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Heart disease is a major global threat. Triglyceride deposit cardiomyovasculopathy (TGCV) is an emerging, noncommunicable, adult-onset heart disease, first identified in Japanese patients with heart failure (HF) requiring cardiac transplantation 1-3 . In TGCV, defective intracellular lipolysis of long-chain triglycerides (TGs) results in cellular steatosis and energy failure mainly in cardiomyocytes 4 and smooth muscle cells 5 , leading to HF, diffuse coronary artery disease with TG deposition and ventricular arrhythmias with high mortality 6 . Tricaprin, a class of medium-chain TGs, recently corrected myocardial TG lipolysis 7 . Here we report remarkable long-term survival and durable recovery of HF in patients with TGCV treated with supplemental tricaprin in registry studies. Our study offers a classification of heart disease caused by defective lipolysis and its possible practical treatment. Because myocardial lipid droplets are a common feature in HF and their potential as therapeutic targets has been discussed worldwide, our findings warrant investigation into other ethnicities., Competing Interests: Competing interests: K.H. has been a Joint Research Chair in collaboration with Toa Eiyo since February 2021 and has served as a medical adviser for Toa Eiyo since December 2021. K.H. has two granted (PCT/JP2012/071594 and PCT/JP2021/008689) and one patent pending (PCT/JP2021/025687). K.H. reports lecture fees from Nihon Medi-Physics. K.H. is the president (unpaid) of the Tochino Foundation and the Society of Triglyceride Biology and Medicine (TGBM). K.S. reports lecture fees from Bayer Yakuhin, Janssen Pharmaceutical, Novartis Pharma and Otsuka Pharmaceutical. H.M. has a patent granted (PCT/JP2023/032407) and is a director (unpaid) of TGBM. H.M. reports lecture fees from Nihon Medi-Physics and Toa Eiyo. Y. Nakano reports a grant from the Japan Society for the Promotion of Science outside the submitted work and lecture fees from Otsuka Pharma, Kowa, Astellas Pharma, AstraZeneca, Daiichi Sankyo and Ono Pharmaceutical. C.H. is a representative caretaker (unpaid) of the TGCV/NLSD Patient Association, a director (unpaid) of TGBM and an employee of the Tochino Foundation. K. Nakajima receives funds for the endowed department (Functional Imaging and Artificial Intelligence, Kanazawa University) from Siemens Healthcare Japan, Nihon Medi-Physics (Japan) and PDRadiopharma (Japan); he has collaborated with Spectrum Dynamics Medical (Israel). S.Y. reports research grants from Nihon Medi-Physics and the Kowa Health Science Council. S.Y. is a representative director (unpaid) of Triglyceride Laboratory and Business and a managing director (paid) of the Tochino Foundation, which paid joint research fees to Osaka University. Y.Y. is a nutritional adviser (unpaid) for the TGCV/NLSD Patient Association. Y.Y., N.Z. and Y. Ikeda have a patent granted (PCT/JP2012/071594). M.H. reports a Takeda Research Grant outside the submitted work and lecture fees from Sumitomo, Novartis, Ono, Kyowa-Kirin, Takeda, Eisai, Otsuka and Chugai. T.A. reports lecture fees from Astellas Pharma, AstraZeneca, Bayer, Daiichi Sankyo and Bristol Myers Squibb. S.F. reports lecture fees from Nihon Medi-Physics and Toa Eiyo Ltd. Y.S. reports collaborative research with TOSHIBA., Fuji Yakuhin and FornesLife, and speaker/lecture fees from AstraZeneca, Ono Pharmaceutical, Eli Lilly, Kowa Pharmaceutical and Nippon Boehringer Ingelheim outside the submitted work. K.K. is a director (unpaid) of TGBM. The other authors declare no competing interests., (© 2025. The Author(s), under exclusive licence to Springer Nature Limited.)

Anti-nuclear matrix protein 2 (NXP2) antibody-positive dermatomyositis (DM) is characterized by extensive and severe myositis. In this study, we evaluated which cytokines/chemokines involved with the activity of the myositis. We performed quantitative immunoassays using the MILLIPLEX ® Multiplex Assays Using Luminex to evaluate serum levels of interferon-γ, interleukin (IL)-1β, IL-6, IL-8, IL-12p40, and tumor necrosis factor-α in samples collected over time from a 9-year-old female with anti-NXP2 antibody-positive DM. In our case, the serum level of IL-8 was elevated when the myositis worsened, and decreased in accordance with the improvement of myositis, suggesting that the serum IL-8 levels were correlated with the myositis activity. Serum levels of IL-8 in samples from five patients with anti-NXP2 antibody-positive DM and five patients with anti-transcriptional intermediary factor 1γ (TIF1γ) antibody-positive DM without both interstitial lung disease (ILD) and malignancy before starting treatments, along with five healthy controls, were also evaluate by an enzyme-linked immunosorbent assay. Serum IL-8 levels were significantly elevated in anti-NXP2 or anti-TIF1γ antibody-positive DM patients with myositis but not ILD, than healthy controls. It was suggested that serum levels of IL-8 correlate with the activity of myositis in DM including anti-NXP2 antibody-positive DM.

Background: Although the development of atrial fibrillation (AF) and the progression of chronic kidney disease are known to be interrelated, it remains unclear when and how renal function changes during the clinical course of AF., Methods: This study retrospectively enrolled 131 patients who were able to collect data on estimated glomerular filtration rate (eGFR) at least five times during the 500 days before and 500 days after the first visit (baseline) of new-onset AF, respectively. To investigate the temporal relationship between the development of AF and the beginning of worsening renal function (WRF), a piecewise regression model was applied to the eGFR time series data. The time point at which the slopes of the two regression lines changed (inflection -point), the slope before and after the inflection-point (β1 and β2, respectively), and the difference in slope (Δβ) were estimated. The presence of WRF was defined as having the inflection-point at which both Δβ and β2 were < - 0.0083 mL/min/1.73 m 2 /day (corresponding to 3.03 mL/min/1.73 m 2 /year), and the corresponding the inflection-point was defined as the beginning of WRF., Results: WRF was detected in 54 (41.2%) patients. The beginning of WRF were distributed at various times, but most frequently (23 of 54 patients) within 100 days before and after baseline. The presence of WRF was not associated with age, heart failure, or baseline eGFR, but was associated with positive β1 (odds ratio 30.5, 95% confidence interval 11.1-83.9, P < 0.01)., Conclusion: In nearly half of AF patients with WRF, the beginning of WRF was observed within a few months before or after the first visit for AF. Patients with a positive eGFR slope before the onset of AF are more likely to develop WRF after the onset of AF, suggesting that potential kidney damage may be underlying., (© 2024. The Author(s).)

Recent studies showed that preoperative functional assessment with fractional flow reserve (FFR) could predict a long-term patency of arterial bypass grafts in patients with coronary artery bypass grafting (CABG). Quantitative flow ratio (QFR) is a novel angiography-based approach to estimate FFR. This study aimed to investigate whether preoperative QFR could discriminate arterial bypass function at 1 year after surgery. The PRIDE-METAL registry was a prospective, multicenter observational study that enrolled 54 patients with multivessel coronary artery disease. By protocol, left coronary stenoses were revascularized by CABG with arterial grafts, whereas right coronary stenoses were treated with coronary stenting. Follow-up angiography at 1 year after surgery was scheduled to assess arterial graft patency. QFR was performed using index angiography by certified analysts, blinded to bypass graft function. The primary end point of this sub-study was the discriminative ability of QFR for arterial graft function, as assessed by receiver-operating characteristic curve. Among 54 patients enrolled in the PRIDE-METAL registry, index and follow-up angiography was available in 41 patients with 97 anastomoses. QFR were analyzed in 35 patients (71 anastomoses) with an analyzability of 85.5% (71/83). Five bypass grafts were found to be non-functional at 1 year. The diagnostic performance of QFR was substantial (area under the curve: 0.89; 95% confidence interval: 0.83 to 0.96) with an optimal cutoff of 0.76 to predict functionality of bypass grafts. Preoperative QFR is highly discriminative for predicting postoperative arterial graft function.Trial registration: Clinical.gov reference: NCT02894255., (© 2023. The Author(s) under exclusive licence to Japanese Association of Cardiovascular Intervention and Therapeutics.)

Dupilumab, a monoclonal antibody that specifically inhibits signal transductions by interleukin (IL)-4 and IL-13, has been used to treat T-helper (Th)2-type allergic disorders, including atopic dermatitis and asthma. We report a 21-year-old female patient with atopic dermatitis who developed systemic lupus erythematosus (SLE) unexpectedly after dupilumab treatment. Her skin lesions partially improved after dupilumab treatment; however, a part of her skin lesions on the face, nape, and upper extremities were refractory even after a 15-month period of dupilumab treatment. These dupilumab-refractory skin lesions were histopathologically diagnosed as cutaneous lupus erythematosus, moreover, subsequent phenomenons, diffuse alopecia, joint pain, lymphopaenia, hypocomplementemia, and positivities for anti-nuclear, anti-double-stranded DNA, anti-U1 ribonucleoprotein, anti-Smith, and anti-Sjögren's syndrome-related antigen A antibodies made a diagnosis of SLE. Our retrospective investigations on her serum samples indicted that these abnormalities of laboratory examinations had not appeared at the initiation of dupilumab treatment. Our case at least indicated that dupilumab was not effective in treating SLE. Moreover, inhibition of Th2-type immune responses by dupilumab may accelerate the pathogenesis of Th1-related inflammatory disorders, including SLE, as observed in our case. Our case also presented another possibility that dupilumab has no effect on the progression of underlying SLE. Because a significant relationship exists between atopic dermatitis/asthma and the risk of SLE, the utility of dupilumab should be carefully considered for each case., (© 2022 Japanese Dermatological Association.)

Background: Although each 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) has been used to diagnose cardiac sarcoidosis (CS), active CS is still misdiagnosed., Methods: Active CS, diagnosed by PET alone, was defined as focal or focal on diffuse FDG uptake pattern. In fusion PET/CMR imaging, using a regional analysis with AHA 17-segment model, the patients were categorized into four groups: (1) PET-/LGE-, (2) PET+/LGE-, (3) PET+/LGE+, and (4) PET-/LGE+. PET+/LGE+ was defined as active CS., Results: 74 Patients with suspected CS were enrolled. Between PET alone and fusion PET/CMR imaging, 20 cases had mismatch evaluations of active CS, and most had diffuse or focal on diffuse FDG uptake pattern on PET alone imaging. 40 Patients fulfilled the 2016 the Japanese Circulation Society diagnostic criteria for CS. The interobserver diagnostic agreement was excellent (κ statistics 0.89) and the overall accuracy for diagnosing CS was 87.8% in fusion PET/CMR imaging, which were superior to those in PET alone imaging (0.57 and 82.4%, respectively). In a sub-analysis of diffuse and focal on diffuse patterns, the agreement (κ statistics 0.86) and overall accuracy (81.8%) in fusion PET/CMR imaging were still better., Conclusions: Fusion PET/CMR imaging with regional analysis offered reliable and accurate diagnosis of CS, covering low diagnostic area by FDG-PET alone., (© 2020. American Society of Nuclear Cardiology.)

A line blotting assay (LB) is currently used to detect myositis-specific autoantibodies (MSAs) in patients with idiopathic inflammatory myopathies (IIMs), because of its simplicity; however, the sensitivity and specificity of this assay is low. The aim of this study is to evaluate the accuracy of the commercial LB in detection of antinuclear matrix protein 2 (NXP2) antibody. Seventy-seven serum samples from patients with IIMs, in which anti-NXP2 antibodies were detected through immunoprecipitation and western blotting (IP-WB) using K562 cell lysate, were enrolled. All samples were assessed by LB and IP-WB using recombinant human NXP2 whole protein (rNXP2) produced by insect cells, and the positive rates of each assay were compared. Thirty-two samples (41.6%) showed false-negativity by LB, which includes 11 samples with negative results by IP-WB using rNXP2. Relative intensities of IP-WB using cell lysate were significantly higher in the samples with positive results by both LB and IP-WB using rNXP2, compared to samples with positive by IP-WB using rNXP2 but negative by LB. Three of 11 samples with negative results by both LB and IP-WB using rNXP2 revealed high antibody titers. Further, differences in post-transcriptional SUMOylation were observed between recombinant and natural NXP2 proteins. In conclusion, the LB showed low sensitivity for detection of anti-NXP2 antibody, an effect exacerbated at low titers of anti-NXP2 antibodies. Moreover, there appears to be differences in the reactivities of antibodies to recombinant and natural NXP2 proteins with different post-transcriptional modifications., (© 2021 Japanese Dermatological Association.)

Objectives: Myositis-specific autoantibodies (MSAs) define distinct clinical subsets of idiopathic inflammatory myopathies (IIMs). The anti-nuclear matrix protein 2 (NXP2) antibody, a MSA detected in juvenile/adult IIMs, has been reported to be associated with a high risk of subcutaneous calcinosis, subcutaneous oedema and internal malignancies. The study aimed to clarify the clinical features of anti-NXP2 antibody-positive IIMs in detail., Methods: This was a multicentre retrospective observational study on 76 anti-NXP2 antibody-positive patients. The antibody was detected via a serological assay using immunoprecipitation and western blotting. The patients were selected from 162 consecutive Japanese patients with IIMs., Results: The cohort of anti-NXP2 antibody-positive IIMs included 29 juvenile patients and 47 adult patients. Twenty-seven (35.5%) patients presented with polymyositis phenotype without dermatomyositis-specific skin manifestations (heliotrope rash or Gottron sign/papules); this was more common in the adults than children (48.9% vs 15.8%, P < 0.01). Nine (11.8%) patients had subcutaneous calcinosis, and 20 (26.3%) patients had subcutaneous oedema. In addition, the proportion of patients with muscle weakness extending to the distal limbs was high (36 patients [47.4%]) in this cohort. Adult patients had a higher prevalence of malignancy than the general population (age-standardized incidence ratio of malignancies: 22.4)., Conclusion: Anti-NXP2 antibody-positive IIMs, which include dermatomyositis sine dermatitis, are characterized by atypical skin manifestations and extensive muscular involvement., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Aims: The clinical impact of the type of atrial fibrillation (AF) has not been completely elucidated in non-ischemic cardiomyopathy (NICM). Although the structure and function of the left atrium (LA) provide prognostic information in patients with heart failure, the relationship of the AF type with LA structure and function in NICM is unclear. Methods: Consecutive patients with NICM who underwent cardiac magnetic resonance were evaluated and followed. Multivariable Cox regression models were used to estimate hazard ratios (HRs) for major adverse cardiovascular events (MACE) related to the AF type, such as paroxysmal AF, chronic AF, and new-onset AF (NOAF). Results: Among 625 patients with NICM (mean age, 64.4 ± 14.2 years; women, 39.7%), 133 had a history of AF at baseline; of these, 60 had paroxysmal AF. Each baseline AF type was associated with higher LA volume and lower LA emptying fraction but not with an increased incidence of MACE ( p = 0.245). New-onset AF developed in 5.9% of patients with sinus rhythm over a median follow-up period of 609 days, and maximum LA volume was a strong and independent predictor [ p < 0.001, area under the ROC curve (AUC): 0.795]. Maximum LA volume was superior to LA emptying fraction and B-type natriuretic peptide (AUC: 0.683 and 0.680, respectively). The use of β-blocker and the age of the patient were associated with the incidence of NOAF (HR: 0.37, 95% CI: 0.16-0.84 and HR: 1.05, 95% CI: 1.01-1.09, respectively). Kaplan-Meier analysis showed that patients with NOAF had a higher incidence of MACE than those with sinus rhythm or baseline AF ( p = 0.002). NOAF and LA emptying fraction were independent predictors of MACE (HR: 2.28, 95% CI: 1.20-3.97 and HR: 0.98, 95% CI: 0.96-0.99, respectively) after adjusting for age, sex, body mass index, and diagnosis. Conclusions: Paroxysmal and chronic AF in patients with NICM were not associated with an increased incidence of MACE despite their association with LA volume and function. NOAF was independently associated with poor prognosis. Higher maximum LA volume predicted the onset and lower LA emptying fraction was independently associated with poor prognosis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Okune, Yasuda, Soejima, Kakehi, Kawamura, Kurita, Nakazawa and Iwanaga.)

Background: We evaluated malignancy according to the characteristics of pericardial fluid in symptomatic Japanese patients undergoing pericardiocentesis and computed tomography (CT)., Methods: This was a retrospective, single-center, observational study of 125 symptomatic patients undergoing pericardiocentesis. The patients were classified into two groups: a malignancy group and a non-malignancy group, according to the primary disease and cytology of the pericardial effusion (PE). We compared the pericardial fluid sample and CT measurements between both groups., Results: All patients were diagnosed as having exudative PE by Light's criteria. PE with malignant cells was demonstrated in 76.8% of the malignancy group patients. Pericardial to serum lactate dehydrogenase (LDH) ratio > 0.6, as one of Light's criteria, was associated with malignancy (p = 0.017). Lower serum brain natriuretic peptide (BNP) concentration was also associated with malignancy (BNP: 126.9 ± 89.8 pg/ml vs 409.2 ± 97.7 pg/ml, malignancy vs non-malignancy groups, respectively; p = 0.037). A significant difference was observed in pericardial fluid glucose level between the malignancy and non-malignancy groups (pericardial fluid glucose: 78.24 ± 48.29 mg/dl vs 98.41 ± 44.85, respectively; p = 0.048). Moreover, CT attenuation values (Hounsfield units (HU)) tended to be higher in the malignancy group vs the non-malignancy group (22.7 [interquartile range (IQR), 17.4-26.0] vs 17.4 [IQR, 13.7-26.4], respectively; p = 0.08). The sensitivity and specificity of pericardial fluid glucose level ≤ 70 mg/dl and CT attenuation values > 20 HU were 40.9% and 89.6%, respectively, in the malignancy group. The positive- and negative predictive values of pericardial fluid glucose level ≤ 70 mg/dl and CT attenuation values > 20 HU were 85.7% and 50.0%, respectively, in the malignancy group. Pericardial fluid glucose level ≤ 70 mg/dl and CT attenuation values > 20 HU were cutoff values associated with malignancy (p = 0.012)., Conclusions: Lower pericardial fluid glucose level with higher CT attenuation values may suggest malignancy-related PE.

Surgical-site infection (SSI) is one of the major postoperative complications in surgery, which can cause significant morbidity. However, factors associated with SSI in dermatological surgery are not well understood. Here, we retrospectively investigated 512 patients who underwent outpatient surgery for skin tumors at the University of Tsukuba Hospital to analyze factors associated with postoperative SSI. The overall incidence of SSI was 28 (5.5%). Univariate logistic regression analysis revealed that SSI was significantly associated with invasive squamous cell carcinoma (iSCC), Bowen's disease (BD), actinic keratosis (AK), longer diameter of defects, presence of ulcer, reconstruction with full-thickness skin graft and local skin flaps, medical history of diabetes mellitus, and use of immunosuppressive agents. However, in the multivariate analysis only iSCC, BD, and AK retained significance. The frequencies of SSI in iSCC, BD, and AK were 22% (13/58 patients), 15.6% (5/32), and 25% (2/8), respectively; however, the frequency of other non-SCC tumors was only 1.9% (8/414). χ 2 -Tests revealed that the frequency of SSI in iSCC, BD, and AK were all significantly higher than in non-SCC tumors, with the frequencies being more than eight times higher. These results suggest that invasive and in situ lesions of SCC are independent risk factors of SSI development after outpatient skin surgery., (© 2021 Japanese Dermatological Association.)

Background: Late gadolinium enhancement imaging (LGE) of the left ventricle (LV) by cardiac magnetic resonance (CMR) has prognostic value for patients with cardiac sarcoidosis (CS). Right ventricle (RV) dysfunction is also associated with adverse outcomes in patients with heart failure. Therefore, we sought to determine if RV LGE and dysfunction predicted adverse events in patients with suspected CS., Methods: In 103 consecutive patients with suspected CS who underwent CMR, functional and remodeling indexes of both the LV and RV were measured and the extent and localization of LGE were also analyzed. Major adverse cardiac events (MACE) were defined as cardiovascular mortality, severe ventricular tachyarrhythmia, hospitalization with heart failure, and advanced atrioventricular block., Results: During a median follow-up of 20.6 months, Kaplan-Meier analysis showed that decreased RV ejection fraction (EF) was associated with MACE (P < 0.001) and receiver operating characteristics curve (ROC) analysis indicated good predictive performance of RV EF for MACE (area under the ROC = 0.834). RV EF operated independently of LV EF or LGE extent for predicting MACE. In addition, the presence of LGE in RV was independently associated with MACE (P = 0.011), and a combined analysis of RV EF and RV LGE showed better risk stratification for MACE (P < 0.001)., Conclusions: Both RV EF and LGE were independently associated with MACE and enhanced risk stratification in patients with suspected CS. CMR may be a useful tool for detecting myocardial function and fibrosis in both the LV and RV.

Background: The clinical significance of left ventricular (LV) trabeculation remains unknown in cardiomyopathies. B-Type natriuretic peptide (BNP) strongly reflects LV end-diastolic wall stress and is a useful prognostic marker of cardiovascular diseases. The enhanced identification of LV trabeculae (T) with the use of cardiac magnetic resonance and the evaluation of its relationship with BNP may elucidate the biologic significance and clinical impact of trabeculation in patients with nonischemic cardiomyopathy (NICM)., Methods: The LV volume and mass of 515 patients with NICM and 36 control subjects were analyzed with the use of a steady-state free precession sequence, and individual T mass was planimetred. Major adverse cardiac events (MACE) were assessed., Results: T mass index correlated with LV end-diastolic volume index (EDVI), LV mass index, and papillary muscle mass index (all P < 0.001). Also, T mass index was positively correlated with BNP level (R = 0.381; P < 0.001) and was an independent determinant of BNP after adjusting for age, sex, body mass index (BMI), etiology, LV ejection fraction, and LV EDVI (P < 0.001). Kaplan-Meier analysis during a median follow-up of 17.3 months showed that higher T mass index and increased BNP level correlated with MACE. On multivariate analysis, T mass index (P = 0.031) and BNP (P < 0.001) remained associated with poor outcomes when combined with age, sex, BMI, and etiology., Conclusions: Increased LV trabeculation was associated with LV dysfunction/remodelling and impaired outcomes in NICM of various etiologies. This may support the biologic significance of LV trabeculation and could be attributed to its association with BNP through LV wall stress., (Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Essential thrombocythemia (ET) is a rare clonal myeloproliferative disorder with a prevalence rate of approximately 1-3 cases per 100 000 individuals per year. ET is characterized by a persistent increase in the platelet count with hyperplasia of bone marrow megakaryocytes. It is difficult to make a diagnosis of ET, because most thrombocythemia are reactive to certain disease conditions including iron deficiency anemia, infection, collagen diseases and malignant tumors. Mutation in the Janus kinase (JAK)2 gene is present in approximately 50-70% of ET patients, and somatic mutations in the calreticulin (CALR) gene were recently discovered in approximately 20-25% of sporadic patients with ET or primary myelofibrosis. Various cutaneous manifestations of ET often occur by microvascular thrombosis and precede severe arterial and venous thromboembolic events in other organs. Therefore, in order to prevent such severe events, it is important to make an early diagnosis of ET based on a number of cutaneous manifestations. Here, we report two cases of ET diagnosed based on livedo racemosa on feet with gene mutations in JAK2 and CALR, respectively, and show the pathological and immunohistological findings of the livedo resulting from platelet thrombosis rather than vasculitis. We also review the cutaneous manifestations in current published reports of Japanese ET patients. Our patients were successfully treated with low-dose aspirin, a vasodepressor and hydroxyurea, following regressed livedo and reduced platelet counts., (© 2016 Japanese Dermatological Association.)

Nivolumab, an anti-programmed death-1-specific monoclonal antibody, has demonstrated a durable response and effect on overall survival and has become one of the standard treatments for patients with advanced melanoma. Reported herein is a case of nivolumab-induced chronic inflammatory demyelinating polyradiculoneuropathy, in which an 85-year-old woman with stage IV melanoma developed grade 1 paresthesia 2 weeks after the initial dose of nivolumab was administered. With continued treatment, the neurological deficiency deteriorated rapidly, mimicking Guillain-Barré syndrome, causing such a dramatic decrease in her activities of daily living that she could no longer function in daily life. Thus, nivolumab treatment was discontinued. A course of intravenous immunoglobulin infusion yielded a dramatic clinical improvement; in particular, improved motor control was observed within a few days. Her initial presentation was suggestive of acute inflammatory demyelinating polyradiculoneuropathy, a subtype of Guillain-Barré syndrome; however, the good response to steroids and exacerbation 8 weeks after the onset were suggestive of chronic inflammatory demyelinating polyradiculoneuropathy induced by nivolumab. This is the first case of Guillain-Barré syndrome-like autoimmune polyradiculoneuropathy induced by programmed death-1/programmed death-ligand 1 inhibitors. Although neurological adverse events related to nivolumab are rare, they can become severe, requiring early diagnosis and intervention. Intravenous immunoglobulin may be considered as an effective initial treatment for patients who develop acute autoimmune nervous system disorders due to nivolumab., (© The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)