Trattamento corticosteroideo versus terapia convenzionale nella corea reumatica

The present retrospective observational study on thirty children with Sydenham chorea shows that steroid treatment seems to be more effective than symptomatic treatment in both clinical remission and clinical improvement of symptoms.

A case report of COVID-19 positive Becker Muscular Dystrophy

Background: Becker and Duchenne muscular dystrophies constitute the most common inherited dystrophinopathies. The chronic steroid treatment predisposes them to any infection, hence, we sought to determine the current COVID-19 infection in them. We conducted an analysis on a real-world database to identify the effect of COVID-19 infection and identified a case of Becker muscular dystrophy who tested positive for COVID-19. For our analysis, we utilized Cerner Real-World DataTM that was provided through Cerner's HealtheDataLab research tool. Case report: A 63-year-old Caucasian male with Becker muscular dystrophy, hyperlipidemia, and atrial fibrillation, was hospitalized with COVID-19 infection. Our search revealed June 22, 2020, as the patient's COVID-19 service date when tested positive. The patient received antibiotics and supportive therapy during hospitalization. Intricate details like oxygen requirement, blood gas analysis, and mechanical ventilation could not be retrieved if used. The patient developed complications like sepsis, pneumonia, acute respiratory failure that resulted in prolonged hospitalization. Our data reported that the patient was alive during discharge. Conclusion: Although patient developed complications during hospitalization, no death from the COVID-19 infection was observed in our analysis.

Immune Thrombocytopenia Induced by the Chimpanzee Adenovirus-Vectored Vaccine against SARS-CoV-2 Infection

We present a case of immune thrombocytopenia (ITP) induced by the chimpanzee adenovirus-vectored vaccine, without evidence of thrombosis, eight days after vaccine administration. The thrombocytopenia condition improved after administering steroid treatment. This adenovirus vaccine had been reported to induce rare side effects, such as immune thrombotic thrombocytopenia. This case report showed that it could also induce immune thrombocytopenia without the presence of thrombosis. Therefore, we should be cautious of this rare side effect as global vaccine administrations against coronavirus disease increase.

Late onset congenital adrenal hyperplasia in pregnancy

Congenital adrenal Hyperplasia (CAH) is a rare disorder to manage in pregnancy as CAH is known to cause infertility. Late onset CAH is more so with 21-hydroxylase deficiency being the most common enzyme deficiency for the same. The mainstay of management in pregnancy is multidisciplinary team management with a consultant Obstetrician and Medical Endocrinologist, steroid treatment and avoiding virilisation of the female patient in early pregnancy is important continuation of dexamethasone is controversial with conflicting evidence and also precipitating or worsening hyperemesis in pregnancy.

Evaluation of Pulmonary Toxicities in Lymphoma Patients Receiving Brentuximab Vedotin

Background: Brentuximab vedotin (Bv) is an anti-CD30 antibody conjugated via a protease-cleavable linker to the anti-microtubule agent monomethyl auristatin E (MMAE). It is FDA approved for the treatment of classic Hodgkin lymphoma (cHL) and CD30-expressing T-cell lymphomas in both upfront and relapsed/refractory (R/R) settings. The most common side effects in the original registration trials (ECHELON-1, ECHELON-2, AETHERA) include peripheral neuropathy and cytopenias. With its expanded use in real world settings, it is imperative to identify less-established adverse events which may also result in dose delays or reductions. Pulmonary toxicity is a rare but potentially life-threatening side effect of Bv, but few studies have characterized this toxicity in the adult and pediatric populations. Here, we characterize the incidence and risk factors of developing Bv-associated pulmonary toxicity in patients with lymphoma. Methods: We conducted a multicenter, retrospective, descriptive study of patients receiving Bv at University of California San Francisco Health in San Francisco, CA and Community Medical Center in Fresno, CA. Adult and pediatric patients were included if they received at least one dose of Bv between June 1, 2015 and September 30, 2020. Retrospective chart review was conducted to identify patients who developed respiratory symptoms concerning for Bv-induced pneumonitis. Past medical history, smoking history, and prior administration of pulmonary toxic agents were collected to assess for risk factors contributing to Bv-associated pulmonary toxicity. Respiratory symptoms were classified as likely related, possibly related, or not related to Bv. Patients were identified to have pulmonary toxicities likely related to Bv if they satisfied the following criteria: development of respiratory symptoms with a temporal relation to Bv, suggestive chest imaging or pulmonary function tests (PFTs), rule out of other etiologies including infectious causes, and relief of symptoms with steroid treatment and/or Bv discontinuation at the physician's discretion. Patients were identified to have pulmonary toxicities possibly related to Bv if they satisfied the following criteria: development of respiratory symptoms with a temporal relation to Bv, equivocal chest imaging or PFTs, inability to fully rule out other etiologies, and relief of symptoms with steroid treatment or Bv discontinuation at the physician's discretion. Data is reported using descriptive statistics. A consort flow diagram of the selection process is depicted in Figure 1. Results: A total of 123 patients were reviewed, of whom 27 were excluded due to pregnancy, prisoner status, or not having received Bv during the study period. 96 patients were included in the final analysis. Baseline characteristics are captured in Table 1. Following Bv administration, 19 of the 96 patients developed pulmonary symptoms (dry cough, shortness of breath, dyspnea on exertion, chest pain, or hypoxic respiratory failure not justified by a competing process). Based on the prespecified definitions, we identified four patients (4.2%) who developed respiratory symptoms concerning for Bv-induced toxicity. The mean age of the four patients was 51 (range 28-76) and one patient was female. One patient received Bv in the upfront setting for cHL, one had R/R cHL, and the remaining two had anaplastic large cell lymphoma (ALCL). One patient previously received pulmonary toxic agents (gemcitabine and carmustine) and two patients had a history of tobacco use. The cumulative doses of Bv the four patients received prior to developing respiratory symptoms ranged from 8.8 to 30.4 mg/kg (6-21 cycles). Three patients were classified as likely related based on supportive findings on chest imaging or PFTs and one patient was classified as possibly related given lack of definitive evidence to differentiate between pneumonitis and disease progression. Three of the four patients developed symptoms requiring steroid treatment and two required Bv dose reduction and/or discontinuation. Conclusions: This study is an effort to raise awareness of the incidence of Bv-induced pulmonary toxicity and describe the potential risk factors associated with this adverse event. Further real world studies in larger and diverse patient populations are necessary to better characterize the incidence and risk factors associated with Bv-associated pulmonary toxicities. Figure 1 Figure 1. Disclosures Lo: EUSA Pharma: Consultancy; Oncopeptides: Consultancy. Ai: Kymria, Kite, ADC Therapeutics, BeiGene: Consultancy. Abdulhaq: BMS, Alexion, Oncopeptides, Morphosys, Pfizer, Norvartis: Honoraria; Oncopeptides, Alexion, Amgen: Speakers Bureau; Morphosys, BMS, Amgen: Membership on an entity's Board of Directors or advisory committees. Fakhri: Loxo/Lilly: Research Funding.

Effect of Steroid Treatment on the Diagnostic Yield of Baseline PET CT in Aggressive B Cell Lymphoma

Aggressive B cell lymphomas often require prompt steroid treatment prior to baseline 18f-fluorodeoxyglucose positron-emission tomography (PET CT) scan and definitive treatment in order to alleviate symptoms and/or prevent organ damage. Since lymphomas are a steroid sensitive malignancy, there is a concern that steroid prophase might affect PET CT results and diagnostic yield. We conducted a retrospective cohort study to evaluate the effect of steroid treatment prior to baseline PET CT scan on the standardized uptake value (SUV) max and additional PET CT parameters by examining two groups of patients: steroid-naïve and steroid-treated patients. The effect of steroid administration on SUV max was examined across different daily and weekly steroid doses and durations of treatment. Between January of 2017 and May 2020, 187 newly diagnosed patients with aggressive B cell lymphoma who had a pre-treatment PET CT scan were evaluated. 160 patients (85.5%) had Diffuse large B-cell lymphoma (DLBCL)/ High-grade B-cell lymphoma, 13 patients (7%) had primary mediastinal (thymic) large B-cell lymphoma, 9 patients (4.8%) had primary DLBCL of the central nervous system and 5 patients (2.7%) had Burkitt lymphoma. 132 patients (70.6%) were included in the steroid-naïve group and 55 patients (29.4%) in the steroid-treated group. In the steroid-treated group, the mean duration of steroid treatment was 10.49 (±9.28) days. Average daily dose of steroid treatment was equivalent to 72.27 (±36) mg of prednisone and the mean cumulative prednisone dose during the week prior to PET CT scan was equivalent to 367.95 (±239.9) mg of prednisone. There was no statistical significant difference between the groups in age, gender or KI67. However, patients in the steroid treated group had a significantly higher stage of disease compared to the steroid-naïve group (mean 3.44 compared to 2.99, respectively, p=0.01). The steroid-treated group also had a trend towards a higher IPI score (mean 2.45 versus 2.08, p=0.08) and a trend towards a higher LDH level (mean 2309.89 U/L, range 250-81374 versus mean 877.65 U/L, range 272-22036, p= 0.07), as depicted in table 1. There was no statistical difference in SUV max between the steroid-naïve and steroid-treated groups (p=0.97). This was consistent across various steroid treatment durations and dosage regimes. Patients in the steroid-treated group had a trend towards a higher tumor burden and a larger tumor volume compared to the steroid-naïve group, however it did not reach statistical significance. Mean tumor volume was 179.04 cm 3 in the steroid naïve group and 337.06 cm 3 in the steroid treated group (p=0.17). Mean tumor burden was 1944.84 in the steroid-naïve group and 3016.94 in the steroid-treated group (p=0.09). There was no difference in additional PET CT parameters including SUV mean, SUV max and SUV mean of liver and mediastinum between the groups as depicted in table 2. In conclusion, in aggressive B cell lymphoma, pre-treatment with steroids prior to initial PET CT scan does not affect SUV max or other PET CT parameters and does not reduce PET CT diagnostic yield. Figure 1 Figure 1. Disclosures Gurion: Medison: Consultancy; Gilead Sciences: Consultancy; Takeda Pharmaceuticals: Consultancy; JC Health Care: Honoraria; Roche: Honoraria.

Giant cell arteritis associated with PD-1 inhibition

A 50-year-old woman was referred to rheumatology for new onset polyarthralgia and headache. She had a history of metastatic lung adenocarcinoma and was started on treatment with the programmed death 1 receptor (PD-1) antagonist pembrolizumab 2 months prior. Examination revealed left temporal artery tenderness and hand synovitis. Investigations revealed enlarged temporal artery on ultrasound imaging. On steroid treatment, she had resolution of symptoms, but due to significant steroid side effects required methotrexate and her PD-1 antagonist therapy was continued in consultation with her oncologist. Her malignant disease has remained stable, and she has improved functional status.