Male Sex Is a Significant Predictor of All-cause Mortality in Patients with Antineutrophil Cytoplasmic Antibodyassociated Vasculitis

Background@#We investigated and compared the initial clinical features at diagnosis and the poor outcomes during follow-up in Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) based on sex. @*Methods@#The medical records of 223 immunosuppressive drug-naïve patients with AAV were reviewed. Age, body mass index (BMI), smoking history, AAV subtypes, ANCA positivity, clinical manifestations, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis were collected. All-cause mortality, end-stage renal disease (ESRD), cerebrovascular accident (CVA) and cardiovascular disease (CVD) were assessed as the poor outcomes of AAV during follow-up. @*Results@#The median age was 59.0 years and 74 of 223 AAV patients (33.2%) were men.Among variables at diagnosis, male patients exhibited higher BMI than female. However, there were no differences in other demographic data, AAV subtypes, ANCA positivity, BVAS, FFS, ESR and CRP between the two groups. Male patients received cyclophosphamide more frequently, but there were no significant differences in the frequencies of the poor outcomes of AAV between the two groups. Male patients exhibited a significantly lower cumulative patients' survival rate than female patients during the follow-up period based on all-cause mortality (P = 0.037). In the multivariable analysis, both male sex (hazard ratio [HR], 2.378) and FFS (HR, 1.693) at diagnosis were significantly and independently associated with allcause mortality during follow-up. @*Conclusion@#Male sex is a significant and independent predictor of all-cause mortality in AAV patients.

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. @*Conclusions@#Evaluation of VAT using CT is useful for estimating disease activity and all-cause mortality in patients with AAV.

Male Sex Is a Significant Predictor of All-cause Mortality in Patients with Antineutrophil Cytoplasmic Antibodyassociated Vasculitis

Background@#We investigated and compared the initial clinical features at diagnosis and the poor outcomes during follow-up in Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) based on sex. @*Methods@#The medical records of 223 immunosuppressive drug-naïve patients with AAV were reviewed. Age, body mass index (BMI), smoking history, AAV subtypes, ANCA positivity, clinical manifestations, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis were collected. All-cause mortality, end-stage renal disease (ESRD), cerebrovascular accident (CVA) and cardiovascular disease (CVD) were assessed as the poor outcomes of AAV during follow-up. @*Results@#The median age was 59.0 years and 74 of 223 AAV patients (33.2%) were men.Among variables at diagnosis, male patients exhibited higher BMI than female. However, there were no differences in other demographic data, AAV subtypes, ANCA positivity, BVAS, FFS, ESR and CRP between the two groups. Male patients received cyclophosphamide more frequently, but there were no significant differences in the frequencies of the poor outcomes of AAV between the two groups. Male patients exhibited a significantly lower cumulative patients' survival rate than female patients during the follow-up period based on all-cause mortality (P = 0.037). In the multivariable analysis, both male sex (hazard ratio [HR], 2.378) and FFS (HR, 1.693) at diagnosis were significantly and independently associated with allcause mortality during follow-up. @*Conclusion@#Male sex is a significant and independent predictor of all-cause mortality in AAV patients.

Antineutrophil Cytoplasmic Antibody Positivity Is Associated with Vascular Involvement in Behçet’s Disease

Purpose@#We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up. @*Materials and Methods@#We retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis. @*Results@#Among the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up. @*Conclusion@#ANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.

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. @*Conclusions@#Evaluation of VAT using CT is useful for estimating disease activity and all-cause mortality in patients with AAV.

Rituximab Biosimilar Prevents Poor Outcomes of Microscopic Polyangiitis and Granulomatosis with Polyangiitis as Effectively as Rituximab Originator

Purpose@#There has been no extensive study to compare the efficacy between rituximab originator (Mabthera®) and its biosimilar (Truxima®) for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Here, we investigated the clinical effects of rituximab on poor outcomes of MPA and GPA in Korean patients, and compared those between Mabthera® and Truxima®. @*Materials and Methods@#We retrospectively reviewed the medical records of a total of 139 patients, including 97 MPA patients and 42 GPA patients. At diagnosis, antineutrophil cytoplasmic antibody positivity and comorbidities were assessed. During follow-up, all-cause mortality, relapse, end-stage renal disease, cerebrovascular accident and acute coronary syndrome were evaluated as poor outcomes. In this study, rituximab was used as either Mabthera® or Truxima®. @*Results@#The median age at diagnosis was 60.1 years and 46 patients were men (97 MPA and 42 GPA patients). Among poor outcomes, patients receiving rituximab exhibited a significantly lower cumulative relapse-free survival rate compared to those not receiving rituximab (p=0.002). Nevertheless, rituximab use did not make any difference in other poor outcomes of MPA and GPA except for relapse, which might be a rebuttal to the fact that rituximab use after relapse eventually led to better prognosis. There were no significant differences in variables at diagnosis and during follow-up between patients receiving Mabthera® and those receiving Truxima®. Patients receiving Truxima® exhibited a similar pattern of the cumulative survival rates of each poor outcome to those receiving Mabthera®. @*Conclusion@#Truxima® prevents poor outcomes of MPA and GPA as effectively as does Mabthera®.

Tuberculous Peritonitis Mimicking Primary Peritoneal Carcinoma

Tuberculous peritonitis is one of the most common extrapulmonary tuberculosis. The presenting signs and symptoms, together with the carbohydrate antigen (CA) 125 status and imaging findings may resemble the primary peritoneal carcinoma or ovarian carcinoma. We herein report a case on a 71-year-old woman who is presented with abdominal distension, abdominal pain, nausea, anorexia. Abdomino-pelvic computed tomography scans reveal large amounts of ascites and mottled omentum with diffuse nodular masses, and the serum CA 125 level is elevated. The initial clinical diagnosis is the primary peritoneal carcinoma, but the final histological diagnosis confirms the tuberculous peritonitis. Thus, we discuss the differential diagnosis of tuberculous peritonitis from primary peritoneal carcinoma and also the problems especially found in old aged patients. In conclusion, although the elderly patients are suspected with malignancy, we should keep in mind the possibility of curable diseases and perform laparoscopic biopsy during the early stage aggressively.

A Case of Bronchial Artery Aneurysm in Cavitary Pulmonary Tuberculosis / 대한내과학회지

A bronchial artery (BA) aneurysm is a rare, life-threatening disease when it ruptures. Recently, we experienced a case of massive hemoptysis due to a BA aneurysm rupture in a pulmonary tuberculosis cavity, treated with BA embolization followed by surgical resection of the cavitary lesion. To our knowledge, this is the first case of a BA aneurysm associated with cavitary pulmonary tuberculosis.

A Case of Bronchial Artery Aneurysm in Cavitary Pulmonary Tuberculosis / 대한내과학회지

A bronchial artery (BA) aneurysm is a rare, life-threatening disease when it ruptures. Recently, we experienced a case of massive hemoptysis due to a BA aneurysm rupture in a pulmonary tuberculosis cavity, treated with BA embolization followed by surgical resection of the cavitary lesion. To our knowledge, this is the first case of a BA aneurysm associated with cavitary pulmonary tuberculosis.

Effective Treatment of Intractable Diarrhea Associated with Common Variable Immunodeficiency with Oral Budesonide / 대한내과학회지

Common variable immunodeficiency (CVID) is the most common symptomatic primary antibody deficiency syndrome and has a high prevalence of gastrointestinal complications. We report the case of a 36-year-old male with CVID who presented with chronic intractable diarrhea and malabsorption. A comprehensive evaluation revealed no secondary causes of his symptoms. He was treated symptomatically without improvement. After receiving systemic steroid treatment, his symptoms improved, but returned soon after tapering the steroid. When he was subsequently administered oral budesonide, his symptoms and quality of life improved; this effect lasted for 20 months without unacceptable side effects.

A Case of a Huge Biliary Bezoar Treated with ESWL / 대한췌담도학회지

A bezoar is a mass formed by the accumulation of digested food. A biliary bezoar, namely a bezoar formed in the bile duct, is rare, which occurs mostly in patients who formerly underwent cholecystectomy. It appears that incompetent sphincter produces occasional reverse flow of foreign bodies including undigested food from the alimentary canal into the bile tract. Surgical treatment is used for huge biliary bezoar which is too huge to be removed by nonsurgical treatment. Extracorporeal shock wave lithotripsy (ESWL) is mostly used to fragment renal or urinary calculi, but may be sparingly used against biliary calculi. This is to report the rare case of a patient with biliary bezoar who formerly underwent open cholecystectomy and choledocoduodenostomy. The patient's huge biliary bezoar was removed by ESWL and the successive execution of endoscopic retrograde cholangio pancreatography (ERCP). ESWL is expected to be a comparatively safe and convenient solution to huge biliary bezoars.

Clinical Utility of Chimerism Status Assessed by Lineage-Specific Short Tandem Repeat Analysis: Experience from Four Cases of Allogeneic Stem Cell Transplantation / 대한진단검사의학회지

Chimerism testing permits early prediction and documentation of successful engraftment, and also facilitates detection of impending graft rejection. In this study, we serially monitored chimerism status by short tandem repeat-based PCR in nucleated cells (NC), T cells and natural killer (NK) cells after myeloablative allogeneic stem cell transplantation (SCT). Four patients with myeloid malignancies showed discrepant chimerism results among those three fractions. Three patients had mixed chimerism (MC) of donor/host T cells at a time point around the onset of chronic graft-versus-host disease (GVHD). In two patients with disease relapse, MC of NK cells preceded a morphological relapse or NK cells showed a higher percentage of patient cells compared to NC. Therefore, our study shows that chimerism analysis in lineage-specific cells might be useful in predicting clinical outcome after allogeneic SCT in certain patients.