Rheumatoid Arthritis-Related Lung Disease and Its Association With Mortality.

PURPOSE: The aim of this study was to determine the association of rheumatoid arthritis-related lung disease (RA-LD) and its subtypes with all-cause mortality. MATERIALS AND METHODS: For the present analyses, patients with RA who underwent computed tomography of the chest (chest-CT) were evaluated. RA-LD was defined in 4 subtypes as follows: interstitial lung disease (RA-ILD), airway disease (RA-AD), rheumatoid pulmonary nodules (RA-PN), and RA-related pleural disease (RA-PD). The date of RA-LD diagnosis was considered the date of the first chest-CT detecting the pathology. To assess the factors associated with mortality, multivariable logistic regression analyses were performed with variables selected based on their causal associations with the outcome. RESULTS: Of 576 RA patients, 253 (43.9%) had RA-LD (38.7% male; mean age at RA-LD diagnosis, 59.9 ± 9.8 years). The most common subtype was RA-AD, which was detected in 119 (47.0%) patients followed by 107 (42.3%) with RA-ILD, 70 (27.7%) with RA-PN, and 31 (12.3%) with RA-PD. Sixty-one (24.1%) patients had 2+ subtypes. After median follow-up of 10.2 years, 97 (16.8%) died. The existence of at least 1 subtype and 2+ subtypes increased the all-cause mortality, as indicated by odds ratios of 1.60 (95% confidence interval [CI], 1.03-2.48) and 2.39 (95% CI, 1.26-4.54), respectively. Among RA-LD patients, RA-ILD and RA-PD were associated with increased mortality (odds ratios were 2.20 [95% CI, 1.18-4.08] and 1.62 [95% CI, 0.70-3.75], respectively). CONCLUSIONS: In this study, RA-AD was the most common subtype, and the presence of RA-LD increased mortality. This effect was particularly pronounced in patients with RA-ILD and RA-PD or those presenting with 2+ subtypes.

Pituitary gland volumes in children with normal endocrine function.

BACKGROUND: Magnetic resonance imaging (MRI) is often the first modality of choice in the detection of pituitary pathologies due to its excellent contrast resolution and high spatial resolution. However, evaluating the size of the pituitary gland is somewhat difficult since the gland morphology varies widely among individuals. OBJECTIVE: The aim of this study was to provide normal reference values for pituitary volumes in the pediatric population using three-dimensional (3-D) MRI data. We also aimed to compare the volumetric data measured through 3-D images with the mathematical volumes obtained by the ellipsoid formula from 2-D images, considering the demographic subgroups of age and sex. MATERIALS AND METHODS: This study was performed retrospectively using PACS-archived brain MRIs obtained in the Pediatric Radiology Unit. Eight hundred children under the age of 18 who underwent brain MRI between January 2014 and May 2018 for headaches, seizures or trauma and who had no brain anomaly or endocrine pathology were recruited. Pituitary volumes were separately calculated from those brain MRIs by means of ellipsoid formula and volumetry. The mean, standard deviation, maximum and minimum and median values were achieved for each 1-year age group in both sexes. Normal reference ranges containing 5th -10th -25th -50th -75th -90th -95th percentiles of volumetric pituitary values were tabulated. RESULTS: Pituitary gland volumes in both sexes were found to increase gradually with age. The average 3-D volume values were greater in girls than boys except for the age groups 1-2 years and 8-9 years. Pituitary volumes showed a growth spurt during early puberty, which was more prominent in girls. Moreover, a strong positive correlation was noted between the 2-D calculated and 3-D measured volumes in both sexes. CONCLUSION: Normative data obtained through this study can be used for clinical diagnostic purposes as well as a reference for future research. The 2-D calculated volumes be used where it is not possible to obtain 3-D volumetric values.

Rituximab experience from a single centre for patients with rheumatoid arthritis-related interstitial lung disease.

Objective: To demonstrate the effects of rituximab (RTX) in patients with rheumatoid arthritis-related interstitial lung disease (RA-ILD). Methods: A total of 165 patients who used RTX for the management of rheumatoid arthritis were retrospectively scrutinised. Among these, 26 patients diagnosed with RA-ILD were analysed (61.5% male, mean age at RTX infusion 61.4 ± 6.5 years). To evaluate the efficacy of RTX on lung response, patients with pulmonary function test results and/or thorax computed tomography (chest-CT) of pre- and post-RTX were compared. Disease progression was defined as either a decline of ≥10% in forced vital capacity (FVC) and/or a decline of ≥15% in diffusion capacity of carbon monoxide (DLCO), or an increase of parenchymal involvement on chest-CT images according to the radiologists' assessment. Results: Among 26 patients, the most common radiologic pattern was usual interstitial pneumonia (42.3%), followed by non-specific interstitial pneumonia (38.5%). Data for lung response was available in 20 patients. Median pre- and post- RTX DLCO values were 71.0% (60.0-77.0) and 63.0% (47.0-74.0), respectively (p= 0.06). Median pre- and post-RTX FVC values were 74.0% (61.0-99.0) and 84.0% (63.0-100.0), respectively (p= 0.28). Overall, stabilization or regression of RA-ILD was provided in 13 (65.0%) patients, whereas 7 patients had progressive RA-ILD. Post-RTX, 5 patients were diagnosed with RA-ILD. Conclusion: Our results suggest that RTX is effective in achieving stabilization or even improvement of RA-ILD. However, considering that it does not cause regression in every patient and some develop RA-ILD under RTX, we still need more effective treatment options.

Clinical characteristics of pulmonary artery involvement in patients with Behçet's syndrome: single-centre experience of 61 patients.

OBJECTIVE: To report the clinical characteristics of pulmonary artery involvement (PAI) in patients with Behçet's syndrome (BS) and to define the predictors of relapses. METHODS: We performed retrospective analysis of BS patients with PAI who fulfilled international study group criteria. Among 460 patients with vascular Behçet's syndrome (VBS), 66 were diagnosed with PAI. For final analyses, 61 patients with PAI were included who had at least 2 follow-up visits (72.1% male, mean age at BS diagnosis 29.34 ± 10.1 years). The patient data were recorded. Relapse was defined as the reoccurrence of vascular event in any vascular structure. Factors associated with relapse were assessed by logistic regression analysis. RESULTS: There were no differences considering demographic and clinical features of the patients with and without PAI in the VBS group, except that intracardiac thrombosis was more common in the patients with PAI (19.7% vs 0.3%). Among 61 patients, 50 (82.0%) had isolated pulmonary artery thrombosis (PAT), whereas 11 (18.0%) had pulmonary artery aneurysm with or without PAT. Twenty-four (39.3%) patients experienced vascular relapse during median follow-up of 65.9 (Q1-Q3: 20.1-109.0) months. To define the factors associated with relapses, patients with isolated PAT were analysed. On multivariable logistic regression analysis, older age at BS diagnosis and anticoagulation usage seemed to be protective (OR: 0.92, 95% CI 0.86-1.02, OR: 0.34, 95% CI 0.09-1.33, respectively). CONCLUSION: Our results indicate a higher frequency of intracardiac thrombosis in BS patients with PAI and possible efficacy of anticoagulation usage in preventing relapses. Key Points • This study shows that intracardiac and intracranial thromboses are seen more frequently in patients with PAI and the prevalence of pulmonary artery thrombosis has been increasing in the case of PAI. Furthermore, our report indicates that anticoagulation might be effective in preventing further vascular relapses.

Benign Hepatic Nodules Mimicking Hepatocellular Carcinoma in the Setting of Fontan-associated Liver Disease: A Case Report.

Fontan procedure, in which systemic circulation is redirected into pulmonary circulation by a baffle, is a palliative surgical strategy for patients born with single ventricle congenital heart disease. Hemodynamic changes secondary to Fontan procedure, also termed as Fontan physiology, result in end-organ damage, especially of the liver. Fontan-associated liver disease (FALD) represents a spectrum of pathologies ranging from mild liver fibrosis to advanced liver cirrhosis and hepatocellular carcinoma (HCC). Hepatic nodules, some of which have been documented as HCC in several case series and reports, are a recognized complicated feature of FALD. Herein, we report a case with benign hepatic nodules mimicking HCC by imaging characteristics, emphasizing the fact that arterially enhancing lesions with delayed washout appearance may reflect benign regenerative or focal nodular hyperplasia-like nodules in patients with Fontan physiology. HOW TO CITE THIS ARTICLE: Çolaklar A, Lehnert SJ, Tirkes T. Benign Hepatic Nodules Mimicking Hepatocellular Carcinoma in the Setting of Fontan-associated Liver Disease: A Case Report. Euroasian J Hepato-Gastroenterol 2020;10(1):42-44.

Infiltrative non-mass-like hepatocellular carcinoma initially presenting with isolated malignant portal vein thrombosis: A case report and review of the literature.

Hepatocellular carcinoma (HCC) shows a rising incidence and mortality rates worldwide. HCC is divided into several distinct subtypes, both morphologically and histopathologically. Among these subtypes, infiltrative HCC may be the most challenging subtype to diagnose, given its characteristic myriad of tumor nodules blended with normal hepatocytes without a distinct mass-like lesion. Herein, we report an unusual case of an infiltrative HCC initially presenting with isolated malignant portal vein thrombosis and provide a brief review of the literature regarding the infiltrative HCC subtype. Additionally, we demonstrate how sonoelastography could aid in detecting the appropriate biopsy area in the infiltrative HCC subtype. To our knowledge, there have not been previously reported cases describing the use of sonoelastography in the evaluation of the appropriate area for the targeted liver biopsy.Hepatocellular carcinoma (HCC) shows a rising incidence and mortality rates worldwide. HCC is divided into several distinct subtypes, both morphologically and histopathologically. Among these subtypes, infiltrative HCC may be the most challenging subtype to diagnose, given its characteristic myriad of tumor nodules blended with normal hepatocytes without a distinct mass-like lesion. Herein, we report an unusual case of an infiltrative HCC initially presenting with isolated malignant portal vein thrombosis and provide a brief review of the literature regarding the infiltrative HCC subtype. Additionally, we demonstrate how sonoelastography could aid in detecting the appropriate biopsy area in the infiltrative HCC subtype. To our knowledge, there have not been previously reported cases describing the use of sonoelastography in the evaluation of the appropriate area for the targeted liver biopsy.

Saccular Aneurysm of the External Jugular Vein: An Unusual Cause of a Neck Mass.

Venous aneurysms are rare cause of neck masses. Among neck veins, external jugular vein aneurysms are incredibly uncommon. We, herein, report the case of a 45-year-old woman who presented with a non-tender, non-pulsatile, progressively enlarging mass in the left supraclavicular region. B-mode and Doppler ultrasound examination showed a saccular dilatation of the external jugular vein. Saccular aneurysms of the external jugular vein are uncommon, and serious complications are rarely encountered. Ultrasonography with color flow duplex imaging can allow early detection of such venous aneurysms of the neck making the other imaging methods unnecessary.