The effect of rituximab on patient reported outcomes in the preclinical phase of rheumatoid arthritis: 2 year data from the PRAIRI study.

OBJECTIVES: Early treatment of individuals at risk of developing rheumatoid arthritis (RA-risk) in the preclinical phase has the potential to positively impact both patients and society by preventing disease onset and improving patients' quality of life. The PRAIRI study was a randomised, double-blind, placebo-controlled trial with the B-cell depleting agent rituximab (RTX), which resulted in a significant delay of arthritis development of up to 12 months in seropositive RA-risk individuals. Here, we report our findings on patient-reported outcomes (PROs) in this study population. METHODS: Seventy-eight RA-risk individuals were treated with one single dose of either placebo (PBO) or 1000 mg RTX plus 100 mg methylprednisolone (MP) and anti-histamines, regardless of treatment allocation, as co-medication. Data on quality of life were collected at baseline and 1, 4, 6, 12 and 24 months using established PRO questionnaires (visual analogue scale (VAS) pain, health assessment questionnaire disability index (HAQ-DI) score, EuroQol five dimension (EQ-5D) and both physical and mental component score of the 36-item short-form heath survey (SF-36)). RESULTS: No significant changes in quality of life over a 2 year follow-up were observed in at-risk individuals treated with RTX compared to PBO given the PRO scores at 24 months (mean difference±SEM: HAQ score=0.07±0.16; EQ-5D=-0.02±0.05; VAS pain=11.11±7.40). Furthermore, no significant effect of treatment on perceived arthritis severity at the time of clinically manifest disease (arthritis) was found. CONCLUSION: One single dose of RTX plus MP administered to RA-risk individuals does not have a meaningful and measurable positive effect on PROs after 2 years of follow-up and/or perceived disease severity at the time of arthritis development. TRIAL REGISTRATION NUMBER: Trial registered at EU Clinical Trial Register, EudraCT Number: 2009-010955-29 (https://www.clinicaltrialsregister.eu/ctr-search/search?query=Prevention+of+RA+by+B+cell+directed+therapy).

A clinical case of pityriasis lichenoides chronica presenting with palpable purpura after streptococcal infection.

Pityriasis lichenoides is a rare inflammatory skin condition presenting with diffuse red-brown papules with evolution polymorphism and mica-like crust on older skin lesions. We present a 60-year-old female patient with pityriasis lichenoides chronica that manifested ten days after streptococcal pharyngitis. Initially, palpable purpura appeared on the lower extremities and later, erythematous-squamous papules and plaques appeared at the site of the palpable purpura and on the upper limbs and trunk. The patient had no history of hematological malignancy, viral hepatitis, kidney involvement, systemic rheumatic disease, or ANCA-associated vasculitis. After administration of methylprednisolone 20 mg for one month and an antimalarial agent (hydroxychloroquine 200 mg, 1 tablet bid) for three months, the skin lesions subsided without recurrence.

Coronavirus-disease-2019-associated Stevens-Johnsons syndrome in a 15-year-old boy: a case report and review of the literature.

BACKGROUND: Stevens-Johnson syndrome (SJS) is a life-threatening condition characterized by high fever and severe mucocutaneous lesions, often triggered by drugs or infection. During the coronavirus disease 2019 pandemic, there was a marked increase in Stevens-Johnson syndrome cases, but relatively few cases were reported in children. The present article reports a pediatric case of Stevens-Johnson syndrome due to coronavirus disease 2019 infection and provides a review of the most relevant literature. CASE PRESENTATION: A previously healthy 15-year-old Han Chinese boy from China presented to the hospital with oral ulcers, conjunctival hyperemia, and widespread maculopapular rash. He had a history of fever 9 days prior and tested positive for coronavirus disease 2019 infection. Upon admission, his rash and mucosal lesions worsened, with the development of blisters on the fingertips of both hands, ocular pain, photophobia, and erosive lesions on the genital mucosa with exudation. He was diagnosed with Stevens-Johnson syndrome and received treatment with methylprednisolone, intravenous immunoglobulin, and dermatological and mucosal care. The patient's condition was managed, and the dosage of high-dose intravenous methylprednisolone was tapered down, followed by a transition to oral prednisolone. He was discharged without sequelae. CONCLUSION: We should be aware that coronavirus disease 2019 infection is associated with the development of Stevens-Johnson syndrome in children and may lead to a wide spectrum of dermatologic presentations. Although Stevens-Johnson syndrome is a relatively rare condition, given its potentially serious consequences, it is crucial to identify it as early as possible and to take appropriate preventive and therapeutic measures to reduce complications and improve the quality of life for patients.

Ruxolitinib plus steroids for acute graft versus host disease: a multicenter, randomized, phase 3 trial.

Newly diagnosed patients with high-risk acute graft-versus-host disease (aGVHD) often experience poor clinical outcomes and low complete remission rates. Ruxolitinib with corticosteroids showed promising efficacy in improving response and failure free survival in our phase I study. This study (ClinicalTrials.gov: NCT04061876) sought to evaluate the safety and effectiveness of combining ruxolitinib (RUX, 5 mg/day) with corticosteroids (1 mg/kg/day methylprednisolone, RUX/steroids combined group) versus using methylprednisolone alone (2 mg/kg/day, steroids-only group). Newly diagnosed patients with intermediate- or high-risk aGVHD were included, with risk levels classified by either the Minnesota aGVHD Risk Score or biomarker assessment. Patients were randomized in a ratio of 1:1 into 2 groups: 99 patients received RUX combined with methylprednisolone, while the other 99 received methylprednisolone alone as the initial treatment. The RUX/steroids group showed a significantly higher overall response rate (ORR) on day 28 (92.9%) compared to the steroids-only group (70.7%, Odds Ratio [OR] = 5.8; 95% Confidence Interval [CI], 2.4-14.0; P < 0.001). Similarly, the ORR on day 56 was higher in the RUX/steroids group (85.9% vs. 46.5%; OR = 7.07; 95% CI, 3.36-15.75; P < 0.001). Additionally, the 18-month failure-free survival was significantly better in the RUX/steroids group (57.2%) compared to the steroids-only group (33.3%; Hazard Ratio = 0.46; 95% CI, 0.31-0.68; P < 0.001). Adverse events (AEs) frequencies were comparable between both groups, with the exception of fewer grade 4 AEs in the RUX/steroids group (26.3% vs. 50.5% P = 0.005). To our knowledge, this study is the first prospective, randomized controlled trial to demonstrate that adding ruxolitinib to the standard methylprednisolone regimen provides an effective and safe first-line treatment for newly diagnosed high-risk acute GVHD.

Severe lupus enteritis: A diagnostic and therapeutic enigma.

Lupus enteritis refers to the gastrointestinal involvement in systemic lupus erythematosus (SLE). It presents with diverse symptoms that frequently overlap with those of other acute abdominal conditions, posing diagnostic challenges. We describe an adolescent female, with lupus pancreatitis and nephritis, who later developed severe lupus enteritis during the course of her illness. She was treated with pulse methylprednisolone and intravenous cyclophosphamide and gradually improved over 3 weeks. Our case highlights the need to consider lupus enteritis in patients with severe pain abdomen and intractable vomiting. Presence of lupus pancreatitis and nephritis are risk factors for development of enteritis.

Recurrent simultaneous central nervous system demyelination with possible peripheral demyelination / nodopathy in a seronegative patient.

Combined central and peripheral demyelination (CCPD) is a rare disease entity. Onset with the simultaneous central nervous system (CNS) and peripheral nervous system (PNS) involvement and its recurrence are exceptional. Anti-neurofascin antibodies have been shown to be present in up to 70% of cases, yet seronegative patients also exist. We present a case of seronegative recurrent CCPD. The PNS involvement was compatible with two episodes of recurrent Guillain-Barre syndrome (GBS), whereas the CNS involvement pattern was not typical for either multiple sclerosis (MS) or acute disseminated encephalomyelitis. The prognosis was excellent with pulse methylprednisolone, intravenous immunoglobulin, and plasmapheresis. This case highlights the varied clinical presentations of CCPD, extending beyond the realms of MS and chronic inflammatory demyelinating polyneuropathy, and underscores the potential for relapse. Importantly, to the best of our knowledge, this represents the inaugural instance of CCPD featuring PNS involvement in the form of recurrent GBS.

A Child with COVID-19 Complicated by Rapidly Progressive Severe Organizing Pneumonia: A Case Report.

A 2-year-old boy tested positive for SARS-CoV-2 and, after 30 days of mild-moderate respiratory symptoms, suddenly deteriorated and required extracorporeal membrane oxygenation. Lung biopsy was performed with findings consistent with organizing pneumonia. He received intensive therapy with high-dose methylprednisolone, intravenous immune globulin, rituximab, and plasmapheresis without improvement. He died after 85 days hospitalization. This case highlights unique presentations of COVID-19 and reaffirms the concept that, while rare in Hawai'i, pediatric COVID-19 is an ongoing problem and that severe, even fatal, disease can occur.

Association of systemic corticosteroid use with prognosis of patients with acute exacerbations of chronic obstructive pulmonary disease in the intensive care unit: a propensity score-matched cohort study.

BACKGROUND: Systemic corticosteroid has been recommended for the treatment of severe acute exacerbations of chronic obstructive pulmonary disease (AECOPD). Little is known about the use of systemic corticosteroid in patients admitted to intensive care units (ICU) since most of previous trials excluded these critically ill patients. METHODS: We conducted a matched cohort study based on the Medical Information Mart in Intensive Care-IV database. Patients with AECOPD in ICUs were included. Patients in the exposure group should be intravenously administrated with methylprednisolone or treated with oral prednisone within 24 h after ICU admission. The propensity score matching and multivariable analyses were used to adjust for covariates. The primary outcome was 28-day mortality, and secondary outcomes included ICU mortality, in-hospital mortality, the duration of ICU stay, and mechanical ventilation. Subgroup analyses for the primary outcome were performed according to age, sex, type of corticosteroid, type of ICU admission, type of mechanical ventilation, and co-morbidities/complications. RESULTS: The entire cohort and the matched cohort included 763 and 412 patients, respectively. In the matched cohort, the use of systemic corticosteroid had no impact on 28-day mortality (OR: 1.00, 95% CI: 0.61-1.64, P = 1.000). The results kept consistent in all subgroups. Additionally, systemic corticosteroid showed no benefits on ICU mortality, in-hospital mortality, the length of ICU stay, and the duration of mechanical ventilation. CONCLUSIONS: The results of this study do not support routine use of systemic corticosteroid in patients with AECOPD admitted to ICUs.

Managing the diagnostic challenge: chronic inflammatory demyelinating polyneuropathy during pregnancy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) during pregnancy presents diagnostic and management challenges. We report a case of a primigravida in her 30s, exhibiting progressive quadriparesis starting in the second trimester. Initially, her symptoms of weakness, numbness and progressive quadriparesis were attributed to vitamin B12 deficiency, leading to the administration of intramuscular methylcobalamin injection. After referral to our tertiary care centre at 37 weeks of pregnancy and after further evaluation, neurological examination revealed findings characteristic of CIDP, which were confirmed by nerve conduction studies showing typical features of the condition. Treatment included intravenous methylprednisolone followed by intravenous immunoglobulin. An elective caesarean section was carried out because the patient was unable to effectively participate in labour due to her condition. The patient exhibited improved limb power postoperatively after immunoglobulin therapy. Challenges include delayed diagnosis, treatment initiation and management decisions. Multidisciplinary collaboration is crucial for optimal outcomes in CIDP during pregnancy.

Systemic lupus erythematosus with podocyte infolding glomerulopathy: A case report and literature review.

RATIONALE: Podocyte infolding glomerulopathy (PIG) is a rare glomerular disease, its diagnosis mainly depends on pathological manifestations of the kidney. Few clinical cases of PIG have been reported, but it is sometimes associated with connective tissue diseases. Here we describe a case of systemic lupus erythematosus (SLE) with PIG and undertake a review of the literature. PATIENT CONCERNS: A 34-year-old female patient was admitted to our hospital in August 2019 with repeated facial erythema and proteinuria for more than 10 years. The patient was previously diagnosed with SLE. DIAGNOSIS: Systemic lupus erythematosus. INTERVENTIONS: Renal biopsy was performed to investigate ongoing proteinuria and the results were consistent with PIG. Treatment with methylprednisolone, hydroxychloroquine sulfate, mycophenolate mofetil, and candesartan ester. OUTCOMES: Improved the patient's condition and resolved the proteinuria. LESSONS: This study reported a case of PIG and SLE. The patient was diagnosed according to biopsy, and the disease remain stable after immunosuppressive therapy. It is recommended to carefully study renal biopsies from patients with proteinuria and underlying autoimmune diseases to identify additional cases.

ANODYNE study: A double-blind randomized trial of greater occipital nerve block of methylprednisolone and lignocaine versus placebo as a transitional preventive treatment for episodic cluster headache.

BACKGROUND: There is inadequate evidence of the efficacy of greater occipital nerve block (GONB) for the preventive treatment of cluster headaches. We assessed the efficacy and tolerability of GONB injections as a transitional preventive treatment for episodic cluster headaches (ECH). METHODS: This randomized, double-blind, placebo-controlled, parallel-group trial conducted at GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India, included ECH patients diagnosed by ICHD-3 criteria, aged 18-65, with one or more attacks per 24 h for seven days before randomization (baseline). ECH patients were either not on preventive medications or on stable doses for at least three months. ECH patients were randomized to receive active GONB (2 ml methylprednisolone (80 mg) and 2 ml lignocaine (2%)) and placebo (4 ml saline injections). Before giving GONB, lignocaine jelly was applied topically to mask the effect of numbness following the GONB. The primary efficacy endpoint was the mean change in weekly attack frequency from baseline to Week 4. Efficacy analyses were performed in a modified intention-to-treat population that included all patients who received at least one injection of GONB and had a follow-up for one week following GONB. The safety analysis included treatment-emergent adverse effects (TEAE) in all patients who received at least one dose of investigational product. The trial was registered with the Clinical Trials Registry of India (CTRI/2021/21/038397). RESULTS: Forty ECH patients were randomized between December 2021 and January 2023. Thirty-nine patients (19 in the active and 20 in the placebo groups) were available for efficacy analysis. The change in weekly attack frequency from baseline to Week 4 was -11.1 (95% CI: -8.5 to -4.4) for the active group compared to -7.7 (95% CI: -11.8 to -9.8) for placebo (mean difference -3.4 (95% CI: -5.2 to -1.7, p < 0.001). We noted TEAE in 18 (90%) of 20 patients who received the active drug and in 18 (90%) of 20 patients who received a placebo (p = 0.38). The common TEAE were local site bleeding and pain, which were mild and transient. No serious adverse events were reported. CONCLUSION: This study found that GONB with methylprednisolone and lignocaine significantly reduced the weekly attack frequency from baseline to Week 1 through Week 4 in ECH patients compared to a placebo. GONB was well tolerated.

Case report: Bullous pemphigoid combined with Sjögren's syndrome complicated by central nervous system infection.

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease in humans, characterized by tense blisters, erosions, urticarial lesions, and itching on normal or erythematous skin. Many autoimmune diseases are considered comorbidities of BP, but clinical case reports of BP complicated by Sjögren's syndrome are very scarce. Furthermore, cases of central nervous system infection secondary to both autoimmune diseases are even rarer. Case presentation: We report a 74-year-old woman diagnosed with bullous pemphigoid, who showed relief of active lesions after treatment with methylprednisolone and dupilumab injections. However, she was admitted for pulmonary infection during which she was diagnosed with Sjögren's syndrome (SS). Subsequently, the patient developed altered consciousness, indicating a central nervous system infection. Adjustment of steroid dosage and aggressive antimicrobial therapy led to alleviation of symptoms. Conclusion: The coexistence of autoimmune subepidermal blistering diseases and SS is rare. The role of SS in the pathogenesis of skin lesions is unclear, and the relationship between these blistering diseases and SS remains elusive. Further research is needed to determine whether there are common pathological mechanisms between the two conditions.

COVID-19-associated autoimmune encephalitis: A case report and literature review.

RATIONALE: This article reports a case of coronavirus disease (COVID-19)-associated autoimmune encephalitis (AE) and reviews the relevant literature to investigate the clinical manifestations, auxiliary inspection, diagnosis and treatment, and prognosis of AE associated with COVID-19. PATIENT CONCERNS: A 68-year-old female with fatigue developed altered consciousness after 2 days of fever, thereafter testing positive for COVID-19. The protein levels in the lumbar puncture cerebrospinal fluid were elevated, and cranial magnetic resonance imaging (MRI) scan indicated T2-weighted hyperintensity in the temporal lobe. DIAGNOSES: The patient was diagnosed with COVID-19-associated AE. INTERVENTIONS: After admission, the patient received pulse steroid therapy with methylprednisolone. Additionally, gastric protection, blood glucose control, nutritional support, and other treatments were administered. OUTCOMES: The symptoms were significantly relieved by steroid pulse therapy. At the 3-month follow-up, the patient had recovered completely without any obvious discomfort. LESSONS: The possibility of AE should be considered if neurological symptoms occur a few days after infection with COVID-19, with early diagnosis and immediate steroid pulse therapy resulting in better outcomes.

An integrated long-acting implant of clinical safe cells, drug and biomaterials effectively promotes spinal cord repair and restores motor functions.

Spinal cord injury (SCI) is incurable and raises growing concerns. The main barrier to nerve repair is the complicated inhibitory microenvironment, where single-targeted strategies are largely frustrated. Despite the progress in combinatory therapeutic systems, the development and translation of effective therapies remain a challenge with extremely limited clinical materials. In this study, mesenchymal stem cells are transplanted in combination with sustained release of methylprednisolone through delivery in one composite matrix of a microsphere-enveloped adhesive hydrogel. All the materials used, including the stem cells, drug, and the matrix polymers gelatin and hyaluronan, are clinically approved. The therapeutic effects and safety issues are evaluated on rat and canine SCI models. The implantation significantly promotes functional restoration and nerve repair in a severe long-span rat spinal cord transection model. Distant spinal cord segments and the urinary system are effectively protected against pathologic damage. Moreover, the local sustained drug delivery mitigates the inflammatory microenvironment when overcoming the clinical issue of systemic side effects. The study presents an innovative strategy to achieve safe and efficient combinatory treatment of SCI.

Evaluation of the Effect of Early-Onset Steroid Treatment in the COVID-19-Positive Pregnant Women on Pregnancy Outcomes.

OBJECTIVE: Coronavirus disease 2019 (COVID-19) is the disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Acute respiratory distress and preterm delivery are the two major complications induced by SARS-CoV-2 infection during pregnancy. In the presence of dyspnea, the use of systemic corticosteroids was recommended in pregnant and non-pregnant groups. Our primary aim was to investigate the effect of early-onset steroid treatment on mortality and adverse effects in pregnant women with COVID-19. Our secondary aim was to investigate the effect of steroid treatment on the length of hospital stay and intensive care unit (ICU) stay, and duration of treatment. The study also investigated infection, preterm birth, and ideal body weight (lbw) in newborns. METHODS: In this retrospective study, 253 patients were divided into three groups according to steroid administration. In Group 1 patients (n:112), treatment was started at the time of hospitalization. In Group 2 patients (n:90), treatment was started at least 24 h after hospitalization. Group 3 consisted of patients (n:51) who did not receive steroid treatment. Methylprednisolone (32 mg/day) was given to pregnant patients with a gestational age below 24 weeks or above 34 weeks, and dexametazone (6 mg/day) was given in four doses followed by 32 mg/day methylprednisolone for the others (whose baby was at a gestational age of 24 weeks and above but less than 34 weeks). RESULT: The hospital stay, ICU stay, and steroid administration time were significantly lower in the Group 1 when compared to the others (p < 0.05). The steroid treatment requirement was 4.4 days in Group 1 and 5.7 days in Group 2 (p < 0.05). While no death was observed in Group 1, one patient died in Group 2 and three patients died in Group 3. There was no difference between the groups in terms of complications, including preterm labor. CONCLUSIONS: No death was also observed with early-onset treatment. Early-onset treatment may be beneficial for fewer hospitalizations, fewer ICU stays, and less mechanical ventilation requirement in pregnant women with COVID-19. In addition, with early treatment, the total number of steroid administration days was reduced, which is important in terms of reducing the risk of side effects.

Evolution of Treatment Modalities for Disseminated HAdV Infection in Neonates.

Human adenovirus (HAdV) infection in newborns is a rare condition that typically affects multiple organ systems and has a high mortality rate. We report a case of neonatal HAdV-D37 infection that presented with fever and respiratory distress that was confirmed by metagenomic next-generation sequencing using blood and bronchoalveolar lavage fluid. We treated the patient with intravenous immunoglobulin, methylprednisolone, and anticoagulants, and the patient recovered. Our review of 41 cases of HAdV found that treatment with intravenous immunoglobin might have improved the outcome of HAdV-D infection. We further suggest that glucocorticoid therapy may have additional therapeutic validity in the setting of severe or disseminated disease and that monitoring coagulation function and timely anticoagulation treatment should be considered to prevent complications associated with disseminated intravascular coagulation.

SARS-CoV-2 as a trigger of IgA vasculitis: a clinical case and literature review.

Coronavirus Disease 2019 (COVID-19), caused by SARS-CoV-2, has negatively affected global health. COVID-19 has been associated with a variety of autoimmune and inflammatory disorders, complicating its respiratory manifestations. SARS-CoV-2 triggers inflammatory reactions which may involve multiple organs and systems. The proof for IgA involvement in the immune reactions to coronavirus infection is growing, particularly in the case of IgA immune complex deposition diseases such as IgA vasculitis (IgAV) and IgA nephropathy.This report presents a case of IgAV caused by SARS-CoV-2 in a 53-year-old man. His symptoms included papillomatous, bright red rashes, urticaria throughout the body, aphthous stomatitis, pain in all joints and muscles, weakness, malaise, abdominal pain, face swelling, and arterial hypertension (160/100 mmHg). He received intravenous methylprednisolone (250 mg) and then oral methylprednisolone (16 mg) treatment, which improved his condition. This improvement included the disappearance of abdominal and joint pain and skin rashes.This article also provides an overview of published cases of IgAV after SARS-CoV-2. It may alert rheumatologists and allied specialists of clinical features of IgAV and guide them how to diagnose and treat this disease.

The usage of immunosuppressant agents and secondary infections in patients with COVID-19 in the intensive care unit: a retrospective study.

Although COVID-19 infection is an immunosuppressant disease, many immunosuppressant agents, such as pulse methylprednisolone (PMP), dexamethasone (DXM), and tocilizumab (TCZ), were used during the pandemic. Secondary infections in patients with COVID-19 have been reported recently. This study investigated these agents' effects on secondary infections and outcomes in patients with COVID-19 in intensive care units (ICUs). This study was designed retrospectively, and all data were collected from the tertiary intensive care units of six hospitals between March 2020 and October 2021. All patients were divided into three groups: Group I [GI, PMP (-), DXM (-) and TCZ (-)], Group II [GII, PMP (+), DXM (+)], and Group III [GIII, PMP (+), DXM (+), TCZ (+)]. Demographic data, PaO/FiO2 ratio, laboratory parameters, culture results, and outcomes were recorded. To compare GI-GII and GI-GIII, propensity score matching (PSM) was used by matching 14 parameters. Four hundred twelve patients with COVID-19 in the ICU were included in the study. The number of patients with microorganisms ≥ 2 was 279 (67.7%). After PSM, in GII and GIII, the number of (+) tracheal cultures and (+) bloodstream cultures detected different microorganisms ≥ 2 during the ICU period, neuropathy, tracheotomized patients, duration of IMV, and length of ICU stay were significantly higher than GI. The mortality rate was similar in GI and GII, whereas it was significantly higher in GIII than in GI. The use of immunosuppressant agents in COVID-19 patients may lead to an increase in secondary infections. In addition, increased secondary infections may lead to prolonged ICU stay, prolonged IMV duration, and increased mortality.

Converging pathways: acquired von Willebrand disease in systemic lupus erythematosus with antiphospholipid antibodies presenting with persistent menstrual bleeding.

We present a case of a woman in her 20s with inadequately treated systemic lupus erythematosus (SLE). She presented with heavy menstrual bleeding, along with nasal and gum bleeding worsening over 3 months. There was no bleeding history in her family, childhood, dental procedures or childbirth. Evaluation ruled out structural causes, revealing prolonged activated partial thromboplastin time (incomplete correction on mixing studies), normal prothrombin time, moderate thrombocytopenia, and lupus anticoagulant and anti-phosphatidylserine/prothrombin antibody positivity twice, 12 weeks apart. Further evaluation showed very low von Willebrand factor (vWF) levels (<5%). She was treated with pulse methylprednisolone for 3 days, resulting in complete symptom resolution and improvement in vWF levels to 130%. The absence of bleeding history, family history, presence of very low vWF and its response to corticosteroids led to a diagnosis of acquired vWF syndrome as the cause of mucosal bleeding in an SLE patient with concomitant positive antiphospholipid antibody. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering oral corticosteroids.

A novel strategy for spinal cord reconstruction via vascularized allogeneic spinal cord transplantation combine spinal cord fusion.

AIMS: Spinal cord injuries (SCI) pose persistent challenges in clinical practice due to the secondary injury. Drawing from our experience in spinal cord fusion (SCF), we propose vascularized allogeneic spinal cord transplantation (vASCT) as a novel approach for SCI, much like organ transplantation has revolutionized organ failure treatment and vascularized composite-tissue allotransplantation has addressed limb defects. MATERIALS AND METHODS: In this study, 24 dogs were paired and underwent vASCT, with donor spinal cord grafts and polyethylene glycol (PEG) application for SCF. The experimental group (n = 8) received tacrolimus and methylprednisolone, while the control group (n = 4) received only methylprednisolone. Safety and efficacy of vASCT were evaluated through electrophysiology, imaging, and 6-month follow-up. RESULTS: The experimental group showed substantial recovery in hind limb motor function. Imaging revealed robust survival of spinal cord grafts and restoration of spinal cord continuity. In contrast, the control group maintained hind limb paralysis, with imaging confirming spinal cord graft necrosis and extensive defects. Electrophysiologically, the experimental group exhibited restored motor evoked potential signal conduction postoperatively, unlike the control group. Notably, PEG application during vASCT led to signal conduction recovery in intraoperative spinal cord evoked potential examinations for all dogs. CONCLUSION: In the vASCT surgical model, the combination of PEG with tacrolimus has demonstrated the ability to reconstruct spinal cord continuity and restore hind limb motor function in beagles. Notably, a low dose of tacrolimus has also exhibited an excellent anti-immune rejection effect. These findings highlight vASCT's potential promise as a therapeutic strategy for addressing irreversible SCI.