newly described condition associated with the virus.

The Effect of Prednisone Dose on the Clinical and Laboratory Course of Henoch–Schönlein Purpura in Children

Objective: Henoch–Schönlein purpura (HSP) is the most common form of systemic vasculitis in children. Most cases are self-limited. Corticosteroids may be considered for HSP patients with severe renal or extrarenal involvement. However, they have some adverse effects related to the dose and the period of treatment. The study aimed to assess the efficacy and safety of dose 1mg/kg/day of prednisone in the treatment of HSP clinical and laboratory manifestations compared with dose 2 mg/kg/day. Methods: A total of 28 patients (14 treated with prednisone 1 mg/kg and 14 treated with prednisone 2 mg/kg, followed up for 6 months) were included in a prospective randomized trial study, at University Children Hospital in Damascus, Syria, between 2020 and 2021. Results: As prednisone was only used in severe manifestations, severe abdominal pain was the most common indication (53.5%), followed by gastrointestinal hemorrhage (32.1%). However, prednisone effect on all symptoms and laboratory findings for all patients was studied. Both doses of prednisone (1 and 2 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) were effective in reducing the intensity of extrarenal symptoms, and no statistical difference was found (rash: P = 0.47, abdominal pain: P = 0.32, gastrointestinal hemorrhage: P = 0.32, arthritis: P = 0.84, and soft-tissues edema: P = 1). In both groups, new renal symptoms and gastrointestinal complications were developed. Elevated inflammatory markers (CRP: 59.3%, ESR: 77.3%) turned up normal at the end of treatment in both groups. Children treated with prednisone 2mg/kg showed increased appetite; three cases developed Cushing’s moon face; one case showed a transient hypertension at the end of treatment. Conclusions: The routine, early use of prednisone in uncomplicated HSP is not recommended. However, when it is indicated, a 2-week course of prednisone 1 mg/kg/day could be an effective alternative to the higher dose (2 mg/kg/day), with less side effects.

Treatment With Cytarabine at Initiation of Therapy With Cyclosporine and Glucocorticoids for Dogs With Meningoencephalomyelitis of Unknown Origin Is Not Associated With Improved Outcomes.

Meningoencephalomyelitis of unknown origin (MUO) is a common disorder of dogs that results in significant morbidity and mortality. The ideal treatment regimen is not known but a second immunosuppressive agent is often utilized in combination with glucocorticoids to increase efficacy and reduce side effects. Recently, a benefit to using a cytosine arabinoside (CA) constant rate infusion (CRI) at the time of diagnosis has been demonstrated. Here, a retrospective study was performed to determine if administration of CA at the time of diagnosis would alter prognosis in dogs receiving cyclosporine and prednisone for treatment of MUO. Medical records of 51 client-owned dogs diagnosed with MUO at one institution were reviewed (2009-2019). All dogs were treated with cyclosporine and a tapering course of prednisone. Twenty-one dogs received a single initial 200 mg/m2 treatment with CA either as a CRI or subcutaneously. Significantly more patients in the CA treatment group were obtunded on presentation but all other baseline parameters were similar between groups. No differences in success (defined as sustained improvement on neurological exam with owner perceived good quality of life), relapse, or death were identified at 1-, 3-, 6-, 9-, 12-, 18-, or 36-month time points. These results do not support treatment with CA (either as a CRI or subcutaneously) at the time of diagnosis in dogs treated with cyclosporine and prednisone.

Evaluation of Tumor Grade and Proliferation Indices before and after Short-Course Anti-Inflammatory Prednisone Therapy in Canine Cutaneous Mast Cell Tumors: A Pilot Study.

Glucocorticoid administration is a common clinical practice that attempts to decrease the inflammation associated with and improve the resectability of canine mast cell tumors (MCTs). However, the impact of neoadjuvant glucocorticoids on the histological features and proliferation indices of canine MCTs is unknown. The objective of this study was to evaluate changes in tumor grade, mitotic count, Ki67, AgNOR, and AgNORxKi67 scores following short-course anti-inflammatory neoadjuvant prednisone in canine patients with MCTs. This was a prospective single-arm pilot study. Client-owned dogs with treatment-naïve cytologically confirmed MCTs were enrolled. Patients underwent an initial incisional biopsy followed by a 10–14-day course of anti-inflammatory prednisone and surgical resection. All histological samples were randomized, masked, and evaluated by a single pathologist. Unstained paired pre- and post-treatment samples were submitted to a commercial laboratory for Ki67 and AgNOR immunohistochemical analysis. There were 11 dogs enrolled with 11 tumors. There were no statistical differences between the pre- and post-treatment histological parameters of mitotic index, Ki67, AgNOR, or Ki67xAgNOR. There were no clinically significant alterations between pre-treatment and post-treatment in the assignment of tumor grades. A short course of anti-inflammatory prednisone does not appear to alter the histological parameters that affect grade determination or significantly alter the proliferation indices in canine MCTs.

Never Feed Birds in the Hot Tub! A Case of Bird-Fancier's Lung

A 50-year-old female non-smoker with a past medical history of rheumatoid arthritis and asthma presented to the pulmonology clinic with a chief complaint of acute on chronic exertional dyspnea for 6 months. The patient was discharged from the hospital 2 weeks prior and was given the diagnosis of acute hypoxemic respiratory failure secondary to asthma exacerbation. She was discharged with a 7-day course of prednisone, which temporarily improved her dyspnea, and an outpatient referral was placed.

A RARE CASE OF BRONCHOLITHIASIS PRESENTING WITH CHRONIC COUGH AND MEDIASTINAL ABSCESS

TYPE: Case Report TOPIC: Disorders of the Mediastinum INTRODUCTION: Broncholithiasis is a rare condition characterized by the erosion of calcified material into the tracheobronchial tree. We present a case of broncholithiasis complicated by mediastinal abscess. CASE PRESENTATION: A 58-year-old male presented with chronic cough and lithoptysis. Chest PET-CT demonstrated calcified right infrahilar and subcarinal soft tissue with avid FDG uptake. Infectious workup was negative, including histoplasma and mycobacterium testing. Bronchoscopy demonstrated mucosal inflammation around the bronchus intermedius. Endobronchial biopsies and EBUS-TBNA were negative for malignancy. Symptoms persisted despite multiple courses of antibiotics and prednisone. Repeat CT showed evolving mediastinal fluid collection and gas. The patient underwent right lower lobectomy and mediastinal lymphadenectomy which showed broncholithiasis, necrotizing granulomas as well as fibrinous and organizing inflammation. Following surgery, he developed right hydropneumothorax requiring chest tube placement with polymicrobial growth consistent with empyema. He was treated with IV antibiotics with subsequent resolution of his symptoms. DISCUSSION: Broncholithiasis is an exceedingly rare cause of chronic cough. Tuberculous and histoplasma necrotizing granulomatous lymphadenitis are the most common causes. Lithoptysis is an uncommon, but a unique symptom of broncholithiasis. Mediastinal abscess is a rare complication as seen in this case. Diagnosis is established by CT imaging and bronchoscopy plays and important role. Depending on disease severity, management options range from observation to bronchoscopic extraction to surgical resection. Caution is advised when bronchoscopic treatment is considered due to the risk of massive bleeding upon manipulation of broncholiths. CONCLUSIONS: Broncholithiasis is a rare complication of mediastinal granulomatous processes that can present with chronic cough, hemoptysis as well as mediastinal abscess. DISCLOSURE: Nothing to declare.

POS0266 EFFECTIVENESS OF A SPACING-UP STRATEGY AFTER ONE-YEAR COURSE OF WEEKLY TOCILIZUMAB IN PATIENTS WITH GIANT CELL ARTERITIS: A SINGLE-CENTRE PROSPECTIVE STUDY

BackgroundAlthough tocilizumab (TCZ) is extensively used for the treatment of giant cell arteritis (GCA), there is still uncertainty with regard to optimal treatment duration. The GiACTA open-label extension phase showed that 58% of patients treated with a 12-month course of weekly TCZ had a disease flare in the 2 years following therapy suspension1, suggesting that some patients may need a more prolonged duration of this therapy.ObjectivesTo evaluate efficacy and safety of low-dose TCZ maintenance in GCA patients who achieved remission after one year of standard TCZ therapy.MethodsGCA patients eligible for TCZ according to the 2018 EULAR recommendations2 and who achieved remission after one year of weekly subcutaneous TCZ were prospectively enrolled. TCZ was administered every-other-week (EOW) for additional 12 months, and eventually suspended. Patients were evaluated 4-monthly during TCZ therapy, and 1 and 6 months after TCZ suspension (Figure 1). In patients with large-vessel (LV) involvement at baseline, PET scan was performed 12 and 24 months after TCZ start. Primary outcome was relapse-free survival at month 6 since TCZ suspension. Relapse-free survival during TCZ therapy and imaging response at PET scan were also evaluated. Adverse events were recorded.Figure 1.Study design.Results17 patients were enrolled (12 women, 71%; mean age 71.5±8.7 years). Disease features at diagnosis and TCZ start are listed in Table 1. Reasons for TCZ start were clinical or imaging disease flare (n=9), persistence of disease activity (n=5), and steroid-related adverse events (n=3). At TCZ start, median disease duration was 8 (3-22) months, serum C-reactive protein (CRP) was 13 (6-22) mg/L, daily prednisone (PDN) dose was 25 (15-37.5) mg; 4 patients were already on methotrexate (MTX) which was maintained in 1 of them; 10 patients (59%) had LV involvement on PET scan. At TCZ EOW start, no patient was on PDN and 1 patient was on MTX; MTX was added in another patient due to persistence of LV involvement at PET scan. All patients completed the 24-month TCZ course. Two patients (12%) had a polymyalgic flare while on EOW TCZ: one patient at month 1 and one patient at month 6; of note, one of them had a polymyalgic flare also while on weekly TCZ. Both flares were managed with a PDN course with complete clinical remission. No patient had active LV involvement at 24-month PET scan. Adverse events on EOW TCZ were zoster reactivation (n=1) and neutropenia (n=1). At TCZ stop, no patient was on PDN and 2 patients were on MTX. One month after TCZ stop, all patients were in remission. Six months after TCZ stop, 4 patients (24%) experienced a flare, which was successfully managed in all cases with TCZ weekly re-introduction; in 2 patients, a PDN course was also started.Table 1.Patients’ characteristics at giant cell arteritis diagnosis and at study entry.GCA diagnosis, n=17n (%)TCZ start, n=17n (%)Headache16 (94)5 (29)Scalp tenderness11 (65)5 (29)Jaw claudication9 (53)2 (12)Ocular ischemic manifestations3 (17)0 (0)Polymyalgia rheumatica8 (47)0 (0)Constitutional symptoms14 (82)7 (47)Active vasculitis on PET10 (59)10 (59)Modality of diagnosisVascular ultrasound7 (47)-Temporal artery biopsy3 (17)-PET10 (59)10 (59)ConclusionIn this proof-of-concept study, low-dose TCZ maintenance in GCA showed excellent disease control, which was maintained in most patients after therapy suspension. Longer follow-up and replication in larger cohorts are required.

Effect of short-term corticosteroid usage on acute urinary toxicity following Cs-131 prostate brachytherapy

To evaluate short-term patient reported urinary quality of life scores in patients with prostate cancer treated at our institution with and without perioperative prednisone following Cesium-131 (131Cs) prostate LDR brachytherapy. We started routinely using a perioperative 7-day course of prednisone at a dose of 5 mg per day, beginning 1 day prior to 131Cs prostate LDR brachytherapy from 2013 with goal of improving acute urinary symptomatology. One hundred consecutive patients treated with prednisone were selected, with comparison to 100 consecutive patients who were not treated with prednisone. We analyzed for differences in mean change with standard deviation (SD) in EPIC and AUA scores at 0.5-1 month and 3 months with or without prednisone by Mann-Whitney U Test. Binary logistic regression was performed to assess for impact of prednisone on postoperative urinary catheter use. Pretreatment EPIC and AUA scores were available in 197 patients. Less reduction in EPIC US score was noted at 0.5-1.0 month in the group who received prednisone with mean change of -22.9 (SD 15.4) when compared to the group who did not receive prednisone with mean change of -31.7 (SD 19.3), p < 0.01, with significance lost at 3 months. There was no significant difference in acute urinary retention requiring postoperative urinary catheter placement with perioperative prednisone (OR 1.13, p = 0.71). A short course of perioperative low-dose prednisone was associated with less severe worsening in urinary symptoms by the EPIC questionnaire at the 0.5-1.0-month timepoint suggesting some improvement in acute urinary quality of life, although differences did not remain statistically significant at 3 months.

54-Year-Old Man With Acute Dyspnea on Exertion

54-Year-Old Man With Acute Dyspnea on Exertion

Transition to Virtual Asthma Care During the COVID-19 Pandemic: An Observational Study

BackgroundThe COVID-19 pandemic increased reliance on virtual care for patients with persistent asthma.ObjectiveThis retrospective cohort study assessed changes from in-person to virtual care during the pandemic. In patients with persistent asthma, compared with the same period before the pandemic.MethodsKaiser Permanente Colorado members aged 18 to 99 years with persistent asthma were evaluated during two periods (March to October 2019 and March to October 2020). Comparison of asthma exacerbations (hospitalizations, emergency department visits, and courses of oral prednisone) and asthma medication metrics were evaluated between the two periods and by type of care received during the pandemic (no care, virtual care only, in-person care only, or a mix of virtual and in-person care). Population characteristics by type of care received during the pandemic were also evaluated.ResultsAmong 7,805 adults with persistent asthma, those who used more virtual care or sought no care during the pandemic were younger and had fewer comorbidities, mental health diagnoses, or financial barriers. Exacerbations decreased (0.264 to 0.214; P <.001) as did courses of prednisone (0.213 to 0.169). Asthma medication adherence (0.53 to 0.54; P <.001) and the asthma medication ratio, a quality-of-care metric (0.755 to 0.762; P = .019), increased slightly. Patients receiving a mix of in-person and virtual care had the highest rate of exacerbations (0.83) and a lower asthma medication ratio (0.74) despite having the highest adherence (.57).ConclusionsDespite an increase in virtual care, asthma exacerbations decreased except among individuals who received both in-person and virtual care, likely because they had more severe disease.

As-Needed Use of Short-Acting β2-Agonists Alone Versus As-Needed Use of Short-Acting β2-Agonists Plus Inhaled Corticosteroids in Pediatric Patients With Mild Intermittent (Step 1) Asthma: A Cost-Effectiveness Analysis

Although the efficacy of the as-needed use of short-acting β2-agonists (SABAs) plus inhaled corticosteroids (ICS) for treating children with mild intermittent asthma has been demonstrated, evidence of its cost-effectiveness is scarce. The aim of the present study was to compare the cost-effectiveness of the as-needed use of SABAs alone versus the as-needed use of SABAs plus ICS in children 5 to 11 years old with mild intermittent (step 1) asthma but suffering from an exacerbation of asthma symptoms. A decision-analysis model was adapted. Effectiveness parameters were obtained from a randomized clinical trial. Cost data were obtained from hospital bills and from the national manual of drug prices in Colombia. The study was carried out from the perspective of the national health care system in Colombia. The main outcome of the model was a first course of prednisone for an asthma exacerbation (AE). Compared with the use of SABAs alone, the as-needed use of SABAs plus ICS was associated with lower overall treatment costs (US$17.99 vs US$27.94 mean cost per patient) and a higher probability of a lack of a requirement for a first course of prednisone (0.6500 vs 0.5100), thus showing dominance. In Colombia, compared with the use of albuterol alone, the use of beclomethasone dipropionate added to albuterol as needed for symptom relief is cost-effective in children 5 to 11 years old with mild intermittent (step 1) asthma, because it involves a higher probability of a lack of a requirement for prednisone for AE at lower total treatment costs.

A258 ROSAI-DORFMAN0-DESTOMBES DISEASE: A RARE CAUSE OF OBSTRUCTIVE JAUNDICE

BackgroundRosai-Dorfman-Destombes Disease (RDD) is rare histiocytic disorder that is most frequently seen in children and young adults. Gastrointestinal involvement is reported in <1% of cases and typically involves the small bowel and colon. Pancreatic and hepatic involvement has been previously reported but is extremely rare.AimsTo describe a case of obstructive jaundice in the setting of a very rare histiocytic disorder known as RDD.MethodsCase ReportResultsA 59-year old previously healthy male of Asian descent presented with obstructive jaundice. Initial imaging demonstrated intra and extrahepatic biliary duct dilation with concurrent diffuse enlargement of the pancreas compatible with autoimmune pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) was performed with stenting and biopsy. ERCP demonstrated a distal common bile duct stricture with biopsies suggestive of low grade reactive changes and inflammation. Subsequent endoscopic ultrasound (EUS) guided biopsy of the pancreas showed active and chronic inflammation, necrosis, and atrophic pancreatic tissue, with no definitive evidence of autoimmune pancreatitis (Figure 1). Ca-19-9 and IgG4 were normal. He was treated with a course of prednisone and responded well, with repeat CT imaging showing almost complete resolution of the previously demonstrated pancreatic changes. A diagnosis of autoimmune pancreatitis was made. He re-presented 6-months later, however, with fatigue and repeat imaging now displayed lymphadenopathy in the neck, chest, and abdomen, and a bulky pancreatic head with associated hepatomegaly. Lymph node excisional biopsy confirmed the diagnosis of RDD with the presence of scattered histiocytic cells showing emperipolesis with a low number of IgG4 positive cells (Figure 1). The patient was promptly initiated on prednisone and rituximab and has since then had excellent clinical response.ConclusionsRDD is a rare non-Langerhans cell histiocytosis of unknown etiology that has a prevalence of 1:200 000. RDD clinically presents with painless bilateral cervical lymphadenopathy and can manifest with both nodal and extra nodal involvement. The most common sites of extra nodal disease are the skin and central nervous system, but rarely, can also present with pancreatic involvement. The use of fine needle guided biopsy in diagnosing RDD with extra nodal disease can be limited by low yield, sclerotic tissue, or non-diagnostic findings. For this reason, RDD with pancreatic involvement can masquerade as autoimmune pancreatitis, pancreatic malignancy and IgG4-related disease.This case report raises awareness about RDD with pancreatic and biliary involvement, a rare entity, that can present with obstructive jaundice. Figure 1: Histological sections of lymph node(left) with hystiocytic cells showing emperipolesis(arrow) and pancreas(right) showing active and chronic inflamation, necrosis and atrophyFunding AgenciesNone

Subungual hematomas after mRNA Covid-19 vaccine administration.

Large bilateral subungual haemorrhages and hematomas were observed in an adult woman after receiving the second dose of a mRNA COVID-19 vaccine. Improvement was achieved after a course of prednisone. Unusual, rare cutaneous adverse effects are likely to be increasingly noticed as the accelerated massive worldwide COVID-19 vaccination campaign intensifies.

P371 The addition of intravenous, high dose, bolus of methyl-prednisolone increases the early clinical response to oral corticosteroids in moderately active ulcerative colitis. Preliminary results of a prospective, controlled, multicentre, randomised, open-label study

Abstract Background Oral corticosteroids remain the treatment of choice for moderately active flares of ulcerative colitis (UC). In early controlled studies, oral corticosteroids achieved clinical remission rates of, 30–60% at, 30 days. Some small prospective studies observed that the administration of high-dose intravenous bolus of methyl-prednisolone accelerated their therapeutic action and increased the rate of clinical response. We aimed to evaluate if the outpatient administration of intravenous, high-dose, methyl-prednisolone bolus increases the efficacy of a conventional course of oral prednisone in moderately active UC. Methods Prospective, multicentre, controlled, randomized, open-label study comparing the therapeutic efficacy of a standard oral prednisone schedule with the same schedule preceded by a three-day regimen of high-dose intravenous bolus of methyl-prednisolone. Inclusion criteria were: Moderately active, left-sided/extensive UC (total Mayo score, 6–10), with no prior exposure to immunosuppressants or biologics, and no corticosteroid treatment in the last, 6 months. Patients were randomized to oral prednisone, 60mg/day (ORAL group) or the same schedule preceded by intravenous bolus of, 500mg of methyl-prednisolone for three consecutive days (BOLUS group), stratified by the baseline use of oral, 5ASA and UC onset. Early clinical response was defined as the reduction of the baseline partial Mayo Score (pMS) by at least three points with at least a decrease by, 1 point in rectal bleeding and with an absolute value of, 0 or, 1, seven days after starting treatment (EUDRA CT:, 2016-001170-15)(Clinicaltrials.gov: NCT02921555) Results In all, 71 patients (38 ORAL, 33 BOLUS) were included. At baseline, the median pMS was, 6 (IQR, 5–7). In, 21% of the patients, the index flare was the onset of UC and, 68% had never received systemic corticosteroids for UC. There were no differences in baseline clinical and demographic features between the two study groups. At day, 7, the proportion of patients fulfilling early clinical response criteria was similar in both groups (BOLUS, 63% vs. ORAL, 58%; p = 0.6), but a significantly greater reduction in pMS at day, 3 (-3.4. vs -1.7; p &amp;lt;0.0001) and a significantly higher proportion of patients in clinical remission (pMS &amp;lt;2) at day, 7 (57% vs, 29%; p = 0.017) were observed in the BOLUS group. Conclusion The addition of three high-dose intravenous bolus of methyl-prednisolone to a standard schedule of oral corticosteroids achieves a faster and more intense response in the short-term in moderately active flares of UC, but it does not increase the clinical response rate at seven days. The impact of these effects on the medium-term outcomes is still to be assessed.

Fractional Exhaled Nitric Oxide Response to Oral Corticosteroids in Children With Mild-to-Moderate Asthma: Influence of Race

To evaluate the magnitude of reduction in fractional exhaled nitric oxide (FeNO) after an oral corticosteroid (OCS) course in children with mild-to-moderate asthma and assess for any differential response from such treatment on the basis of race.In the study, the researchers included 92 children (aged 11 ± 3.3 years; White: n = 46 [50%]; Hispanic: n = 30 [33%]; African American [AA]: n = 16 [7%]) who presented to 1 urgent care in Denver, Colorado, for an acute asthma exacerbation from June 2010 to May 2011.In this prospective cross-sectional cohort study, children aged 7 to 18 years with a physician diagnosis of mild-to-moderate persistent asthma and FeNO measurements within the past 6 months who received a short course of prednisone for acute exacerbation were enrolled. FeNO measurements were obtained at the time of exacerbation and at a 1 week follow-up.At baseline, AA children had significantly higher mean FeNO values, compared with that of both White (48.9 vs 25.6 parts per billion; P < .5) and Hispanic children (22.5 parts per billion; P < .5), despite similar inhaled corticosteroid doses. During exacerbation, AAs had the highest FeNO values, without a difference in lung function compared with that of non-AAs. After OCSs, there was a 56.6% reduction in FeNO among all children, whereas the relative reduction was similar between that in AAs and non-AAs (53.9% vs 57.8%).FeNO values were reduced by >50% after a short course of OCSs among all children. AA children showed higher levels of FeNO at baseline and after treatment of exacerbation, although the relative reduction in FeNO was similar among groups.FeNO is a noninvasive tool for evaluating type 2 airway inflammation and predicting responses to inhaled corticosteroids, but less is known about how FeNO levels change in acute exacerbation or from OCSs. This study reveals clearly that such inflammation is likely alleviated by OCSs across racial and ethnic groups. Given the significantly higher FeNO levels in AA children, however, this also suggests that further research into racial differences in FeNO and how they are clinically correlated is warranted. It may help to explain disparities in health outcomes seen among AA children with asthma or guide therapies. Knowledge of participant environmental factors would be helpful in assessing the significance of the baseline results.

NCMP-08. CHALLENGING DIAGNOSIS AND MANAGEMENT OF PCNSL IN AN IMMUNOSUPPRESSED PATIENT ON MYCOPHENOLATE MOFETIL FOR MYASTHENIA GRAVIS

Abstract INTRODUCTION Primary CNS lymphoma (PCNSL) typically presents with non-focal neurologic symptoms including disorientation, poor balance and memory and singel or multifocal periventricular MRI lesions. Deviations in characteristic findings can delay diagnosis and require additional diagnostic tests. OBSERVATION A 68-year-old man with a recent zoster infection and acetylcholine receptor antibody positive myasthenia gravis (MG) on Mycophenolate Mofetil for 22 years presented at another institution with left eye vision changes, and focal neurological deficits. A brain MRI showed an enhancing lesion within his left medulla extending to the cerebellum. Cerebrospinal fluid (CSF) analysis was positive for EBV and negative for malignancy. He was diagnosed with VZV vasculopathy and discharged home on IV Acyclovir for 14 days and a 5-day course of oral prednisone 60 mg. Three months later, a repeat brain MRI showed multiple new enhancing lesions bilaterally along the periventricular white matter with involvement of the corpus callosum with several lesions in peripheral locations of the cerebrum, cerebellum, and brainstem. He presented to local ER with intermittent encephalopathy, acute left eye vision blurriness and was started on steroids. He was transferred to our institution and had CSF analysis which was positive for EBV and negative for malignancy. Due to rapid progression of his symptoms, he underwent gross total resection of the left frontal lesion which showed EBV-induced diffuse large B-cell lymphoma (DLBCL). His Mycophenolate Mofetil was discontinued and he had a dramatic improvement in his left eye vision and cognitive deficits within 24 hours after one dose of Rituximab IV 500 mg/m2. DISCUSSION In the setting of periventricular lesions and EBV positivity on CSF, EBV-induced DLBCL should be highly considered. CONCLUSION Misdiagnosis or delay in diagnosis of PCNSL due to the presence of atypical features in disease presentation and radiographic findings could lead to progression of PCNSL.