Severe TAFRO Syndrome Mimicking Hepatorenal Syndrome Successfully Treated with a Multidisciplinary Approach: A Case Report and Literature Review
Shinya YamamotoKen WellsKeisuke MoritaKatsuya TanigakiKoji MuroMinami MatsumotoHirotsugu NakaiYasuyuki AraiShuji AkizukiKen TakahashiSachiko MinamiguchiShingo FukumaMotoko Yanagita
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Abstract:
Finding the ideal balance between efficacy and safety of immunosuppression is challenging, particularly in cases of severe TAFRO syndrome. We herein report a 60-year-old man diagnosed with grade 5 TAFRO syndrome mimicking hepatorenal syndrome that was successfully treated by glucocorticoid, tocilizumab, and cyclosporin despite virus infection. Furthermore, by examining 14 peer-reviewed remission cases, we revealed that the recovery periods among inflammation, renal dysfunction, and thrombocytopenia were quite different, with recovery from thrombocytopenia notably slow. All patients requiring dialysis were successfully withdrawn from dialysis, and the reversibility from kidney injury was good. This clinical information will help clinicians plan treatments and tailor the intensity of immunosuppression.Keywords:
Tocilizumab
Immunosuppression
Introduction: The aim of this study was to analyse the impact of tacrolimus-based immunosuppression on outcome after heart transplantation with special regard towards long-term side effects, since our center has experience with tacrolimus after HTx since 1993.
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Posttransplantation lymphoproliferative disorder (PTLD) is a well-described complication of the systemic immunosuppression required for successful organ transplantation. Lesions of PTLD often occur in the region of the head and neck and require otolaryngologic evaluations. Although the majority of reported cases of PTLD are associated with cyclosporine immunosuppression, recently, PTLD has been described in patients treated solely with the newer systemic immunosuppressive agent tacrolimus (FK 506). As an introduction to tacrolimus and to PTLD as one of its complications, a case of PTLD presenting as airway obstruction in a child treated solely with tacrolimus immunosuppression is described. In addition, a review of tacrolimus and PTLD in patients under tacrolimus immunosuppression is presented to familiarize the otolaryngologist with this important new immunosuppressive agent and a potential complication of its use. (Arch Otolaryngol Head Neck Surg 1995;121:1037-1041)
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Background and Objectives: Older adults are more susceptible to coronavirus disease 2019 (COVID-19). Interleukin-6 (IL-6) is an important cytokine in the cytokine release syndrome (CRS), and tocilizumab blocks the IL-6 receptor. The objective is to analyze the effect of tocilizumab on CRS in older patients with severe COVID-19. Materials and Methods: Between February 10 and March 21, 2020, a total of 19 patients aged ≥60 years with severe or critical COVID-19 met the study inclusion criteria at the Tongji Hospital in Wuhan City, Hubei Province, China. The patients were divided into two groups: the tocilizumab group, with IL-6 levels, which exceeded the upper limit of normal by >10-fold and non-tocilizumab group. Results: Patients in the tocilizumab group were older (73.20 ± 4.44 vs. 66.21 ± 5.06 years, P = 0.014), had lower lymphocyte counts (0.71 ± 0.18 vs. 1.18 ± 0.59 × 109/L, P = 0.016), and higher high-sensitivity C-reactive protein (hsCRP) levels (94.04 ± 57.24 vs. 51.65 ± 45.37 mg/L, P = 0.035). Increases in ferritin (FER) and hsCRP levels in patients in the tocilizumab group were marked. Except for one patient who died, IL-6, FER, hsCRP levels, and the neutrophil/lymphocyte ratio in the remaining four patients decreased following treatment with tocilizumab. Tocilizumab did not cause any serious adverse reactions. There were no differences in mortality, days until lung computerized tomography improvement, or renal function between the two groups. The total mortality rate was 10.53%. Conclusions: Our results support the therapeutic efficacy and safety of tocilizumab in older patients with severe COVID-19.
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Introduction: Based on the known relevance of IL-6 in juvenile idiopathic arthritis (JIA) pathophysiology, tocilizumab has been investigated and approved for use in the treatment of systemic and polyarticular JIA. Tocilizumab is a humanized monoclonal antibody that inhibits signaling through both membrane bound and soluble IL-6R.Areas Covered: The purpose of this article is to summarize the available clinical data on the efficacy and safety of tocilizumab in JIA and to provide our opinion on the place of tocilizumab in current treatment regimens. A literature search for all relevant clinical trials and studies with regards to tocilizumab, JIA and IL-6 was performed through PubMed, in addition to a review of recent conference abstracts.Expert Opinion: Tocilizumab has an important and evolving role in controlling disease activity in patients with JIA. It has proven useful even in patients whose JIA has previously been refractory to other biologic disease modifying anti-rheumatic drugs (DMARDs) and also appears quite effective as monotherapy. Tocilizumab is relatively well tolerated amongst JIA patients, with systemic JIA patients experiencing more serious adverse events overall.
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Abstract Background/Aims Giant cell arteritis (GCA) has been revolutionised by fast track pathways that ensure early secure diagnosis. The GiACTA study provided evidence for the beneficial role of tocilizumab in the treatment of GCA and encouraged us all to reconsider the role of corticosteroids. These data relate to real life experience of the use of tocilizumab, including current status of patients after completing the initial year of treatment. In Scotland, tocilizumab was initially used for patients with relapsing disease or steroid complications. However, since 2018, the Scottish Medicines Consortium has supported use from disease onset and it has become increasingly common to consider adding at diagnosis. Methods The case notes of 23 patients treated with tocilizumab were reviewed back to 2017. Patient demographics were collated including whether cranial (C) or large vessel vasculitis (LVV) variant. The diagnosis of GCA had to be confirmed by biopsy or imaging with GCA rheumatology clinic review. Any patients who discontinued tocilizumab prior to completing one year had the reason recorded. Duration on tocilizumab was intended to be one year. Data were reviewed to capture outcomes after treatment was withdrawn. Results 2 patients with non-relapsing disease discontinued tocilizumab within the first month due to early relative neutropenia (1.4). 5 of 21 patients who completed treatment have since had additional tocilizumab treatment. 1 patient with particularly severe relapsing disease and steroid complications remains on long term tapered tocilizumab monotherapy without attempt to stop (C and LVV). 4 patients (LVV=C) flared within 1-9 months (median 6 months) and resumed with steroids and subsequent tapers; all had commenced for relapsing disease. 1 patient (C), commenced on tocilizumab at diagnosis, opted for MTX when flared. 15 remaining patients are on no steroid, methotrexate or tocilizumab and are free of disease activity as yet, but undergoing monitoring for flare (12C 3 LVV variant). Conclusion Tocilizumab is effective, limits steroid exposure and is well-tolerated by patients. The most common reason for discontinuing tocilizumab was relative neutropenia (neutrophils 1-1.4). The majority of patients discontinued tocilizumab and have not yet flared off all treatment. Cranial variant was less associated with relapse after tocilizumab treatment versus LVV. New diagnoses appear less prone to relapse off tocilizumab than patients already known to have relapsing disease prior to using tocilizumab. Patients discontinuing tocilizumab benefit from ongoing monitoring to recognise the early stages of returning disease; this would need to be provided for at least one year. Patients discontinuing treatment, their GPs and carers need advice on routes to escalate concerns about returning symptoms. Fast track services need to provide a flare service in addition to a diagnosis pathway if there is to be safety, confidence and expert support for this patient group and primary care colleagues. Disclosure L.M.M. Hutton: Other; 2022 Roche educational grant to attend BSR.
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Results Forty-two SJIA patients, 131 blood samplings were included in this study. Seventeen patients (40%) were treated with tocilizumab during the study. Serum IL-6 levels in patients without tocilizumab treatment significantly elevated in active disease with systemic features and arthritis [median (IQR) = 101.8 (303.2) pg/mL] when compared to active disease with only arthritis [median (IQR) = 4.5 (23) pg/mL], and remission on medication [median (IQR) = 1.5 (0.55) pg/mL], whereas serum IL-6 levels in patients with tocilizumab treatment were not different between groups but there were significantly different when compared to healthy children (p < 0.05). In addition, the correlation between serum IL-6 levels and JADAS-71 in patients without tocilizumab treatment (r = 0.71, p < 0.001) was stronger than patients with tocilizumab treatment (r = 0.42, p = 0.01). Serum sIL-6R levels in SJIA patients with and without tocilizumab treatment were significantly higher when compared to healthy children (p < 0.05). Interestingly, in patients with tocilizumab treatment, serum sIL-6R levels were extremely higher [median (IQR) = 1,110.3 (840.2) ng/mL] than patients without tocilizumab treatment [median (IQR) = 94.2 (82.7) ng/mL]. Conclusion The correlation between serum IL-6 levels and disease activity in patients without tocilizumab treatment was stronger than patients with tocilizumab treatment. In addition, serum sIL-6R levels in patients with tocilizumab treatment were extremely higher than patients without tocilizumab treatment.
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Clinical and Radiologic Improvement Following Tocilizumab Administration in Patients With SARS-CoV-2
Cytokine release syndrome and acute respiratory distress syndrome are the major complications of coronavirus disease 2019 (COVID-19) and tocilizumab has shown efficacy in this setting. We report on 2 severely ill patients with COVID-19 treated with tocilizumab within 7 to 10 days of onset of symptoms. Tocilizumab markedly improved their clinical condition and was associated with regression of abnormalities on chest computed tomography within 1 week of tocilizumab administration.
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Currently, COVID-19 is one of the most pressing healthcare problems across the world. With no definitive pharmacological guidelines, multiple drugs were used to treat critical patients with little success. of Tocilizumab, a monoclonal antibody has shown some role in the treatment of Covid-19 infection. The study was a cross-sectional prospective observational study. It was conducted in the COVID-19 Intensive Care Unit (ICU) of Liaquat National Hospital and Medical College, and National Medical Centre Karachi. The study was conducted from 15th June 2020 to 31st July 2020. Objective of the study was to compare the outcomes of severe to critical COVID-19 patients with established Cytokine release Syndrome (CRS), who received Tocilizumab with the group received Tocilizumab followed by intravenous immunoglobulins (IVIG). Two groups were made with one receiving Tocilizumab alone while the other received IVIG after Tocilizumab. Comparison was then made based on frequency of mortality as well the need of mechanical ventilation and its range of days. In results 4 (15.4%) patients in Tocilizumab only group died while Tocilizumab followed by IVIG treated group had 10 (38.46%) deaths. 20 (76.9%) patients needed mechanical ventilation in Tocilizumab only group while Tocilizumab followed by IVIG treated group consisted of 23 (88.46%) patients. This concludes that the group with only Tocilizumab therapy has better outcome as compare to the group who received both Tocilizumab and intravenous immunoglobulins.
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Neurotoxicity is a well-known side effect of tacrolimus-based immunosuppression after liver transplantation. Until now, only 31 cases of immunosuppression-associated leukoencephalopathy in liver transplant recipients reported in the literature are related to tacrolimus therapy. We report a patient who developed a posterior leukoencephalopathy syndrome, secondary to tacrolimus-based immunosuppression, after living donor liver transplantation. The special features of this case are the sudden and late onset of neurologic symptoms, a persistent comatose state, and increased signal intensity in follow-up MRI.
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