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.
Background:Rheumatoid arthritis-related interstitial lung disease (RA-ILD) is the most common type of lung involvement in rheumatoid arthritis (RA). The existence of RA-ILD is associated with worse survival. There is no mainstay treatment for RA-ILD. However, in recent studies, rituximab (RTX) seems to be effective in RA-ILD.Objectives:To determine the effects of RTX in patients with RA-ILD from a single-centre.Methods:In our biological treatment outpatient clinic, a retrospective study was conducted in patients with RA who were treated with RTX. Among them, patients with RA-ILD were analysed. For lung response to RTX, progression was defined as a decline of 10% ≥ in forced vital capacity (FVC) and/or a decline of 15% ≥ in diffusion capacity of carbon monoxide (DLCO). Computed tomography of the chest (chest-CT) were integrated to lung response so as to constitute missing data of pulmonary function tests (PFTs).Results:A total of 165 patients who are followed-up in our biological treatment outpatient clinic have been using RTX for their RA diagnosis. Among 165 patients, 26 (15.8%) patients with RA-ILD were initiated RTX. Five patients were diagnosed with RA-ILD while using RTX (incidence rate 3%). Patients‘ characteristics were demonstrated in table 1. Of 26 patients, the most commonly used concomitant disease-modifying antirheumatic drugs was leflunomide (46.2%) followed by mycophenolate mofetil (11.5), methotrexate (11.5%) and azathioprine (3.8%). Twenty-four (92.3%) patients used steroids. For the evaluation of lung response, 20 patients who had follow-up PFTs and/or chest-CT were compared. Median DLCO values for pre- and post RTX were 71.0% (60.0-77.0) and 63.0% (47.0-74.0), respectively (p= 0.061). Median pre and post-RTX FVC were 74.0% (61.0-99.0) and 84.0% (63.0-100.0), respectively (p= 0.28). After a median of 2.7 years, 11 (55.0%) patients had stable disease, 2 (10.0%) patients had regression of RA-ILD and 7 (35.0%) patients had progressive disease. Sex, age, seropositivity and radiographic patterns were not associated with progression. In total, 23 adverse events were detected in 11 (42.3%) patients. Five of them were serious infections requiring hospitalization, mostly pneumonia. Other adverse events were simple infections and surgeries such as arthroplasty. RTX was stopped in 4 (15.4%) patients due to infections secondary to hypogammaglobulinemia in 2, malignancy in 1 and allergic reaction in 1.Conclusion:RTX seems to achieve stabilization/improvement of RA-ILD with fewer adverse events. However, given that RA-ILD develops post-RTX, it is not a panacea; therefore, we need more effective treatment modalities in the case of RA-ILD.Table 1.Characteristics of 26 patients with RA-ILD treated with rituximabAge at first RTX infusion, median (IQR), years61.2 (57.1-65.1)RA disease duration at first RTX, median (IQR), years10.1 (4.3-29.7)ILD disease duration at first RTX, median (IQR), years1.9 (0.1-4.5)Age at RA diagnosis, median (IQR), years51.1 (40.0-60.6)Age at ILD diagnosis, median (IQR), years58.3 (53.9-63.3)Male patient, n (%)16 (61.5)RF positivity, n (%)25 (96.2)ACPA positivity, n (%)20 (90.9)CRP levels,mg/L, mean (SD)40.3 (58.9)ESR levels, mean (SD)37.7 (18.2)Smoking status, n (%)16 (64.0)Prior TNF inhibitor treatment, n (%)5 (19.2)DAS28 at first RTX infusion, mean (SD)5.3 (1.5)Radiographic pattern, n (%)UIP7 (26.9)NSIP8 (30.8)OP2 (7.7)Indeterminate8 (30.8)Others1 (3.8)Follow-up duration, median (IQR), years2.5 (1.2-3.7)RA-ILD= Rheumatoid arthritis-related interstitial lung disease, RTX= rituximab, RA= rheumatoid arthritis, RF= rheumatoid factor, ACPA= anti-citrullinated protein antibody, TNF= tumor necrosis factor, CRP= C-reactive protein, ESR= erythrocyte sedimentation rate, UIP= usual interstitial pneumonia, NSIP= non-specific interstitial pneumonia, OP= organizing pneumoniaDisclosure of Interests:None declared
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.
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