systemic steroids
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2022 ◽  
Vol 0 ◽  
pp. 1-5
Author(s):  
David Pudukadan

Coronavirus disease-2019 (COVID-19) has affected countries around the world. The introduction of COVID-19 vaccines has proved the most effective arsenal in the fight against the disease. However, with the vaccination of billions of people, data on vaccine-induced adverse reactions are also emerging. We report a 32-year-old woman who manifested papulopustular rash 7 days after receiving Moderna COVID-19 (mRNA-1273) vaccine. The patient responded to a short course of systemic steroids and antihistamines. Awareness regarding the possible adverse events that can be anticipated after the COVID-19 vaccination may help the healthcare professionals to offer prompt and effective care to the affected.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4253-4253
Author(s):  
Syed Mujtaba Naqvi ◽  
Carrisa Schwartz ◽  
Rachel Whittaker ◽  
Ghayth Hammad ◽  
Rishi Agarwal

Abstract Introduction: Type I cryoglobulinemia (Type I CG) usually develops in the setting of protein-secreting monoclonal gammopathies [1,2]. The estimated frequency of renal involvement is 30% in Type 1 CG. Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening disease in which there is a deficiency in ADAMTS13, a protease which normally functions to cleave von Willebrand factor (vWF). A deficiency leads to persistence of large vWF multimers and the formation of platelet-rich thrombi in the microvasculature. We present a case of 52 years old female with Type 1 CG and acquired autoimmune TTP secondary to mild Covid-19 disease. Case Report: A 52-year-old woman with medical history significant for CKD stage 2, with baseline creatinine (Cr) of 2.5, Diffuse Large B-Cell Gastric Lymphoma (DLBCL), in complete remission, and recent diagnosis with COVID-19 presented to the ED for evaluation of three days of a petechial rash on her bilateral extremities. Significant labs on arrival included hemoglobin (Hgb) 8.9, platelet count 65, Cr of 3.65, and K+ 6.6, meeting criteria for acute kidney injury (AKI). Peripheral blood smear showed thrombocytopenia and normochromic normocytic anemia with no schistocytes or microangiopathic changes. ADAMTS13 was 43% and there was no clinical suspicion for TTP. The patient's Cr continued to worsen up to 8.1 during admission, prompting renal biopsy. Renal biopsy showed Type 1 cryoglobulinemic glomerulonephritis with vasculitis and 30 to 40% fibrosis of glomeruli. The patient was started on systemic steroids, plasma exchange every 2-3 day (completed 6 sessions) and hemodialysis. Her platelet counts steadily increased to 212 but had a significant drop to 32 when rechecked seven days later, prompting further investigation. ADAMTS13 was rechecked and found to be 5% with presence of inhibitor, Hgb was 7.7, haptoglobin was <20, and a diagnosis of TTP was made. The patient was started on daily plasmapheresis, rituximab, Caplacizumab and high dose systemic steroid therapy. Our patient had prior history of DLBCL and was treated with radiation therapy. Restaging PET/CT and EGD with biopsy showed no evidence of recurrence. Bone marrow biopsy was negative for evidence of lymphoma. Several serologic tests were used to rule out other etiologies of Type I CG and came back negative including: SLE, HIV, hepatitis C, multiple myeloma, and Waldenstrom's macroglobulinemia. The patient had elevations in multiple immune markers including IgG, IgM, free kappa light chains, and free lambda light chains. She also had hypocomplementemia of both C3 and C4. Discussion: The relationship of Type I CG and COVID-19 is unclear, though viral infections can trigger autoimmune diseases [3]. Previously, all patients with Type I CG had either a hematologic malignancy, solid-organ malignancy, infection, or autoimmune syndromes. Interestingly, in our patient, several serologic tests were performed and negative, which ruled out these etiologies of Type I CG. Our patient's manifestation of Type I CG was petechial rash on extremities and acute on chronic renal disease. Specific treatment of CG can include plasma exchange, corticosteroids, rituximab, and hemodialysis, depending on underlying cause. Our patient was started on systemic steroids, plasma exchange every 2-3 days, and hemodialysis due to worsening renal functions. Despite cases of covid-19 and TTP being reported, the relationship between the disease processes remains unclear. COVID-19 infection can cause disproportionate activation of the complement system and lead to excessive coagulation and thrombotic events. Systemic infection can decrease ADAMTS-13 activity, and this has also been noted in COVID studies. ADAMTS-13 deficiency has no pathophysiologic relevance specific to Type 1 CG. Our patient developed anemia and thrombocytopenia, and ADAMTS13 activity testing was found to be 5% with presence of an inhibitor. Considered as high-risk disease, our patient was started on steroids, rituximab, Caplacizumab and daily plasmapheresis. Repeated weekly ADAMTS13 level was 26% and platelets were stabilized at 69k/microL. Conclusion: Suggests that Covid-19 virus has the capability to induce cryoglobulinemia through an unknown mechanism • There are two other case reports of an association between Covid-19 and TTP. • May be the first case indicating a possible association of Covid-19 with cryoglobulinemia. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3899-3899
Author(s):  
Egor A. Kulagin ◽  
Alisa G. Volkova ◽  
Anna A. Dotsenko ◽  
Valeria R. Yanbukhtina ◽  
Julia D. Rabik ◽  
...  

Abstract Introduction: Bronchiolitis obliterans syndrome (BOS) is an extremely unfavorable chronic GVHD manifestation with limited therapeutic options. Recent small retrospective series and the only prospective randomized trial have demonstrated different response rates (8.6 - 66.7%) in patients with BOS to JAK1/2 inhibitor ruxolitinib (Streiler C. et al. BMT 2020, Zhao Y. et al. Front. Pharmacol. 2021, Zeiser R. et al. NEJM 2021). Here we report a single-center study of the ruxolitinib efficacy for BOS in adults after HSCT. Methods: The study included 52 patients (median age 33 years (18-58), female 48%) diagnosed with BOS according to the NIH (2014) criteria between 2008 and 2020. The median %FEV1 at BOS diagnosis was 41.6% (20.0-74.1). Severe BOS was present in 44% patients. Due to the previous extrapulmonary cGVHD, the vast majority of patients (81%) had a history of various treatment options, including calcineurin inhibitors, systemic steroids, extracorporeal photopheresis (ECP), tyrosine kinase inhibitors (TKIs imatinib, dasatinib) and ruxolitinib. After BOS diagnosis the following treatments were administered: a FAM-like regimen (fluticasone, azithromycin and montelukast) (94%), CNIs (50%), mTOR inhibitor (35%), systemic corticosteroids (52%), ECP (25%) or low-dose rIL-2 (19%) as the first and subsequent lines of therapy. Seventeen patients (33%) received TKIs. A total of 20 BOS patients received ruxolitinib (median dose of 15 mg/day). The remaining 32 patients were included in the control group. The response was assessed by the dynamics of FEV1 according to the NIH scale (2014): partial response (PR, ≥10% increase in FEV1), stabilization (<10% change), progression (≥10% decrease) (n=45), as well as clinically by the degree of dyspnea according to the NIH symptom-based lung score and mMRC scale with PR defined as a decrease in dyspnea by at least 1 point (n=52). The overall survival (OS) was assessed with the Kaplan-Meier method from the moment of BOS diagnosis. Results: At a median follow up of 27 months (5-102) in the ruxolitinib group, 5 (26%) patients achieved PR criteria, while BOS stabilization and progression was observed in 7 (37%) cases each. In the control group, PR, stabilization and progression were documented in 3 (12%, p=0.253), 17 (65%) and 6 (23%) cases respectively (Fig.1). The clinical evaluation of dyspnea also did not reveal statistically significant differences in PR rate: 15% vs 19% according to NIH-CC and 30% vs 31% according to mMRC scale (p = 1.0). Systemic steroids were used less frequently in the ruxolitinib group (35% versus 63%, p = 0.049). As of July 1, 2021, 32 (62%) patients were alive. Twenty patients died due to cGVHD and infectious complications (n = 16, 80%) or underlying disease relapse (n = 4, 20%). As a result, the 5-year OS was 54.4% (95% CI, 36.7-69.1) with no statistically significant differences between ruxolitinib and control groups: 59.1% (95% CI, 25.6-81.6) vs 50.2 (95% CI, 29.5-67.7) (p=0.387). Conclusions: In comparison with the retrospective control group, ruxolitinib showed no significant benefit in FEV1-based and clinical response rate in patients with BOS after HSCT. However, ruxolitinib therapy provides a more frequent systemic steroid-free strategy. These data are consistent with the results of REACH3 (Zeiser R. et al. NEJM 2021). Despite very positive results of ruxolitinib in the general cGVHD population, BOS remains an unsolved problem. Novel approaches are required. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare. OffLabel Disclosure: Ruxolitinib for BOS in the settings of cGVHD


Author(s):  
Nitin Goel ◽  
Nitesh Goyal ◽  
Ravishankar Nagaraja ◽  
Raj Kumar

Some patients even 4 weeks after Corona Virus Disease 2019 (COVID-19) remain to be symptomatic and are known as “long-COVID”. In the present study we performed the follow up evaluation at 3 months of long-COVID patients, after treatment with systemic steroids. During the study duration, out of the 4,542 patients managed in the outpatient department of the particular unit, there were 49 patients of Long-COVID. The patients having abnormal computed tomography (CT) alongwith resting hypoxia or exertional desaturation were treated with systemic steroid (deflazacort) in tapering doses for 8-10 weeks. We retrospectively analysed the clinical and radiological findings of these patients at first presentation and at about 3 months of follow up visit. On follow up, all the 49 long-COVID patients showed improvement. The occurrence of breathlessness decreased from 91.83% to 44.89% (p<0.001) and cough from 77.55% to 8.16% (p<0.001). Twenty-four patients were prescribed systemic steroids. Out of these, nearly 58% patients had MMRC grade 4 breathlessness, which decreased to < 2 MMRC in about 86% of these patients. MMRC grade (median) decreased from 3 to 1 (p<0.001). Majority of patients who were tachypnoeic and hypoxic at rest (n=7) showed improvement (71%), post-treatment with corticosteroids. Occurrence of normal chest X-ray increased from 12% to 71% (p<0.001). All these patients had abnormal CT thorax initially, and post-treatment 25% had normal CT thorax. Hence, we conclude that systemic steroids are helpful in hastening recovery of select subset of long-COVID patients. Simultaneously, we should be cautious of immunosuppressive effects of steroids like tuberculosis reactivation, especially in tuberculosis endemic countries. These findings have therapeutic implications and may serve as guidance for future approach to the management of ‘long-COVID’ with pulmonary sequalae.


2021 ◽  
pp. 17-25
Author(s):  
N. P. Kniajeskaia ◽  
E. H. Anaev ◽  
A. S. Belevskiy ◽  
A. A. Kameleva ◽  
E. V. Safoshkina ◽  
...  

Clinical and molecular heterogeneity of bronchial asthma has been documented in recent years. The search for novel solutions to enhance efficient patient support related first of all to understanding of asthma heterogenic nature and allows to personalize each patient treatment. Biological therapy application can influence to achieve better control at greater extent for patients with severe uncontrolled asthma. Nowadays 5 biological drugs are registered on Russian Federation territory and implemented according to severe asthma phenotypes: anti-IgE, anti-IL-4,13 and anti-IL-5 class therapies. Omalizumab become the first target drug for uncontrolled allergic asthma patients (monoclonal antibody against IgE). This medication is prescribed for uncontrolled moderate and severe allergic (atopic) asthma in patients on basic asthma therapy according GINA step 4 and 5 (Level of evidence A). Clinical trials confidently reported that anti- IgE-therapy reduces the rate of asthma exacerbations, severity of disease in patients with chronic severe asthma on high doses of inhaled steroids or systemic steroids and allows to reduce or withdraw systemic steroids doses in case of steroid-dependent asthma. For the last years special attention led to and demonstrated omalizumab positive effect on airways remodeling and modification of bronchial asthma natural course in adults and children. Antiinflammatory effect of omalizumab is documented. Omalizumab significantly reduces eosinophilic infiltration of submucosal bronchi layer among patients with atopic asthma, sputum eosinophilia, which correlates with reduction of FeNO during biologic treatment, reduces mast cells infiltration of smooth muscle cells in bronchi. Omalizumab significantly reduces the thickness of the bronchial wall, increases the lumen of the bronchi (positive dynamics of CT-scan parameters), which is clinically manifested by increased of FEV1.


Author(s):  
Celine Miyazaki ◽  
Yukata Ishii ◽  
Natalia M. Stelmaszuk

Abstract Introduction/objectives Since new consensus on polymyositis (PM) and dermatomyositis (DM) were released in Japan, an updated evidence on treatment landscape and PM/DM burden was essential. This study evaluates treatment burden and overall treatment cost of PM/DM-related inpatient and outpatient visits, treatments, and procedures/patient/year. Method This retrospective, observational study analyzed insurance claims from Japan Medical Data Center (JMDC) database. Patients with at least one PM/DM diagnosis/one dispensation of treatment between 1 January 2009 and 31 December 2019 were enrolled. Patient characteristics, treatment patterns and sequence, treatment choices, healthcare resource utilization (HCRU), and related costs were assessed. Chi-square test and linear regression model were used to assess impact of patient characteristics on treatment choice. Results Patients (836/4,961) receiving a relevant treatment were analyzed. Heart disease (35%), interstitial lung disease (27%), and diabetes mellitus (26%) were frequently identified as comorbidities. Concomitant dispensation of immunosuppressants and systemic steroids was largely found in first and second line of treatment (LoT) while systemic steroids remained as single dominant treatment across all LoTs. HCRU was very low for inpatient visits (0.68 [1.43]) or rehabilitation (4.74 [14.57]). The mean (SD) number of inpatient visits decreased from first (1.23 [2.32]) to third year (0.11 [0.54]). Total mean (SD) healthcare cost per patients per year was ¥ 3,815,912 (7,412,241), with overall drug dispensation compounding to 80% of total cost. Conclusions High concomitant immunosuppressant and systemic steroid prescriptions in first LoT recommend early optimal treatment to manage PM/DM. Although inpatient costs are low, outpatient dispensation costs increase overall economic burden. Key Points• Japan faces treatment challenges in the prognosis of polymyositis (PM) and dermatomyositis (DM) and thus, we assessed the real-world treatment landscape, practice, patterns, and healthcare resource utilization as an evidence to support healthcare outcome improvement and treatment burden reduction.• Systemic steroids were the dominant preferred choice of treatment, but it increases the overall cost of the treatment due to the resultant comorbidities considering possible side-effects promotion.• Thus, an increased awareness towards the disease management among patients and medical doctors is required for better management goals based on this real-world practice evidence.


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