Lupus enteritis as a lone manifestation of SLE: rare but possible

2021 ◽  
Vol 14 (8) ◽  
pp. e239072
Author(s):  
Eduardo Quintero ◽  
Jerald Pelayo ◽  
Grace Salacup ◽  
Kevin Bryan Lo
Keyword(s):  
Watery Diarrhoea ◽  
High Dose ◽  
Radiological Imaging ◽  
Biliary Leak ◽  
Double Stranded Dna ◽  
Positive Antinuclear Antibody ◽  
Autoimmune Pathology ◽  
Lupus Enteritis ◽  
Systemic Symptoms

A 28-year-old Southeast Asian non-pregnant woman with asthma and prior cholecystectomy presented to the emergency department with acute watery diarrhoea, intermittent abdominal pain and vomiting. Apart from abdominal tenderness, the rest of the physical examination was unremarkable. She had leucocytosis, alkaline phosphatase elevation and exudative ascites. Radiological imaging ruled out biliary leak and was only significant for circumferential small and large bowel thickening. Upper endoscopy and colonoscopy showed normal duodenal and colonic mucosae. Both infectious and malignancy workup were also unremarkable. Bereft of other systemic symptoms, autoimmune pathology was initially deemed unlikely; however, autoimmune workup revealed positive antinuclear antibody, double-stranded DNA, anti-Smith antibody, antinuclear ribonucleoprotein and hypocomplementaemia. With multidisciplinary collaboration, the patient was initiated on high-dose steroids, which dramatically improved her symptoms. She was discharged home with a steroid taper, and at 3 months of follow-up with her rheumatologist, she was continued on steroids and hydroxychloroquine.

When fever is more than infection: two cases of vancomycin-induced drug reaction with eosinophilia and systemic symptoms (DRESS)

2021 ◽  
Vol 14 (1) ◽  
pp. e238006
Author(s):  
Mitchell Cox ◽  
Sophie Paviour ◽  
Sophie Gregory ◽  
Rusheng Chew
Keyword(s):  
Drug Reaction ◽  
Cytomegalovirus Reactivation ◽  
First Case ◽  
Metropolitan Hospital ◽  
Orthopaedic Ward ◽  
Systemic Hypersensitivity ◽  
Systemic Symptoms ◽  
Inflammatory Syndrome ◽  
Intravenous Glucocorticoids

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, but serious systemic hypersensitivity reaction associated with a range of medications. We present two cases of vancomycin-induced DRESS, which occurred simultaneously in the orthopaedic ward in an outer metropolitan hospital. These cases demonstrate the complexity in the diagnosis and management of this inflammatory syndrome on the background of known infection as well as evidence for linezolid as an alternative to vancomycin. The first case was managed conservatively, but developed progressive renal and liver injury along with demonstrated cytomegalovirus reactivation and recurrent colitis, and was eventually palliated. The second was commenced on intravenous glucocorticoids and achieved remission, although had ongoing renal dysfunction at the time of discharge from outpatient follow-up.

scholarly journals Safety of High-Dose Dabigatran in Elderly and Younger Patients with a Low Bleeding Risk: A Prospective Observational Study

Cardiology ◽  
2021 ◽  
pp. 1-5
Author(s):  
Aharon Erez ◽  
Gregory Golovchiner ◽  
Robert Klempfner ◽  
Ehud Kadmon ◽  
Gustavo Ruben Goldenberg ◽  
...  
Keyword(s):  
Observational Study ◽  
Elderly Patients ◽  
Prospective Observational Study ◽  
Bleeding Risk ◽  
The Elderly ◽  
High Dose ◽  
World Population ◽  
Younger Patients ◽  
End Point

<b><i>Introduction:</i></b> In patients with atrial fibrillation (AF) at risk for stroke, dabigatran 150 mg twice a day (DE150) is superior to warfarin for stroke prevention. However, there is paucity of data with respect to bleeding risk at this dose in elderly patients (≥75 years). We aimed to evaluate the safety of DE150 in comparison to warfarin in a real-world population with AF and low bleeding risk (HAS-BLED score ≤2). <b><i>Methods:</i></b> In this prospective observational study, 754 consecutive patients with AF and HAS-BLED score ≤2 were included. We compared outcome of elderly patients (age ≥75 tears) to younger patients (age &#x3c;75 years). The primary end point was the combined incidence of all-cause mortality, stroke, systemic emboli, and major bleeding event during a mean follow-up of 1 year. <b><i>Results:</i></b> There were 230 (30%) elderly patients, 151 patients were treated with warfarin, and 79 were treated with DE150. Fifty-two patients experienced the primary endpoint during the 1-year follow-up. Among the elderly, at 1-year of follow-up, the cumulative event rate of the combined endpoint in the DE150 and warfarin was 8.9 and 15.9% respectively (<i>p</i> = 0.14). After adjustment for age and gender, patients who were treated with DE150 had a nonsignificant difference in the risk for the combined end point as patients treated with warfarin both among the elderly and among the younger population (HR 0.58, 95% C.I = 0.25–1.39 and HR = 1.12, 95% C.I 0.62–2.00, respectively [<i>p</i> for age-group-by-treatment interaction = 0.83). <b><i>Conclusions:</i></b> Our results suggest that Dabigatran 150 mg twice a day can be safely used among elderly AF patients with low bleeding risk.

scholarly journals AB0601 RAPID AND SUSTAINED EFFICACY OF AN INDUCTION TREATMENT WITH A TRIPLE THERAPY INCLUDING HIGH-DOSE INTRAVENOUS IMMUNOGLOBULINS, METHOTREXATE AND GLUCOCORTICOIDS IN ANTI-3-HYDROXY-3-METHYLGLUTARYL-COENZYME A REDUCTASE MYOPATHY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1597.1-1597
Author(s):  
E. Treppo ◽  
M. Infantino ◽  
M. Benucci ◽  
V. Ravagnani ◽  
B. Palterer ◽  
...  
Keyword(s):  
Triple Therapy ◽  
Antibody Titer ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Sustained Remission ◽  
Red Rice ◽  
Gradual Improvement ◽  
High Doses ◽  
High Dose Ivig

Background:Anti-3-hydroxy-3-methylglutaryl-coenzime A reductase (HMGCR) myopathy is a new entity, which has been clearly associated to statin use, even if it can be diagnosed in patients without a history of exposure to statin or even in the childhood (1).Objectives:The aim of the study is to describe the efficacy of a triple therapy regimen consisting in high-doses of intravenous immunoglobulins (IVIG), methotrexate (MTX), and glucocorticoids (GC) in 16 patients with Anti-HMGCR myopathy enrolled in 6 specialized centres.Methods:A total of 16 patients with anti-HMGCR myopathy (7 females; 9 males) were collected. Mean (±standard deviation) age at the onset of disease was 72.4±10.3 years old. All patients were diagnosed having anti-HMGCR myopathy [anti-HMGCR antibodies were measured by chemiluminescence assay (BioFlash, Inova, CA)] (2). Median follow-up was 29.5 months (interquartile range: 15.75-60 months). Anti-HMGCR antibodies were available in the follow-up in 8/16 patients.Results:Thirteen out of 16 patients (81.3%) had been exposed to statin (1/13 to red rice), 3/16 (18.7%) were not exposed. As induction therapy, 11/16 patients have been treated with triple therapy (high-dose IVIG, MTX and GC), 2/16 with double therapy (high-dose IVIG and GC), 2/16 have been treated with GC alone, the patient exposed to red rice resolved only with red rice suspension. Clinical remission and normalization of CPK values within month +24 were obtained in all the patients. All the patients were in remission at the last follow-up. Gradual improvement started soon from the first month, and among the 13 patients treated with an aggressive immunosuppresssive therapy including IVIG (13/13), GC (13/13) and methotrexate (11/13), 9/13 normalized the CPK value within 6 months. Clinical and laboratory response was accompanied by significant decrease or normalization of the anti-HMGCR antibody titer. All the patients were either not taking GC (56.3%), or were taking low doses of GC (43.7%) at the last follow-up. Four patients had stopped GC within 6 months. No serious side effects were recorded. After persistent remission, a maintenance immunosuppressive therapy was then administered. Only 3 relapses in 3 different cases were recorded, all of them during drug-free remission in long-term follow-up. Reinduction was again effective in all.Conclusion:Anti-HMGCR myopathy is a rare and serious myopathy which usually affects older people during statin treatment. After statin suspension, a rapid and sustained remission can be achieved by induction with a triple aggressive therapy consisting in medium-to high doses of GC, high-dose IVIG, and MTX (3). GC should be tapered as soon as possible. Relapse appears infrequent during maintenance treatment. Monitoring anti-HMGCR antibody titer may be clinically relevant.References:[1]AL Mammen et al. N Engl J Med. 2016;374:664-9[2]Musset L et al. Autoimmun Rev. 2016;15:983-93.[3]Aggarwal A et al. Scand J Rheumatol. 2019; 1-7.Acknowledgments:We thank MD Francesca Grosso and MD Valentina Mecheri from the University of Florence, MD Angela Zuppa and MD Chiara De Michelis, from San Martino Hospital, Genova, for their valued collaboration in data collectionDisclosure of Interests:Elena Treppo: None declared, Maria Infantino: None declared, Maurizio Benucci: None declared, Viviana Ravagnani: None declared, Boaz Palterer: None declared, Marina Grandis: None declared, Martina Fabris: None declared, Paola Tomietto: None declared, Mariangela Manfredi: None declared, Arianna Sonaglia: None declared, Maria Grazia Giudizi: None declared, Francesca Ligobbi: None declared, Daniele Cammelli: None declared, Paola Parronchi: None declared, Salvatore De Vita Consultant of: Roche, GSK, Speakers bureau: Roche, GSK, Novartis, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer

scholarly journals 346. Eastern Equine Encephalitis and Use of Intravenous Immunoglobulin Therapy and High-Dose Steroids

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S242-S243
Author(s):  
Sarah I Collens ◽  
Douglas R Wilcox ◽  
Shibani Mukerji ◽  
Farrah J Mateen ◽  
Isaac H Solomon
Keyword(s):  
Cerebrospinal Fluid ◽  
Temporal Lobe ◽  
Standard Of Care ◽  
High Dose ◽  
High Morbidity ◽  
Intravenous Immunoglobulin Therapy ◽  
Eastern Equine Encephalitis ◽  
Neuroanatomical Region ◽  
Ivig Administration

Abstract Background Eastern equine encephalitis (EEE) is a mosquito-borne viral infection with significant neurological morbidity and mortality. The clinical presentation and patient outcomes after treatment with IVIG, high-dose steroids, or standard of care alone in EEE remains unclear. Methods A retrospective observational study of patients admitted to two tertiary academic medical centers in Boston, Massachusetts with EEE from 2005 to 2019. Results Of 17 patients (mean [SD] age, 50 [26] years; 10 (59%) male, and 16 (94%) White race), 17 patients had fever (100%), 15 had encephalopathy (88%), and 12 had headache (71%). Eleven of 14 patients with cerebrospinal fluid (CSF) cell count differential had a neutrophil predominance (mean [SD], 60.6% of white blood cells [22.8]) with an elevated protein level (mean [SD], 112 mg/dL [48.8]). Affected neuroanatomical regions included the basal ganglia (n=9/17), thalamus (n=7/17), and mesial temporal lobe (n=7/17). A total of 11 patients (65%) received IVIG; 8 (47%) received steroids. Of the patients who received IVIG, increased time from hospital admission to IVIG administration correlated with worse long-term disability as assessed by modified Rankin Score (mRS) (r=0.72, p=0.02); steroid use was not associated with mRS score. The mortality was 12%. Figure 1. Imaging Characteristics: Typical Pattern of MRI Involvement and Affected Neuroanatomical Regions in Patients with Eastern Equine Encephalitis. All images displayed are the T2-FLAIR sequence. (A) Representative images of pattern of typical neuroanatomical region involved in one patient with demonstrated involvement of the temporal lobe and pons, temporal lobe and midbrain, and basal gangial by T2-FLAIR hyperintensity (panels left to right). (B) Representative images of patients with mild (mRS 0–2), moderate (mRS 3–4), and severe (mRS 5–6) disability score at discharge. (C) Representative images of one patient over course of hospitalization at days 1, 4, and 10 after admission. (D) Quantification of neuroanatomical region involvement in initial MRI of patients with EEE as determined by T2-FLAIR hyperintensity. An area was scored as abnormal only once per patient. Figure 2. Outcomes in Patients with Eastern Equine Encephalitis. Patient disability by modified Rankin Score (mRS) of EEE patients at admission to the hospital, discharge from the hospital, and last recorded follow-up (A). Time to IVIG administration compared to mRS at discharge (B), and most recent clinical follow-up (C). Table 1. Demographics, Clinical Characteristics, and Laboratory Data in Patients with Eastern Equine Encephalitis. Abbreviations: CSF = cerebrospinal fluid, WBC = white clood count, EEG = electroencephalogram, ALT = alanine aminotransferase, AST = aspartate transaminase. Demographic data was collected for all patients with confirmed EEE. Altered mental status included any description of encephalopathy, confusion, or difficulty with attention. Seizures were defined as clinical events with a high-degree of suspicion to be true seizures, and were entirely comprised of generalized tonic-clonic seizures. Conclusion Clinicians should suspect EEE in immunocompetent patients with early subcortical neuroimaging abnormalities and CSF neutrophilic predominance. This study suggests a lower mortality than previously reported, but a high morbidity rate in EEE. IVIG as an adjunctive to standard of care may be considered early during hospitalization. Disclosures All Authors: No reported disclosures

scholarly journals AB0531 GOLIMUMAB IN THE TREATMENT OF SEVERE AND/OR REFRACTORY VASCULO-BEHÇET’S DISEASE: A SINGLE-CENTRE EXPERIENCE IN CHINA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1562.1-1563
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
High Dose ◽  
Efficacy And Safety ◽  
Behcet's Disease ◽  
Severe Aortic Regurgitation ◽  
Tnf Α

Background:Vascular involvement is one of the leading causes of mortality and morbidity in Behcet’s Disease (BD)1. Surgical treatments are difficult for Vaculo-BD (VBD) patients due to the high risk of serious postoperative complications without effective and promptly perioperative immunotherapy2, 3. Anti-tumor necrosis factor alpha (TNF-α) therapy has been reported as a potential treatment in severe VBD, e.g. infliximab (IFX) and adalimumab (ADA). However, only few case reports are available regarding the fully humanized monoclonal antibody to TNF-α, golimumab (GOL), in the management of VBD4.Objectives:The objective of this study was to report the efficacy and safety of GOL for the treatment of severe and/or refractory VBD.Methods:We retrospectively analyzed the efficacy and safety profile of patients with severe and/or refractory VBD treated with GOL in our medical center between 2018 to 2020.Results:Nine VBD patients (8 male and 1 female) were enrolled, with a mean age and median course of 37±8.6 years and 72 months (range 12 to 300), respectively. Cardiac involvements (severe aortic regurgitation secondary to BD) were presented in 7 patients, including 2 patients with post-operative paravalvular leakage (PVL) after aortic valve replacement surgery. Multiple vascular lesions were documented in the other 2 patients, including one patient with life-threatening multiple pulmonary aneurysms, pulmonary thromboembolism and recurrent deep vein thrombosis, and another patient with abdominal aortic pseudoaneurysm and multiple artery stenosis and occlusion. Prior to GOL therapy, all patients experienced disease progression despite high-dose glucocorticoids combined with multiple immunosuppressants. Moreover, seven patients required effective and fast control of inflammation and a decrease of glucocorticoid dose during the perioperative period. They were treated with GOL, 50mg every 4 weeks, in combination with background low-or medium-dose glucocorticoids and immunosuppressants, for a median of 6 (range 3-15) months. After a mean duration of follow-up of 10 (range 2-6) months, all patients achieved improvement both in clinical symptoms and serum inflammation markers. The ESR level [4.88±4.94 mm/h vs 31.13±31.78mm/h, P<0.01] and CRP level [1.9 (0.11-3.73)mg/L vs 24.3 (0.4-85.57)mg/L, P<0.01] significantly decreased. The dosage of glucocorticoid[10 (0-15) vs 40 (0-100)mg/d, P<0.01] effectively tapered, indicating a potential steroid-sparing effect. No newly-onset aneurysm and recurrent venous thrombosis were observed. Also, one patient had a marked reduction in size and number of pulmonary aneurysms. No post-operative PVL was observed in the five patients after Bentall operation with a median follow-up of 10 months. One patient with severe aortic regurgitation remained stable and without surgical intervention with the treatment of GOL for 16 months. No severe complication occurred in one patient after underwent endovascular repair of abdominal aorta for 8 months. GOL was well-tolerated, and no serious adverse event was observed.Conclusion:Our results suggested that GOL is safe and effective for the treatment of patients with severe and / or refractory VBD. Further controlled studies are warranted to confirm the therapeutic potential of GOL in VBD patients.Disclosure of Interests:None declared

scholarly journals SEOM clinical guideline for management of adult medulloblastoma (2020)

2021 ◽  
Author(s):  
R. Luque ◽  
M. Benavides ◽  
S. del Barco ◽  
L. Egaña ◽  
J. García-Gómez ◽  
...  
Keyword(s):  
Residual Tumor ◽  
Large Cell ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Molecular Alterations ◽  
Adult Medulloblastoma ◽  
Group 4 ◽  
Risk Patients ◽  
Standard Risk

AbstractRecent advances in molecular profiling, have reclassified medulloblastoma, an undifferentiated tumor of the posterior fossa, in at least four diseases, each one with differences in prognosis, epidemiology and sensibility to different treatments. The recommended management of a lesion with radiological characteristics suggestive of MB includes maximum safe resection followed by a post-surgical MR < 48 h, LCR cytology and MR of the neuroaxis. Prognostic factors, such as presence of a residual tumor volume > 1.5 cm2, presence of micro- or macroscopic dissemination, and age > 3 years as well as pathological (presence of anaplastic or large cell features) and molecular findings (group, 4, 3 or p53 SHH mutated subgroup) determine the risk of relapse and should guide adjuvant management. Although there is evidence that both high-risk patients and to a lesser degree, standard-risk patients benefit from adjuvant craneoespinal radiation followed by consolidation chemotherapy, tolerability is a concern in adult patients, leading invariably to dose reductions. Treatment after relapse is to be considered palliative and inclusion on clinical trials, focusing on the molecular alterations that define each subgroup, should be encouraged. Selected patients can benefit from surgical rescue or targeted radiation or high-dose chemotherapy followed by autologous self-transplant. Even in patients that are cured by chemorradiation presence of significant sequelae is common and patients must undergo lifelong follow-up.

scholarly journals Recombinant Human Thyrotropin-Stimulated Radioiodine Therapy in Patients with Multinodular Goiters: A Meta-Analysis of Randomized Controlled Trials

2020 ◽  
Vol 52 (12) ◽  
pp. 841-849
Author(s):  
Chunmei Xu ◽  
Ping Wang ◽  
Huikai Miao ◽  
Tianyue Xie ◽  
Xiaojun Zhou ◽  
...  
Keyword(s):  
Fixed Effects ◽  
Dose Group ◽  
Low Dose ◽  
Radioiodine Therapy ◽  
Meta Analysis ◽  
High Dose Group ◽  
High Dose ◽  
Fixed Effects Model ◽  
Recombinant Human Thyrotropin

AbstractA potential reduction of goiter volume (GV) of recombinant human thyrotropin (rhTSH) on multinodular goiters (MNG) was previously reported but controversial. Hence we conducted a meta-analysis to estimate the effect of rhTSH-stimulated radioiodine therapy in patients with MNG. PubMed, Cochrane, CNKI, VIP, and Wanfang databases were searched. Mean difference (MD) and odds ratios with 95% confidence intervals (95% CI) were derived by using an inverse variance random-effects model and fixed-effects model, respectively. Six studies (n=237) were involved in the analysis. For 12 months follow up, high dose (>0.1 mg) of rhTSH significantly reduced GV (MD=17.61; 95% CI=12.17 to 23.04; p<0.00001) compared with placebo. No effective pooled results of low dose of rhTSH (<0.1 mg) were applicable for only one study included. For 6 months follow up, the source of heterogeneity was determined by subgroup and sensitivity analysis. High dose group showed vast improvement in GV reduction (MD=16.62; 95% CI=1.34 to 31.90; p=0.03). The reduction of low dose group compared with placebo was inferior to high dose group. No available data were obtained to assess the influence of rhTSH after 36 months follow up for the only included study. Hypothyroidism incidence was higher for rhTSH group. No publication bias was seen. High dose of rhTSH treatment-stimulated radioactive 131I therapy after 6 months and 12 months follow up had a better effect in reducing GV, but with higher incidence of hypothyroidism. Owing to the limited methodological quality, more clinical researches are warranted in the future.

scholarly journals Interstitial HDR Brachytherapy in the Treatment of Non-Melanocytic Skin Cancers around the Eye

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1425
Author(s):  
Paweł Cisek ◽  
Dariusz Kieszko ◽  
Mateusz Bilski ◽  
Radomir Dębicki ◽  
Ewelina Grywalska ◽  
...  
Keyword(s):  
External Beam Radiotherapy ◽  
Late Complication ◽  
Target Volume ◽  
Lower Eyelid ◽  
High Dose Rate ◽  
High Dose ◽  
Hdr Brachytherapy ◽  
Lateral Canthus ◽  
Skin Cancers

Background: Eyelid tumors are rare skin cancers, the most common of which is basal cell carcinoma characterized primarily by local growth. In addition to surgery, radiotherapy is among the basic methods of treatment. External beam radiotherapy is associated with the risk of complications within ocular structures, especially the lens. In the case of interstitial brachytherapy, it is possible to administer a high dose to the clinical target volume (CTV), while reducing it in the most sensitive structures. Methods: This paper presents the results of an analysis of 28 patients treated with interstitial high dose rate (HDR) brachytherapy for skin cancers of the upper and lower eyelid; medial and lateral canthus; and the cheek, nose and temples with the infiltration of ocular structures. The patients were treated according to two irradiation schedules: 49 Gy in 14 fractions of 3.5 Gy twice a day for 7 days of treatment, and 45 Gy in 5 Gy fractions twice a day for 5 days. The mean follow-up was 22 months (3–49 months). Results: two patients (6%) had a relapse: a local recurrence within the irradiated area in one of them, and metastases to lymph nodes in the other. The most common early complication was conjunctivitis (74%), and the most common late complication was dry eye syndrome (59%). Conclusions: Interstitial HDR brachytherapy for skin cancers of the upper and lower eyelid; medial and lateral cants; and the cheek, nose and temples with infiltration of ocular structures is a highly effective, short and relatively low burden type of treatment.

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