Comparison of the Characteristics of Three Acellular Dermal Matrices Subjected to Distinct Processing Methods Using Five Types of Histochemical Staining.

BACKGROUND: Acellular dermal matrix (ADM) is treated using various devitalization and aseptic processing methods. The processing effects on ADM were evaluated by histochemical tests. METHODS: From January 2014 to December 2016, 18 patients [average age, 43.0 (range, 30-54) years] who underwent breast reconstruction with an ADM and tissue expander were prospectively enrolled. During the permanent implant replacement, a biopsy of the ADM was performed. We used three different human-derived products, namely, Alloderm®, Allomend®, and Megaderm®. Hematoxylin and eosin, CD68, CD3, CD31, and smooth muscle actin were used to evaluate the collagen structure, inflammation, angiogenesis, and myofibroblast infiltration. Each ADM was semi-quantitatively analyzed. RESULTS: Significant differences in collagen degradation, acute inflammation, and myofibroblast infiltration were observed among the ADMs. Collagen degeneration (p<0.001) and myofibroblast infiltration (smooth muscle actin-positive, p=0.018; CD31-negative, p=0.765) were the most severe in Megaderm®. Acute inflammation, represented by CD68, was most severe in Alloderm® (p=0.024). Both radiation and freeze-drying treatment physically damaged the collagen structure. Collagen degeneration was most severe in Megaderm®, followed by Allomend® and Alloderm®. Since Alloderm® is treated using chemicals, an assessment of the chemical irritation is warranted. CONCLUSIONS: The biopsy results were inconclusive. Therefore, to better interpret processing, more large-scale, serial, histochemical studies of each ADM are needed. LEVEL OF EVIDENCE IV: This journal requires that authors 38 assign a level of evidence to each article. For a full 39 description of these Evidence-Based Medicine ratings, 40 please refer to the Table of Contents or the online 41 Instructions to Authors www.springer.com/00266 .

Modified Keystone Perforator Island Flap Techniques for Small- to Moderate-Sized Scalp and Forehead Defect Coverage: A Retrospective Observational Study.

We aimed to demonstrate the effective application of keystone perforator island flap (KPIF) in scalp and forehead reconstruction by demonstrating the authors' experience with modified KPIF reconstruction for small- to moderate-sized scalp and forehead defects. Twelve patients who underwent modified KPIF reconstruction of the scalp and forehead from September 2020 to July 2022 were enrolled in this study. In addition, we retrospectively reviewed and evaluated the patient's medical records and clinical photographs. All defects (size range, 2 cm × 2 cm to 3 cm × 7 cm) were successfully covered using four modified KPIF techniques (hemi-KPIF, Sydney Melanoma Unit Modification KPIF, omega variation closure KPIF, and modified type II KPIF) with ancillary procedures (additional skin grafts and local flaps). All flaps (size range, 3.5 cm × 4 cm to 7 cm × 16 cm) fully survived, and only one patient developed marginal maceration that healed with conservative management. Furthermore, through the final scar evaluation with the patient satisfaction survey and Harris 4-stage scale, all patients were satisfied with their favorable outcomes at the average final follow-up period of 7.66 ± 2.14 months. The study showed that the KPIF technique with appropriate modifications is an excellent reconstructive modality for covering scalp and forehead defects.

Modified Keystone Perforator Island Flap for Tension-Reducing Coverage of Axillary Defects Secondary to Radical Excision of Chronic Inflammatory Skin Lesions: A Retrospective Case Series.

Axillary defect coverage is often challenging after radical excision of chronic inflammatory skin lesions, such as complicated epidermoid cysts and hidradenitis suppurativa. This retrospective case series aims to demonstrate our experience with axillary reconstruction using the modified keystone perforator island flap (KPIF) technique, emphasizing its tension-reducing effects. All patients who presented for axillary reconstruction after radical excision of chronic inflammatory skin lesions between May 2019 and December 2020 were identified using the medical record database. Eleven patients ranging in age from 17 to 71 years underwent modified KPIF axillary reconstruction. Four types of modifications (modified type II KPIF, omega variation closure, Sydney melanoma unit modification, and hemi-KPIF) were used. All defects (size range, 2.5 × 3 cm2 to 8 × 13 cm2) were successfully covered using these modified KPIF techniques. All flaps (size range, 3.5 × 3.5 cm2 to 11 × 30 cm2) fully survived without complications. All patients exhibited favorable functional outcomes, and no cases of recurrence or limitations in joint range of motion were observed during the follow-up period (range, 4-5 months). Modified KPIF techniques may represent a reliable, effective alternative reconstructive modality for managing axillary defects.

Modified Mini-Keystone Flaps for Coverage of Tiny Volar Pulp Defects of the Fingertips in Cases with Missing Amputation Skin Stumps: A Retrospective Study.

This study aimed to demonstrate the expanding versatility of keystone flap reconstruction in fingertips. Fifteen patients who underwent the modified mini-keystone flap reconstruction for tiny volar pulp defects of the fingertip between September 2020 and February 2021 were included in this study (average age: 43.4 ± 13.52 years, range: 19-61 years). Patient data were retrospectively collected from their medical records. The two-point discrimination test was used to evaluate the degree of sensory recovery. All defects were successfully covered with the modified mini-keystone flap. The defect sizes ranged from 0.5 cm × 1 cm to 1.2 cm × 2.0 cm, and the flap sizes ranged from 0.7 cm × 1.5 cm to 1.5 cm × 3.0 cm. Although one patient showed a small distal margin maceration, all flaps survived fully. The overall outcomes were favorable at the mean follow-up period of 5.73 ± 0.79 months. We suggest that the modified mini-keystone flap technique is a promising alternative modality for covering tiny volar pulp defects of the fingertip, with few complications and favorable outcomes.

Clinical and imaging findings suggestive of histopathological immunoglobulin G4-related disease: a single-center retrospective study.

OBJECTIVE: To investigate the clinical and imaging features predicting the histopathological diagnosis of immunoglobulin G4 (IgG4)-related disease (IgG4RD) in patients with suspected IgG4RD on computed tomography (CT). METHODS: We retrospectively reviewed the medical records of 178 patients with CT findings suspicious of IgG4RD from January 2015 to December 2017. Patients who underwent tissue biopsy were included to evaluate the association between patient characteristics and histopathological diagnosis of IgG4RD. The histopathological diagnosis was classified into IgG4RD and non-IgG4RD. Clinical, laboratory, and imaging features were compared between patients with IgG4RD and non-IgG4RD, and logistic regression analysis was performed to identify the predictors for histopathologically confirmed IgG4RD. RESULTS: Of the 103 patients with histopathologically proven diseases, 46 and 57 patients were classified as IgG4RD and non-IgG4RD, respectively. The median age was 64 years; 65% of patients were male. There were significant differences in sex (P = 0.035), fever (P = 0.039), serum IgG4 level (P < 0.001), renal involvement (P = 0.036), lacrimal or salivary glands involvement (P = 0.050), swelling pattern on CT (P = 0.001), and positron emission tomography (PET)-CT findings (P < 0.001) between patients with IgG4RD and non-IgG4RD. Multivariate analysis revealed elevated IgG4 level > 135 mg/dL (odds ratio [OR] = 5.418, P < 0.001), kidney involvement (OR = 6.170, P = 0.044), and the swelling feature on CT (OR = 4.301, P = 0.012) to be independent factors for histopathological diagnosis of IgG4RD. CONCLUSION: This study suggests that elevated IgG4 level, renal involvement, and swelling pattern on CT are associated with histopathological diagnosis of IgG4RD. The clinical and imaging features might help to decide further evaluation in patients with clinically suspected IgG4RD. Key Points • Computed tomography (CT) is not sufficient to discriminate between IgG4-related disease (IgG4RD) and non-IgG4RD conditions. • Histopathological diagnosis of IgG4RD is associated with elevated IgG4 level, renal involvement, and swelling pattern on CT. • Positron emission tomography-CT may be a useful diagnostic tool in patients with clinically suspected IgG4RD. >.

Evaluation of body composition using computed tomography in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

BACKGROUND/AIMS: Measures of body composition, including visceral adipose tissue (VAT), subcutaneous adipose tissue (SAT), and skeletal muscle area (SMA), are considered important prognostic factors in chronic diseases. The association of these measures with auto-inflammatory disorders, such as anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), remains unclear. We investigated the clinical significance of VAT, SAT, and SMA in patients with AAV. METHODS: Patients with AAV subjected to chest computed tomography (CT), abdominal CT, or positron emission tomography-CT on diagnosis of AAV were evaluated. Quantitative assessment of VAT, SAT, and SMA was performed at the third lumbar vertebral level and computed by summing the pixel attenuation for tissue-specific Hounsfield units in the corresponding region. Associations of VAT, SAT, and SMA with clinical and laboratory data and clinical outcome measures were evaluated. RESULTS: Of the 117 patients, 61 (52.1%) were classified as having microscopic polyangiitis, 28 (23.9%) as granulomatosis with polyangiitis, and 28 (23.9%) as eosinophilic granulomatosis with polyangiitis. VAT significantly correlated with age, weight, body mass index (BMI), and Birmingham Vasculitis Activity Score, whereas SAT correlated with weight, BMI, and creatinine levels. A significant association was found between SMA and age, height, weight, BMI, and the Five-Factor Score. Cox proportional hazards analysis showed that creatinine levels (odds ratio [OR], 1.346; 95% confidence interval [CI], 1.034 to 1.753; p = 0.027) and high VAT (OR, 7.137; 95% CI, 1.343-37.946; p = 0.021) were independently associated with all-cause mortality during follow-up. CONCLUSION: Evaluation of VAT using CT is useful for estimating disease activity and all-cause mortality in patients with AAV.

Prevalence of Osteopenia in Drug-Naive Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Monocentric Study.

OBJECTIVES: To examine the bone mineral density and prevalence of osteoporosis and osteopenia in glucocorticoid- and immunosuppressive drug-naive patients younger than 55 years with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: This was a cross-sectional study. We reviewed the medical records of 35 AAV patients and 35 age-, sex-, and body mass index (BMI)-matched control subjects. We collected clinical data such as AAV-related variables and conventional risk factors for osteoporosis and assessed bone mineral density and the prevalence of osteoporosis and osteopenia in both groups. Categorical and continuous variables were compared between the 2 groups using the χ2 or Fisher exact test and Mann-Whitney U test, respectively. Multivariate logistic regression analysis was used to calculate the odds ratio (OR). RESULTS: There were no statistically significant differences between the demographical data of AAV patients and control subjects. Patients with AAV showed significantly higher frequencies of conventional risk factors for osteoporosis than the control subjects, except for hyperthyroidism. Osteopenia was found more commonly in AAV patients than in control subjects (57.1% vs. 31.4%, p = 0.030). In the univariate logistic regression analysis, BMI (OR, 0.813) and AAV (OR, 2.620) were associated with osteopenia in all participants. In the multivariate analysis, both BMI and AAV were associated with osteopenia, but this was not statistically significant. In contrast, when analyzing AAV patients only, neither conventional risk factors nor AAV-related variables were associated with the prevalence of osteopenia. CONCLUSIONS: Antineutrophil cytoplasmic antibody-associated vasculitis and BMI were both associated with osteopenia.

Clinical features of Korean elderly patients with antineutrophil cytoplasmic antibody-associated vasculitis.

BACKGROUND/AIMS: We compared the clinical and laboratory data between elderly and non-elderly patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) at diagnosis; further, we investigated the predictors at diagnosis for all-cause mortality and end-stage renal disease (ESRD) occurrence during follow-up in Korean elderly patients with AAV. METHODS: We reviewed the medical records of 191 AAV patients regarding clinical manifestations and laboratory results at diagnosis and during follow-up. The follow-up duration was defined as the period from diagnosis to death for deceased patients or to the time of dialysis for ESRD patients, or to the last visit. Elderly (n = 67) and non-elderly (n = 124) patients were grouped based on an age threshold of 65 years. RESULTS: At diagnosis, elderly patients exhibited higher median Birmingham Vasculitis Activity Score (BVAS) and higher frequencies of ANCA positivity and pulmonary manifestations than non-elderly patients. Furthermore, elderly patients exhibited increased median white blood cell count, blood urea nitrogen (BUN), alkaline phosphatase, erythrocyte sedimentation rate, and C-reactive protein and decreased median hemoglobin. However, there were no significant differences in all-cause mortality and ESRD occurrence between elderly and non-elderly patients. Meanwhile, elderly patients exhibited lower cumulative patients' and ESRD-free survival rates than non-elderly patients. In the multivariable Cox hazards model, BUN, creatinine and serum albumin at diagnosis were independent predictors for ESRD occurrence, whereas there were no independent predictors at diagnosis for all-cause mortality. CONCLUSION: Elderly AAV patients exhibited substantially higher rates of all-cause mortality and ESRD occurrence during follow-up compared than non-elderly AAV patients.

Serum Mannose-Binding Lectin Levels Are Correlated with the Disease Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Single-Center Study.

Mannose-binding lectin (MBL) is a soluble pattern-recognition molecule, which plays a crucial role in the innate immune system and the activation of lectin complement pathway. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease affecting the small vasculatures and is characterized by the alteration of innate and adaptive immunity and complement activation. In this study, we investigated whether serum MBL is associated with disease activity of AAV, which was measured by ELISA. Associations between serum MBL and AAV-specific indices, as well as clinical and laboratory data were assessed using Kendall's tau. Among the 80 patients, 42 (52.5%), 21 (26.3), and 17 (21.3%) patients were classified as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), respectively. The median values of erythrocyte sedimentation rate, C-reactive protein, and serum MBL were 36.5 (normal range < 20) mm/h, 2.4 (normal range < 8) mg/dL, and 8.6 ng/mL, respectively. The median serum levels of MBL in MPA, GPA, and EGPA patients were 8.4, 9.3, and 8.2 ng/mL. Correlation analysis showed that serum MBL was associated with Birmingham Vasculitis Activity Score (BVAS) (R = 0.169, p = 0.027), but not with other AAV-specific indices and clinical and laboratory data. In addition, serum MBL was significantly associated with the pulmonary manifestation score based on BVAS (R = 0.247, p = 0.001). In summary, among the AAV-specific indices and clinical and laboratory variables analyzed, serum MBL is correlated with BVAS and pulmonary manifestation score based on the BVAS.

Clinical characteristics and long-term outcomes of Libman-Sacks endocarditis in patients with systemic lupus erythematosus.

OBJECTIVE: Treatment of Libman-Sacks (LS) endocarditis in patients with systemic lupus erythematosus (SLE) is challenging due to the lack of data. This study aimed to identify the clinical characteristics of SLE patients and LS endocarditis, and to investigate the treatment and prognosis of LS endocarditis. METHODS: Of all the patients with SLE who underwent echocardiography between 2010 and 2019, 11 and 29 patients with and without LS endocarditis, respectively, were included. We compared the inflammatory and thrombotic profiles between patients with and without LS endocarditis, and investigated the treatment and long-term outcome of LS endocarditis. RESULTS: No significant differences were observed in disease activity, clinical characteristics and inflammatory marker levels between patients with and without LS endocarditis. Patients with LS endocarditis had a significantly higher prevalence of antiphospholipid antibody (aPL) but a lower prevalence of SLE-specific antibody. Triple positivity of aPL was found in 72.7% and 13.8% of patients with and without LS endocarditis, respectively. Of 11 patients with LS endocarditis, six patients received anticoagulation therapy, and five patients received augmented immunosuppressive therapies. One patient who did not receive anticoagulation therapy developed cerebral infarction. Nine (82%) patients with LS endocarditis were classified as having antiphospholipid syndrome (APS). Despite the residual vegetation and valve dysfunction, surgical intervention was not required during the follow-up period of 56.8 months. CONCLUSION: A significant correlation was observed between APS and LS endocarditis. Anticoagulation therapy should be considered to prevent thromboembolic complications in SLE patients with LS endocarditis.

Clinical implication of plasma exchange on life-threatening antineutrophil cytoplasmic antibody-associated vasculitis.

BACKGROUND: We assessed the rate of and predictors for all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) receiving plasma exchange (PLEX) and evaluated the survival benefit of PLEX for diffuse alveolar haemorrhage (DAH) between AAV patients receiving PLEX and those not receiving PLEX. METHODS: We retrospectively reviewed the medical records of 212 patients with AAV. Demographic, clinical and laboratory data at the time of PLEX were collected from nine patients receiving PLEX, six of whom had DAH. The follow-up duration was defined as the period from the time of PLEX or DAH occurrence to death for the deceased patients and to the last visit for the survived patients. RESULTS: The median age of nine AAV patients receiving PLEX was 71.0 years, and five patients were men. Four of nine patients receiving PLEX died at a median follow-up duration of 92.0 days. Three patients died of sepsis and one died owing to a lack of response to PLEX. When patients with DAH receiving or not receiving PLEX were compared, there were no significant differences in variables between the two groups. The cumulative patients' survival rate between patients with DAH receiving and not receiving PLEX were also compared using the Kaplan-Meier survival analysis; however, no survival-benefit of PLEX for DAH was observed. CONCLUSION: The rate of all-cause mortality in nine AAV patients receiving PLEX was found to be 44.4% and the notion that PLEX is beneficial for the improvement in the prognosis of AAV-related DAH was deemed controversial.

Hyperuricemia is associated with decreased renal function and occurrence of end-stage renal disease in patients with microscopic polyangiitis and granulomatosis with polyangiitis: a retrospective study.

Current evidence suggests that high uric acid levels are associated with accelerated renal damage. However, the clinical impact of serum uric acid level on patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) is unknown. We aimed to evaluate the impact of hyperuricemia on such patients. A retrospective study was performed to obtain patients' demographic, clinical, and laboratory data from when they were diagnosed with MPA and GPA. Multivariable logistic regression and Cox hazard model analyses were performed to evaluate factors associated with hyperuricemia at diagnosis and predictive factors of end-stage renal disease (ESRD) development. Among 156 patients, 35 (22.4%) had hyperuricemia at baseline. Hyperuricemic patients had renal manifestation and impaired renal function more frequently than non-hyperuricemic patients. Logistic regression analysis revealed that serum creatinine was significantly associated with hyperuricemia at diagnosis [odds ratio 1.995; 95% confidence interval (CI), 1.503-2.648; P < 0.001]. Cox hazard model analysis revealed that body mass index and serum creatinine were significantly associated with ESRD when all variables were included, but hyperuricemia was independently associated with ESRD [hazard ratio (HR), 3.799; 95% CI 1.719-8.222; P < 0.001) when serum creatinine was excluded. Additionally, in a subgroup analysis of patients with decreased glomerular filtration rates (GFRs), serum uric acid was the sole predictor of ESRD (HR, 1.243; 95% CI 1.048-1.475; P = 0.013). Hyperuricemia is associated with renal damage and ESRD occurrence in MPA and GPA patients. Serum uric acid level is associated with ESRD occurrence in patients with decreased GFRs.

Double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane antibody could predict end-stage renal disease in ANCA-associated vasculitis: a monocentric pilot study.

OBJECTIVES: To investigate the detection rate of double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody at diagnosis and its clinical implication during follow-up in Korean patients with ANCA-associated vasculitis (AAV). METHODS: We retrospectively reviewed the medical records of 96 Korean AAV patients. We obtained data at diagnosis and assessed the poor outcomes of AAV such as all-cause mortality, relapse and end-stage renal disease (ESRD). Comparison of cumulative survivals were analysed by the Kaplan-Meier survival analysis, and hazard ratios (HRs) were obtained by the multivariable Cox hazard model. RESULTS: Seven of 96 AAV patients (7.3%) had double positivity for ANCA and anti-GBM. Among variables at diagnosis, there were no significant differences between patients with double positivity for ANCA and anti-GBM and those without. In the cumulative survival analysis, AAV patient with double positivity for ANCA and anti-GBM at diagnosis exhibited the lower cumulative ESRD-free survival rate than those without (P = 0.044) and furthermore, than those with positive for only ANCA and those with double negativity (P = 0.022). Myeloperoxidase (MPO)-ANCA, renal manifestation and five-factor score at diagnosis were also associated with ESRD occurrence in AAV patients. In the multivariable Cox hazards model using these 4 variables, only double positivity for ANCA and anti-GBM exhibited the significant association with ESRD occurrence during follow-up (HR 3.831). CONCLUSIONS: Double positivity for ANCA and anti-GBM at diagnosis were observed in 7.3% of AAV patients, and it could predict ESRD occurrence during follow-up in Korean patients with AAV.Key Points• 7.3% of AAV patients had double positivity for ANCA and anti-GBM at diagnosis (total n = 96)• Double positivity for ANCA and anti-GBM could predict ESRD occurrence during follow-up (HR 9.021, P = 0.004)• AAV patients with double positivity for ANCA and anti-GBM exhibited the lower cumulative ESRD-free survival rate compared with those without (P = 0.044).

Predictors of mortality in autoimmune disease patients with concurrent cytomegalovirus infections detected by quantitative real-time PCR.

OBJECTIVE: Cytomegaloviruses (CMV) can have a significant impact on the prognosis of immunocompromised patients. Unlike in the transplantation and AIDS fields, only a few studies on CMV infections have been published in the field of autoimmunity. In this study, we examined the clinical outcomes of CMV infections in patients with autoimmune diseases at a single tertiary medical institution. METHODS: A retrospective study was performed to identify the mortality risk factors associated with CMV infections in patients with autoimmune diseases. We reviewed the medical records of patients with autoimmune diseases who were diagnosed with CMV infections using real-time quantitative polymerase chain reaction between December 2005 and March 2016. Clinical and laboratory parameters as well as treatment outcomes were analyzed. RESULTS: Seventy-three CMV infected patients were separated into survivors and non-survivors. Non-survivors had significantly higher median CMV-DNA copy numbers than survivors (95,500 vs 6,700 copies/mL, p = 0.005) and demonstrated significantly more frequent incidents of CMV pneumonitis (69.2 vs 36.2%, p = 0.007). After adjusting for multiple confounding covariates, the log CMV-DNA copies/mL (hazard ratio, 1.48; 95% confidence interval, 1.14-1.92; p = 0.003) and the presence of concurrent infections (hazard ratio, 22.00; 95% confidence interval, 2.75-175.97, p = 0.004) were identified as independent mortality risk factors. Furthermore, patients with high CMV copy numbers (> 60,000 copies/mL) had higher in-hospital mortality than those with low CMV copy numbers (p < 0.05). CONCLUSIONS: CMV-DNA copy numbers and concurrent infections are predictors of in-hospital mortality in CMV-infected patients with autoimmune diseases. Therefore, serial measurements of CMV-DNA copy numbers and close observation for signs of other infections are recommended for patients with autoimmune diseases who have concurrent CMV infection.

Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease.

OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). METHODS: We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016. The patients with AOSD were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features, laboratory findings, and overall survival were analyzed. Logistic regression analysis was used to evaluate the factors associated with in-hospital mortality. RESULTS: Among 64 patients with AOSD, 36 (56.3%) were classified as having MAS. The overall survival rate was significantly lower in patients with MAS than in those without (67% vs 100%, p < 0.001). Multivariate analysis showed that a low erythrocyte sedimentation rate, a low albumin level, an increase in ferritin of over 2 folds, and the development of MAS on admission were significantly associated with mortality in patients with AOSD. CONCLUSION: The 2016 EULAR/ACR/PRINTO classification criteria for MAS are potentially useful for the identification of patients with AOSD at high risk for a poor outcome. Febrile patients with AOSD should be monitored with the 2016 classification criteria for MAS in the early diagnosis and proper treatment of MAS.

In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome.

OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with febrile systemic lupus erythematosus (SLE). METHODS: We performed a retrospective analysis of SLE patients with fever, who were admitted to Severance Hospital between December 2005 and May 2016. Patients were evaluated for MAS using the 2016 classification criteria for MAS. Clinical features and laboratory findings were compared and overall survival rate was analyzed. Forward and backward stepwise logistic regression analysis was used to evaluate the factors associated with in-hospital mortality. RESULTS: Among 157 patients with SLE, 54 (34.3%) were considered to have MAS on admission (n = 42) and during admission (n = 12). For patients who already have MAS on admission, their baseline laboratory findings demonstrated lower CRP, platelets, total protein, albumin, complement C3, fibrinogen and higher AST, ALT, total bilirubin, ferritin, and triglyceride. The overall survival rate was significantly lower in patients with MAS than without MAS (64.8% vs. 97.0%, p < 0.001). Multivariate analysis showed that the presence of MAS was significantly associated with in-hospital mortality in febrile SLE patients (OR = 64.5; 95% CI: 7.6-544.4; p < 0.001). CONCLUSIONS: The 2016 classification criteria for MAS is useful to identify febrile SLE patients at high risk for in-hospital mortality. Monitoring febrile SLE patients with the new 2016 classification criteria might aid in the early detection of MAS.

Anti-Sm is associated with the early poor outcome of lupus nephritis.

AIM: We investigated whether anti-Smith (Sm) is associated with the outcome of kidney biopsy-proven lupus nephritis. METHODS: We retrospectively analyzed clinical, laboratory and histological results in 90 patients with kidney biopsy-proven lupus nephritis. We defined persistent administration of immunosuppressants for more than 3 months after the kidney biopsy as early poor outcome of lupus nephritis. We compared baseline variables and delta values of lupus nephritis-related variables between patients with and without anti-Sm. The independent predictive values for early poor outcome were analyzed using logistic regression analysis. RESULTS: The median age was 32.0 years old, and 77 patients (85.5%) were women. Anti-Sm was found in 44 of 90 patients (48.8%). When we analyzed the differences in delta values of variables reflecting the kidney function or the early poor outcome between patients with and without anti-Sm, we found significant difference in the early poor outcome between the two groups (80.0% of patients having anti-Sm vs. 56.5% of those not having anti-Sm, P = 0.022). In multivariate logistic regression analysis, along with age and Systemic Lupus Erythematosus Disease Activity Index, the presence of anti-Sm increased the potential of the early poor outcome of lupus nephritis (odds ratio 2.870, 95% confidence interval, 1.033, 7.976, P = 0.043). CONCLUSION: Our data suggest that anti-Sm identified at kidney biopsy might have a predictive value for the early poor outcome of biopsy-proven lupus nephritis during the follow-up period.

Effects of Dietary Supplementation of Barodon, an Anionic Alkali Mineral Complex, on Growth Performance, Feed Utilization, Innate Immunity, Goblet Cell and Digestibility in Olive Flounder (Paralichthys olivaceus).

A 15-wk feeding trial was conducted to examine the supplemental effects of Barodon on growth performance, gastrointestinal histology, feed digestibility and innate immunity in olive founder. A basal commercial diet was used as a control and two other diets were prepared by spraying 0.1% or 0.2% of Barodon. Triplicate groups of fish (BW, 145 g) were fed one of the test diets to apparent satiation twice daily. At the end of the feeding trial, fish growth performance was not significantly affected by dietary treatments; however, feed utilization was significantly improved (linear and quadratic, p<0.05) by Barodon supplementation. Significantly higher (p<0.05) survival rates were obtained in fish fed Barodon containing diets. Hepatosomatic index increased significantly in Barodon treated groups. Also, the use of Barodon resulted in significant increase (linear and quadratic, p<0.05) of intestine length and number of goblet cells. Significantly higher (Quadratic, p<0.05) apparent digestibility coefficient of DM was obtained by supplementation of Barodon. Lysozyme and myeloperoxidase activities increased quadratically and linearly, respectively, in Barodon treated fish. Also, significantly higher (linear and quadratic, p<0.05) superoxide dismutase activity was found in Barodon fed fish. The findings in this study show that inclusion of Barodon in diets for olive flounder improves feed utilization and digestibility, and positively affects digestive tract histology and innate immunity.