An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab: a case-based review.

IgG4-related disease (IgG4-RD) is a systemic condition in which IgG4+ plasma cell infiltration and fibrosis cause organ swelling and lead to diverse clinical manifestations. Although IgG4-RD typically responds to glucocorticoids (GCs), relapse during tapering occurs and an early GC-sparing approach might therefore be beneficial. Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiple symptoms that is also treated with GCs as a first-line therapy. Recently, belimumab, a recombinant human IgG-1λ monoclonal antibody that inhibits B-cell activating factor, was approved, but reports of use for IgG4-RD are scarce. Here, we present a rare case of IgG4-RD complicated with SLE which was successfully treated with belimumab. A 67-year-old man was diagnosed with IgG4-RD based on a high serum IgG4 level and histopathological findings. Furthermore, he had pericardial effusion on echocardiography, and laboratory tests revealed thrombocytopenia, autoimmune hemolysis, positive anti-nuclear antibodies, positive anti-DNA antibodies, and hypocomplementemia. These data led to an SLE diagnosis. Treatment was started with prednisolone at 40 mg/day, plus hydroxychloroquine, which initially improved both the SLE and IgG4-RD symptoms. During the GC tapering, belimumab was added and clinical symptoms resolved completely. Our case and the literature review summarize reported rare overlapping cases of IgG4-RD and SLE and suggest that belimumab is a promising candidate for the treatment of IgG4-RD.

Efficient detection of somatic UBA1 variants and clinical scoring system predicting patients with variants in VEXAS syndrome.

OBJECTIVES: To efficiently detect somatic UBA1 variants and establish a clinical scoring system predicting patients with pathogenic variants in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. METHODS: Eighty-nine Japanese patients with clinically suspected VEXAS syndrome were recruited [81 males and 8 females; median onset age (IQR) 69.3 years (62.1-77.6)]. Peptide nucleic acid-clamping PCR (PNA-PCR), regular PCR targeting exon 3 clustering UBA1 variants, and subsequent Sanger sequencing were conducted for variant screening. Partitioning digital PCR (pdPCR) or targeted amplicon deep sequencing (TAS) was also performed to evaluate the variant allele frequency (VAF). We developed our clinical scoring system to predict UBA1 variant-positive and ­negative patients and assessed the diagnostic value of our system using receiver operating characteristic (ROC) curve analysis. RESULTS: Forty patients with reported pathogenic UBA1 variants (40/89, 44.9%) were identified, including a case having a variant with VAF of 1.7%, using a highly sensitive method. Our clinical scoring system considering >50 years of age, cutaneous lesions, lung involvement, chondritis, and macrocytic anaemia efficiently predicted patients with UBA1 variants (the area under the curve for the scoring total was 0.908). CONCLUSIONS: Genetic screening with the combination of regular PCR and PNA-PCR detected somatic UBA1 variants with high sensitivity and specificity. Our scoring system could efficiently predict patients with UBA1 variants.

Self-remitting Bartonella neuroretinitis without a cat scratch.

•Bartonella infection is a leading cause in patients with neuroretinitis.•Ocular involvement occurs in 5-10% of people with cat-scratch disease (CSD).•Bartonella neuroretinitis presents without systemic signs and symptoms of CSD.•Some cases with bartonella neuroretinitis could be self-remitted.

Kidney biopsy in patients with antineutrophil cytoplasmic antibody-associated vasculitis with mild renal abnormality.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic inflammation of small or medium blood vessels that includes microscopic polyangiitis. A diagnosis of ANCA-associated vasculitis can be aided by histological identification of vasculitis, and identification of renal impairment can help predict outcomes. However, kidney biopsy is not generally indicated in the absence of renal findings. We report two cases of ANCA-associated vasculitis diagnosed by kidney biopsy despite the absence of remarkable urinary abnormality and renal impairment. These patients had fever of unknown origin and were positive for myeloperoxidase (MPO)-ANCA but showed few findings that would suggest small-vessel vasculitis in the kidney. Nevertheless, kidney biopsies revealed small-vessel arteritis, necrotizing glomerulonephritis, and interstitial nephritis. Immunofluorescent antibody tests performed using samples of glomeruli were all negative, suggesting microscopic polyangiitis. Therefore, kidney biopsy may be useful in confirming the diagnosis, even if patients have completely normal urinary findings in the absence of other organ lesions.

Comparison of simultaneous and separate processes: saccharification and thermophilic L-lactate fermentation of catch crop and aquatic plant biomass.

Catch crop candidates (corn, guinea grass) for recovering nutrients from farm soil and aquatic plants (water caltrop, water hyacinth) were utilized to produce L-lactic acid. The efficiencies ofpre-treatment methods for enzymatic saccharification and L-lactate production of two fermentation processes, thermophilic simultaneous saccharification and fermentation (SSF), as well as separate saccharification and fermentation, were compared. Conditions were set at 55 degrees C and pH 5.5 for non-sterile fermentation. Alkaline/peroxide pre-treatment proved the most effective for saccharification in pre-treated corn, guinea grass, water caltrop and water hyacinth with glucose yields of 0.23, 0.20, 0.11 and 0.14 g/g-dry native biomass (24-hour incubation period), respectively. Examination of the two types of thermophilic L-lactate fermentation employed following alkaline/peroxide pre-treatment and saccharification demonstrated that the L-lactate yield obtained using SSF (0.15 g/g in the case of corn) was lower than that obtained using separate saccharification and fermentation (0.28 g/g in the case of corn). The lower yield obtained from SSF is likely to have resulted from the saccharification conditions used in the present study, as the possibility of cellulase deactivation during SSF by thermophilic L-lactate producing bacteria existed. A cellulase that retains high activity levels under non-sterile conditions and a L-lactate producer without cellulose hydrolysis activity would be required in order for SSF to serve as an effective method of L-lactate production.

Fatal thrombotic microangiopathy with rhabdomyolysis as an initial symptom after the first dose of mRNA-1273 vaccine: A case report.

We report a case of a Japanese man with severe rhabdomyolysis and multiple thrombosis of arterioles after the first dose of mRNA-1273 vaccine. He developed rapidly progressive rhabdomyolysis and infarctions of multiple organs. Antiplatelet factor 4 antibody test was negative. Despite the intensive supportive care, including aggressive fluid administration, hemodialysis, administration of anticoagulants, high-dose steroid, and eculizumab, the patient ultimately died of multiple organ failure. Autopsy revealed multiple thrombosis in the arterioles and organ necrosis. Low serum complements and C3 deposition in the renal glomeruli detected by immunofluorescence suggested a possible immune-mediated mechanism. To our knowledge, this is the first case report of rhabdomyolysis and multiple thrombosis of the arterioles as an adverse event following COVID-19 vaccination.

The effect of soil sample size, for practical DNA extraction, on soil microbial diversity in different taxonomic ranks.

To determine the optimal soil sample size for microbial community structure analysis, DNA extraction, microbial composition analysis, and diversity assessments were performed using soil sample sizes of 0.2, 1, and 5 g. This study focused on the relationship between soil amount and DNA extraction container volume and the alteration in microbial composition at different taxonomic ranks (order, class, and phylum). Horizontal (0.2 and 1 g) and vertical (5 g) shaking were applied during DNA extraction for practical use in a small laboratory. In the case of the 5 g soil sample, DNA extraction efficiency and the value of α-diversity index fluctuated severely, possibly because of vertical shaking. Regarding the 0.2 and 1 g soil samples, the number of taxa, Shannon-Wiener index, and Bray-Curtis dissimilarity were stable and had approximately the same values at each taxonomic rank. However, non-metric multidimensional scaling showed that the microbial compositions of these two sample sizes were different. The higher relative abundance of taxa in the case of the 0.2 g soil sample might indicate that cell wall compositions differentiated the microbial community structures in these two sample sizes due to high shear stress tolerance. The soil sample size and tube volume affected the estimated microbial community structure. A soil sample size of 0.2 g would be preferable to the other sample sizes because of the possible higher shearing force for DNA extraction and lower experimental costs due to smaller amounts of consumables. When the taxonomic rank was changed from order to phylum, some minor taxa identified at the order rank were integrated into major taxa at the phylum rank. The integration affected the value of the ß-diversity index; therefore, the microbial community structure analysis, reproducibility of structures, diversity assessment, and detection of minor taxa would be influenced by the taxonomic rank applied.

Nitrous oxide emissions during biological soil disinfestation with different organic matter and plastic mulch films in laboratory-scale tests.

Nitrous oxide (N2O), which is a greenhouse gas, may be more emitted as an intermediate product of denitrification during biological soil disinfestation. The biological soil disinfestation is a method to suppress soil-borne pathogens under reductive soil conditions produced by the application of organic matter and water irrigation with plastic film. The objective of the study was to determine the effects of different organic matter and mulch films on N2O emissions during biological soil disinfestation. Grey lowland soil amended with cattle compost plus rice bran (0.2%), rice husk (0.2%) or dent corn (0.1%, 0.2% and 0.4%) was incubated at 100% water-holding capacity with or without plastic films made of polyvinyl chloride (PVC) and triple-layer polyolefin (3PO) for 72 h at 50°C. Permeation of the two films was also measured at 25°C and 50°C. Results showed that incorporation of organic matter increased N2O emissions compared with no organic matter addition at 50°C. Incorporation of rice bran and dent corn with easily decomposable C and low C:N ratios increased N2O emissions for the first 12 h, but thereafter, available C supply from these amendments suppressed N2O emissions. Permeability of mulch films increased at a higher temperature and was larger for PVC than for 3PO. Our study indicated that rice husk should not be used for soil disinfestation and that application rates of organic matter must be determined based on their decomposability. Moreover, mulch film covering would not suppress N2O emission in biological soil disinfestation because of high temperature.

Utilization of water chestnut for reclamation of water environment and control of cyanobacterial blooms.

Overgrowth of water chestnut (Trapa spp.) is a regional problem throughout Asia and North America because of waterway blockage and water fouling upon decomposition. In the present study, we investigated the potential of water chestnut to control cyanobacterial blooms, via a high content of phenolic compounds. In addition, we assessed the impact of biomass harvesting and crude extract application on nutrient balance. We showed that the floating parts of water chestnut contained high concentrations of total phenolics (89.2 mg g(-1) dry weight) and exhibited strong antioxidant activity (1.31 mmol g(-1) dry weight). Methanol-extracted phenolics inhibited growth of Microcystis aeruginosa; the half maximal effective concentration (EC50) of the extracted phenolics was 5.8 mg L(-1), which was obtained from only 103 mg L(-1) of dry biomass (the floating and submerged parts). However, the crude extracts also added important quantities of nitrogen, phosphorus, and potassium (1.49, 1.05, and 16.3 mg g(-1), respectively; extracted dry biomass weight basis); therefore, in practice, nutrient removal before and/or after the extraction is essential. On the other hand, biomass harvesting enables recovery of nitrogen, phosphorus, and potassium from the water environment (23.1, 2.9, and 18.7 mg g(-1), respectively; dry biomass weight basis). Our findings indicate that water chestnut contains high concentrations of phenolics and exhibits strong antioxidant activity. Utilization of these resources, including nutrients, will contribute to reclamation of the water environment, and also to disposal of wet biomass.

Cascade utilization of water chestnut: recovery of phenolics, phosphorus, and sugars.

Overgrowth of aquatic plants, such as water chestnut, has been reported as a regional problem in various areas. We proposed cascade utilization of water chestnut through the recovery of phenolics, phosphorus, and sugars. Phenolics were extracted using 50 g (wet weight) of biomass with 300 mL of acetone, methanol, or hot water, and the yields of total phenolics were 80.2, 56.2, and 49.7 mg g(-1) dry weight of native biomass, respectively. The rate of eluted phosphorus in the phenolic extraction step was 8.6, 14.8, and 45.3 % of that in the native biomass, respectively, indicating that the use of polar organic solvents suppressed phosphorus elution at the phenolic extraction step. Extraction of phosphorus following the phenolic extraction was combined with alkaline pretreatment (1 % NaOH solution) of biomass for saccharification; 64.1 and 51.0 % of phosphorus in the native biomass were extracted using acetone and methanol for the phenolic extraction, respectively. Saccharification following the alkaline pretreatment showed that the glucose recovery rates were significantly increased (p<0.05) with the phenolic extraction step compared to alkaline pretreatment alone. This finding indicates that extraction of phenolics not only provides another useful material but also facilitates enzymatic saccharification.