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Frontiers in Immunology 2024A 54-year-old Japanese man presented with headache and fever the day after SARS-CoV-2 vaccination. He became deeply unconscious within a week. Brain MRI showed...
A 54-year-old Japanese man presented with headache and fever the day after SARS-CoV-2 vaccination. He became deeply unconscious within a week. Brain MRI showed periventricular linear enhancements and a few spotty lesions in the cerebral white matter. Cerebrospinal fluid (CSF) testing showed mild pleocytosis. He was treated with intravenous methylprednisolone and plasma exchange. However, the white matter lesions enlarged to involve the brainstem and cerebellum, and long cord spinal lesions appeared. Anti-glial fibrillary acidic protein (GFAP) antibody was positive in the CSF and serum, and he was therefore diagnosed as autoimmune GFAP-astrocytopathy (GFAP-A). In addition, high-dose immunoglobulin therapy was administered twice, but his symptoms did not improve; the white matter lesions enlarged further, and modified Rankin Scale score increased to 5. A brain biopsy specimen showed infiltration of macrophages and CD lymphocytes together with neuron and oligodendrocytic injuries and glial scar. Although GFAP-A generally responds well to steroids, the present case developed GFAP-A following SARS-CoV-2 vaccination, with refractory to intensive immunosuppressive therapy and atypical pathologic findings of infiltration of CD lymphocytes and demyelination.
Topics: Humans; Male; Middle Aged; Glial Fibrillary Acidic Protein; COVID-19; SARS-CoV-2; Immunosuppressive Agents; Astrocytes; COVID-19 Vaccines; Autoantibodies; Vaccination; Brain
PubMed: 38665914
DOI: 10.3389/fimmu.2024.1361685 -
Acute Medicine & Surgery 2024Nitrogen dioxide (NO) is known to cause lung injury, but there is no established treatment for acute respiratory distress syndrome (ARDS) caused by NO inhalation.
BACKGROUND
Nitrogen dioxide (NO) is known to cause lung injury, but there is no established treatment for acute respiratory distress syndrome (ARDS) caused by NO inhalation.
CASE PRESENTATION
A 35-year-old man was accidentally exposed to NO fumes and presented to the emergency department. On admission, his oxygen saturation was 87% on ambient air and he was diagnosed with ARDS caused by NO inhalation and immediately intubated; however, hypoxemia and hypercapnia were not ameliorated. Hence, veno-venous extracorporeal membrane oxygenation (V-V ECMO) was introduced and the ventilator settings were set for lung-protective ventilation. Methylprednisolone was also administered. After the initiation of these treatments, oxygenation gradually improved. Therefore, ECMO was weaned off on day 11 and he was extubated on day 12.
CONCLUSION
Lung injury caused by NO inhalation can cause ARDS, and lung-protective ventilation with V-V ECMO induction, as well as glucocorticoid administration, may be effective for this condition.
PubMed: 38665593
DOI: 10.1002/ams2.957 -
BMC Medicine Apr 2024There is an urgent unmet need for effective initial treatment for acute graft-versus-host disease (aGVHD) adding to the standard first-line therapy with corticosteroids... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
There is an urgent unmet need for effective initial treatment for acute graft-versus-host disease (aGVHD) adding to the standard first-line therapy with corticosteroids after allogeneic haematopoietic stem cell transplantation (allo-HSCT).
METHODS
We performed a multicentre, open-label, randomized, phase 3 study. Eligible patients (aged 15 years or older, had received allo-HSCT for a haematological malignancy, developed aGVHD, and received no previous therapies for aGVHD) were randomly assigned (1:1) to receive either 5 mg/m MTX on Days 1, 3, or 8 and then combined with corticosteroids or corticosteroids alone weekly.
RESULTS
The primary endpoint was the overall response rate (ORR) on Day 10. A total of 157 patients were randomly assigned to receive either MTX plus corticosteroids (n = 78; MTX group) or corticosteroids alone (n = 79; control group). The Day 10 ORR was 97% for the MTX group and 81% for the control group (p = .005). Among patients with mild aGVHD, the Day 10 ORR was 100% for the MTX group and 86% for the control group (p = .001). The 1-year estimated failure-free survival was 69% for the MTX group and 41% for the control group (p = .002). There were no differences in treatment-related adverse events between the two groups.
CONCLUSIONS
In conclusion, mini-dose MTX combined with corticosteroids can significantly improve the ORR in patients with aGVHD and is well tolerated, although it did not achieve the prespecified 20% improvement with the addition of MTX.
TRIAL REGISTRATION
The trial was registered with clinicaltrials.gov (NCT04960644).
Topics: Humans; Graft vs Host Disease; Female; Male; Methotrexate; Middle Aged; Adult; Methylprednisolone; Hematopoietic Stem Cell Transplantation; Young Adult; Treatment Outcome; Drug Therapy, Combination; Aged; Adolescent; Acute Disease
PubMed: 38664766
DOI: 10.1186/s12916-024-03395-y -
World Journal of Clinical Cases Apr 2024Syphilis is an infectious disease caused by that can invade the central nervous system, causing encephalitis. Few cases of anti-N-methyl-D-aspartate receptor autoimmune...
BACKGROUND
Syphilis is an infectious disease caused by that can invade the central nervous system, causing encephalitis. Few cases of anti-N-methyl-D-aspartate receptor autoimmune encephalitis (AE) secondary to neurosyphilis have been reported. We report a neurosyphilis patient with anti-γ-aminobutyric acid-B receptor (GABAR) AE.
CASE SUMMARY
A young man in his 30s who presented with acute epileptic status was admitted to a local hospital. He was diagnosed with neurosyphilis, according to serum and cerebrospinal fluid (CSF) tests for syphilis. After 14 d of antiepileptic treatment and anti- therapy with penicillin, epilepsy was controlled but serious cognitive impairment, behavioral, and serious psychiatric symptoms were observed. He was then transferred to our hospital. The Mini-Mental State Examination (MMSE) crude test results showed only 2 points. Cranial magnetic resonance imaging revealed significant cerebral atrophy and multiple fluid-attenuated inversion recovery high signals in the white matter surrounding both lateral ventricles, left amygdala and bilateral thalami. Anti-GABAR antibodies were discovered in CSF (1:3.2) and serum (1:100). The patient was diagnosed with neurosyphilis complicated by anti-GABAR AE, and received methylprednisolone and penicillin. Following treatment, his mental symptoms were alleviated. Cognitive impairment was significantly improved, with a MMSE of 8 points. Serum anti-GABAR antibody titer decreased to 1:32. The patient received methylprednisolone and penicillin after discharge. Three months later, the patient's condition was stable, but the serum anti-GABAR antibody titer was 1:100.
CONCLUSION
This patient with neurosyphilis combined with anti-GABAR encephalitis benefited from immunotherapy.
PubMed: 38660543
DOI: 10.12998/wjcc.v12.i11.1960 -
Respirology Case Reports Apr 2024A 65-year-old man presented with intermittent fever and progressive shortness of breath. He responded poorly to antibiotics and corticosteroids (methylprednisolone...
A 65-year-old man presented with intermittent fever and progressive shortness of breath. He responded poorly to antibiotics and corticosteroids (methylprednisolone 40 mg/d). Chest computed tomography scans showed diffuse consolidations and ground glass density patchy opacities in both lungs and these lesions progressed rapidly. The diagnosis of acute fibrinous and organizing pneumonia (AFOP) was confirmed through transbronchial cryobiopsy. This patient had prostate cancer with bone metastasis for 4 months and took the anti-prostate cancer medications including apalutamide and leuprorelin acetate. Considering his medication history, the patient was diagnosed with AFOP induced by anti-prostate cancer medications through panel discussion of multidisciplinary teams. Intravenous methylprednisolone of 500 mg/day was administered for 3 days and then slowly tapered. The patient's shortness of breath gradually subsided. In addition, the lesions in the lungs improved significantly on follow up imaging. AFOP induced by anti-prostate cancer medications is rare. To our knowledge, this is the first reported case and high-dose glucocorticoid treatment may be required in some of these cases.
PubMed: 38660340
DOI: 10.1002/rcr2.1357 -
Internal Medicine (Tokyo, Japan) Apr 2024A 68-year-old woman was admitted to our hospital because of a rapid progression of renal dysfunction with positive myeloperoxidase antineutrophil cytoplasmic antibody...
Successful treatment for life threatening recurrent non-traumatic rectus sheath hematoma in a case with microscopic polyangiitis with rapidly progressive glomerulonephritis.
A 68-year-old woman was admitted to our hospital because of a rapid progression of renal dysfunction with positive myeloperoxidase antineutrophil cytoplasmic antibody and was diagnosed with rapidly progressive glomerulonephritis associated with microscopic polyangiitis (MPA). Severe right rectus sheath hematoma (RSH) bleeding from the inferior epigastric artery developed after starting hemodialysis, which required 4 transarterial embolizations due to recurrent bleeding. After additional treatment with methylprednisolone pulse therapy and rituximab, no rebleeding occurred. Although the giant hematoma reached the pelvis, it shrank spontaneously without any intervention. Nontraumatic RSH should therefore be considered when treating patients with multiple risk factors.
PubMed: 38658341
DOI: 10.2169/internalmedicine.3239-23 -
Qatar Medical Journal 2024Previous studies have delineated different neurological manifestations associated with coronavirus disease 2019 (COVID-19). Myelitis is identified as a rare neurological...
BACKGROUND
Previous studies have delineated different neurological manifestations associated with coronavirus disease 2019 (COVID-19). Myelitis is identified as a rare neurological complication resulting from a COVID-19 infection. Limited information is available regarding the treatment of patients experiencing this condition.
CASE REPORT
This report extracts data from the medical record of a post-COVID-19 myelitis patient at Buriram Hospital and follows up prospectively on the patient's symptoms after treatment. A 61-year-old man, previously vaccinated for COVID-19 and with a history of hypertension and dyslipidemia, experienced progressive bilateral lower-extremity weakness (recorded as muscle strength grade 2/5 in both lower extremities) for 6 weeks. He had a mild case of COVID-19 2 months earlier, which resolved in 10 days without specific treatment. However, 2 weeks after being diagnosed with COVID-19, he developed weakness in his lower limbs, numbness below the nipple, and urinary retention. Spinal magnetic resonance imaging revealed multifocal longitudinal myelitis. Despite initial treatment with methylprednisolone, the patient showed no clinical improvement. Consequently, he underwent five cycles of plasmapheresis. Three months after discharge, a notable improvement was observed, with his muscle strength graded at 4/5 in both lower extremities and the resolution of sensory and urinary symptoms.
CONCLUSIONS
We presented the case of a COVID-19-vaccinated patient, in whom COVID-19 infection might have led to myelitis. We found promising results in treating prolonged COVID-19-related myelitis symptoms through the use of plasmapheresis.
PubMed: 38654819
DOI: 10.5339/qmj.2024.19 -
Orphanet Journal of Rare Diseases Apr 2024Multisystem childhood Langerhans cell histiocytosis (LCH) patients, especially those with risk organ (RO) involved, had not been satisfactorily treated under the...
BACKGROUND
Multisystem childhood Langerhans cell histiocytosis (LCH) patients, especially those with risk organ (RO) involved, had not been satisfactorily treated under the international traditional schemes as high incidences of reactivation with late sequelae were largely reported. Over years, we have observed that LCH patients with varied clinical symptoms responded differently to different drugs, suggesting the current grouping strategies based only on the number of organs involved might be inadequate. LCH has been defined as an inflammatory myeloid tumor, thus this study has innovatively divided LCH pediatric patients into inflammatory or malignant symptoms group, and given different intensity treatment regimens to different groups.
AIM
This clinical study aimed to explore a more appropriate patient grouping system according to the LCH symptom presentations and examine the clinical outcomes of treatment strategies in different groups.
METHODS
According to the clinical manifestations, 37 cases of children were divided into Group A (only inflammatory symptoms) and Group B (malignant symptoms with or without inflammatory symptoms). Patients in Group A and B were initially treated with vindesine (VDS) and methylprednisolone (PSL), and VDS, PSL, pirarubicin (THP) and cyclophosphamide (CTX), respectively. Treatment responses were evaluated six weeks after the induction therapy in all patients, and the criteria were disease status and clinical scores of symptoms.
RESULTS
Pre- and post-treatment scores were 1.22 ± 0.547 and 0.00 ± 0.00 in Group A, and 14.79 ± 1.686 and 1.00 ± 1.563 in Group B, respectively. All patients had subsequentlly received maintenance therapy without progressive disease. The 4-year overall survival (OS) rate was 100% in both groups and the 4-year event-free survival (EFS) was 94.4% in Group A and 89.5% in Group B, respectively. There were no obvious adverse events (AE) in Group A, whereas the main AE in Group B were alopecia and non-lethal hematological toxicity.
CONCLUSION
Stratification according to patients' clinical symptoms, with low-intensity treatment for inflammatory symptoms (mild manifestations) and intensive treatment with multiple drugs for malignant symptoms (severe manifestations), is a positive exploration that simplifies stratification method, achieves good long-term remission of the disease, and obtains a higher survival rate and quality of life, which seemed to be more appropriate for LCH patients.
Topics: Humans; Histiocytosis, Langerhans-Cell; Female; Male; Pilot Projects; Child, Preschool; Child; Infant; Inflammation; Adolescent
PubMed: 38654381
DOI: 10.1186/s13023-024-03151-8 -
Frontiers in Neurology 2024There is growing evidence that severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) or COVID-19 infection is associated with the development of immune mediated...
BACKGROUND
There is growing evidence that severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) or COVID-19 infection is associated with the development of immune mediated neuropathies like chronic inflammatory demyelinating polyneuropathy (CIDP), but the impact of SARS-CoV-2 vaccination and COVID-19 infection on genetic disorders such as Charcot-MarieTooth (CMT) remains unclear.
CASE PRESENTATION
A 42-year-old male with occulted CMT neuropathy type lA (CMT1A) who developed limb numbness and weakness after the second SARS-CoV-2-vaccination was confirmed by identifying characteristic repeats in the p11.2 region of chromosome 17. Due to the progressive deterioration of muscle strength over 8 weeks, limb atrophy, moderately elevated protein counts in the cerebrospinal fluid, and significant improvement with intravenous human immunoglobulin, which were characteristic of acquired inflammatory neuropathies, he was eventually diagnosed with CIDP superimposed on CMT1A. However, after a three-month plateau, the patient contracted COVID-19, which led to repeated and worsening symptoms of limb weakness and atrophy, thus was diagnosed with a recurrence of CIDP and treated with Intravenous immunoglobulin and methylprednisolone 500 mg/d for 5 consecutive days, followed by oral prednisone and mycophenolate mofetil tablets. On 2 month follow-up, he exhibited remarkable clinical improvement and could walk independently with rocking gait. After 1 year of follow-up, the patient's condition was stable without further change.
CONCLUSION
Our case indicates that CMT1A can deteriorate after SARS-CoV-2 vaccination. Thus, SARS-CoV-2 vaccination should be considered a potential predisposing factor for CMT1A worsening. The possible superposition of CMTIA and CIDP in the context of SARS-CoV-2 infection or immunity suggests that any clinical exacerbation in patients with CMT1A should be carefully evaluated to rule out treatable superposition inflammation. In addition, electrophysiological and imaging examination of the proximal nerves, such as the axillary nerve, is helpful for the diagnosis of CIDP.
PubMed: 38651106
DOI: 10.3389/fneur.2024.1358881 -
Infection and Drug Resistance 2024Hemophagocytic lymphohistiocytosis (HLH), whether primary or secondary, is a rare and fatal clinical syndrome of uncontrolled immune activation and inflammatory cascade....
Hemophagocytic lymphohistiocytosis (HLH), whether primary or secondary, is a rare and fatal clinical syndrome of uncontrolled immune activation and inflammatory cascade. Immune checkpoint inhibitors (ICIs) induced HLH has no standard diagnostic and treatment guidelines. Early diagnosis and appropriate treatment according to different disease backgrounds are crucial. Herein, we first report 2 cases of patients with chronic active Epstein-Barr virus infection (CAEBV) who developed HLH after the use of sintilimab, a monoclonal antibody against programmed cell death protein 1 (PD-1), and the DEP (liposomal doxorubicin, etoposide, methylprednisolone) chemotherapy regimen in combination with ruxolitinib were used to successfully control the disease.
PubMed: 38650754
DOI: 10.2147/IDR.S441460