Course Of Steroids Research Articles

P016: Reduced steroid exposure is safe and does not reduce disease control among Hodgkin Lymphoma patients treated with escalated BEACOPP (eBEACOPP)

Figure 1: PFS (Kaplan-Meier curves): The outcome of EU and AS patients - 5-y PFS 95% vs. 98%, respectively. Full-length vs. shorter steroid courses - 5 y PFS 98% vs. 95%, respectively. Background: eBEACOPP is the most effective chemotherapy regimen for younger patients with early unfavourable (EU) and advanced stage (AS) Hodgkin lymphoma (HL), but is burdened with early and late toxicities. The original 14 days of steroids contributes to side effects, including severe osteoarticular events, like avascular bone necrosis (AVN). We have been using eBEACOPP since 2009 for AS and since 2014 also for EU patients. We started reducing the length of steroid treatment to 8–10 days in 2016, primarily to reduce the risk of AVN. Methods: We analysed outcomes of our patients, focusing on the comparison of EU and AS patients and those receiving full length and shorter steroid courses. Data was obtained retrospectively, from the hospital database. Results: 162 patients received eBEACOPP as front-line treatment, 130 with AS and 32 with EU HL. Median age was 31 y, range 19–59; 88 (54%) were male. After a median follow-up of 58 mo, 5-y PFS of the whole cohort was 97% and OS 98%. The outcome of EU and AS patients was indistinguishable with a 5-y PFS of 95% vs. 98%, respectively (Fig). Outcome of patients receiving full-length or shorter steroid courses was also indistinguishable, with a 5 y PFS of 98% vs. 95% respectively (Fig). The incidence of AVN was numerically, but statistically insignificantly lower in patients receiving 6 cycles of eBEACOPP with a shorter steroid course (1/42 vs. 4/72, p=0.65). There were no differences in emergency hospital admissions and episodes of febrile neutropenia between the two cohorts. Conclusion: eBEACOPP provides excellent and durable first line disease control. Our data confirms the findings of GHSG of lack of outcome differences between different prognostic groups if eBEACOPP is used as primary treatment. Reducing the duration of steroid treatment to 8 days per cycle is safe, but longer follow-up and more patients are needed to confirm that it reduces serious acute and chronic toxicities.

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

REACTIVATION OF LATENT TB PRESENTING WITH BILATERAL PLEURAL EFFUSIONS

REACTIVATION OF LATENT TB PRESENTING WITH BILATERAL PLEURAL EFFUSIONS

WHEN TREATING ACNE GOES WRONG: A CASE OF BACTRIM-INDUCED ARDS IN A HEALTHY YOUNG WOMAN

WHEN TREATING ACNE GOES WRONG: A CASE OF BACTRIM-INDUCED ARDS IN A HEALTHY YOUNG WOMAN

MULTIPLE SCLEROSIS AFTER COVID-19: A SIGN OF THINGS TO COME?

MULTIPLE SCLEROSIS AFTER COVID-19: A SIGN OF THINGS TO COME?

FROM ALL LUNG TO ALL AIR: A CASE OF IATROGENIC FUNCTIONAL PNEUMONECTOMY

FROM ALL LUNG TO ALL AIR: A CASE OF IATROGENIC FUNCTIONAL PNEUMONECTOMY

POST-COVID PULMONARY COMPLICATIONS IN THE OUTPATIENT SETTING: ARE WE PREPARED FOR THE AVALANCHE?

POST-COVID PULMONARY COMPLICATIONS IN THE OUTPATIENT SETTING: ARE WE PREPARED FOR THE AVALANCHE?

S2489 Differentiating CMV Colitis From GVHD in Post-Transplant Patients: A Case Study

Introduction: Cytomegalovirus (CMV) infection can complicate the clinical course of immunocompromised patients including allogeneic hematopoietic stem cell transplant (allo-HSCT) recipients, where the infection can be serious. CMV can involve different organs including lungs, brain, eyes, and the GI tract. Signs and symptoms of CMV infection may overlap with other conditions including graft-versus-host disease (GVHD), a multisystem disorder commonly seen in allo-HSCT recipients. Case Description/Methods: A 57-year-old female presented with abdominal pain and diarrhea. Past medical history included relapsed acute myeloid leukemia, treated with azacitidine and venetoclax followed by allo-HSCT. She was recently hospitalized for suspected GVHD with skin, liver, and gastrointestinal involvement. She was also found to have a CMV viral load of 920 IU/mL. Endoscopy showed diffuse inflammation across the GI tract, with biopsies revealing apoptotic bodies consistent with acute GVHD. She was treated with ruxolitinib and prednisone for GVHD, and valganciclovir prophylactically for CMV viremia. At this presentation, labs were notable for lymphocytes of 39 x103/uL and platelets of 22 x 103/uL, and CMV viral load of 1,822,242 IU/mL. CT imaging revealed thickened distal small bowel loops, cecum, and right colon, and edematous gastric walls with submucosal enhancement. Infectious work-up, including stool culture, ova/parasite, and clostridium, was negative. She underwent endoscopic evaluation; EGD showed white discoloration that washed off in the esophagus and diffuse erythematous mucosa in the stomach (Figure). Biopsies from gastric mucosa and right colon mucosa revealed focal nuclear changes. Immunohistochemical stains were positive for CMV. She was diagnosed with refractory CMV colitis and treated with maribavir with improvement of symptoms and reduction of viral load, and prior course of steroids was tapered. Discussion: Immunosuppression for GVHD has been identified as an important risk factor for CMV reactivation. Differentiating CMV colitis and GI-GVHD poses a diagnostic challenge, and endoscopic evaluation is needed to address this. Endoscopy can identify cecal ulcers, a reliable finding for CMV colitis in patients with GVHD after allo-HSCT.1 It is important to timely identify CMV colitis to prevent post-transplant morbidity and mortality. This case also demonstrates effectiveness of maribavir, a novel antiviral, for refractory post-transplant CMV colitis.Figure 1.: EGD images showing white discoloration in the entire esophagus and diffuse erythematous mucosa in the entire examined stomach.

S2743 Mesalamine-Induced Interstitial Lung Disease in a Crohn’s Patient With COVID-19

Introduction: Extraintestinal manifestations of Crohn’s disease (CD) generally correlate to the disease activity and may be seen in 1 of 4 patients with CD. Though the prevalence of bronchopulmonary manifestations in CD is 0.4%, subclinical pulmonary changes may be seen in up to half of adults with CD, with involvement of the tracheobronchial tree, lung parenchyma or the pleura. Case Description/Methods: A 61-year-old man, with a history of CD diagnosed 2 years ago, currently in remission with mesalamine, was admitted to the ICU with acute hypoxic respiratory failure due to COVID-19. Inflammatory markers were elevated and he needed high-flow nasal cannula. He was started on a 5-day course of dexamethasone. By HOD-9 O2 requirement and inflammatory markers had trended down. However, around HOD-10 his O2 requirement increased to 6-10 L at rest. CT-chest showed peripherally distributed ground-glass opacities and superimposed early diffuse fibrotic lung changes. Superadded bacterial infection was ruled out. Mesalamine was discontinued and the patient was started on a course of iv methylprednisolone, after which oxygen requirements dramatically declined. He was discharged on HOD 24 with 2L O2 at rest and 4L on ambulation and a prednisone taper. Chest x-ray in 8 weeks showed improving infiltrates. On follow-up 5 months later, he was not on O2 anymore and was doing well. Discussion: Latent interstitial pulmonary involvement is seen in around 20%-55% of patients with IBD, with the most common manifestation being drug-induced lung disease, due to sulfasalazine, mesalamine, methotrexate, or anti-TNF agents. Pulmonary function tests can help in early identification of lung injury since respiratory involvement is latent many times. Onset of drug reaction can occur from 5 days to 44 months after initiation of mesalamine therapy and has been reported in the form of eosinophilic pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and hypersensitivity pneumonitis. The absence of other extra-intestinal manifestations, and the remission of CD itself, can be helpful in excluding other pulmonary manifestations of CD from drug-induced lung disease. Discontinuation of the causative drug, along with a course of steroids, can help in reversing the symptoms. Though the differential of mesalamine-induced lung injury, cannot be confirmed nor excluded here, this patient probably had underlying lung pathology from his CD or its treatment, making his recovery from COVID-19 more complicated (Figure).Figure 1.: A: Portable Chest X-ray showing multifocal pneumonia, low lung volumes with bilateral patchy airspace disease; B, C: CT-Chest showing peripherally distributed ground-glass opacities, distributed in upper and lower lobes. There were superimposed early diffuse lung fibrotic changes, without any evidence of pulmonary thromboembolism; D: CT Chest, 5 months post-discharge, showing improvement in opacities.

S3093 Monotherapy for Multiple Autoimmune Diseases: The Novel Use of Ofatumumab for Concurrent Multiple Sclerosis and Autoimmune Hepatitis

Introduction: Autoimmune hepatitis (AIH) is a rare acute or chronic inflammatory liver disease, that can be associated with a variety of extrahepatic disorders, such as multiple sclerosis (MS). AIH has been reported both during and after treatment with immunomodulatory drugs for MS, such as interferon-beta and steroids, with its incidence in MS being 10-fold higher than the general population. Typically, those patients are treated with steroids and azathioprine as the first line of treatment according to AIH guidelines. However, in comparison to newer disease-modifying therapies (DMTs) for MS, azathioprine is less effective and requires further study. Notably, several studies have suggested a role for B-cell-driven autoimmune liver injury in AIH, and there have been reports of refractory AIH being successfully treated with rituximab, an anti-CD20 monoclonal antibody. Based on these results and the well-known role of B-cells in the underlying pathophysiology of MS, we postulated that anti-CD20 therapy may be effective in treating patients with concurrent MS and AIH, thereby helping to limit steroid duration and avoid the use of azathioprine. Case Description/Methods: We report the case of a 49-year-old previously healthy female who presented with symptoms and magnetic resonance imaging (MRI) findings consistent with relapsing-remitting MS (RRMS). On routine labs at initial presentation, she was incidentally found to have ALT/AST in the 500's range. An extensive work-up was completed, including a liver biopsy that showed a centrilobular necroinflammatory injury pattern suggest of early AIH without cirrhosis. The patient was started on oral budesonide followed by ofatumumab, an anti-CD20 monoclonal antibody approved for treatment of RRMS, as steroids were weaned. The patient’s liver enzymes returned to normal ranges within 12 weeks and have remained so now for approximately 8 months. Additionally, she has had no new clinical or imaging findings suggestive of MS disease activity. We present this as the first-reported case of the use of an anti-CD20 monoclonal antibody to treat both MS and AIH concurrently. Discussion: This case furthers our understanding of AIH by suggesting that the pathophysiology contributing to AIH is predominately B-cell related as this patient’s AIH was successfully treated with ofatumumab and a short course of budesonide. It highlights a possible alternative treatment modality for AIH that avoids a prolonged course of steroids and/or azathioprine. More research is still needed however.

Unilateral Acute Parotitis

Background: Severe acute respiratory virus coronavirus-2 infection or coronavirus disease (COVID) is categorized into acute illness and late multiinflammatory syndrome in children (MISC). This has recently been challenged with recognition of presentations with mucocutaneous-enteric symptoms that display considerable overlap between the two. We recognized a similar overlap of manifestations when encountered the three cases of fever associated with unilateral parotitis. Clinical Description: The three patients were of different age groups ranging from 2 months to 7 years of age, all of whom presented with fever, unilateral swelling of face and neck consistent with the region of the parotid gland, and absence of other localizing symptoms or abnormalities on examination. All of them were positive for COVID antibodies, had negative COVID real-time polymerase chain reaction test, did not satisfy the diagnostic criteria of MISC, but had raised inflammatory markers. Since the workup for other common causes of acute parotitis was negative, a clinical diagnosis of post-COVID immune-mediated acute parotitis was kept. Management: All the three patients were managed with systemic steroids (oral or parenteral) and showed complete resolution of symptoms and normalization of laboratory parameters within a few days, a therapeutic response in alignment with an immune-mediated phenomenon. Conclusion: Acute unilateral parotitis with pyrexia may be a hitherto unreported late post-COVID manifestation that is immune mediated and shows an excellent therapeutic response to a short course of steroids.

S2228 A Segmental Colitis Associated With Diverticulosis versus Inflammatory Bowel Disease: A Diagnostic Dilemma Despite Total Proctocolectomy

Introduction: Patients with diverticular disease can develop segmental colitis associated with diverticulosis (SCAD). It can range from mild inflammatory changes to chronic active inflammation that mimics inflammatory bowel disease (IBD). SCAD usually presents a more benign outcome, with a low rate of complications. Here, we present a complex case in which a diagnosis was still unclear till the final stage of the management. Case Description/Methods: A 60-year-old female was admitted for hypovolemic shock. She was resuscitated, and the GI was consulted for the management of IBD. She was recently diagnosed with ulcerative colitis about 2 months ago. She has abdominal pain in the right lower side, and examination revealed mild abdominal tenderness. She was already on prednisone 60 mg daily for 2 weeks and infliximab maintenance dose (completed 2 doses) and mesalamine from the last admission. She has had 3 colonoscopies in the past. The first colonoscopy 6 months ago showed a normal rectum, unable to traverse the sigmoid colon due to stricture and diverticulosis. The biopsy showed a normal rectum and a very focal area of cryptitis. The last colonoscopy 2 months ago showed a severely inflamed ulcerated mucosa in the sigmoid colon (Figure, left). Colon biopsy showed mild acute cryptitis and distorted and branched crypts. No crypt abscesses or granulomas were seen. On this admission, CT enterography showed pancolitis. Serology for Crohn's disease (CD) was negative, including ASCA IgA and ASCA IgG. The strong differential diagnosis was SCAD vs.IBD. The decision was to continue with the medical treatment (i.e., completion of steroid course, infliximab, and follow the response) and was discharged. The patient again presented with a shock one month after this admission. Unfortunately, she had colonic perforation and had total proctocolectomy with ileostomy creation. Pathology of surgical specimens revealed mild chronic active colitis and severe diverticular disease with associated transmural inflammation (Figure, right). Ileum excision showed no significant pathologic changes unlikely to be CD. The patient was doing well till the last follow-up. Discussion: This is a unique case where the diagnosis of SCAD vs. IBD was unclear regardless of the availability of serology, endoscopic, surgery, and histopathological examinations. In this complex case, we suggest a multidisciplinary team approach to improve patient-centered care.Figure 1.: Left: colonoscopy showing inflamed ulcerated mucosa in the sigmoid colon. Right: Pathology shows mild chronic active colitis and severe diverticular disease.

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DISSEMINATED HISTOPLASMOSIS PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE IN A 32-YEAR-OLD WOMAN

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO DISSEMINATED HISTOPLASMOSIS PRESENTING AS DIFFUSE ALVEOLAR HEMORRHAGE IN A 32-YEAR-OLD WOMAN

P14 Challenges in treating anti-melanoma differentiation associated gene 5 (MDA5) antibody positive juvenile dermatomyositis (JDM) with interstitial lung disease (ILD) complicated by pneumocytis jirovecii pneumonia (PCP): a case report

Introduction/BackgroundWe describe a case of MDA5 antibody positive JDM associated with ILD who developed worsening in breathing effort requiring oxygen support. We highlight the challenges associated with the risk of rapidly progressing ILD (RPILD) versus infection as the patient deteriorated when her oral prednisolone was tapered. Whilst investigating for other potential causes of RPILD, immunosuppression was intensified. A suboptimal response to treatment prompted extensive opportunistic infection screening identifying pneumocystis pneumonia (PCP). Here, we describe the clinical course of this patient, outline the management, and ask whether immunosuppressed JDM patients with ILD should be prophylactically treated for fungal disease.Description/MethodA 12-year-old girl presented with a 4 months history of oral ulcers, exercise intolerance, joint stiffness, and erythematous palmar rash. On examination, she had a weak swallowing with swelling and restriction in her joints, Gottron’s papules, vasculitic rash with skin ulceration and dilated nailfold capillaries. Manual muscle testing (MMT)-8 and Childhood Muscle Assessment Scale (CMAS) scores were 54/80 and 42/52 respectively. Based on this clinical presentation and further tests she was diagnosed with MDA5 positive JDM.She developed symptoms suspicious of pulmonary embolism, prompting a CT pulmonary angiogram. This identified coincidental scattered areas of ground-glass consolidation with atelectasis consistent with ILD. Lung function testing (LFT) was normal (FEV1 2.89L (100.2%), FVC 2.94L (89.9%), DLCO 87%, KCO 98.4%).She was treated with pulses of IV methylprednisolone (IVMP), IV immunoglobulin (IVIG), methotrexate, and rituximab. Methotrexate was stopped as she developed transaminitis and switched to ciclosporin. She was discharged with prednisolone and ciclosporin.She presented 6 weeks later with dyspnoea, fatigue and new skin rashes, but had no cough. Examination found reduced breath sounds and crackles.Investigations showed CRP <5, ESR 80mm/hour, WCC 15.45 (x10*9/L), ALT 43 (U/L), AST 80 (U/L), CK 21 (U/L), LDH 639 (U/L). Chest radiograph on admission was reported as normal and CT thorax showed non-specific patchy mild ground-glass opacification. LFT showed worsening restrictive picture with decreased diffusing capacity with FEV1 1.56L (53%), FVC 1.79L (54%), DLCO 46%, and KCO 73%. Progression of ILD was suspected thus she was pulsed with IVMP and given IV cyclophosphamide.Fever with worsening respiratory distress ensued requiring PICU admission, prompting an extensive infection screen. PCP PCR and β -D glucan were positive, thus she was treated for PCP with co-trimoxazole and caspofungin. She made good recovery and was discharged well.Discussion/ResultsJuvenile dermatomyositis (JDM) is a rare systemic autoimmune condition in childhood. It primarily affects the proximal muscles and skin but may also affect the heart, respiratory, GI system, joint and other organs. It is a complex condition and is potentially life threatening if treated inadequately. The emergence of myositis specific antibodies (MSA) have enhanced our understanding of both the clinical and laboratory features of JDM. The phenotype of children with anti-MDA5 autoantibodies has yet to be clearly established unlike their adult counterpart however there have been increased recognition that it is associated with risk of developing ILD. In our case, MDA5-associated ILD was diagnosed at presentation and she was treated aggressively with immunosuppressants. She developed deterioration in her respiratory symptoms 6 weeks later whilst on weaning course of steroids triggering the possibility of worsening ILD. This has led to her receiving a course of intravenous cyclophosphamide in addition to on-going immunosuppressants. However, as she worsened despite optimal treatment for ILD, she was screened for opportunistic infections and was positive for PCP. This highlights challenges faced by clinicians in identifying the cause of worsening respiratory function in a children with established ILD who are immunosuppressed. It maybe multi-factorial however opportunistic infections such as PCP should always be considered in these children to ensure it is treated appropriately to prevent further complications.Key learning points/ConclusionAnti-MDA5 positive ILD represents a cohort of children requiring a more aggressive immunosuppressive therapies to optimise and prevent further deterioration in their clinical respiratory function. However, since there are no proven biomarkers able to differentiate indolent ILD from RPILD, it may be favourable to assume RPILD and treat aggressively.Balancing the risks and benefits caused by aggressive immunosuppression in these children continues to be a challenge. Therefore, clinicians should be cognisant of opportunistic infections such as PCP when faced with children with underlying ILD whose respiratory function deteriorates. Chemoprophylaxis with co-trimoxazole is generally well tolerated with minimal side effects and therefore should be considered in these children at diagnosis.

P32 Therapeutic dilemma due to gut complications with spondyloarthritis

Introduction/BackgroundTumor necrosis factor (TNF) inhibitor therapy is one of the primary modalities of treating spondyloarthritis. However, infections secondary to biologic use is a common complication. Clostridium difficile is a gut colonizer which may become pathogenic in the presence of dysbiosis and antibiotic use. Symptomatic clostridium difficile infection(CDI) due to adalimumab(ADA) use causing lymphocytic colitis(LC) is rarely reported. We present a case of axial plus peripheral spondyloarthritis on adalimumab who developed CDI and presented a therapy related dilemma.Description/MethodWe present a case of a 52-year-old lady who was diagnosed as HLAB27 positive axial spondyloarthritis with bilateral sacroiliitis on MRI bilateral sacroiliac joint in 2016. She failed two non-steroidal antinflammatory drugs (NSAID) with high disease activity scores(BASDAI 8.6, BASFI 6.2, Spinal VAS 9). She was eventually initiated on injection adalimumab in 2017. There was clinical improvement after 6 months of initiating ADA. Repeat MRI spine and SI joint showed resolution of inflammatory changes with adalimumab. Sulfasalazine was added in the treatment protocol due to peripheral arthralgia, however she developed a drug rash to it and it was stopped. In September 2021, she developed recurrent non-bloody watery diarrhoeal episodes. Adalimumab was stopped and she tested positive for Clostridium difficile on three occasions. She received multiple courses of vancomycin, fidoxamycin and metronidazole with partial resolution of diarrhoea. Adalimumab was rechallended in January 2022 since her disease activity was worsening in the absence of NSAID and biologic use. However, it was paused again due to worsening of diarrhoea. Gastroenterology conducted a Colonoscopy and biopsy of colonic polyps which showed increased number of intraepithelial lymphocytes with no thickening of subepithelial collagen plate suggestive of lymphocytic colitis (LC). It was postulated that CDI may have been the trigger for her LC. She has been started on local corticosteroid therapy(budesonide) for her LC. At this junction, we were faced with the decision of restarting adalimumab for her which may have been the original cause of her CDI.Discussion/ResultsWe present this case in view of a dilemma regarding which problem occurred first Did immunosuppression due to adalimumab lead to CDI? Literature is not suggestive of increased risk of CDI with adalimumab, especially in a non-inflammatory bowel disease (IBD) background. Was the lymphocytic colitis triggered by the CDI or was clostridium difficile just incidentally detected on a background of LC? There is a well documented associated of LC with autoimmune conditions and use of NSAIDs along with proton pump inhibitor is another trigger for it. TNF alpha inhibitor is one of the treatment modalities of refractory LC. Would it be appropriate to restart ADA for her since she responded well despite there being a risk of recurrence of a CDI? Finally, what is the role of gut dysbiosis in the role of CDI and LC in this situation. Presently, she is planned to be treated with local steroid therapy for her LC. ADA will be rechallenged once her gut has responded to the steroid course. In the event of her gut worsening on a rechallenge, she will be given a trial of Infliximab.Key learning points/ConclusionThe case highlights the crucial point of thinking of causes of diarrhea apart from well documented conditions of ulcerative colitis/Crohn’s disease in an immunosuppressed spondyloarthritis patient. A detailed stool examination including bacterial and parasitic examination is crucial in recurrent diarrhea. The threshold for colonoscopy should be low when episodes are refractory to treatment. The case-based conference gives us an opportunity to receive feedback regarding further management approaches in this case.

Association of Antenatal Steroid Exposure at 21 to 22 Weeks of Gestation With Neonatal Survival and Survival Without Morbidities

The provision of antenatal corticosteroids to pregnant patients at gestational age (GA) 22 6/7 weeks or less remains controversial and lacks support from randomized clinical trials. To compare rates of survival and survival without major morbidities among infants born at GA 22 0/7 to 23 6/7 weeks after exposure to antenatal steroids at 22 6/7 weeks' gestation or less vs no exposure to antenatal steroids. This cohort study enrolled infants born at GA 22 0/7 to 23 6/7 weeks between January 1, 2016, and December 31, 2019, at centers in the National Institute of Child Health and Human Development Neonatal Research Network. Infants who did not receive intensive care and infants with antenatal steroid exposure after GA 22 6/7 weeks were excluded. Infants were classified as having no, partial, or complete exposure to antenatal steroids. The primary outcome was survival to discharge. The main secondary outcome was survival without major neonatal morbidity. The associations of differential exposures to antenatal steroids with outcomes were evaluated using logistic regression, adjusting for GA, sex, race, maternal education, small for GA status, mode of delivery, multiple birth, prolonged rupture of membranes, year of birth, and Neonatal Research Network center. A total of 431 infants (mean [SD] GA, 22.6 [0.5] weeks; 232 [53.8%] boys) were included, with 110 infants (25.5%) receiving no antenatal steroids, 80 infants (18.6%) receiving partial antenatal steroids, and 241 infants (55.9%) receiving complete antenatal steroids. Seventeen infants were exposed to antenatal steroids at GA 21 weeks. Among infants exposed to complete antenatal steroids, 130 (53.9%) survived to discharge, compared with 30 infants (37.5%) with partial antenatal steroid exposure and 239 infants (35.5%) with no antenatal steroids. Infants born after complete antenatal steroid exposure, compared with those without antenatal steroid exposure, were more likely to survive to discharge (adjusted odds ratio [aOR], 1.95 [95% CI, 1.07-3.56]) and to survive without major morbidity (aOR, 2.74 [95% CI, 1.19-6.30]). In this retrospective cohort study, among infants born between GA 22 0/7 and 23 6/7 weeks who received intensive care, exposure to a complete course of antenatal steroids at GA 22 6/7 weeks or less was independently associated with greater odds of survival and survival without major morbidity. These data suggest that the use of antenatal steroids in patients at GA 22 6/7 weeks or less could be beneficial when active treatment is considered.

Cardiorespiratory adaptation to low-dose dexamethasone for lung disease in extremely preterm infants: A prospective echocardiographic study.

The cardiovascular impact of dexamethasone (Dex) is not well understood. Most data are obtained from a 6week, high-dose regimen, and are limited to findings of hypertension and cardiac hypertrophy. The present study ascertained the impact of low-dose Dex on cardiac indices when administered to extremely preterm infants for lung disease. A pre-post intervention prospective echocardiographic (Echo) study was undertaken, with cardiac assessments performed before and within 24h after completion of first course of therapy (10day regimen, cumulative 0.89mgkg-1 ). Thirty infants with a gestational age of 24.6±1.1weeks and birthweight of 612±125g, respectively, were studied. The age at Dex administration was 20±9days. Fractional inspired oxygen decreased from 0.7±0.23 to 0.35±0.14 (P<0.001). Patent ductus arteriosus was noted in 20 infants at Echo1. At Echo2, the ductal diameter decreased from 2.16±0.8 to 1.1±0.8mm (P= 0.0003), with complete closure in 7/20 (35%). A reduction in left pulmonary artery end-diastolic velocity was noted (17±12 to 9±10cms-1 , P<0.001). Pulmonary vascular resistance decreased (increased time to peak velocity/right ventricular ejection time, 0.2±0.03 to 0.23 ±0.03, P= 0.0001) and right ventricular systolic performance improved (tricuspid annular plane systolic excursion, 4.9±0.8 to 5.5±0.9mm, P= 0.02). No significant changes in fractional shortening and left ventricular mass were noted. A significant increase in blood pressure was noted. As a percentage of pre-treatment baseline, the mean increase for systolic blood pressure was 20.3% (95% confidence interval=14-26) on day 2 (P= 0.008). Low-dose Dex influenced cardiovascular parameters related to pulmonary circulation. KEY POINTS: Corticosteroid therapy is frequently used in preterm infants who are dependent on ventilator support. Echocardiographic studies in infants administered a 6week course of steroids have noted left ventricular hypertrophy, outlet obstruction and hypertension, but no information is available on right heart indices. The cardiopulmonary effects of the current, significantly lesser cumulative dose (10day regimen, commonly described as 'DART') have not been evaluated. The present study noted a significant influence on ductal and pulmonary circulation indices. Left heart architecture and function was maintained, whereas a significant but transient increase in blood pressure was noted.

Isolated Central Nervous System Vasculitides in COVID-19: A Systematic Review of Case Reports and Series

Cerebral vasculitides, both isolated or in systemic disorders, could be triggered by infections, and few cases have been associated to coronavirus disease 2019 (COVID-19). This study searched for publications in Pubmed, EMBASE, and Cochrane library databases for case reports and series of isolated central nervous system (CNS) vasculitides triggered by severe acute respiratory syndrome coronavirus-2. We included 12 studies (published from June 2020 to July 2022) and collected 39 adult patients (5/39 pathologically or radiologically proven, 34/39 suggestive for primary CNS vasculitis or PCNSV). All cases had a positive real-time polymerase chain reaction on a nasopharyngeal swab or a respiratory tract specimen. About the 85% of the included cases were males, and disease onset occurred later than 50 years old in all but three subjects. In total, 33/39 patients presented severe COVID-19 pneumonia, frequently requiring intensive care unit care. The most common neurological features were headache, obnubilation, and coma. PCNSV was suspected mainly on radiological findings, whereas the cerebrospinal fluid analysis was minimally altered. Magnetic resonance imaging showed vessel wall enhancement in 32/39 cases, generally with the concomitant presence of microbleeds, subarachnoid haemorrhages, and/or multiple ischemic lesions. Despite the severe respiratory and neurological disease course, most cases (93%) improved spontaneously or after a course of high-dose intravenous steroids with no need for immunosuppression. In conclusion, PCNSV could rarely relate to COVID-19 and independently from pulmonary disease severity. Adults with COVID-19-related PCNSV could have a favourable prognosis.

DRESS syndrome: Tricky to diagnose but easy to treat

A 27-year-old female presented to us with a short history of fever, jaundice, rash, and worsening hepatic dysfunction subsequent to treatment with intravenous antibiotics and alternative medicine for a urinary tract infection. The eosinophilia, lymphadenopathy, and transaminitis prompted us to consider a diagnosis of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) which can be fatal if not treated. The patient showed improvement in clinical and laboratory parameters after a course of steroids. This case is presented as DRESS syndrome that can prove rapidly fatal if not diagnosed and treated immediately

Metastatic neuroendocrine tumor masquerading as orbital cysticercosis.

Orbital metastases secondary to neuroendocrine tumors are exceedingly rare. We present a unique case of a 30-year-old female initially presenting with fever, chills, periorbital swelling, and painful proptosis. CT orbits revealed two ovoid-shaped ring-enhancing lesions in the right lateral and superior rectus muscles and clear sinuses, atypical for infectious post-septal cellulitis. Further work-up included serologic analysis, auto-immune panel, and MRI. Further imaging showed pseudocystic orbital lesions mimicking orbital cysticercosis. Additionally, given the bilateral nature of the lesions and patient's country of origin, this parasitic process was highly suspected. A course of albendazole and steroids led to resolution of symptoms. With a presentation at age 30, this is by far the youngest case reported in literature to date.