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1.
Curr Res Pharmacol Drug Discov ; 3: 100101, 2022.
Article in English | MEDLINE | ID: covidwho-1944730

ABSTRACT

Background: Patients with inflammatory bowel disease (IBD) often require the use of immunosuppressant medications that increase infection risk, leading to concerns over the safe use of IBD medications during the Coronavirus 19 (COVID-19) pandemic. Objectives: To summarize available evidence on the safety and appropriate use of IBD medications during the COVID-19 pandemic, particularly in regard to risk of severe COVID-19 outcomes such as hospitalization, respiratory failure, or death for patients on IBD therapeutics. Conclusions: The majority of IBD medications are safe to continue during the COVID-19 pandemic, with a few notable exceptions. Patients with IBD who do not have COVID-19 should continue their prescribed IBD therapies, although steroids are associated with severe COVID-19 outcomes and should be weaned when possible. Corticosteroids should be tapered and discontinued when possible in patients with IBD who test positive for COVID-19 as well. Patients with IBD who test positive for COVID-19 should hold biologics, thiopurines, methotrexate, and tofacitinib for at least 2 weeks, and those who have symptoms should not restart these medications until symptom resolution. During the COVID-19 pandemic, all patients with IBD should continue to follow public health guidance including social distancing, masking, and COVID-19 vaccination recommendations.

2.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927837

ABSTRACT

Introduction: The Fungitell assay is an in vitro diagnostic test for the qualitative detection of (1-3)-beta-D-Glucan (BDG) in serum. It can be particularly useful in early diagnosis of fungal infections that would otherwise take weeks to finalize in culture.Description:This is a case of a 73 year old Filipino female with a history of diffuse large B-cell lymphoma status post RCHOP therapy, currently maintained on Ritixumab, and rheumatoid arthritis treated with Methotrexate who was admitted to the hospital with increasing shortness of breath for several weeks. In the Emergency Department she was hypoxic and required 2 liters of oxygen via nasal cannula and with 92% oxygen saturation. Her vital signs were otherwise normal. She was afebrile and WBC was 9.4. She had a negative respiratory viral PCR which included COVID-19. Infectious work up including sputum culture and urine antigens were also sent. A CT chest was performed and showed bilateral ground glass opacities suspicious for atypical pneumonia.There was concern for drug toxicity from Methotrexate which was subsequently suspended. A bronchoscopy and bronchoalveolar lavage (BAL) was performed to rule out infection prior to starting steroids for suspected pneumonitis. Cell count from the BAL revealed low neutrophils. There was negative growth over the next 48 hours. Steroids were initiated at 1 mg/kg daily and patient was discharged home with close outpatient follow up scheduled. A fungitell (serum beta D glucan) that was collected from the BAL had resulted after the patient was discharged home. The level returned very elevated (>500). The patient was contacted and she reported that her symptoms did not improve with the steroids. She was still requiring up to four liters of oxygen at home. She was asked to return to the hospital to work up an undiagnosed fungal or PJP pneumonia. A repeat bronchoscopy was performed and a PJP PCR was tested on the BAL. This returned positive. She was started on Bactrim for 14 days to treat PJP pneumonia. She was weaned down to 2 liters of oxygen and was doing well from a pulmonary standpoint at her outpatient follow up visit 2 weeks later. Discussion: The Fungitell assay test in this case was crucial to help guide us to the correct diagnosis. In patients who are immunocompromised, physicians should utilize specialty testing such as Fungitell when it is available. Compared to microbial fungal culture, Fungitell results faster, has a higher sensitivity and a higher negative predictive value. (Figure Presented).

3.
Value in Health ; 25(7):S498, 2022.
Article in English | EMBASE | ID: covidwho-1926727

ABSTRACT

Objectives: Mycosis Fungoides (MF) and Sézary syndrome (SS) are the two most common subtypes of cutaneous T cell lymphomas. Given their low prevalence, real-world treatment patterns of existing and newly approved therapies for MF and SS remain unknown, especially during the COVID pandemic. This study examined treatment patterns among patients with MF or SS between 2018-2020 in the United States. Methods: Patients in the Symphony Health Solutions database were classified into 6 groups: ≥1 MF diagnosis (no SS diagnosis) in 2018, 2019, and 2020, and ≥1 SS diagnosis in 2018, 2019, 2020, respectively. Utilization of treatments recommended by the current National Comprehensive Cancer Network guidelines was examined: skin-directed therapy (SDT;topical, local radiation, total skin electron beam therapy, or phototherapy), systemic therapy (extracorporeal photopheresis [ECP], parenteral, or oral), and bone marrow transplant (BMT). Results: Overall(mean age;male), 10,527(62.9 years;54.6%), 10,078(63.2 years;54.3%) and 9,414(63.2 years;53.9%) patients had ≥1 MF diagnosis and 869(66.3 years;54.4%), 882(66.9 years;54.8%) and 853(67.3 years;55.6%) patients had ≥1 SS diagnosis in 2018, 2019 and 2020, respectively. From 2018-2020, 56.1-56.6% of MF (SDT 52.1-52.5%;systemics 12.1-13.5%;BMT 0.1-0.2%) and 64.6-68.8% of SS patients (SDT 48.9-52.9%;systemics 41.8-46.5%;BMT 0.8-1.5%) had ≥1 treatment claims. Among MF patients with any systemic therapies, bexarotene was the most common in 2018 (28.1%) and 2019 (27.5%), methotrexate in 2020 (27.2%), and mogamulizumab (2018 MF/SS approval) the 6th most common in 2019 (6.8%) and 2020 (6.6%). Among SS patients with any systemic therapies, ECP was the most common in 2018 (40.8%) and 2019 (33.3%), and mogamulizumab in 2020 (29.2%). Conclusions: Using claims from 2018-2020, approximately half of MF and SS patients had SDT each year, without major change during the COVID pandemic. Since 2018 in SS, there was an increasing systemic usage, with increasing mogamulizumab but decreasing ECP usage.

4.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925512

ABSTRACT

Objective: To describe outcomes of the coronavirus disease 2019 (COVID-19) in stiff person syndrome (SPS). Background: The COVID-19 pandemic has impacted people with autoimmune disorders to varying degrees. Several risk factors for more severe COVID-19 outcomes in different populations have been identified including medical comorbidities and immunosuppressant therapies (IST). These risk factors are often present in people with SPS, however, little is known about the impact of COVID-19 in the SPS population. Design/Methods: Individuals with a diagnosis of SPS seen at Johns Hopkins Hospital are followed as part of a longitudinal observational study. Data collection includes demographics, disease characteristics, laboratory studies, and outcomes. Individuals who tested positive for COVID-19 were identified and information on their disease severity and COVID-19 outcomes were obtained. Results: Fourteen SPS patients were identified as testing positive for COVID-19. Mean (standard deviation) age at COVID-19 diagnosis was 52(±12) years, most were female (86%) and half were non-Caucasian. The most common COVID-19 medical comorbidities noted were obesity(n=4), diabetes(n=3), and cardiovascular disease(n=2). Four individuals were on IST (rituximab, mycophenolate mofetil, or methotrexate). The majority(79%) of patients did not require hospitalization. Three individuals required hospitalization including intensive care and mechanical ventilation, and all had diabetes. One individual on rituximab with underlying diabetes, obesity, and hypertension died from COVID-19 complications. Two others received monoclonal antibody infusions in the outpatient setting. There was one breakthrough infection following full COVID-19 vaccination;occurred 4 months after vaccination and did not require hospitalization. Conclusions: The majority of people with SPS in this case series did not require hospitalization due to COVID-19. However, the individuals that required hospitalization also had underlying comorbidities that have been associated with more severe COVID-19 outcomes in other populations. One breakthrough COVID-19 case was reported thus far, hence, public health preventative measures are critical no matter vaccination status.

5.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925323

ABSTRACT

Objective: To present an unusual presentation of CLIPPERS that was responsive to rituximab Background: CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a neuroinflammatory disorder typically affecting the brainstem and cerebellum with clinical and radiographic improvement with steroids. One reported case showed improvement with rituximab. We present a case of CLIPPERS with supra- and infratentorial involvement that improved with rituximab. Design/Methods: A 30-year-old male presented with several months of headaches, dizziness, and face and arm numbness, then developed diplopia and gait ataxia. Serial MRI's showed worsening punctate enhancing and T2-hyperintense lesions in the brainstem and supratentorial white matter over months. Workup showed normal CSF cell count, and negative CSF cytology, ACE, VDRL, oligoclonal bands, serologic IgG4, Coccidioidomycosis, Lupus, rheumatoid arthritis, and COVID. Vascular imaging showed no evidence of vasculitis. Biopsy showed a dense perivascular lymphocytic infiltrate including B and T cells, without evidence of vasculitis or lymphoma. He received IVIG and IV solumedrol, with symptom resolution and some improvement on imaging. Rituximab was started with subsequent resolution of the lesions on MRI. Results: NA Conclusions: CLIPPERS has a variable clinical presentation but typically includes gait ataxia and diplopia. MRI shows multiple punctate/curvilinear enhancing lesions in the brainstem and cerebellum, rarely in the spinal cord or supratentorially. Differential diagnoses include neurosarcoidosis, Behcet's disease, vasculitis, lymphoma, chronic infections, glioma, and demyelinating disease. It is characterized by responsiveness to steroids, and patients require long-term steroid or steroid-sparing agent treatment, at least until resolution of enhancement. Methotrexate, hydroxychloroquine, and cyclophosphamide are most commonly used. There was one report of treatment with rituximab with 4 years stability. Our case was unusual as he had supra and infratentorial lesions, and he had good response to rituximab. Rituximab should be considered in the treatment of CLIPPERS.

6.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925183

ABSTRACT

Objective: N/A Background: The full scope of the mid- and long-term effects of SARS-CoV2 infection is currently being reported. The immune response might contribute not only to the development of ARDS, but also to other systemic complications after the acute setting. Some disorders, including those of autoimmune or presumed autoimmune etiology, have been reported to be triggered, exacerbated, or unmasked during the COVID-19 pandemic. Sarcoidosis is a multi-systemic inflammatory disorder believed to occur due to an exaggerated immune response to unknown antigens in the setting of genetic susceptibility. We present a case of neuro-sarcoidosis after COVID-19. Design/Methods: Descriptive study, case report. Results: A 51-year-old right-handed female presented with multiple cranial neuropathies and paresthesia after a mild case of COVID-19. Her symptoms included vertigo, hypoacusis, balance issues, left facial palsy, and paresthesia in her upper extremities. Her brain MRI with contrast showed bilateral enhancement of the VII and VIII cranial nerves. CSF analysis showed mild protein elevation and elevated CD4:CD8 ratio. Serum sIL-2R was also elevated. Her chest CT scan was abnormal, prompting a lymph node biopsy that was consistent with non-caseating granulomas. A diagnosis of probable neuro-sarcoidosis was made and she showed improvement with steroids. She was later started on methotrexate as a steroid sparing agent in the outpatient setting. Conclusions: To our knowledge, neuro-sarcoidosis has not been previously described in temporal association with COVID-19. It might be that this infection acts as one of the triggers for sarcoidosis. Some common pathways shared by these conditions could explain the possibility of such a trigger. These pathways include the ACE2 receptor, the TMRPPS gene, and certain cytokines. When aberrant, causing incomplete clearance of an antigen, these pathways might lead to the formation of granulomas. Further research surrounding the non-immediate effects of the novel coronavirus is needed to better delineate possible autoimmune consequences of this serious infection.

7.
Obstetrics and Gynecology ; 139(SUPPL 1):3S, 2022.
Article in English | EMBASE | ID: covidwho-1925135

ABSTRACT

INTRODUCTION: Compared to misoprostol alone, medical management of early pregnancy loss (EPL) with mifepristone and misoprostol has a 25% higher success rate, reduces subsequent uterine aspiration, and is cost-effective. During the COVID-19 pandemic, we began providing mifepristone and misoprostol for EPL in our urban emergency departments (EDs), aiming to increase access to essential services and to minimize repeated health care visits. We sought to describe the safety and efficacy of this novel practice. METHODS: With institutional review board approval, we retrospectively reviewed the charts of all patients receiving mifepristone and misoprostol for first trimester pregnancy management in our EDs between April 2020 and March 2021 (n=33). Effective treatment was defined as pregnancy resolution after a single treatment with mifepristone and misoprostol. Safety outcomes included need for additional management, emergent surgical interventions, and blood transfusion. Descriptive statistics and univariate analysis were performed. RESULTS: The prevalence of effective treatment was 70% (n=23). Among the 10 subjects (30%) requiring additional management, interventions were: one additional dose of misoprostol (n=4), uterine aspiration (n=4), methotrexate (n=1), and uterine artery embolization (n=1). Three interventions (30%) were emergent, and two of these patients also required transfusion. Demographic characteristics were not associated with effective treatment nor with safety outcomes. Twenty-eight subjects (84%) participated in follow-up, 17 via telemedicine and 11 in person. CONCLUSION: Providing mifepristone and misoprostol for EPL in the ED may be an effective method to increase health care access. Further research is needed to determine whether this population is truly at increased risk for serious complications.

8.
Pediatric Dermatology ; 39(SUPPL 1):25-26, 2022.
Article in English | EMBASE | ID: covidwho-1916269

ABSTRACT

Objectives: To describe the clinical and laboratory characteristics of pediatric patients diagnosed with dermatomyosis during the COVID pandemic. Method: Description of the clinical and laboratory findings in patients under 15 years of age, who were admitted at our hospital with signs and symptoms suggestive of dermatomyositis, from March 2020 until November 2021. Results: Five patients, three boys and two girls, aged between 8 and 13 years, were diagnosed with juvenile dermatomyositis (JDM). The most frequent symptom was asthenia. Heliotrope erythema, malar rash, periungual erythema, and papules on elbows and knees were present in all cases. Three of our patients had perniosis on their toes. Four patients presented lesions in the oral mucosa, such as geographic tongue or gingivitis. The time between the onset of symptoms and the first visit with the pediatrician ranged from 1 to 6 months. In all cases, GOT/GPT enzymes, and aldolase were elevated at diagnosis, and the SARS-CoV-2 PCR was negative. Two patients had anti-TIF1 antibodies, and two had anti-MDA5 antibodies. In one girl, no specific autoantibodies for JDM were detected. Magnetic resonance imaging showed muscle oedema in all patients. All cases are in remission after systemic treatment with steroids, methotrexate or immunoglobulins. Discussion: JDM is a severe disease of childhood. Our cases did not present symptoms suggestive of COVID and the PCR for SARS-CoV-2 was negative on admission in all of them, but the presence of perniosis on the toes of three patients could correspond to “COVID toes,” and be a late manifestation of an asymptomatic or oligosymptomatic infection of SARS-CoV-2. It has been suggested that SARS-CoV-2 infection could trigger the development of JDM, possibly through the induction of IFNα. Long-term follow-up is necessary to establish a relationship between the prognosis of specific autoantibodies, the involvement of acral and mucosal areas, and the possible relation with COVID.

9.
European Heart Journal, Supplement ; 24(SUPPL C):C181, 2022.
Article in English | EMBASE | ID: covidwho-1915564

ABSTRACT

Myxoma (mx) is the most frequent adult cardiac tumour, that often poses a difficult diagnostic challenge due to the variety and aspecifity of presenting clinical signs. Alongside the more typical clinical onset caused by intracardiac obstruction and systemic embolization, mainly at cerebral level, a mx may initially manifest itself with nonspecific systemic symptoms such as fever, weight loss, fatigue, skin rash, myalgia and arthralgia. We present the case of a 58-year-old woman diagnosed in December 2018 with idiopathic, serum-negative arthritis of the metacarpophalangeal joint of the first finger of the right hand, treated with methotrexate (Reumaflex 10 mg/week s.c.), hydroxychloroquine sulphate (Paquenil 200 mg/day per os) and corticosteroids cycles;in January 2021, this therapy was suspended by the patient (pt) as ineffective. In April 2021, pt was admitted for bilateral SARS-CoV-2 pneumonia. As dyspnoea and fatigue persisted during moderate physical activity (NYHA class II), the pt underwent a cardiological examination on 13 August, when echocardiographic diagnosis of a left atrial mass with the appearance of a mx was made. Interleukin-6 (IL-6) was assayed for a suspected relationship between the cardiac tumour and rheumatic symptoms and was found to be elevated (490 pg/m)l. On 23 August, cardiac surgery was performed to remove the left atrial mass by right minithoracotomy. Histopathological examination confirmed that the 3.5 x 2.5 x 1.5 cm neoformation was a mx. On 1 September, strong attenuation of joint pain and IL-6 reduction to 107 pg/ml was detected. On 9 October, resolution of rheumatic symptoms and normalisation of IL-6 to 3.7 pg/ml (N.V < 7) occurred. This clinical case is emblematic of the long time that sometimes can elapse between the first clinical manifestation and the diagnosis of mx. Also the widespread use of echocardiography did not significantly reduce the diagnostic delay when cardiac symptoms are absent. In a large number of cases, onset symptoms mimicking autoimmune connective tissue disease are reported for 5% (“Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases” Medicine (Baltimore) 2001 May;80(3):159-729). The monoarticular localisation we describe is however unusual. Finally, our observation confirms that the association between mx and systemic symptoms is most likely due to IL-6 synthesis by tumour cells. (Figure Presented).

10.
Age and Ageing ; 51, 2022.
Article in English | ProQuest Central | ID: covidwho-1901103

ABSTRACT

Introduction An 88-year-old previously independent lady presented with progressive proximal weakness of all limbs and multiple falls for 10 months. Her swallowing got difficult lately. She lost weight gradually in that duration but denied any other symptoms suggestive of malignancy. PMH—Hypothyroid, Mild cognitive impairment Drg History—Atorvastatin for years. Stopped in this admission. On examination, she showed signs of proximal muscle weakness and areflexia in both upper and lower limbs without muscle tenderness. She had no facial nor eye muscle weakness. Sensory functions were intact. There were no rashes. Investigations CK 2000 TSH 24 Inflammatory markers, white cell counts—non-significant Vitamin D—18 CT-Chest Abdomen Pelvis, CT-colonography—No evidence of malignancy Paraneoplastic antibodies—Negative Anti-OJ and anti-Ro52 Antibodies—positive ENA profile—negative Anti-RNP—equivocal HMG-CoA reductase antibodies (HMCR)—Positive. Progress Prednisolone was commenced and gradually increased to 60 mg once a day but there is no significant improvement clinically though creatinine kinase level had improved. She was unfortunately infected with COVID-19 infection in the stay which delayed the plan for muscle biopsy and EMG. It affected the plan for Intravenous Immunoglobulins and was later decided to be non-beneficial due to high risks of thromboembolic events and superimposed infections. Steroid was later switched to methotrexate. She was discharged to a rehab unit. Conclusion Necrotising Autoimmune Myopathy is a rare form of idiopathic inflammatory myopathy. Risk factors include statins, cancer, connective tissue diseases, autoimmune diseases, and infections such as HIV2. Diagnosis includes clinical features, serum creatine kinase, HMG CoA reductase antibody (HMGCR-Ab), electromyography, and muscle biopsy. The first-line treatment options are steroids and immunosuppressive agents. Early use of immunoglobulin achieves good outcome. It is still under investigation for the recommended choice for immunosuppressive therapy and the duration of the therapy.

11.
Pediatric Blood and Cancer ; 69(SUPPL 2):S202-S203, 2022.
Article in English | EMBASE | ID: covidwho-1885446

ABSTRACT

Background: Transplant-associated thrombotic microangiopathy (TA-TMA) is an increasingly recognized complication of hematopoietic stem cell therapy (HSCT) with incidence rates ranging from 10-35%. The predominant mechanism leading to TA-TMA is endothelial cell damage leading to complement dysregulation and microvascular hemolysis. Complement dysregulation is particularly important in the pathophysiology of TA-TMA as initial trials have shown response to complement blockade using eculizumab, a humanized monoclonal antibody targeting the terminal complement pathway. Ravulizumab is a longer acting monoclonal antibody with the same target as eculizumab that is increasingly used for treatment of atypical hemolytic uremic syndrome. Herein, we describe the case of an African American female with relapsed/refractory infantile B-cell acute lymphoblastic leukemia (B-ALL) who underwent 10/10 HLA-matched sibling donor allogeneic transplant (conditioning: busulfan/fludarabine/thiotepa;GVHD prophylaxis: tacrolimus/methotrexate) who developed TA-TMA marked by pericardial effusion, elevated LDH, proteinuria, hypertension, thrombocytopenia, anemia, and evidence of microangiopathy. Upon diagnosis, as ravulizumab was on formulary and readily available unlike eculizumab, she was treated with ravulizumab instead of eculizumab. Objectives: To describe the therapeutic response to ravulizumab in one patient diagnosed with TA-TMA. Design/Method: A retrospective chart review was performed regarding this patient's ravulizumab treatment course, and direct discussions were had with the patient's care team. Results: Ravulizumab (loading dose of 600 mg followed 2 weeks later by maintenance dosing of 600 mg every 4 weeks) was administered. Pre-treatment CH50 was >75 U/mL (range: 30-75 U/mL) with sC5b9 and C3 complement levels at the upper limit of normal at 220 ng/mL (range: ≤244 ng/mL) and 143 mg/dL (range: 72-164 mg/dL), respectively. Clinical normalization of the patient's TA-TMA was achieved two weeks after loading dose administration with normalization of LDH and blood pressure values, improved proteinuria, decreased transfusion requirements, absence of schistocytes on peripheral smear, and complete resolution of pericardial effusion. A total of 5 maintenance doses of ravulizumab were administered approximately every 4 weeks with CH50 ranging <3-33 U/mL during this time period. Five maintenance doses were administered as the optimal duration was unknown and the patient's TA-TMA treatment course was complicated by COVID-19 infection, for which there was concern could lead to TA-TMA reactivation (which did not occur). The ravulizumab was well tolerated throughout with amoxicillin used for meningococcal prophylaxis. Conclusion: While studies evaluating ravulizumab for treatment of TA-TMA are ongoing, ravulizumab successfully led to complement blockade and clinical improvement in this patient with TA-TMA.

12.
Journal of Oncology Pharmacy Practice ; 28(2 SUPPL):21-22, 2022.
Article in English | EMBASE | ID: covidwho-1868958

ABSTRACT

Introduction: Desmoid-type fibromatosis (DF) are a group of rare (0.03% of total neoplasms) benign tumours arising from fibroblasts that can exhibit locally aggressive behaviour.1 Single agent oral vinorelbine (90 mg weekly for three consecutive weeks of each 28-day cycle) recently emerged as a simplified, less toxic and more manageable alternative to the standard pharmacological approach with two-weekly IV methotrexate and vinorelbine in patients with aggressive or refractory disease.2,3 This study aimed at describing the efficacy and safety of oral vinorelbine in a cohort of patients with DF in any line of treatment. Methods: This was a single-centre, retrospective study of patients treated with oral vinorelbine for DF over a period of 18 months (Jan 2020 to Jun 2021) within a specialised cancer centre. Patients who received at least 3 cycles of treatment (or until the first documented radiographic assessment, whatever occurred first) were included. The following information was collected from the electronic health care records system (Epic®): patient demographics (age, gender), number of previous treatment lines, and number of number of months on treatment (until closure of observations). Efficacy: clinical and radiographic reports were used to determine disease status as per RECIST A subgroup analysis of patients who were transitioned from other pharmacological options was also performed. Safety: routine full blood count and biochemistry weekly for the first three cycles, and once monthly thereafter were assessed for blood dyscrasias. Other side effects were also recorded. Results: Twenty-four patients were initially started on oral vinorelbine, of which 19 met the inclusion criteria (baseline and demographic characteristics summarised in table 1). Efficacy: Symptomatic improvement was observed in all cases (100%) shortly after initiation. Favourable radiographic findings (SD or better) were documented in 18 cases (94.73%). Only one patient (5.26%) experienced marginal increase in tumour size, but was maintained on treatment until further assessment, as still deriving clinical benefit. Additionally, all subjects in the subgroup who were transitioned from other pharmacologic options (9 patients) showed disease response. Safety: one episode of grade 3 neutropenia observed after seven cycles. Full blood counts and biochemistry parameters remained otherwise stable throughout treatment: one patient had low haemoglobin requiring multiple blood transfusions;this occurred in the context of poor baseline renal function and remained essentially unchanged throughout. The main reported toxicities were: diarrhoea (5 cases), nausea (grade 1 in 7 patients and grade 2 in one), fatigue (8), constipation (grade 2, 4 patients), and headaches and muscle pain (one case each). Discussion/conclusion: Single agent oral vinorelbine was found to be an effective treatment option for the management of newly diagnosed or refractory DF;this was also true for a subgroup of patients who received this to reduce hospital visits or after failure of a previous treatment line. Safety-wise, patients had stable blood counts and biochemical values throughout treatment, whereas most toxicities were mild and did not require discontinuation. These findings are in line with previous reports and underpin a paradigm shift in the management of DF in the post-COVID era.

13.
Diabetic Medicine ; 39(SUPPL 1):75, 2022.
Article in English | EMBASE | ID: covidwho-1868630

ABSTRACT

A 64 year-old Caucasian woman was admitted profoundly unwell with lethargy and generalised weakness. She had rheumatoid arthritis, with no history of diabetes or long-term steroid-use. Blood tests revealed pancytopaenia secondary to methotrexate (stopped on admission), acute kidney injury and severe metabolic acidosis. CT demonstrated lung base ground-glass changes. The patient had been double-vaccinated for covid-19 and all covid swabs were negative. Despite receiving Tazocin, respiratory symptoms worsened. High-dose co-trimoxazole was commenced to treat potential Pneumocystis infection from being immunocompromised. Subsequently she developed new confusion. Random blood glucose at midday was 1.2mmol/l. After treatment, hypoglycaemia recurred and persisted despite repeated intravenous dextrose boluses and glucagon injection. Blood glucose only improved with continuous 10% dextrose infusion. Causes were explored -a recent CT scan showed no pancreatic or intra-abdominal pathology;morning cortisol was normal. Literature review revealed very rarely Co-trimoxazole causes hypoglycaemia;hence this was stopped. Hypoglycaemia resolved within 48 hours;confusion improved. When serum glucose was 3.3mmol/l, c-peptide measured was inappropriately high (5175pmol/L). Co-trimoxazole is biochemically similar to sulfonylureas, mimicking their action on pancreatic beta-cells. Endogenous insulin hypersecretion raises c-peptide levels during hypoglycaemia. As Co-trimoxazole is renally excreted, when renal function is impaired it accumulates, with exacerbation of side effects such as protracted hypoglycaemia, especially at higher doses, as in our case. Hypoglycaemia will likely resolve after a 24-48h washout period, especially if renal function improves back to baseline. We recommend awareness of hypoglycaemia risk with co-trimoxazole treatment and blood glucose monitoring for inpatients taking high doses, especially in the setting of renal impairment.

14.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i95, 2022.
Article in English | EMBASE | ID: covidwho-1868406

ABSTRACT

Background/Aims Cogan's syndrome is a rare vasculitis, characterised by progressive sensorineural bilateral hearing loss, vestibular symptoms and nonsyphilitic interstitial keratitis. We present a paediatric case which was refractory to initial treatment and subsequently successfully treated with Tocilizumab. Methods Case report. Results A previously well 14-year-old boy presented with sudden onset hearing loss, tinnitus and vertigo. There was no history of new medication, trauma or tick bite. There was no family history of autoimmune disease or genetic hearing losses. On examination his cranial nerves except for vestibulocochlear were grossly intact. He had a steady gait with no cerebellar signs. Otoscopy was unremarkable. Audiology showed a moderate to severe sensorineural hearing loss bilaterally, worse on the right. He had normal type A tympanograms. An autoimmune screen was carried out which showed normal FBC, inflammatory markers, ACE and Chitotriosidase. ANCA, Rheumatoid factor and lyme serology were negative. MRI of the internal auditory meati was normal. He subsequently developed visual disturbance and was diagnosed with bilateral interstitial keratitis. A unifying diagnosis of Cogan's syndrome was made. An MRI scan of his head, neck and upper thorax looking for evidence of large vessel vasculitis was normal. Genetics looking for evidence of a monogenic autoinflammatory disorder and primary immunodeficiency were negative. He was initially treated with a weaning course of oral prednisolone with improvement in symptoms. Unfortunately, 4 months later had clinical and audiological deterioration in hearing. He therefore received pulsed IV methylprednisolone and commenced subcutaneous methotrexate and adalimumab. 3 months later, there was both clinical and audiological improvement and he started to wean prednisolone. 7 months later, he presented with a 24-hour history of reduced hearing on the right, confirmed on audiogram. He received a further pulse of IV methylprednisolone and a short course of high-dose oral prednisolone, followed by a slowly weaning course. 3 months later, again he felt his hearing had deteriorated and this was confirmed on audiogram, with a 40-decibel loss in his previously good ear. He received two doses of IV methylprednisolone and background steroids were increased to 20mg daily. Due to frequent relapses, adalimumab was changed to IV tocilizumab at 10mg/kg 2-weekly, alongside methotrexate. IV tocilizumab was changed to the subcutaneous route during the COVID-19 pandemic and was tolerated well. His hearing subsequently improved and tocilizumab interval was extended to 3-weekly in Feb 2021. At last review he was stable and successfully transitioned to adult services. Conclusion Evidence regarding treatment options in paediatric patients is lacking due to the rarity of the condition and consequent difficulty in arranging high-quality trials. This is the first case report of use of tocilizumab for Cogan's syndrome in children, highlighting it as a well-tolerated and successful treatment modality.

15.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i72, 2022.
Article in English | EMBASE | ID: covidwho-1868395

ABSTRACT

Background/Aims Infections on conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) are an important concern for rheumatoid arthritis (RA) patients, especially during the COVID-19 pandemic. However comparative safety data between csDMARDs have been conflicting and limited in power. The objective was to assess the comparative safety of serious, opportunistic and all infections (including nonserious) of first-line csDMARDs in RA through a large multinational observational study. Methods We evaluated first-line new users of methotrexate (MTX), hydroxychloroquine (HCQ), sulfasalazine (SZ) and leflunomide (LEF) monotherapy. Data was obtained from four US databases (IQVIA US Ambulatory EMR (AMBER), Optum® De-identified Clinformatics® Datamart (Optum), IBM MarketScan® Medicare Supplemental Database (MDCR), and IBM MarketScan Commercial Database (CCAE)), one from Germany (IQVIA Disease Analyser Germany EMR (Germany)), and another from the UK (IQVIA UK The Health Improvement Network). Patients included were ≥18 years with a RA diagnosis between 2005-2019, without prior inflammatory arthritis, cancer or infection (in the preceding 30 days). Serious infections were defined as those requiring hospitalisation or resulting in death within 30 days;opportunistic infections were defined as per published EULAR consensus. Patients were followed from 1-day following treatment initiation to the earliest of treatment discontinuation, switching, or addon plus 14 days, or loss to follow-up. Cox proportional-hazards models for MTX against each csDMARD with large-scale propensity score stratification were performed. A large set of negative control outcomes were used to calibrate hazard ratios (cHR) to account for potential residual confounding. Estimates were pooled where homogeneity across sources was adequate (I2<0.4). Results A total of 247,511 patients were included (MTX: 141,647;HCQ: 73,286, SSZ: 16,521, LEF: 16,057), with pooled incidence rates of serious, opportunistic and all infections across sources for MTX users of 33.7, 20.1 and 311.8 per 1,000 pyrs, respectively. With MTX as the referent, for all infections, the pooled cHR (with 95% Confidence Intervals) for SSZ was 0.73 (0.62, 0.86);HCQ, 0.96 (0.89, 1.04);and LEF, 0.74 (0.50, 1.08). The serious infection pooled cHR for SSZ was 0.75 (0.58, 0.97) and for LEF, 0.93 (0.61, 1.40). For opportunistic infections, pooled cHR for HCQ was 1.04 (0.92, 1.19). Conclusion SSZ, LEF and less consistently HCQ had a lower risk of all (including non-serious) infections, compared to MTX. SSZ and LEF were associated with a 25% reduction in the expected risk of all infections. SSZ was associated with a 25% lower risk of serious infections relative to MTX. In the first large scale observational network study assessing comparative risk of infection with csDMARDs there were differences between drugs in risk for all infections, with potential implications for clinical care.

16.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i55, 2022.
Article in English | EMBASE | ID: covidwho-1868382

ABSTRACT

Background/Aims The aim of this study is to describe demographics features and outcomes of patients with rheumatic diseases diagnosed with COVID- 19 in a single hospital. Methods Patients with rheumatic diseases and COVID-19 were identified via rheumatology outpatient and inpatient hospital admissions between February 2020 and March 2021. Data was collected retrospectively using the electronic medical records system and in-person and telephone consultations. The data was entered into the COVID-19 Global Rheumatology Alliance (GRA) Registry. Data collected included age, gender, ethnicity, smoker status, rheumatic disease, co-morbidities, drug history and vaccine status. Patient outcome was recorded as mortality, recovered (including days to recovery) or symptoms persisting over 90 days (>90). Requirement for hospital admission was also recorded. Comparison was made to the published GRA Registry data. Results Forty-three patients were identified;33 Female (77%),10 (23%) male. Median age 52. 22 Caucasian, 12 Black, 3 mixed race, 2 Asian, 2 Hispanic and 5 unknown. Rheumatoid arthritis (14 patients;33%) was the most common disease. Other diagnoses included psoriatic arthritis (6;14%), systemic lupus erythematosus (4: 9%), Sjogrens syndrome (4: 9%) and ankylosing spondylitis (4;9%). The most common disease modifying antirheumatic drug (DMARD) was methotrexate (35%) followed by hydroxychloroquine (33%). Eight patients were taking steroids (19%). Factors associated with hospitalisation were older age (57% age ≥ 52 vs 40% < 52), multiple co-morbidities (71% ≥ 2 comorbidities vs 35% < 2 co-morbidities) and black ethnicity (75% black vs 26% of caucasian). These risk factors for morbidity are similar to the UK background population and published COVID-19 GRA data. There was no increased risk of hospitalisation between different DMARDs (53% on methotrexate required admission vs 50% on hydroxychloroquine). 71% of patients on steroids required admission. The overall study cohort had a 49% hospital admission rate. Similar risk factors were identified for persistence of symptoms > 90;27% of black patients vs 5% of caucasian and 24% of patients with > 2 comorbidities vs 10% of patients with < 2 co-morbidities. Age did not follow the same trend as hospitalisation;10% of patients age ≥ 52 vs 20% of patients < 52 had symptoms > 90. 15% on methotrexate had a recovery time > 90 vs 7% on hydroxychloroquine and 14% on steroids. The overall cohort had a 17% rate of patients having symptoms > 90. Mortality rate within the cohort was 5% (2 patients). Conclusion A case-series of 43 patients with rheumatic diseases and COVID-19 was conducted. The risk factors for hospitalisation, mortality and persistence >90 were similar to other studies. Most significantly the findings show a correlation between black ethnicity and increased risk of all mortality, hospitalisation and symptoms > 90. There was no difference in hospitalisation and different DMARDs.

17.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i53-i54, 2022.
Article in English | EMBASE | ID: covidwho-1868379

ABSTRACT

Background/Aims Current British Society for Rheumatology guidance suggests 3- monthly blood monitoring for patients prescribed methotrexate whose disease, drug dosage and blood results are stable, although evidence for this recommendation is limited. Anecdotal reports suggest monitoring may have reduced during the pandemic. The study aims were, in patients with rheumatoid arthritis (RA) prescribed methotrexate, to determine: 1) interval length between blood tests during the pandemic, and 2) whether prolonged intervals were associated with abnormal blood test results. Methods Data came from the Greater Manchester Care Record, a database containing electronic health records from both primary and secondary care for people across Greater Manchester. Inclusion criteria were: a diagnosis of RA;regular blood monitoring (<=91 days between blood tests) and regular methotrexate prescriptions in the year prior to the pandemic (01/03/2019-01/03/2020). Blood test intervals during the pandemic were determined, a prolonged interval was defined as>91 days. Cytopenia and transaminitis events were identified between March-December 2020 and March-December 2019 (as a comparator). Proportions of events are presented for each time-period and stratified by whether the interval was prolonged. Results 1011 patients met the inclusion criteria, with a median of 5 blood tests (interquartile range (IQR): 3-7) during the pandemic and a median interval of 57 days (IQR: 34-84). 612 (61%) patients had at least one prolonged interval, with 241 (23.8%) having multiple. Prolonged intervals had a median length of 112 days (IQR: 99-135). 115 (11.4%) people had a prolonged interval but no subsequent blood test before the study end date (median 120 days [IQR: 104-150]). The proportion of cytopenia and transaminitis events was low and similar across time-periods and when stratified by interval length (Table 1). Conclusion Nearly two-thirds of patients on stable methotrexate had at least one prolonged interval and 1 in 10 had a prolonged interval with no subsequent blood test. Initial analyses of test results do not indicate increased occurrence of methotrexate blood toxicity in those with prolonged blood test intervals during the pandemic. Further work is required to determine whether those with prolonged intervals represent a group at lower risk where less frequent blood tests would be appropriate.

18.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i33, 2022.
Article in English | EMBASE | ID: covidwho-1868362

ABSTRACT

Background/Aims To understand the impact of COVID-19 on UK paediatric rheumatology services, to determine the learning points that could change future practice and provide information for trainees to plan further quality improvement projects. Methods Survey details with a Microsoft forms link were emailed to consultants in each UK paediatric rheumatology centre. Consultants were asked to forward the survey onto any regional units linked. The survey was also sent to the paediatric rheumatology trainee's network. The questionnaires covered blood monitoring, joint injections, oral corticosteroid use, modes of consultations, positive and negative impacts and what changes should be carried forward. Results 20 staff responded to the survey;17 consultants and three trainees (registrar level). 85% (17) reported changes had occurred to the frequency of blood monitoring to rheumatology patients on Disease Modifying Anti-Rheumatic Drugs (DMARDs) and/or biologics during the pandemic. 60% (12) reported this was due to patient/family reluctance to attend or due to unavailability of appointments. 75% (15) recorded no complications following changes of blood frequency on disease activity or flare. Of those that did have a flare, a number of factors were described including: parents not contacting their service, patient/family discontinuation of treatment, lack of clinic attendance, running out of treatment and safety concerns re prescribing from primary care or local department. One patient on methotrexate experienced significantly deranged liver function tests.75% (15) reported changes with provision of joint injections due to theatre unavailability. As a consequence, 60% (12) reported an increase in the use of oral corticosteroids with 20% (4) performing more ward-based injections with local anaesthetic or Entonox on younger children. 100% (20) changed their mode of consultation. Between 5 and 40% of all appointments are now virtual (telephone or video) and 60-95% are face-face. 60% (12) report that frequency of review appointments has now returned to normal. Major disruption has occurred in seeing new patients due to a backlog of patients created by the pandemic. Some patients have been referred to units with possible arthritis that haven't been examined or seen face-face in primary care prior to referral. Conclusion COVID-19 caused significant changes to UK paediatric rheumatology services. The results highlighted the use of virtual consultations where appropriate, consideration of joint injections without general anaesthetic as much as possible, enabling virtual teaching/conferences and also raising the need to review blood monitoring frequency for patients on DMARDs/biologics. Longer waiting lists, increased anxiety amongst children and young people, increase in disease activity and the impact on learning opportunities for trainees were concerning negative aspects of the pandemic. It is likely the impact of these negative consequences will continue to be experienced for some time. Future planning and consideration is required to minimise the negative impact of these aspects on our patients and colleagues.

19.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i26-i27, 2022.
Article in English | EMBASE | ID: covidwho-1868358

ABSTRACT

Background/Aims Rituximab (RTX) is a chimeric monoclonal antibody against CD20, a transmembrane protein on B lymphocyte surfaces. It is now established as an effective treatment for rheumatoid arthritis (RA). The timing of re-treatment is not well defined and varies widely in clinical practice. Our aim was to assess departmental practice on retreatment doses and frequency of RTX in RA patients. We proposed retreatment with reduced dose (1gm) as compared to the standard dose (1gm x 2, 2 weeks apart). We envisage this would maintain efficacy and lead to significant cost saving for the CCG and Rheumatology department. Methods Data were collected retrospectively over 3 years (2017- 2020) on patients who had received at least one re-treatment RTX dose no less than 24 weeks after first course of standard regime. Information was obtained on patient demographics, serology, disease activity scores, previous treatment, concurrent therapy including steroids, frequency and dosing of RTX. Results 50 patients were included with an average age 66 years (range 32- 85), 58% were females, 83% were seropositive and 59% had established erosive disease. Most patients had received prior therapy of nonbiologic and biologic DMARDs, methotrexate and anti-TNF being the most used agents in 70% and 52%, respectively. 40% were biologic DMARD naïve mostly due to anti-TNF contraindication. 78% patients were receiving concomitant non-biologic DMARDs, methotrexate being the commonest in 50% patients. 16% were on steroids with dose ≤ to 5mg in 87% of patients. 43% of patients received RTX 6 monthly, 33% 6- 9 monthly and 24% > 9 months. 96% of the patients received 1gm infusion x2, 2 weeks apart. Conclusion Our data showed most patients were receiving standard retreatment regime (1gm x2 fortnightly) at 6 monthly intervals. Previous studies including SMART (Study of Re-treatment With MabThera), SERENE, IMAGE and MIRROR trials suggested reduced dose of RTX had similar clinical efficacy to the standard dose. We optimised retreatment schedule of RTX from 2g to 1g, thereby reducing infection risk, especially in view of the COVID-19 pandemic and achieving significant cost savings. (Table Presented).

20.
Rheumatology (United Kingdom) ; 61(SUPPL 1):i23, 2022.
Article in English | EMBASE | ID: covidwho-1868355

ABSTRACT

Background/Aims Patient initiated follow up (PIFU) is an initiative that allows patients to access follow-up when required by initiating their own appointments. The disruptive impact of the COVID-19 pandemic has created significant capacity constraints on many services that were already experiencing pressures prior to the pandemic, piquing interest in PIFU as a sustainable model of care. The BSR has produced draft PIFU guidance to support rheumatology units to develop and deliver safe, robust and sustainable PIFU care models. We audited our PIFU pathway in line with the BSR's recommended standards to establish its safety and efficacy since its implementation in 2018. Methods This was a retrospective analysis of patients transferred to an active PIFU access plan within UHMBT between February 2018 and November 2019. Patients were identified by the informatics team from the active PIFU access plans. We captured data to include treatments used. The electronic case notes were reviewed to establish reasons for appointment triggers in those who contacted the service, analyse waiting times for clinical review further to making contact and assess subsequent outcomes. Data were entered and analysed in Microsoft Excel 2016. Results We had a total of 420 patients on PIFU. We audited 100 of these. 56 were female. Mean age was 63.4 years (24-96). The most common diagnosis was inflammatory arthritis (n=78). The majority of patients (n=53) were on a single disease-modifying anti-rheumatic drug (DMARD). 17 patients were on>1 DMARD and 5 patients were on prednisolone monotherapy, mean steroid dose 4.9mg daily. 25 patients were not on any treatment. Of those on DMARDS, 63% (44) were on methotrexate, either as monotherapy or in combination with other DMARDs. 68% (30) of those on methotrexate were on a dose of ≤15mg weekly. Of the 100 patients, 9 triggered a review within the follow up period. This was usually via the nurse helpline (n=7). Occasionally the GP triggered a review on behalf of the patient via the advice and guidance line (n=2). The most common reason for a trigger was a flare of inflammatory arthritis (n=7) and the remaining two appointments were due to side effects of DMARDs. Patients were contacted via nurse telephone call back within 48 hours of contacting the helpline (n=8). One patient required an urgent face-to-face consultant review and was seen within 7 days. After contacting the service, all nine patients were reverted to regular follow-up. Conclusion Our results confirmed a robust PIFU pathway with appropriate safety nets ensuring prompt access to clinician input when needed. The use of PIFU pathway did not compromise care or result in any worsening clinical outcomes. After validation at 2 years, the majority of our patients on this pathway were onwardly managed through the PIFU model.

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