Use Of Steroids In Patients Research Articles

Dose finding study of the intratumoral administration of the oncolytic polio/rhinovirus recombinant (PVSRIPO) against WHO grade IV malignant glioma (MG).

e13533 Background: The live attenuated oral poliovirus vaccine was modified to contain a heterologous internal ribosomal entry site stemming from human rhinovirus type 2, creating PVSRIPO. PVSRIPO recognizes CD155, an oncofetal cell adhesion molecule and tumor antigen widely expressed ectopically in malignancy. We report results of the dose finding trial evaluating PVSRIPO delivered intratumorally by convection-enhanced delivery (CED). Methods: Eligible patients were adults with recurrent supratentorial WHO grade IV MG; solitary tumor 1-5.5cm in diameter; ≥4 weeks after chemotherapy, bevacizumab or study drug; adequate organ function; KPS≥70%; and positive anti-polio titer. The original two-step continual reassessment method dose escalation was amended to decrease to dose level(DL) -1 and DL -2 after observing prolonged steroid use in patients treated on higher DLs. Results: As of 2/01/2017, 52 pts were treated on study (1 each at DL1 and DL3, 7 at DL2, 2 at DL4, 4 at DL5, 24 at DL -1 and 13 at DL -2). Only one DLT was observed, a grade 4 intracranial hemorrhage at the time of catheter removal on DL5. Grade 3 or higher adverse events possibly, probably or definitely related to PVSRIPO include: lymphopenia (grade 3, n = 1), steroid myopathy (grade 3, n = 1), cerebral edema (grade 4, n = 1), headache (grade 3, n = 1), dystonia (grade 3, n = 1), pyramidal tract syndrome (grade 3, n = 6), seizure (grade 3, n = 1; grade 4, n = 1), delusions (grade 3, n = 1), hypertension (grade 3, n = 1), and thromboembolic events (grade 3, n = 2). At a median follow-up of 20.1 months, 20.8% of pts remain alive at 36-month post PVSRIPO infusion, compared to 4% of an historical control. Four pts remain alive more than 22 months post treatment without having received any additional intervention following PVSRIPO at 57.5+, 56.4+, 27.9+ and 23.2+ months. Conclusions: Infusion of PVSRIPO via CED is safe and encouraging efficacy results are observed. The dose finding study is now completed and we are initiating clinical trials evaluating combination of PVSRIPO with other therapies. Clinical trial information: NCT01491893.

Staphylococcus Aureus Colonization Is Associated with Increased Atopy and Inhaled Steroid Use in Patients with Atopic Dermatitis and Asthma

Staphylococcus Aureus Colonization Is Associated with Increased Atopy and Inhaled Steroid Use in Patients with Atopic Dermatitis and Asthma

Development of the Systemic Lupus Erythematosus Steroid Questionnaire (SSQ): a novel patient-reported outcome tool to assess the impact of oral steroid treatment

BackgroundOral glucocorticoids (steroids) are the mainstay of treatment for systemic lupus erythematosus (SLE), but their use is often associated with short- and long-term side effects. Following a literature review and discussions with patients with SLE, clinicians, and payers, a need was identified for a comprehensive SLE-specific tool that can be used to evaluate the side effects and benefits of steroids over time from a patient perspective. The objective of this study was to develop a patient-reported outcome (PRO) measure to assess general impact (baseline burden), benefits, side effects, and impacts associated with the use of oral steroids in patients with SLE.MethodsA qualitative research protocol was developed in which adults with SLE currently receiving or who had received steroids in the past year were recruited from six US rheumatology practices to participate in concept elicitation (CE) interviews. The SLE Steroid Questionnaire (SSQ) was developed based on CE interview results and clinical input. Cognitive debriefing interviews with a second group of patients with SLE evaluated the content, clarity, and relevance of the items. The SSQ was refined using patient feedback, clinician review, and a translatability assessment. The protocol received central independent review board approval.ResultsThirty-three patients (52% moderate disease severity; 58% currently receiving steroids, mean dose 8.7 mg/day) completed CE interviews. Patients reported benefits, side effects, and impacts from steroids. The refined SSQ contains 50 items assessing steroid dose/duration (4 items), general impact (baseline burden; 19 items), benefits (7 items), work/productivity (3 items), side effects (10 items), emotions (6 items), and overall satisfaction (1 item).ConclusionThe SSQ is a unique PRO, developed using robust scientific methodology in accordance with the Food and Drug Administration PRO Guidance. It was designed to comprehensively assess the patient experience with steroid therapy and better understand the benefits and burden of steroids for patients with SLE.

Steroids Abrogate BiTE® Antibody Construct-Mediated Cytotoxicity in Primary AML Cells

Antibody based immunotherapy represents a promising strategy to eliminate chemoresistant cells in acute myeloid leukemia (AML). Clinical experience in acute lymphoblastic leukemia (ALL) has shown a clear correlation of leukemic burden and the occurrence of a cytokine release syndrome (CRS) during treatment with blinatumomab (CD19/CD3 BiTE®). A cytoreductive phase prior to or in combination with antibody therapy might be beneficial to reduce the severity of adverse events like CRS. The latter is often treated with steroids (dexamethasone, DEX) or less commonly, with the IL-6R antibody tocilizumab (TOC). As T-cell proliferation and function are of crucial importance for BiTE® activity (Zugmaier 2015), the effect of the drugs on effector cell function will dictate clinical response to therapy. In this study, we evaluated the influence of cytoreductive- (azacythidine, AZA; decitabine, DEC), and immunmodulatory (DEX and TOC) drugs on antibody-mediated cytotoxicity and T-cell proliferation. A CD33/CD3 BiTE® antibody construct (AMG 330) served as model T-cell recruiting antibody in this study. To address this question we set up the following experimental approaches: AML cells were cocultured with healthy donor (HD) T cells for up to 14 days ex vivo. T cells were either incubated with the specific drug for 3 days prior to coculture or the drugs were simultaneously added to AML-T cell cultures. Drug concentrations were chosen based on published serum concentrations in AML patients and their ex vivo stability in culture, validated by mass spectrometry. BiTE® mediated cytotoxicity and T-cell proliferation were assessed by flow cytometry. Preincubation of T cells with AZA and DEC impaired antibody mediated cytotoxicity of HL60 cells in a concentration dependent manner (% lysis control (ctrl) vs AZA at 1, 5, 10 µM: 99.9 vs 99.2 vs 52.1 vs 28.7, n=7; ctrl vs DEC at 0.2, 2, 5 µM: 98.4 vs 71.3 vs 60.0 vs 50.0, n=3). Similarly, T-cell proliferation was also markedly decreased (fold change (FC) T cells ctrl vs AZA at 1, 5, 10 µM: 2.9 vs 2.8 vs 1.5 vs 0.7; ctrl vs DEC at 0.2, 2, 5 µM: 3.8 vs 3.0 vs 2.3 vs 1.2). For DEX it was shown that incubation of T cells with steroids prior to cocultures had no negative effect on BiTE® mediated cytotoxicity (Brandl 2007). However, as steroids are often used simultaneously with T-cell recruiting immunotherapies, we tested the influence of DEX in combination with AMG 330. The addition of DEX to primary AML-T cell cultures (75 ng/ml) significantly impaired AMG 330 mediated cytotoxicity (% lysis AMG 330 vs AMG 330+DEX day (d) 6: 95.9 vs 47.5, n=9). This correlated to a markedly reduced T-cell proliferation (FC T cells AMG 330 vs AMG 330+DEX d6: 11.2 vs 1.2, n=9). Correspondingly, secretion of IFNγ was also decreased (n=3). Upon discontinuation of DEX an increase in AMG 330 mediated cytotoxicity was observed. Nevertheless, cytotoxicity was still considerably lower compared to control cultures (%lysis AMG 330 vs AMG 330+DEX d9: 95.6 vs 77.0). In contrast to DEX, TOC (110 µg/ml) had no negative effect on T-cell proliferation (FC T cells d6: AMG 330 vs AMG 330+TOC: 42.3 vs 36.9, n=4). Similarly, secretion of IFNγ was not affected through the simultaneous addition of TOC to primary AMG 330 cultures (pg/ml AMG 330 vs AMG 330+TOC d6: 543.9 vs 345.8 n=2). Importantly, drugs might not only interfere with effector cell function but also modulate target antigen expression. As we have previously demonstrated that antigen expression levels influence BiTE® mediated cytotoxicity (Krupka 2016), we analysed the effect of the drugs on CD33 expression. None of the drugs induced a significant up- or downregulation of CD33 on AML celllines as detected by flow cytometry. Hence our data support the notion that these drugs do not modulate antigen expression dependent lysis kinectics. We conclude, that drugs given prior or concomitant to BiTE® therapy have the potential to reduce T-cell proliferation and cytotoxicity. In particular, we observed a negative impact of AZA and DEC when given prior to AML-T cell cocultures. Importantly, even short exposure to DEX led to a significanly reduced T-cell responsiveness. Our data suggest the careful evaluation of concomitant drugs in T-cell recruiting antibody therapies and support the restrictive use of steroids in patients receiving BiTE® antibody therapy. For management of severe CRS, TOC could be considered as a targeted biologic therapy that preserves BiTE®-dependent T cell function. DisclosuresKrupka:AMGEN Research Munich: Research Funding. Kufer:AMGEN Research Munich: Employment, Equity Ownership, Patents & Royalties. Kischel:AMGEN Research Munich: Employment, Equity Ownership, Patents & Royalties. Subklewe:AMGEN Research Munich: Research Funding.

Patterns Between Multi-Year Steroid Use in Patients with Inflammatory Bowel Disease and Quality of Life, Disease Severity, and Healthcare Utilization

Introduction: Quality improvement measures for Inflammatory Bowel Disease (IBD) providers emphasize limiting steroid use, as prolonged use is associated with complications including infection, osteoporosis. The aim of this study was to characterize patterns of steroid use in IBD patients over multi-year time periods and the relationship with quality of life (QOL), healthcare use, and disease severity. Methods: Patients followed in a prospective IBD natural history registry for >4 years from 2009 to 2015 were grouped based on low (<1 year), medium (2 to 3 years), and high steroid use (>4 years). Measures of disease activity, including Harvey-Bradshaw Index (HBI), Ulcerative Colitis Activity Index (UCAI); QOL using the Short Inflammatory Bowel Disease Questionnaire (SIBDQ) (with scores < 50 indicating poor QOL); healthcare use, including total charges, ER and clinic visits, hospital admissions; narcotic use; antidepressant use were organized. Steroid usage groups were compared using ANOVA analysis for parametric measures and Kruskal-Wallis H for non-parametric ones. Results: 1,457 patients were followed (47.0+15.5 years old, 52.4% female, 56.6% with Crohn's Disease, 34.8% with Ulcerative Colitis) and divided into low (63.7%, n=928), medium (24.3%, n= 354), and high steroid use (12.0%, n=175) without a difference in age (p=0.44), disease type (p=0.094), mean BMI (p=0.25). Mean SIBDQ scores varied amongst groups (54.4+10.8 for low, 50.4+11.3 for medium, 48.6+10.9 for high use, p0.001). The groups varied in narcotic and anti-depressant use, chronic abdominal ratings, as well as feeling discouraged, unable to conduct regular activities (all p < 0.001). Steroid use correlated with Clostridium difficile infection and vancomycin exposure (all p < 0.001). Disease activity indices (UCAI, HBI) and healthcare utilization, including total charges, ER and clinic visits, and hospital admissions were found different amongst the groups (all p < 0.001). Conclusion: Most IBD patients require low steroid use, but a subset requires prolonged use given more severe and refractory disease. Quality measures for steroid use need to account for the difference amongst patients when assessing providers. Steroid use also correlates with potentially modifiable measures, such as worse QOL measures and narcotic and anti-depressant use. Through multi-faceted care that focuses on pain control and psychiatric care, IBD providers may be able to improve steroid use amongst their patients.

Partial remission by cyclosporine monotherapy in a patient with membranous nephropathy superimposed diabetic nephropathy.

It has been noted that cyclosporine A (CsA) is an effective drug for membranous nephropathy (MN). Diabetes is a common disease that sometimes causes nephrotic syndrome. We report the case of an 89-year-old woman with type 2 diabetes mellitus who exhibited nephrotic syndrome. Examination of a renal biopsy indicated MN and she was prescribed CsA as monotherapy. Her edema subsided and she achieved partial remission. This is the first report of a patient in diabetic condition with MN having achieved partial remission after CsA monotherapy without steroid therapy. The use of steroid in patients with diabetes may worsen their diabetic condition, especially if they are of very advanced age. CsA monotherapy may be useful for diabetic patients with MN.

Steroid-exacerbated HIV-associated cutaneous Kaposi’s sarcoma immune reconstitution inflammatory syndrome: ‘Where a good intention turns bad’

A 51-year-old man with head and neck skin lesions was diagnosed with Kaposi's sarcoma (KS) as his initial presentation of acquired immunodeficiency syndrome. Following initiation of antiretroviral therapy and subsequent full virologic suppression, his facial lesions worsened, consistent with immune reconstitution inflammatory syndrome (IRIS). He was started on glucocorticoids in an attempt to ameliorate the KS-IRIS but experienced paradoxical worsening of the KS lesions. Steroids were subsequently discontinued and he required chemotherapy for severe and cosmetically disfiguring skin lesions. This article describes the potential for worsening of KS lesions in individuals started on glucocorticoids for KS-IRIS as this has been reported rarely in published literature. The mechanisms underlying this phenomenon remain poorly understood but potential explanations are offered in the case discussion. This article aims to raise clinician awareness on the harms of steroid use in patients with KS-IRIS.

Hip Arthroscopy Surgical Volume Trends and 30-Day Postoperative Complications

To determine hip arthroscopy surgical volume trends from 2006 to 2013 using the National Surgical Quality Improvement Program (NSQIP) database, the incidence of 30-day complications of hip arthroscopy, and patient and surgical risk factors for complications. Patients who underwent hip arthroscopy from 2006 to 2013 were identified in the NSQIP database for the over 400 NSQIP participating hospitals from the United States using Current Procedural Terminology and International Classification of Diseases, Ninth Revision codes. Trends in number of hip arthroscopy procedures per year were analyzed. Complications in the 30-day period after hip arthroscopy were identified. Univariate and multivariate regression analyses were performed to identify risk factors for complications. We identified 1,338 patients who underwent hip arthroscopy, with a mean age of 39.5 ± 13.0 years. Female patients comprised 59.6%. Hip arthroscopy procedures became 25 times more common in 2013 than 2006 (P < .001). Major complications occurred in 8 patients (0.6%), and minor complications occurred in 11 patients (0.8%); overall complications occurred in 18 patients (1.3%) (1 patient had 2 complications). The most common complications were bleeding requiring a transfusion (5, 0.4%), return to the operating room (4, 0.3%), superficial infection not requiring return to the operating room (3, 0.2%), deep venous thrombosis (2, 0.1%), and death (2, 0.1%). Multivariate analysis showed that regional/monitored anesthesia care as opposed to general anesthesia (P= .005; odds ratio, 0.102) and a history of patient steroid use (P= .05; odds ratio, 8.346) were independent predictors of minor complications in the 30days after hip arthroscopy. Hip arthroscopy is an increasingly common procedure, with a 25-fold increase from 2006 to 2013. There is a low incidence of 30-day postoperative complications (1.3%), most commonly bleeding requiring a transfusion, return to the operating room, and superficial infection. Regional/monitored anesthesia care and steroid use were independent risk factors for minor complications. Level III, retrospective comparative study.

Corticosteroids and occurrence of and mortality from infections in severe alcoholic hepatitis: a meta-analysis of randomized trials.

Prednisolone is the first-line therapy for severe alcoholic hepatitis (AH). Patients with severe alcoholic hepatitis often develop severe infections that negatively impact short-term prognosis. We performed this meta-analysis to assess the effect of corticosteroids on the occurrence of and mortality from infections in patients with severe alcoholic hepatitis. Randomized controlled trials examining the use of corticosteroids in severe alcoholic hepatitis and reporting data on infection rates and mortality were included. Random effects model was used to pool the data comparing arms with and without steroids for the occurrence of infection, 28-day mortality and cause specific mortality. Of 1062 patients (528 steroids treated) without infection at baseline from 12 studies, infection was reported in 213 (113 steroids treated) patients without differences comparing arms with and without steroids (OR: 0.98; CI: 0.49-1.94). However, frequency was higher for occurrence of fungal infections among steroid-treated patients (eight of 528 vs. one of 534; P = 0.02). Steroids provided mortality benefit at 28 days (OR: 0.55; CI: 0.34-0.90) mainly for liver failure-related death (OR: 0.46; CI: 0.24-0.87) without differences on mortality from infection (OR: 1.19; CI: 0.38-3.73) or gastrointestinal bleeding (OR: 0.90; CI: 0.43-1.87). Three of nine patients with fungal infections died, all in corticosteroid arm. Corticosteroids do not increase occurrence of or mortality from bacterial infections in patients with severe alcoholic hepatitis. Further studies are needed to develop strategies of reducing the risk of fungal infection with use of steroids for patients with severe alcoholic hepatitis.

A Phase II study of the efficacy and safety of rontalizumab (rhuMAb interferon-α) in patients with systemic lupus erythematosus (ROSE)

ObjectivesTo examine the safety and efficacy of rontalizumab, a humanised IgG1 anti-interferon α (anti-IFN-α) monoclonal antibody, in patients with moderate-to-severe systemic lupus erythematosus (SLE).MethodsPatients with active SLE were randomised (2:1)...

Su1339 The Frequency of Steroid Use in Patients With Ulcerative Colitis Who Have Concomitant Primary Sclerosing Cholangitis

Su1339 The Frequency of Steroid Use in Patients With Ulcerative Colitis Who Have Concomitant Primary Sclerosing Cholangitis

Side effects of steroid use in patients with traumatic spinal cord injury

OBJECTIVE: Indicate and identify potential complications in our unit associated with the use of steroids in patients over 16 years of age with traumatic acute spinal cord injury managed with NASCIS II, III scheme compared with patients with the same characteristics who did not receive this management. METHODS: To conduct a research study with reports of cases and controls in patients over 16 years of age and with an established diagnosis of acute spinal cord injury, treated definitively in our unit, performing the comparison of evolutionary process between those treated with steroids and those who were not, based on the development of a data collection sheet with several variables.. The results were encoded, tabulated and analyzed. RESULTS: A total of 30 patients were analyzed from January to December 2012 and it was found that 16% of the patients managed with the steroid scheme required admission to the intensive care unit, 40% developed hospital-acquired pneumonia, 17% had urinary tract infection, 3% progressed to respiratory failure and 20% of this group had gastrointestinal bleeding. CONCLUSIONS: It was concluded that steroid management is not a risk-free therapy and the recommendation is to make a direct assessment of the potential benefit to its use in relation to the possible complications that can ensue before choosing this option in patients with traumatic spinal cord injury.

Severe community-acquired pneumonia: Severity and management

Community-acquired pneumonia (CAP) is a leading cause of morbidity and mortality worldwide. Severe CAP'' (sCAP) identifies a group of patients who have severe disease and require Intensive Care Unit admission. Recommendations for antibiotic treatment for sCAP are based on illness severity, frequency of specific pathogens and local microbial resistance patterns. The challenges to patient management include the emergence of the high prevalence of multidrug-resistant in CAP, mainly from institutionalizing patients. A new approach is the evaluation of biomarkers as C-reactive protein, procalcitonin on the diagnosis, prognosis and therapy duration of sCAP with promising results . Implementation of guidelines for CAP treatment should be emphasized in order to increase survival. The benefits of steroid use in patients with severe pneumonia have not been proven yet by current literature.

Corticosteroids in Stevens-Johnson Syndrome/toxic epidermal necrolysis: current evidence and implications for future research.

To review the evidence for the use of steroids in adults presenting with Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), or overlap. EMBASE (1974 to April 2014), MEDLINE (1946 to April 2014), Cochrane Database of Systematic Reviews, and International Pharmaceutical Abstracts (1970 to January 2014) were searched using the terms: prednisone, methylprednisolone, dexamethasone, prednisolone, steroids, glucocorticoids, corticosteroids, Stevens-Johnson Syndrome, toxic epidermal necrolysis, and SJS/TEN overlap. English-language, full reports of experimental and observational studies were included. Bibliographies from pertinent publications were reviewed for additional references. Prespecified outcomes included survival, survival to discharge, hospitalization without intensive care, length of intensive care stay, duration of hospitalization, ophthalmological complications, infection rates, and adverse events. Six studies that used steroids for SJS, TEN, and/or overlap were included. All studies were retrospective cohort studies with no case-control or cross-sectional studies; 5 studies reported on steroid doses, and 2 studies reported time from disease onset to steroid use (2-4 days). Only 1 of 6 studies reported a statistically significant impact on mortality with steroids use (odds ratio = 0.4; 95% CI = 0.2-0.9). Adverse event rates were not reported in any of the studies. A review of the current evidence reveals a need for prospective, randomized controlled studies to provide more definitive conclusions on steroid use in patients with SJS, TEN, and/or overlap.

Steroid Exposure, Acute Coronary Syndrome, and Inflammatory Bowel Disease: Insights into the Inflammatory Milieu

BackgroundSteroids are anti-inflammatory agents commonly used to treat inflammatory bowel disease. Inflammation plays a critical role in the pathophysiology of both inflammatory bowel disease and acute coronary syndrome. We examined the relationship between steroid use in patients with inflammatory bowel disease and acute coronary syndrome. MethodsIn 177 patients with inflammatory bowel disease (mean age 67 years, 75% male, 44% Crohn's disease, 56% ulcerative colitis), we performed a 1:2 case-control study matched for age, sex, and inflammatory bowel disease type, and compared 59 patients with inflammatory bowel disease with acute coronary syndrome to 118 patients with inflammatory bowel disease without acute coronary syndrome. Steroid use was defined as current or prior exposure. Acute coronary syndrome was defined as myocardial infarction or unstable angina, confirmed by cardiac biomarkers and coronary angiography. ResultsIn patients with inflammatory bowel disease, 34% with acute coronary syndrome had exposure to steroids, vs 58% without acute coronary syndrome (P < .01). Steroid exposure reduced the adjusted odds of acute coronary syndrome by 82% (odds ratio [OR] 0.39; 95% confidence interval [CI], 0.20-0.74; adjusted OR 0.18; 95% CI, 0.06-0.51) in patients with inflammatory bowel disease, 77% in Crohn's disease (OR 0.36; 95% CI, 0.14-0.92; adjusted OR 0.23; 95% CI, 0.06-0.98), and 78% in ulcerative colitis (OR 0.41; 95% CI, 0.16-1.04; adjusted OR 0.22; 95% CI, 0.06-0.90). There was no association between other inflammatory bowel disease medications and acute coronary syndrome. ConclusionsIn patients with inflammatory bowel disease, steroid use significantly reduces the odds of acute coronary syndrome. These findings provide further mechanistic insight into the inflammatory processes involved in inflammatory bowel disease and acute coronary syndrome.

A81: Spectrum of Mevalonate Kinase Deficiency: Is Colitis More Common Than We Think?

Background/Purpose:Mevalonate kinase deficiency (MKD) is an autosomal recessive inflammatory disorder caused by a mutation in the mevalonate kinase (MVK) gene. This leads to decreased enzymatic activity, resulting in impaired cholesterol biosynthesis and increased production of inflammatory mediators such as interleukin 1‐β. There are two known forms of MKD, with Hyperimmunoglobulinemia D Syndrome (HIDS) carrying a milder phenotype and Mevalonic Aciduria (MA) representing a severe form. HIDS is characterized by recurrent febrile episodes accompanied by varying clinical features that include lymphadenopathy, gastrointestinal symptoms, oral ulcers, and arthralgia. A recent report identified early onset colitis as a feature of MKD in two patients (Levy M et al, 2013). Here we report the clinical and laboratory phenotypes of patients cared for at Boston Children's Hospital who were diagnosed with HIDS/MKD.Methods:We conducted a retrospective chart review of children with HIDS/MKD treated from 2002–2012 at the Rheumatology Clinic at Boston Children's Hospital. Charts were first identified by searching for key words or billing codes related to autoinflammatory syndromes. A manual chart review was then performed to confirm appropriate case identification.Results:Four patients were diagnosed with HIDS/MKD over the 10‐year period. Age of symptom onset varied from 2 months to 1 year. Fever duration ranged from 1–5 days, with duration between fevers ranging from 9 days to 12 weeks. Symptoms included sore throat, oral lesions, vomiting, diarrhea, abdominal pain, rash, and arthralgia. Prodromal symptoms were identified in all patients, and included irritability, anorexia, fatigue, geographic tongue, rash, and neck pain. Three of the four patients had a history of bloody stools beginning in infancy/early childhood, and were followed by gastroenterology. All patients had lymphadenopathy on exam, and splenomegaly was detected in two patients by ultrasound. Labs obtained during febrile episodes showed elevated inflammatory markers, however, in two out of three patients evaluated in between fevers, the ESR was persistently elevated. All four patients had elevated IgA when compared to age matched normal values, and two patients had elevated IgD levels. Genetic testing was performed in all patients, with all four having two heterozygous mutations within the MVK gene. In addition, one patient also had single variant mutations of unclear significance in two other genes associated with autoinflammatory syndromes. Two patients were treated with steroids and in each case this aborted febrile episodes, however, in one patient steroid use was also associated with increased fever frequency. One patient had decreased severity of symptoms when treated with colchicine as well as with montelukast, and had rapid resolution of fever and associated symptoms when treated with anakinra.Conclusion:The persistence of ESR elevation outside of febrile episodes seen in patients with HIDS/MKD may indicate the presence of chronic subclinical inflammation. Hematochezia was commonly seen, and could indicate the presence of colitis. Of the immunomodulatory medications used in this small patient population, anakinra appeared to be effective.

Management of Bell's palsy in children: an audit of current practice, review of the literature and a proposed management algorithm

ObjectiveWe carried out a complete audit cycle, reviewing our management of paediatric patients with Bell's palsy within 72 h of symptom onset. Our protocol was published after the initial audit...

Anti-SSA Ro52/Ro60 antibody testing by immunodot could help the diagnosis of Sjogren’s syndrome in the absence of anti-SSA/SSB antibodies by ELISA

The objective of this study was to assess the diagnostic value of anti-Ro52/60 antibodies by immunodot in patients with suspected SS. All patients between 2008 and 2012 with suspected SS without anti-SSA/SSB antibodies by ELISA and who had a determination of anti-SSA (Ro52/Ro60) antibodies by immunodot (Euroimmun, Germany) were retrospectively analysed. Eighty-four patients (median age 62 years; males 35 cases) were included. Forty-five patients had associated interstitial lung disease (ILD). American-European Consensus Group (AECG) criteria for SS were fulfilled in 10 patients (12%) with a positive salivary gland biopsy, and among them 2 patients had anti-Ro52 antibodies by dot (20%). Among 74 patients with a negative salivary gland biopsy, 16 (22%) had anti-Ro52/60 antibodies and 10 (14%) fulfilled AECG criteria when including Ro52/60 antibodies. A comparison of patients with and without anti-Ro52/60 antibodies revealed no differences, except gamma globulin levels and more frequent steroid use in patients with anti-Ro52/60 antibodies (P < 0.05). In 45 patients with ILD and dry eye/mouth syndrome, 10 (22%) had positive anti-Ro52/60 antibodies. In these patients 2 (4%) fulfilled AECG criteria without anti-Ro52/60 antibodies vs 4 (8%) patients if anti-Ro52/60 antibodies were included. In patients with dry eye/mouth syndrome without anti-SSA/SSB antibodies by ELISA, the detection of anti-Ro52/Ro60 antibodies by dot could help in the diagnosis of SS. In patients with ILD, the lower frequency of a Chisholm score ≥3 enhances the interest of anti-Ro52/Ro60 screening.

The Management of HCV Recurrence after Liver Transplantation

Liver transplantation (LT) for hepatitis C virus (HCV)-related liver diseases has been increasing in Korea. HCV recurrence, a serious complication after LT, can accelerate liver cirrhosis and fatal graft loss. Therefore, the prevention and appropriate treatment for HCV recurrence can improve a LT patient’s quality of life and survival. In considering the relationship between immunosuppressants and HCV recurre nce, there is no clear difference in HCV recurrence between the immunosuppressant tacrolimus or cyclosporine, and the use of steroids for patients with HCV is still under debate. In the management of HCV recurrence, direct-acting antivirals, such as protease inhibitors, polymerase inhibitors, or other nonstructural protein inhibitors will open a new era in HCV treatment. However, their safety and drug interactions with immunosuppressants should be evaluated for patients after LT.

The Effects of Alar Batten Grafts on Nasal Airway Obstruction and Nasal Steroid Use in Patients With Nasal Valve Collapse and Nasal Allergic Symptoms

Clinical management of nasal airway obstruction (NAO) in patients with and without nasal allergic symptoms and nasal valve collapse (NVC). To examine the impact that autologous alar batten grafts have on patients with NAO owing to NVC and their affect on nasal steroid use and allergic symptoms. A prospective study. Indiana University Medical Center, Indianapolis. Patients with NAO due to NVC with or without symptoms of nasal allergic symptoms. All of the patients had placement of autologous batten grafts during the study period. Prospective study of patients with dynamic NVC undergoing alar batten graft treatment. Nasal Obstruction Symptom Evaluation survey preoperatively and postoperatively, prospective outpatient questionnaire to determine use of nasal steroids and presence of nasal allergic symptoms preoperatively and postoperatively. A total of 126 patients underwent surgical intervention for the treatment of NAO due to NVC. All of these patients were using nasal steroid sprays, and 78 patients (62%) also reported nasal allergic symptoms at their initial presentation. At 6-month and 1-year postoperative evaluations, 118 (94%) and 122 (97%), respectively, reported significant improvement of their NAO, regardless if they had presented with or without allergic nasal symptoms. Sixty-two of the 78 patients (79%) who initially presented with NAO owing to NVC and nasal allergic symptoms preoperatively reported significant improvement in their NAO and nasal allergic symptoms postoperatively. Eight of 126 (6%) restarted their use of nasal steroids postoperatively. All 8 of these patients reported nasal allergic symptoms preoperatively. No patients in the nonallergic group continued the use of nasal steroids postoperatively. There was no increase in nasal steroid use at the 12-month follow-up visit. Nasal airway obstruction due to NVC in patients can be surgically treated with autologous alar batten grafts. In addition, the use of alar batten grafts may improve NAO in patients with nasal allergic symptoms and reduces their use of nasal steroids. These results support the idea of potential surgical repair of the nasal valve to treat patients with NAO due to nasal allergic symptoms and NVC. 4.