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Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies. [ABSTRACT FROM AUTHOR]

To evaluate the specific prognostic value of CAs, we conducted an analysis of 923 symptomatic multiple myeloma patients. Among this cohort, 480 patients had complete data set of high-risk CAs by interphase fluorescent in situ hybridization at diagnosis. In the high-risk group analysis, the median OS of patients without CAs (n = 338, 72 %) was 6.5 years, patients with del(17p) (n = 42, 9 %) was 4.4 years, patients with t(4;14) or t(14;16) (n = 72, 15 %) was 4.4 years, and patients with double-positive CAs(del(17p) and t(4;14) or t(14;16)) (n = 18, 4 %) was 2.1 years (p = 0.032). Patients with double-positive CAs had a significantly worse prognosis.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) affects kidneys mainly in the form of acute kidney injury but rarely can cause glomerular disease. On a very rare occasion, SARS-CoV-2 infection can be associated with anti-neutrophil cytoplasmic antigen (ANCA)-associated vasculitis and anti-glomerular basement membrane glomerulonephritis (anti-GBM GN). We report a case of a 59-year-old man who presented with progressive renal failure 8 weeks after contracting the viral infection, which progressed slowly to severe renal dysfunction. Renal biopsy showed crescentic glomerulonephritis (CrGN) accompanied by interrupted linear IgG deposits along the glomerular basement membrane (GBM) on immunofluorescence (IF) staining with associated mild acute tubular injury. The serology for anti-myeloperoxidase (MPO), as well as anti-GBM antibodies, was positive. He was treated with steroid and pulse intravenous cyclophosphamide, following which there was a significant improvement in the renal function and serological resolution of both the antibodies 6 months post-treatment. To the best of our knowledge, this is the first reported case of "double-antibody" positive CrGN following SARS-CoV-2 infection. [ABSTRACT FROM AUTHOR]

We report a case of a patient double-seropositive for anti-glomerular basement membrane (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) who reported retrosternal chest pain during a regular hemodialysis session associated with ST-segment depression in electrocardiogram and an increase of serum high-sensitivity troponin T. Urgent coronary angiography excluded obstructive coronary artery disease, suggesting the diagnosis of ischemia with non-obstructive coronary arteries. This case illustrates an unusual presentation of cardiovascular involvement in a patient with double-positive ANCA/anti-GBM disease, emphasizing the possible relevance of coronary microvascular dysfunction and the need for close cardiovascular follow-up in this patient population.

ObjectivesThe clinical data of patients with double-positive for leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies is limited, particularly for children. This study aimed to investigate and summarize the clinical features and long-term prognosis of children’s LGI1 and CASPR2 antibodies related to neurological disorders.MethodsWe collected the clinical data and prognosis of patients with dual positive antibodies of CASPR2 and LGI1, hospitalized in the Department of Neurology, Children’s Hospital of Chongqing Medical University. Furthermore, we summarized the clinical phenotypes of this disorder in children by reviewing the published literature.ResultsTwo patients presenting with variable neurological symptoms including pain, hypertension, profuse sweating, irritability, and dyssomnia from Children’s Hospital of Chongqing Medical University were enrolled in this study. Together with the two patients, we identified 17 children with dual CASPR2 and LGI1 antibodies, including 12 males and 5 females. At the onset, the median age was 4.1 years (range 1–16, interquartile range 2.5–13.5), with 9 children younger than 5 years and 6 adolescents. Of the 17 patients, 11 were diagnosed with Morvan syndrome, 4 with acquired neuromyotonia, 1 with Guillain-Barré syndrome, and 1 with Guillain-Barré syndrome combined with Morvan syndrome. Dysautonomia (14/17, 82.3%), pain (13/17, 76.4%), sleep disorders (13/17, 76.4%), encephalopathy (12/17, 70.5%), and weight loss (10/17, 58.8%) were the most frequently described symptoms overall. No tumors were identified. Of the 17 patients, 13 received immunotherapy comprising IVIG combination of IVMP during the acute symptomatic phase followed by oral prednisolone to maintain remission (n = 7), the combination of IVIG, IVMP, oral prednisolone and methotrexate (n = 1), the combination of IVIG, IVMP, and mycophenolate mofetil (n = 1), the combination of IVIG, IVMP, oral prednisolone, and rituximab (n = 1), IVIG only (n = 2), IVMP only (n = 1). Median modified Rankin Scale (mRS) scores in the acute phase were 3 (range 1–4) and improved gradually. Over the follow-up (median 8.6 months, range 1–36 months), 52.9% (9/17) of the patients recovered completely; one patient relapsed and showed immunotherapy-dependent.ConclusionLGI1 and CASPR2 double-positive antibodies associated with the neurological diseases can occur in children of all ages and involve multiple nervous systems. Morvan syndrome is the most common phenotype of this disorder. The long-term outcomes are mostly favorable upon immunotherapy.

To evaluate the specific prognostic value of CAs, we conducted an analysis of 923 symptomatic multiple myeloma patients. Among this cohort, 480 patients had complete data set of high-risk CAs by interphase fluorescent in situ hybridization at diagnosis. In the high-risk group analysis, the median OS of patients without CAs ( n  = 338, 72 %) was 6.5 years, patients with del(17p) ( n  = 42, 9 %) was 4.4 years, patients with t(4;14) or t(14;16) ( n  = 72, 15 %) was 4.4 years, and patients with double-positive CAs(del(17p) and t(4;14) or t(14;16)) ( n  = 18, 4 %) was 2.1 years ( p  = 0.032). Patients with double-positive CAs had a significantly worse prognosis., Competing Interests: All authors received research funding from ONO PHARMACEUTICAL CO., LTD., Bristol-Myers Squibb Company. JKU is appointed as an officer or advisor at Kansai Medical Net. JKU received lecture honoraria from Bristol-Myers Squibb Company, Janssen Pharmaceutical K. K., Sanofi K.K., ONO PHARMACEUTICAL CO., LTD., AbbVie GK., Takeda Pharmaceutical Company Limited, and Novartis Pharma KK. AT received lecture honoraria from Bristol-Myers Squibb Company, Novartis Pharma KK, Kyowa Kirin Co., Ltd., Astellas Pharma Inc., Nippon Shinyaku Co., Ltd., JANSSEN PHARMACEUTICAL K.K., Otsuka Pharmaceutical Co., Ltd. MH received lecture honoraria from Novartis Pharma K.K., Takeda Pharmaceutical Company, Limited, Bristol-Myers Squibb., Otsuka Pharmaceutical Co., Ltd. IM received lecture honoraria from Novartis Pharma KK, Bristol-Myers Squibb Company, Pfizer Japan Inc., DAIICHI SANKYO COMPANY, LIMITED., Otsuka Pharmaceutical Co., Ltd., Astellas Pharma Inc., Janssen Pharmaceutical K.K, AbbVie GK., Takeda Pharmaceutical Company Limited, ONO PHARMACEUTICAL Co., Ltd., SymBio Pharmaceuticals Limited. HS received lecture honoraria from Takeda Pharmaceutical Company, Limited., ONO PHARMACEUTICAL CO., LTD., Novartis Pharma K.K., Bristol-Myers Squibb K.K., JANSSEN PHARMACEUTICAL K.K., CHUGAI PHARMACEUTICAL CO., LTD., Sanofi K.K., AstraZeneca K.K. TI received lecture honoraria from Bristol-Myers Squibb, Mundipharma K.K., Novartis Pharma K.K., Pfizer Japan Inc., Takeda Pharmaceutical Company, Limited., AbbVie GK., CHUGAI PHARMACEUTICAL CO., LTD., Sanofi K.K., Nippon Shinyaku Co., Ltd., CSL Behring K.K., Otsuka Pharmaceutical Co., Ltd., NIHON PHARMACEUTICAL CO., LTD., JANSSEN PHARMACEUTICAL K.K.. SF received lecture honoraria from Takeda Pharmaceutical Company, Ltd., Sanofi K.K., JANSSEN PHARMACEUTICAL K.K., ONO PHARMACEUTICAL CO., LTD., Bristol-Myers Squibb K.K. TT received lecture honoraria from Sanofi K.K.. JKU received writing fees from JANSSEN PHARMACEUTICAL K.K., Bristol-Myers Squibb K.K. JKU received grants for research from Bristol-Myers Squibb Company, ONO PHARMACEUTICAL CO., LTD., Sysmex Corporation, Takeda Pharmaceutical Company, Limited, Otsuka Pharmaceutical Co., Ltd., Incyte Corporation, JANSSEN PHARMACEUTICAL K.K., Pfizer Japan Inc., CHUGAI PHARMACEUTICAL Co., Ltd., Kyowa Kirin Co., Ltd., IQVIA Services Japan K.K. AT received grants for research from ONO PHARMACEUTICAL Co., Ltd., COGNANO, Inc., DKS Co., Ltd.. IM received grants for research from Chugai Pharmaceutical Co., Ltd., Novartis Pharma K.K., Astellas Pharma Inc., AbbVie GK., Pfizer Japan Inc., Takeda Pharmaceutical Company Limited., Alexion, Otsuka Pharmaceutical Co., Ltd., Janssen Pharmaceutical K.K. NH received grants for research from Otsuka Pharmaceutical Co., Ltd. HS received grants for research from JANSSEN PHARMACEUTICAL K.K., ONO PHARMACEUTICAL CO., LTD., Bristol-Myers Squibb K.K., Novartis Pharma K.K., AstraZeneca K.K., AbbVie GK., Eisai Co., Ltd., HUYABIO International, CHUGAI PHARMACEUTICAL CO., LTD., PharmaEssentia Japan KK. TI received grants for research from Bristol-Myers Squibb. JKU recieved scolarship grant from Kyowa Kirin Co., Ltd., CHUGAI PHARMACEUTICAL CO., LTD., DAIICHI SANKYO COMPANY, LIMITED, ONO PHARMACEUTICAL CO., LTD., Sanofi K.K., Eisai Co., Ltd., Dainippon Sumitomo Pharma Co., Ltd., Takeda Pharmaceutical Company, Limited., Nippon Shinyaku Co., Ltd. AT received scholarship grants from Takeda Pharmaceutical Company, Limited, Nippon Shinyaku Co., Ltd., Kyowa Kirin Co., Ltd., CHUGAI PHARMACEUTICAL CO., LTD., Sanofi K.K., Otsuka Pharmaceutical Co., Ltd., Astellas Pharma Inc., Eisai Co., Ltd., OHARA Pharmaceutical Co., Ltd., Popuri, Kinshikouraininjin, AbbVie, SHIONOGI & CO., LTD., Asahi Kasei Pharma Corporation., the Japanese Society of Hematology. MH recieved scholarship grants from CHUGAI PHARMACEUTICAL CO., LTD., Kyowa Kirin Co., Otsuka Pharmaceutical Co., Ltd., TAIHO PHARMACEUTICAL CO., LTD., Takeda Pharmaceutical Company, Limited., ONO PHARMACEUTICAL CO., LTD. IM received scholarship grants from Chugai Pharmaceutical Co., Ltd., Kyowa Kirin Co., Ltd, Sumitomo Dainippon Pharma Co., Ltd., Takeda Pharmaceutical Company Limited., Shionogi & Co., Ltd., ASAHI KASEI PHARMA CORPORATION, Eisai Co., Ltd., TAIHO PHARMACEUTICAL CO., LTD., NIPPON SHINYAKU CO.,LTD., Otsuka Pharmaceutical Co., Ltd, AbbVie GK., ONO PHARMACEUTICAL CO., LTD., Sanofi K.K., Mitsubishi Tanabe Pharma Corporation. IM received scholarship grants from Chugai Pharmaceutical Co., Ltd., Kyowa Kirin Co., Ltd, Sumitomo Dainippon Pharma Co., Ltd., Takeda Pharmaceutical Company Limited., Shionogi & Co., Ltd., ASAHI KASEI PHARMA CORPORATION, Eisai Co., Ltd., TAIHO PHARMACEUTICAL CO., LTD., NIPPON SHINYAKU CO.,LTD., Otsuka Pharmaceutical Co., Ltd, AbbVie GK., ONO PHARMACEUTICAL CO., LTD., Sanofi K.K., Mitsubishi Tanabe Pharma Corporation. HS received scholarship grants from Astellas Pharma Inc., TEIJIN PHARMA LIMITED, SHIONOGI & CO., LTD., Eisai Co., Ltd., TAIHO PHARMACEUTICAL CO., LTD., Nippon Shinyaku Co., Ltd. CS received lecture honoraria from Sanofi K.K., JANSSEN PHARMACEUTICAL K.K., Bristol-Myers Squibb K.K., (© 2023 The Author(s).)

Rationale: Vaccines remain central to the management of COVID-19 pandemic, including the need for repeat doses of vaccines to boost immunity. There has been an accumulating case count of glomerulopathies temporally associated with COVID-19 vaccination. This case series presents 4 patients who developed double-positive anti-glomerular basement membrane antibody (anti-GBM) and myeloperoxidase (MPO) antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis after COVID-19 mRNA vaccination. This report contributes to our collective knowledge about the pathophysiology and clinical outcomes associated with this rare complication., Presenting Concerns of the Patient: Four patients developed nephritic syndrome within 1 to 6 weeks after receiving a COVID-19 mRNA vaccine (3 post Pfizer-BioNTech and 1 post Moderna vaccination). Three of the 4 patients also had hemoptysis., Diagnosis: Three of the 4 patients had double-positive serology, whereas the fourth patient had renal biopsy findings consistent with double-positive disease, although anti-GBM serology was negative. All patients had renal biopsy findings consistent with double-positive anti-GBM and ANCA-associated glomerulonephritis., Interventions: All 4 patients were treated with pulse steroids, cyclophosphamide, and plasmapheresis., Outcomes: Of the 4 patients, 1 demonstrated complete remission, 2 remained dialysis-dependent, and the fourth is deceased. Of the 2 patients who received repeat vaccination with COVID-19 mRNA vaccine, 1 patient had second serologic flare of anti-GBM in response to the vaccine., Novel Findings: This case series reinforces growing evidence that COVID-19 mRNA vaccine-induced glomerulonephritis is a rare but real phenomenon. Dual ANCA and anti-GBM nephritis can present after the first dose of COVID-19 mRNA vaccine or after several administrations of the vaccine. We are the first to report cases of double-positive MPO ANCA and anti-GBM nephritis after Pfizer-BioNTech vaccination. To our knowledge, we are also the first to report outcomes of repeat COVID-19 vaccination in patients with de novo flare of ANCA and anti-GBM nephritis temporally associated with COVID-19 vaccination., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

Objectives: The clinical data of patients with double-positive for leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies is limited, particularly for children. This study aimed to investigate and summarize the clinical features and long-term prognosis of children's LGI1 and CASPR2 antibodies related to neurological disorders., Methods: We collected the clinical data and prognosis of patients with dual positive antibodies of CASPR2 and LGI1, hospitalized in the Department of Neurology, Children's Hospital of Chongqing Medical University. Furthermore, we summarized the clinical phenotypes of this disorder in children by reviewing the published literature., Results: Two patients presenting with variable neurological symptoms including pain, hypertension, profuse sweating, irritability, and dyssomnia from Children's Hospital of Chongqing Medical University were enrolled in this study. Together with the two patients, we identified 17 children with dual CASPR2 and LGI1 antibodies, including 12 males and 5 females. At the onset, the median age was 4.1 years (range 1-16, interquartile range 2.5-13.5), with 9 children younger than 5 years and 6 adolescents. Of the 17 patients, 11 were diagnosed with Morvan syndrome, 4 with acquired neuromyotonia, 1 with Guillain-Barré syndrome, and 1 with Guillain-Barré syndrome combined with Morvan syndrome. Dysautonomia (14/17, 82.3%), pain (13/17, 76.4%), sleep disorders (13/17, 76.4%), encephalopathy (12/17, 70.5%), and weight loss (10/17, 58.8%) were the most frequently described symptoms overall. No tumors were identified. Of the 17 patients, 13 received immunotherapy comprising IVIG combination of IVMP during the acute symptomatic phase followed by oral prednisolone to maintain remission ( n = 7), the combination of IVIG, IVMP, oral prednisolone and methotrexate ( n = 1), the combination of IVIG, IVMP, and mycophenolate mofetil ( n = 1), the combination of IVIG, IVMP, oral prednisolone, and rituximab ( n = 1), IVIG only ( n = 2), IVMP only ( n = 1). Median modified Rankin Scale (mRS) scores in the acute phase were 3 (range 1-4) and improved gradually. Over the follow-up (median 8.6 months, range 1-36 months), 52.9% (9/17) of the patients recovered completely; one patient relapsed and showed immunotherapy-dependent., Conclusion: LGI1 and CASPR2 double-positive antibodies associated with the neurological diseases can occur in children of all ages and involve multiple nervous systems. Morvan syndrome is the most common phenotype of this disorder. The long-term outcomes are mostly favorable upon immunotherapy., 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 © 2022 Jiang, Tan, Li, Song, Ma, Yao, Hong, Li, Jiang and Luo.)

• Properties of optimized TMOKE of all-types of bi-gyrotropic metamaterials for both s- and p-polarized waves have been investigated and compared. • By optimizing the structure, wide-angle giant transverse magneto-optical Kerr effect can be observed in double-positive and double-negative metamaterials. • The results of double-positive metamaterials for s-polarization wave are same as ones for double-negative ones for p-polarization, and vice versa. • A giant TMOKE can be occurred in near-zero refractive index metamaterials, too. The properties of optimized transverse magneto-optical Kerr effect (TMOKE) of all-types of bi-gyrotropic metamaterials have been investigated and compared for both s- and p-polarized waves. We demonstrated that double-positive and double-negative ones are capable of achieving maximum TMOKEs, but with the opposite signs. On the other hand, single-negative metamaterials could not provide large TMOKE. In addition, a giant TMOKE can be observed in a special case of metamaterials whose are exhibiting near-zero refractive index. A large TMOKE response is important for applications in magneto-optical data storage and high-definition imaging. [ABSTRACT FROM AUTHOR]

Mucosal-associated invariant T (MAIT) cells have been reported to play an antimicrobial role in infectious diseases. However, little is known about age- and gender-related changes in circulating MAIT cell level and function in healthy population. The purposes of this study were to examine the level and cytokine production of circulating MAIT cells and their subsets in healthy adults and to investigate potential relationships between clinical parameters and MAIT cell levels or their subset levels. One hundred thirty-three healthy subjects were enrolled in this study. MAIT cells, their subset, and cytokine levels were measured by flow cytometry. Circulating MAIT cell levels were found to vary widely (0.19% to 21.7%) in the study subjects and to be significantly lower in elderly subjects (age, 61-92 years) than in young subjects (age, 21-40 years) (p<0.0005). No significant difference was found in the circulating MAIT cell levels between male and female subjects. A linear regression analysis revealed that circulating MAIT cell levels declined annually by 3.2% among men and 1.8% among women, respectively. Notably, the proportion of CD4+ MAIT cells increased with age, whereas that of CD8+ MAIT cells decreased with age. In addition, the production of interleukin (IL)-4 by MAIT cells was found to be significantly increased in elderly subjects and the ratio of interferon (IFN)-γ/IL-4 was lower as compared with young subjects, showing a Th1 to Th2 shift in cytokine profile in elderly subjects. Our data suggest that aging is associated with a reduction in circulating MAIT cells, accompanied with alterations in subset composition and cytokine profile., (Copyright © 2013 Elsevier Inc. All rights reserved.)