Dose Prednisone Therapy Research Articles

S2025 Eosinophilic Enterocolitis Improved After Breast Reduction

Introduction: Hypereosinophilic Syndrome (HES) is a group of disorders characterized by an accumulation of eosinophils within peripheral tissues or blood, with manifestations dependent on organ involvement. Eosinophilic gastrointestinal disorders (EGIDs) is one manifestation. In our case, it is believed that antigenic leakage from ruptured silicone breast implants was the proximate cause, as the patient had complete resolution of symptoms and normalization of labs after implant removal. Case Description/Methods: Our patient is a 59-year-old female with a history of asthma, who presents with new-onset sharp epigastric pain worsened with oral intake and associated with diarrhea. Recent evaluation at another hospital for leg weakness revealed peripheral eosinophilia and electromyography showed chronic inflammatory demyelinating neuropathy. After a full diagnostic evaluation for HES, she was diagnosed with idiopathic HES and treated with low dose steroids, IVIG and plasmapheresis without clinical improvement. At our hospital, labs revealed a white count of 35,000/μL, eosinophil count of 21,960/mm3 and IgE of 1299 IU/mL (Table). A CT scan revealed descending colon wall thickening, and intracapsular rupture of the right breast implant. Enteroscopy demonstrated erosions and ulcerations of the duodenum and jejunum. Colonoscopy showed areas of focal ulcerations with surrounding inflammation separated by normal mucosa and histology showed eosinophilic infiltration throughout. Video capsule endoscopy demonstrated diffuse inflammation and ulceration of the entire small bowel. She was empirically treated for strongyloides before starting high dose prednisone therapy. A month later, her breast implants were removed elsewhere. After a short interval, she was successfully tapered off steroids and achieved resolution of both her GI symptoms and lower extremity weakness, as well as normalization of lab work (Figure). Discussion: EGIDs are believed to be multifactorial with an emphasis on immunologic factors. Eosinophilic infiltration seen on biopsies and exclusion of secondary causes is required. Treatment is aimed at symptomatic improvement and removal of offending agent, as seen with our patient who experienced complete clinical resolution after removal of her silicone breast implants. Our case highlights a rare case of eosinophilic enterocolitis associated with a ruptured breast implant in an otherwise healthy patient and emphasizes the importance of remaining vigilant of all external factors that may be contributing to the disease process.Figure 1.: A, B: Small intestine duodenum & Jejunum. C, D: Colonic areas of inflammation. Table 1. - Initial labs versus labs post implant removal Units Reference Labs Initial Labs Labs post implant removal White Blood Count μL 4.0 - 10.5 35 x 103 7.1 Hemoglobin g/dL 11.2 - 15.7 9.6 11.2 Platelets μL 150-400 508 x 103 369 Eosinophil # μL 0.04 - 0.36 21,960 2.5 Eosinophils % 0.7-5.8 61 35 IgE IU/mL < 100 1299

Virus-Negative Necrotizing Coronary Vasculitis with Aneurysm Formation in Human SARS-CoV-2 Infection.

We report a case of myopericarditis associated to SARS-CoV-2 infection with necrotizing coronary vasculitis of intramural vessels, giving rise to biventricular apical microaneurysms and to electrical instability. Negativity of myocardial polymerase chain reaction for the most common cardiotropic viruses and for SARS-CoV-2 suggested an immune-mediated myocardial and pericardial inflammatory disease. High dose (1 mg/Kg daily) prednisone and anti-viral (Remdesivir, IDA Business, Carrigtohill, County Cork, T45 DP77, Ireland) therapy led to resolution of cardiac inflammation and ventricular arrhythmias. Morpho-molecular characterization of endomyocardial tissue may improve the outcome in subjects with SARS-CoV-2-associated myopericarditis and coronary vasculitis.

Frequency of anergy in a group of patients with rheumatoid arthritis on immunosuppressive therapy

BackgroundTuberculin is the globally accepted delayed cutaneous hypersensitivity test for the diagnosis of latent tuberculosis. The alteration of cellular immunity induced by disease-modifying drugs used in rheumatoid arthritis may give a false negative result, also known as cutaneous anergy. There are no studies that determine the frequency of anergy in patients with rheumatoid arthritis and on immunosuppressive therapy. ObjectiveTo determine the frequency and possible factors associated with cutaneous anergy in a group of patients with rheumatoid arthritis and on immunosuppressive therapy. MethodsCross-sectional analytical observational study including 100 patients with rheumatoid arthritis on immunosuppressive therapy. They were tested for delayed cutaneous hypersensitivity with tuberculin, and a control test with tetanus toxoid. The non-reactivity of both tests was defined as anergy. ResultsThe overall frequency of cutaneous anergy was 9% (n = 11). It occurred in 33% of men versus 6% of women. The mean age was 57 years, and 89% were over 50 years-old. Being female behaved as a protective variable for the generation of anergy, OR 0.795 [95% CI, 0.658 - 0.959, P<.05]. All patients with anergy were being treated with corticosteroids, 44% with methotrexate, and 33% with biological therapy. Treatment with moderate to high dose prednisone and biological therapy were independently associated as risk factors for presenting with anergy, OR 1.044 [95% CI, 1.008-1.080 P<.05] and OR 1.096 [95% CI, 1.016-1.182, P<.05], respectively. The overall positivity for tuberculin was 13%. Symptoms associated with disease activation were present in 38% of these. All cases (n= 1) of confirmed active tuberculosis were excluded. ConclusionsThe high prevalence of cutaneous anergy in patients with RA in the present study, and the evidence presented here, supports the recommendation of a second diagnostic test (tuberculin booster or Interferon-Gamma Release Assays) for the diagnosis of latent TB in patients with RA on immunosuppressive therapy.

Identification of Lupus Podocytopathy with Coexistent Glomerular Diseases – A Case Report

Abstract Introduction/Objective Lupus podocytopathy (LP), featured by nephrotic syndrome, is a unique subtype of lupus nephritis that mimics minimal change disease or primary focal segmental glomerulosclerosis (FSGS) on renal biopsy with diffuse podocyte foot process effacement and no capillary-loop immune deposits. LP usually presents on a background of ISN/RPS class I or class II lupus nephritis, and very rarely may present without immune deposits. Diagnosis of LP, when confounded by other glomerular diseases associated with nephrotic syndrome, can be very challenging and requires thorough clinical and pathology correlations. Methods Here we report a case of LP in a patient with nephrotic syndrome and multiple comorbidities affecting kidneys. A 24-year-old female with type-I diabetes, psoriasis, and intermittent arthritis/rash of unknown etiology, presented with abrupt onset of nephrotic proteinuria attributed to recent low dose prednisone therapy, and renal insufficiency. A renal biopsy showed nodular glomerulosclerosis and FSGS. No immune deposits were identified by immunofluorescence or electron microscopy. Ultrastructurally there was also diffuse glomerular basement membrane thickening and over 90% podocyte foot process effacement. With no established systemic lupus erythematosus (SLE), the case was initially diagnosed as diabetic nephropathy with coexistent FSGS as the etiologies for nephrotic proteinuria, and the patient was put on ACEI and diuretics. However, massive proteinuria persisted, and the patient also developed pancytopenia. Serology concurrent with the biopsy came out later showing positive autoantibodies against dsDNA, Smith, and Histone. With continued worsening of creatinine, a renal biopsy was repeated revealing essentially similar findings to the first biopsy. Results Integrating the serology results and clinical presentation, SLE was favored. The pathology findings were re- evaluated and considered to be most consistent with LP and coexistent diabetic nephropathy, with FSGS either a component of LP or an independent lesion secondary to diabetes or hypertension. The patient was started with high dose prednisone at 60 mg/day. One month later, her proteinuria, serum creatinine, pancytopenia, skin rash, and arthritis were all significantly improved. Conclusion LP can be easily masked by coexistent glomerular diseases. Sufficient awareness of the entity is necessary for the appropriate diagnosis and treatment.

MONOCLONAL ANTIBODY GONE ROGUE: ALEMTUZUMAB CAUSING ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Anti-Glomerular basement membrane disease (anti-GBM), is a rare autoimmune process that can cause irreversible renal or lung injury. Here we discuss a case of Alemtuzumab induced anti-GBM. CASE PRESENTATION: A 46 year-old caucasian female with history of Multiple Sclerosis presented with flu-like symptoms, dizziness, nausea and vomiting ongoing for two weeks. Her physical exam was significant for dry mucous membranes and tachycardia. Her labs were significant for a BUN of 48 mg/dL and a creatinine of 6.8 mg/dL without any history of prior kidney disease. Renal biopsy revealed more than 90% glomerular involvement with necrotizing and crescentic proliferation, as well as positive linear staining along the glomerular basement membranes for IgG, Kappa, Lambda and C3. Anti-glomerular basement membrane antibodies were 189 units; thereby, Anti-GBM disease was diagnosed. Patient reported being on Alemtuzumab eight months prior to presentation. She was started on plasmapheresis, high dose prednisone and cyclophosphamide but therapy was stopped early due to nightly agitation and confusion likely related to sundowning, but the patient's family attributed it to the new therapy. Patient was discharged to inpatient rehab where she developed a right internal jugular vein thrombosis and was started on rivaroxaban. She later developed hemoptysis and had to be readmitted. Computed tomography scan of her lungs showed bi-apical and basilar dense airspace opacities with air bronchograms suggesting hemorrhagic infiltrates, findings were most consistent with diffuse alveolar hemorrhage. [Images A-C] Anti-GBM antibodies were elevated at 217 units. Patient was then restarted on plasmapheresis, steroids and cyclophosphamide and was able to complete therapy with slow but definite improvement. DISCUSSION: Alemtuzumab is a humanized monoclonal antibody that binds to CD52 (a cell-surface antigen present on T and B cells) and can be associated with anti-GBM disease that may present up to 40 months after the last dose.[1] Our case illustrates this lingering side effect and highlights the importance of taking a thorough history including previous and current medications. Our case shows the second reported case of Alemtuzumab induced anti-GBM with both renal and lung involvement [2] and the seventh case with renal involvement.[3-6] Our case is unique in the timing and course of development of kidney and lung injury, with about a month in between, while the first reported case showed a more rapid succession of kidney and lung injury. CONCLUSIONS: Alemtuzumab can be associated with autoimmune syndromes but clear screening tools and monitoring is not currently available. Further investigation is needed to identify a possible role for antibody monitoring in patients on Alemtuzumab. Reference #1: [1] Devonshire, V et al. Monitoring and management of autoimmunity in multiple sclerosis patients treated with alemtuzumab: practical recommendations. J Neurol 2018. Reference #2: [2] Lapointe, E., Moghaddam, B., Barclay, K., Traboulsee, A., & Neufeld, P. (2018). Goodpasture’s Syndrome Following Alemtuzumab Therapy in Multiple Sclerosis. Canadian Journal of Neurological Sciences / Journal Canadien Des Sciences Neurologiques, 45(6), 712-714. Reference #3: [3] Coles, AJ, Cox, A, Le Page, E, et al. The window of therapeutic opportunity in multiple sclerosis: evidence from monoclonal antibody therapy. J Neurol. 2006;253(1):98-108 [4] Meyer, D, Coles, A, Oyuela, P, et al. Case report of anti-glomerular basement membrane disease following alemtuzumab treatment of relapsing-remitting multiple sclerosis. Mult Scler Relat Disord. 2013;2(1):60-63. [5] Phelps, R. Autoimmune events: nephropathies. Scientific exchange forum 2013. [6] Singer, BA, Alroughani, R, Brassat, D, et al. Durable improvements in clinical outcomes with alemtuzumab in patients with active RRMS in the absence of continuous treatment: 7-year follow-up of CARE-MS II patients (TOPAZ study). Poster 736. 7th joint ACTRIMS-ECTRIMS meeting, Paris. 2017. DISCLOSURES: No relevant relationships by Youssef Abouleish, source=Web Response No relevant relationships by Paul Marik, source=Web Response

S3007 What Lies Beneath: A Curious Case of Eosinophilic Gastroenteritis

INTRODUCTION: The serosal form of eosinophilic gastroenteritis (EG) is the least common but typically the most responsive to corticosteroids. The diagnosis can be elusive, as there are no standard diagnostic criteria for this form of EG, and endoscopic mucosal biopsies may frequently fail to reveal tissue eosinophilic infiltrates. This case demonstrates that the diagnosis of serosal eosinophilic gastroenteritis requires a high index of suspicion that incorporates clinical presentation, laboratory data, radiologic findings, and response to corticosteroids. CASE DESCRIPTION/METHODS: A 50-year-old Caucasian male with a history of ADHD and GERD presented with progressive abdominal distention and pain radiating to the back. Vital signs were notable for a temperature of 100.5o F. Physical exam was notable for decreased breath sounds at the lung bases, mild distention of the abdomen and mild diffuse abdominal tenderness. Laboratory studies were all normal including WBC count except for absolute eosinophil count >5,000. CT imaging revealed a right > left sided pleural effusion, small bilateral pulmonary nodules, hilar and mediastinal lymphadenopathy, long-segment small bowel thickening, ascites, and distal esophageal thickening. Paracentesis revealed eosinophilic ascites. Evaluation for parasitic, nematodal, neoplastic, allergic, and hematologic etiologies for eosinophilia was unrevealing including flow cytometry. Subsequent bone marrow evaluation excluded an eosinophilic myeloproliferative disorder. Endoscopy and biopsies from the esophagus to jejunum were normal except for Barrett’s esophagus. Prednisone was initiated for presumed serosal EG with a complete clinical response within one week and complete normalization of eosinophilia. Over the course of several years, prednisone tapering was inevitably followed by symptomatic recurrence of ascites and systemic eosinophilia. He has remained in complete remission 6 years from initial presentation on low dose prednisone maintenance. DISCUSSION: Eosinophilic ascites (EA) is a rare presentation of EG and is due to serosal involvement. Peripheral eosinophilia may or may not be present. Exclusion of other etiologies of EA is important before initiating therapy. Serosal EG may have a relapsing course (as in our patient) in 25% of patients who then require chronic low dose prednisone or immunomodulator therapy. Laparoscopic full-thickness gastric or small bowel biopsies may be necessary to make the diagnosis in cases without a prompt corticosteroid response.

Corticosteroid therapy for nephrotic syndrome in children.

Corticosteroid therapy for nephrotic syndrome in children.

Frequency of anergy in a group of patients with rheumatoid arthritis on immunosuppressive therapy

BackgroundTuberculin is the globally accepted delayed cutaneous hypersensitivity test for the diagnosis of latent tuberculosis. The alteration of cellular immunity induced by disease-modifying drugs used in rheumatoid arthritis may give a false negative result, also known as cutaneous anergy. There are no studies that determine the frequency of anergy in patients with rheumatoid arthritis and on immunosuppressive therapy. ObjectiveTo determine the frequency and possible factors associated with cutaneous anergy in a group of patients with rheumatoid arthritis and on immunosuppressive therapy. MethodsCross-sectional analytical observational study including 100 patients with rheumatoid arthritis on immunosuppressive therapy. They were tested for delayed cutaneous hypersensitivity with tuberculin, and a control test with tetanus toxoid. The non-reactivity of both tests was defined as anergy. ResultsThe overall frequency of cutaneous anergy was 9% (n = 11). It occurred in 33% of men versus 6% of women. The mean age was 57 years, and 89% were over 50 years-old. Being female behaved as a protective variable for the generation of anergy, OR 0.795 [95% CI, 0.658 - 0.959, P<.05]. All patients with anergy were being treated with corticosteroids, 44% with methotrexate, and 33% with biological therapy. Treatment with moderate to high dose prednisone and biological therapy were independently associated as risk factors for presenting with anergy, OR 1.044 [95% CI, 1.008-1.080 P<.05] and OR 1.096 [95% CI, 1.016-1.182, P<.05], respectively. The overall positivity for tuberculin was 13%. Symptoms associated with disease activation were present in 38% of these. All cases (n= 1) of confirmed active tuberculosis were excluded. ConclusionsThe high prevalence of cutaneous anergy in patients with RA in the present study, and the evidence presented here, supports the recommendation of a second diagnostic test (tuberculin booster or Interferon-Gamma Release Assays) for the diagnosis of latent TB in patients with RA on immunosuppressive therapy.

Nephrological disorders and neurological involvement in pediatric primary Sjogren syndrome:a case report and review of literature

BackgroundSjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients. To date, there are few cases concern about neurological and nephrological disorders in childhood Sjögren syndrome. We describe a case of Sjögren syndrome in a 12-year-old girl who developed neurological disorders and interstitial nephritis and review the literature currently available on this topic.Case presentationA 12-year-old girl was admitted to our hospital for arthritis and glucosuria. She was required to do labial gland and renal biopsy, because the positive for anti-nuclear antibody and anti-Sjögren syndrome B (anti-SSB) antibody. Then the biopsy was performed revealing the lymphocytic infiltrate in the small area and renal tubular interstitial damage,thus the diagnosis of Sjögren syndrome with tubular interstitial damage was made. Three months later, she presented again with headache, fever, nausea, vomiting and was recovered without drug therapy. Based on the patient’s medical history, laboratory and imaging examination, and treatment, we speculate that the disorders of the nervous system were caused by the Sjögren syndrome. The girl has stable renal function and no residual nervous system damage in the next 1.5 years, but she underwent low dose prednisone therapy because of persistent renal glucosuria.ConclusionsNephrological disorders and neurological involvement are rare manifestations of Sjögren syndrome in children, and rarely presented as the initial symptoms. It should be suspected in children presenting with unexplained renal diseases, neurological abnormalities, or unexplained fever. Although there is no guidelines on the diagnosis and treatment of children Sjögren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications.

SUN-201 Adrenal Insufficiency After Treatment for Vaping Associated Pulmonary Injury

Background: In 2019 a record number of patients were admitted to our children’s hospital with e-cigarette or vaping-associated lung injury (EVALI). The majority of patients were treated with high-dose prednisone therapy including 3 days of 1000 mg daily followed by a 4 week wean. Because of the concern for iatrogenic adrenal insufficiency, all patients were then placed on a 3-4 weak hydrocortisone taper followed by ACTH stimulation testing. The purpose of this study is to document the incidence of iatrogenic adrenal insufficiency following a 2 months glucocorticoid wean. Methods: All patients seen by the Pulmonary team for EVALI who also received high dose prednisone therapy were referred to Pediatric Endocrinology for ACTH testing. A low dose (1 mcg) ACTH stimulation test was performed with cortisol measurements at baseline, 30 minutes, and 60 minutes. A passing result was any cortisol over 18 ug/dl. Results: An anticipated 20-25 patients will have had ACTH stimulation testing by the time of abstract presentation. Of those that have already completed testing, three of four patients failed the first time. Discussion: High-dose prednisone therapy for EVALI is associated with significant rates of iatrogenic adrenal insufficiency, even after slow hydrocortisone wean. Structures need to be implemented to teach patients about adrenal insufficiency prior to hospital discharge and organize adequate post-discharge follow-up until HPA function returns.

A CASE OF HYPEREOSINOPHILIC SYNDROME AFTER MEPOLIZUMAB CESSATION

Introduction Little is known about potential consequences of stopping mepolizumab therapy in asthmatic patients. This is a case of a 43 yr female diagnosed with eosinophilic myocarditis six months after cessation of mepolizumab. Case Description A 43 yr female with severe eosinophilic asthma was started on mepolizumab. She remained compliant for 2 yr with significant improvement of symptoms but due to a lapse of insurance coverage, therapy was interrupted. Six months later, she developed low-grade fever, cough, and dyspnea with infiltrates on CXR. She was diagnosed with atypical pneumonia and treated with antibiotics without improvement. Within a few days, she developed hypoxic respiratory failure requiring noninvasive ventilation support. WBC was 26.2 K/uL with eosinophilia of 9.69 K/uL. She had elevated troponin (7.58); EKG had lateral ST elevation myocardial infarction, and echocardiogram showed pericardial effusion with EF of 20-25%. Infectious workup was unremarkable. Heart catheterization revealed normal coronaries. Pericardiocentesis cytology demonstrated 89% eosinophils. Cardiac MRI showed circumferential subendocardial hyperenhancement of left ventricle and global ventricular hypokinesis. Bone marrow biopsy and cytogenetic testing were negative. She was diagnosed with eosinophilic myocarditis and started on IV methylprednisolone with marked improvement in symptoms. The patient was discharged on high dose prednisone and heart failure therapy. Three months later, she resumed mepolizumab and has been tolerating a slow prednisone taper. Discussion To our best knowledge, this is the first reported case of eosinophilic myocarditis and pneumonia after mepolizumab cessation. This patient may have had a rebound hypereosinophilia or an underlying hypereosinophilic syndrome which was masked by mepolizumab therapy.

Analysis of High Dose and Long-Term Prednisone Therapy on Trap 5B Level Change in Children with Steroid Sensitive Nephrotic Syndrome

Nephrotic syndrome is a condition which is characterized by protein leakage from the blood to the urine through glomeruli. It leads to hypoproteinemia and generalised oedema. Patients with nephrotic syndrome need high dose and long term glucocorticoid such as prednisone. High dose and long term glucocorticoid can increase bone resorption. Biological marker is a valuable tool to evaluate efficacy of therapy. TRAP 5B is a sensitive biological marker for bone resorption because it reflects the number of osteoclasts. TRAP 5B is not affected by renal dysfunction and food. It also has a low diurnal variation than other bone resorption marker. The aim of this study was to analyze the changes of TRAP 5B levels at induction and alternate phase in children with steroid sensitive nephrotic syndrome. This observational prospective study was conducted from May to October 2016. Venous blood samples obtained at 08.00-10.00 am. TRAP5B levels were measured before and after induction phase and after alternate phase using ELISA. Fifteen patients were included in this study (60% boys). Majority of their age was 6 - &lt;12 years and 40% were dependent steroid NS. TRAP 5B serum levels in induction phase increased by 37.41%±56.22%. In alternate phase, TRAP 5B serum levels increased by 28.75%±66.55% compared to the induction phase. However, the level change of both phases were not significant. As a conclusion, TRAP 5B levels increased in induction and alternate phase after high dose and long-term prednisone treatment in nephrotic syndrome.

Children with Standard Risk Acute Lymphoblastic Leukemia in Induction And Consolidation Phase

Prednisone has an important role in the therapy of patient with standard risk ALL. Patients with standard risk ALL receiving high dose prednisone as therapy and supraphysiology dose of prednisone are expected to cause suppression in HPA-axis (Hypothalamic Pituitary Adrenal axis). This suppression could reduce immune system in children with ALL and increase infection risk because reduction of cortisol level. In Indonesia, we did not find study about the incident of adrenal suppression after high dose prednisone therapy, especially in induction to consolidation phase ALL patient. The aim of this study was to analyze adrenal suppression after high dose prednisone therapy on children with standard risk acute lymphoblastic leukemia in induction and consolidation phase. This study has received a certificate of Ethical Clearance No. 588/Panke.KKE/X/2016, a longitudinal observational, prospective, non-randomized trial involving children with ALL who received prednisone for 49 days during the induction phase. We collected and compared laboratory result of cortisol level in children with ALL and received prednisone therapy during induction to consolidation phase. Sample was taken at week 0,4,5,6,7,8,10,12 in the course of ALL chemotheraphy Indonesian protocol year 2013. Serum was examined using methods CLIA ADVIA Centaur® XP. Between June 2016 – January 2017, 13 patients (8 males, 5 females) were included in this study. Decrease of cortisol level after prednisone therapy occured in week-10 as much as 53% compared with week-0 (p=0.027). Cortisol level increased 64% of week-12 compared with week-10 (p=0.003). In conclusion, high dose prednisone is not significant to causing adrenal suppression in induction phase of ALL patients, and the reducing cortisol level is reversible.

Analysis of ACTH Levels After High Dose and Long-Term Prednisone Therapy in Children with Steroid Sensitive Nephrotic Syndrome

Background The use of high dose and long-term prednisone as glucocorticoid in steroid-sensitive nephrotic syndrome patients can cause the suppressive effect on endogenous steroid production, namely HPA axis suppression which is characterized by the decrease of ACTH levels. This can decrease cortisol levels so can affect metabolism process, immune response, and brain function. Objective To analyze ACTH levels in the induction and alternating phase, and to relate with the patient’s condition both clinical and laboratory data. Methods ACTH levels were measured before and after induction phase and four weeks after alternating phase at 08.00-09.30 a.m. Results 15 patients consisted of 9 boys and 6 girls showed there were no significant differences between ACTH levels in each phase. ACTH levels were increased 23.6% from 22.2 ± 13.1 pg/mL to 27.4 ± 23.0 pg/mL during the induction phase (p&gt;0.05) and alternating phase also showed that ACTH levels were increased 1.7% from 27.4 ± 23.0 pg/mL to 27.9 ± 22.2 pg/mL (p&gt;0.05). The clinical manifestation of HPA axis suppression such as hypoglycemia, hypotension, weight loss, appetite loss, and acute dehydration were not found in the patients. Weakness, fatigue, nausea, vomiting, and abdominal pain were found only 7% after the induction phase. Conclusion HPA axis suppression did not occur after the high dose and long-term prednisone therapy in the induction and alternating phase which showed ACTH levels average in normal range. It was also supported by the absence of clinical and laboratory data that showed signs of HPA axis suppression.

Alveolar Microcirculation Injury in an Immunocompromised Patient With Influenza

SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a life threatening condition of alveolar microcirculation injury from vasculitic disorders, connective tissue diseases and some infections, like Influenza. We present a case of DAH in a patient with metastatic prostate cancer on chronic low dose prednisone therapy and a history of chemotherapy and radiation. CASE PRESENTATION: A 72 year old male presented with fever, cough and altered mental status in the setting of metastatic prostate cancer with bony and liver metastasis diagnosed nine years ago. He underwent palliative radiation five weeks prior to admission and was being treated with Abiraterone and Prednisone 10mg daily. The patient was febrile, tachypnic and tachycardic. Physical exam revealed a lethargic male with diffuse crackles on lung auscultation. Chest X-ray revealed bibasilar interstitial opacifications. Rapid flu nasal swab returned influenza A positive and Oseltamivir was started in addition to empiric antibiotics. CT chest (figure 1) showed diffuse bilateral airspace disease. The patient subsequently developed increased oxygen requirements with PaO2/FiO2 of 59 and hemolytic anemia requiring transfusion. Repeat chest x-ray (figure 1) showed worsening diffuse interstitial infiltrates and nodular densities. He was electively intubated and transferred to the ICU. Low tidal volume ventilation was initiated and bedside bronchoscopy was performed revealing diffuse airway petechiae. Lingular bronchoalveolar lavage returned blood without clearance after multiple sequential aliquots (figure 2). Cell count showed 700 WBC (50% neutrophils, 16% lymphocytes and 34% monocytes) and 150,000 RBC. Pathology returned with pigment-laden macrophages. Bronchial cultures were negative for bacteria, fungi and viruses. Rheumatologic serology was negative. Patient suffered a prolong ICU stay but was ultimately successfully extubated. DISCUSSION: Although Influenza A is one etiology of alveolar damage which may result in DAH, many medical professionals are not aware that the presence of malignancy may compound the risk for this complication. There are few case reports of H1N1 pneumonia complicated by alveolar hemorrhage. Patients with malignancies are more susceptible for the acquisition of infections than the general population. To that extent, disruptions in both innate and acquired immunity place cancer patients at higher risk for complications related to infectious organisms. CONCLUSIONS: Our intention is to shed light on the connection between influenza A and DAH in immunocompromised patients so that prompt and appropriate treatment can be initiated in order to reduce morbidity and mortality associated with this disease process. Reference #1: Hajjar, L. A, et al. (2010). Severe novel influenza A (H1N1) infection in cancer patients.Annals of Oncology, 21, 2333-2341. Reference #2: Mussi von Ranke, F, et al.(2013). Infectious Diseases Causing Diffuse Alveolar Hemorrhage in Immunocompetent Patients: A State-of-the-Art Review. Lung,191, 9-18. DISCLOSURE: The following authors have nothing to disclose: Clarissa Castanon, Christopher Jordan, Stephen Milan, Saleem Shahzad No Product/Research Disclosure Information

Metastatic Gastric Signet Ring Cell Carcinoma Mimicking Crohnʼs Disease

Background: Signet-ring cell carcinoma (SRCC) is a rare adenocarcinoma characterized by mucin producing cells that commonly arises in the stomach. Here we describe the first case of metastatic gastric SRCC mimicking Crohn's disease (CD). Case: A 40 year-old Puerto-Rican man without significant medical history presented to another facility with 3 months of abdominal pain, associated with loose brown stool, and 20 lbs. weight loss. Stool studies were negative and computed tomography (CT) revealed thickening of the transverse and descending colon. Colonoscopy showed marked mucosal inflammation with stenosis starting at 30 cm from the anal verge and extending to the distal transverse colon. Biopsies were negative for malignancy. Over the course of a year the patient was repeatedly admitted for obstructive symptoms. Due to concern for inflammatory bowel disease (IBD), mesalamine and high dose prednisone therapy was initiated and later escalated to azathioprine and infliximab with minimal relief. Subsequent imaging and colonoscopy showed no significant changes beyond a few terminal ileum ulcers. At our institution, magnetic resonance enterography (MRE) showed a thickened transverse colon without the previously noted mesenteric stranding. The patient underwent a total colectomy with ileorectal anastomosis. Pathology revealed a poorly differentiated carcinoma with signet-ring cell features involving the hepatic flexure, transverse, and proximal descending colon. Immunohistochemical staining favored metastatic disease. Upper endoscopy confirmed the suspected gastric origin. Discussion: Primary gastric signet-ring cell carcinoma is an uncommon diagnosis that presents at a younger age and often late in the disease course. Colonic SRCC can share features of colitis including long segments of concentric bowel wall thickening and ulcerated mucosa with regions of sparing. Our patient underwent two colonoscopies with biopsies showing no evidence of malignancy. This misdiagnosis is likely due to endoscopic sampling error and resulted in treatment for presumed CD. The diagnosis of SRCC was only made after the colectomy was performed. Our case typifies some of the challenges of endoscopists and pathologists in detecting colonic neoplasia in younger populations which impacts survival. Physicians need to have a higher index of suspicion for malignancy in patients with presumed IBD that present with high-risk strictures, and are refractory to conventional treatment.Figure 1Figure 2

Relapse episodes in childhood primary nephrotic syndrome treated by alternate or three consecutive daily dose prednisone therapy

Background Prednison is still the drug of choice for the treatmentof nephrotic syndrome, especially for those with minimal change.Methods of treatment to optimize the effectiveness and efficacyare still in discussion.Objectives To evaluate the episode of relapsing minimal changenephrotic syndrome patients who received prednisone therapy byalternate or by three consecutive dose methods.Methods We performed a retrospective cohort study using medicalrecords of the patients with primary nephrotic syndrome admittedto Division of Nephrology, Sardjito Hospital, Yogyakarta fromJanuary 1995 to January 2005. Subjects were divided into twogroups, the first group treated with alternate days while thesecond group with three consecutive days prednisone program.Evaluation had been done to compare both treatment program(alternate days or consecutive days).Results Relapse episodes after six month recovery periods withalternate days treatment was 33% while those with consecutivedays was as high as 83% (P&gt;O.Ol).Conclusion Alternate dose group has a lower relapse eventcompared to three consecutive dose group in children withnephrotic syndrome.

Anti-glomerular basement membrane antibody disease treated with rituximab: A case-based review

To report the successful use of rituximab in a patient with anti- glomerular basement membrane (GBM) antibody disease and to review the literature regarding rituximab use in anti-GBM mediated disease. We report a case of anti-GBM antibody disease with both anti-GBM antibodies and anti-myeloperoxidase (MPO) specific p-ANCA, who developed thrombotic thrombocytopenic purpura (TTP) on high dose prednisone, plasmapheresis, and cyclophosphamide therapy. The patient was then treated with rituximab. We analyzed the clinical features of five additional patients of anti-GBM disease treated with rituximab identified through a systematic literature review. Our patient was 68-year-old female who presented with acute renal failure. Renal biopsy showed crescentic glomerulonephritis with linear deposits of IgG antibody along the glomerular basement membrane. Treatment was initiated with high dose prednisone, plasmapheresis and oral cyclophosphamide, with subsequent development of leukopenia and TTP and discontinuance of cyclophosphamide. Treatment with rituximab was initiated with clinical improvement of her hematological parameters but not her renal function. Among the five previously reported cases of anti-GBM disease treated with rituximab, three received brief course of IV cyclophosphamide prior to use of rituximab. Except one patient, all recovered renal function and remained dialysis independent. The anti-GBM antibody level remained undetected in all patients. Combination of prednisone, plasmapheresis, and rituximab can be an effective therapy in patients with an anti-GBM antibody disease complicated with TTP.

Lenalidomide for the Treatment of Cryoglobulinemia and Undifferentiated Spondyloarthropathy in a Patient With Multiple Myeloma

Cryoglobulinemia refers to the presence of one or more immunoglobulins that precipitate at temperatures below 37°C and redissolve on rewarming. It is classified into 3 types based on immunochemical characteristics of the paraprotein. Type I is a monoclonal immunoglobulin that is most often associated with Waldenstrom macroglobulinemia, lymphoma, multiple myeloma (MM), and monoclonal gammopathy of unknown significance (MGUS).1 The cutaneous manifestations of cryoglobulinemia include palpable purpura, livedo reticularis, ischemic necrosis, and cutaneous ulcerations.1 Here, we report a case of cryoglobulinemia in a patient with undifferentiated spondyloarthropathy and multiple myeloma, who was successfully treated with lenalidomide. A 43-year-old white man presented with history of recurrent episodes of anterior uveitis and progressive limping secondary to lower back and buttock pain. His physical examination showed only mild tenderness and swelling of the right midfoot. Erythrocyte sedimentation rate (ESR) was 54 (<15), human leukocyte antigen B27 was positive, and magnetic resonance imaging of the lumbosacral spine was consistent with left sacroiliitis (Fig. 1). A diagnosis of undifferentiated spondyloarthropathy was made and he was managed conservatively with nonsteroidal anti-inflammatory medications.2 FIGURE 1 Magnetic resonance imaging of the sacroiliac joints, T2-FLAIR sequence showing edema in the left SI joint (arrow). One year later, he developed recurrent sinopulmonary infections. Extensive workup revealed elevated serum monoclonal IgG to 2.82 g/dL ( 13), calcium level was 9.0 mg/dL (<10.4), and no overt lytic lesions were detected on the skeletal survey. Therapy was deferred given the absence of end organ damage.3 Three months later, he developed a flare of the inflammatory arthritis manifested by worsening right foot and left hip pain and new rashes. Physical examination noted limited left hip range of motion secondary to pain, right foot swelling, and livedo reticular type of rashes associated with necrotic plaques and ulcerations on the bilateral lower extremities. ESR was now 134 and hepatitis C viral antibody was negative. Type I cryoglobulinemia was diagnosed with a cryocrit of 8% (<0.5% volume) and pathologic findings of ischemic and thrombotic vasculopathy with extensive fibrin deposition and red blood cell extravasations on skin biopsy (Fig. 2). FIGURE 2 Skin biopsy showing fibrin deposition and red blood cell extravasations (arrows). Low dose prednisone therapy was first attempted without significant clinical improvement in several weeks and his cryocrit was now 25%. Plasmapheresis was initiated and his cutaneous symptoms partially improved. Lenalidomide, 25 mg by mouth once a day 3 weeks on and 1 week off, and weekly dexamethasone pulse, 40 mg by mouth, were then started and continued for 4 cycles. The skin ulcers healed, his livedo reticular rash resolved, and his cryocrit became negative. Interestingly, his hip and foot pain also resolved; physical examination revealed diminished right foot swelling; and the ESR post-treatment became undetectable. The serum monoclonal IgG was now is 0.76 g/dL and repeat bone marrow biopsy showed 3% plasma cells. He subsequently underwent consolidative bone marrow transplantation and remained symptom free 8 months after treatment with lenalidomide. Treatment options for patients with Type I cryoglobulinemia occurring in the setting of MM or MGUS include systemic corticosteroid therapy with or without alkylating agents.1 Plasmapheresis has been used in severe or life threatening cases of cryoprecipitation or serum hyperviscosity syndrome. However, it is inconvenient and produces only transient decrease in cryoglobulin level.4 Lenalidomide is a new generation antiangiogenic and immunomodulatory drug related to thalidomide, an inhibitor of the tumor necrosis factor (TNF) alpha pathway.5 It has enhanced TNF-alpha inhibitory activity and tumor cytotoxicity, and fewer side effects of deep vein thrombosis and peripheral neuropathy than thalidomide. It is now used as a first-line treatment for MM and myelodysplastic syndrome.3 This is the first reported case of human leukocyte antigen B27 spondyloarthropathy and Type I cryoglobulinemia treated successfully with lenalidomide. Review of the literature revealed only 2 other cases of paraproteinemia-associated Type I cryoglobulinemia treated with thalidomide. Neither of these 2 cases had associated inflammatory arthritis.6,7 The first unique feature of our case is that lenalidomide appears to have activity in both HLA B27 spondyloarthropathy as well as Type I cryoglobulinemia. This could be explained mechanistically by the involvement of TNF-alpha pathway in both disease processes, and supported by clinical observations that inflammatory arthritis can be associated with MGUS and MM.8 The second unique feature is that the exacerbation of his inflammatory spondyloarthropathy was temporally associated with the development of cryoglobulinemia. It is possible that cryoglobulin crystals may be contributing to the worsening of arthropathy as suggested by a recent report.9 The arthropathy in that patient responded to oral and intra-articular corticosteroids, and MM was subsequently diagnosed and treated with lenalidomide.9 In summary, on the basis of our limited experience and literature review, lenalidomide may have a potential role in the treatment of inflammatory spondyloarthropathy as well as Type I cryoglobulinemia associated with paraproteinemia.

A Prospective, Randomized, Double-Blind, Placebo-Controlled Multicenter Trial Comparing Early (7 Day) Corticosteroid Cessation Versus Long-Term, Low-Dose Corticosteroid Therapy

To compare outcomes with early corticosteroid withdrawal (CSWD) and chronic low dose corticosteroid therapy (CCS). Final, 5-year results from the first randomized, double-blind, placebo-controlled trial of early CSWD (at 7 days posttransplant) are presented. Adult recipients of deceased and living donor kidney transplants without delayed graft function were randomized to receive prednisone (5 mg/d after 6 months posttransplant) or CSWD. Blinding was maintained for 5 years. This clinical trial is registered at www.clinicaltrials.gov (NCT00650468). Results in 386 patients CSWD (n = 191), CCS (n = 195) are presented (CSWD; CCS). No differences were observed at 5 years in the proportion of patients experiencing: primary end point (composite of death, graft loss, or moderate/severe acute rejection) (30/191 (15.7%); 28/195 (14.4%)), patient death (11/191(5.8%);13/195 (6.7%)), death-censored graft loss (11/191 (5.8%); 7/195(3.6%)), biopsy confirmed acute rejection (BCAR) (34/191 (17.8%); 21/195 (10.8%), P = 0.058), moderate/severe acute rejection (15/191 (7.9%); 12/195 (6.2%)). Kaplan Meier analyses of the primary end point and its components also showed no differences; but BCAR was higher with CSWD (P = 0.04). Increased BCAR episodes were primarily corticosteroid-sensitive Banff 1A rejections: the incidence of antibody-treated BCAR was similar between groups (11/191 (5.8%); 13/195 (6.7%)). No differences in renal function were observed at 5 years: mean serum creatinine (1.5 +/- 0.6; 1.5 +/- 0.7 mg/dL), or Cockroft Gault calculated creatinine clearance (58.6 +/- 19.7; 59.8 +/- 20.5 mL/min). CSWD was associated with improved serum triglycerides (evaluated by mean and median change from baseline) at all time points (except at 5 years measured by mean change). Weight change also demonstrated changes favoring CSWD (median change from baseline at 5 years: 5.1 vs. 7.7 kg, P = 0.05). New onset diabetes after transplant (NODAT) was similar with respect to proportions who required treatment (23/107 (21.5%)); 18/86 (20.9%); however, fewer CSWD patients required insulin for NODAT at 5 years (4/107 (3.7%)); 10/86 (11.6%), P = 0.049). Changes in HgA1c values (from baseline) were lower in CSWD patients at all time points except 4 years. Early CSWD, compared with CCS, is associated with an increase in BCAR primarily because of mild, Banff 1A, steroid-sensitive rejection, yet provides similar long-term renal allograft survival and function. CSWD provides improvements in cardiovascular risk factors (triglycerides, NODAT requiring insulin, weight gain). Tacrolimus/MMF/antibody induction therapy allows early CSWD with results comparable to long-term low dose (5 mg/d) prednisone therapy.