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.ope

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.ope

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.ope

Ifosfamide-induced Fanconi syndrome with diabetes insipidus

Ifosfamide-induced Fanconi syndrome is a rare complication that typically occurs in young patients due to a cumulative dose of ifosfamide > 40–60 g/m2, a reduction in kidney mass, or concurrent cisplatin treatment. It is usually characterized by severe and fatal progression accompanied by type II proximal renal tubular dysfunction, as evidenced by glycosuria, proteinuria, electrolyte loss, and metabolic acidosis. Diabetes insipidus is also a rare complication of ifosfamide-induced renal disease. We herein describe a case involving a 61-year-old man who developed ifosfamide-induced Fanconi syndrome accompanied by diabetes insipidus only a few days after the first round of chemotherapy. He had no known risk factors. In addition, we briefly review the mechanisms and possible therapeutic options for this condition based on other cases in the literature. Patients who receive ifosfamide must be closely monitored for renal impairment to avoid this rare but fatal complication.ope

Phenotypic and functional characteristics of swine T lymphocyte subpopulations activated by nonspecific immunostimulator Barodon[ⓡ]

학위논문(박사)--서울대학교 대학원 :수의학과 수의미생물학전공,2000.Docto

D-dimer predicts poor hospitalisation outcomes in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis

Objectives: The in-hospital mortality rate among patients with antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is high. Unfortunately, there is no reliable prognostic biomarker. This study aimed to investigate whether elevated D-dimer levels can predict hospitalisation outcomes among patients with AAV. Methods: We performed a retrospective analysis at a tertiary medical centre in Seoul, South Korea, between 2005 and 2019. Patients with AAV requiring hospitalisation, whose D-dimer levels were available within one week of hospitalisation, were included; patients with known alternative reasons for elevated D-dimer were excluded. Death and intensive care unit requirements were defined as adverse outcomes. Results: In total, 61 AAV patients with a total of 100 episodes of hospitalisation were included. Median D-dimer levels were significantly higher in patients with adverse outcomes than in those without adverse outcomes (1.84 vs. 0.42 mg/dL; p=0.006). Consistently, the incidence of the adverse outcomes was significantly higher in the high D-dimer group (≥0.699 mg/dL; n = 40) than in the low D-dimer group (<0.699 mg/dL; n = 60) (35% vs. 10%; p=0.002). Multivariate logistic regression analysis revealed that a high D-dimer level was a significant risk factor for adverse outcomes (hazard ratio, 4.852; 95% confidence interval, 1.320-17.833; p=0.017). Kaplan-Meier survival analysis revealed that the high D-dimer group was associated with more 30-day in-hospital adverse outcomes than the low D-dimer group (p=0.008). Conclusions: High D-dimer levels on admission are significantly associated with adverse outcomes among patients with AAV.restrictio

Will the HALP score help to assess the activity and predict the prognosis of antineutrophil cytoplasmic antibody-associated vasculitis?

restrictio

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.restrictio

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.restrictio

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 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. >.restrictio