Lower Extremity Edema Research Articles

Real life efficacy and safety of direct-acting antiviral therapy for treatment of patients infected with hepatitis C virus genotypes 1, 2 and 3 in northwest China.

BACKGROUNDRegimens involving direct-acting antiviral agents (DAAs) are recommended for the treatment of infection with hepatitis C virus (HCV) genotypes 1, 2 and 3. But real-world data is still not enough, especially in Asia.AIMTo investigate the efficacy and safety of DAA-based regimens in a real-life setting in China.METHODSThis study included 366 patients infected with HCV genotypes 1, 2 and 3, with or without cirrhosis, who were observed between May 2015 and December 2018. They were treated with ledipasvir and sofosbuvir (SOF) (genotype 1) with or without ribavirin (RBV), SOF and RBV (genotype 2), or SOF and daclatasvir (genotype 3), with or without RBV, for 12 or more wk. The participants’ sustained virological responses (SVR) at post-treatment week 12 (SVR12) was the primary endpoint. The occurrence of adverse events and drug-drug interactions were recorded.RESULTSIn the 366 patients, genotype 1 (59.0%) was the most common genotype, followed by genotypes 2 (34.4%) and 3 (6.6%). Liver cirrhosis was diagnosed in 154 (42.1%) patients. Fifty (13.7%) patients were treatment-experienced. Intention-to-treat analysis revealed that SVR12 was 86.3% (316/366). For modified intention-to-treat analysis, SVR12 was achieved in 96.6% of overall patients (316/327), 96.3% in patients with genotype 1, 97.5% in those with genotype 2, and 95.0% in those with genotype 3. Most of the treatment failures were due to lack of follow-up (3 cases had non-responses, 1 had virological breakthrough, 11 relapsed and 36 did not participate in the follow-up). There was no significant difference in SVR between different genotypes and liver statuses (P < 0.05). Patients with lower alanine aminotransferase levels at baseline who achieved an end of treatment response were more likely to achieve SVR12 (P < 0.05). High SVR was observed regardless of age, gender, liver status, alpha-fetoprotein, HCV RNA levels or history of antiviral therapy (P > 0.05 for all). The cumulative hepatocellular carcinoma occurrence and recurrence rate after using the DAAs was 0.9%. Most of the adverse events were mild. We found two cases of special adverse events. One case involved facial and bilateral lower extremity edema, and the other case showed an interesting change in lipid levels while on medication. No severe adverse events were noted.CONCLUSIONThe DAA-based regimens tested in this study have excellent effectiveness and safety in all patients infected with HCV genotypes 1, 2 and 3, including those with cirrhosis.

Acute Systolic Heart Failure as the only Risk Factor for Cardioembolic Cerebrovascular Accident in the Setting of Nonischemic Cardiomyopathy and Normal Sinus Rhythm: A Case Report with Literature Review

The association between atrial fibrillation and stroke is well-known by use of the CHA2DS2VASc risk assessment. However, little is known about the thromboembolic risk in patients who have isolated systolic heart failure (HF) with sinus rhythm. Despite growing literature which shows how HF in the setting of normal sinus rhythm is a risk factor for thromboembolic disease, the recommendation for use of anticoagulation, whether warfarin or direct oral anticoagulation (DOAC) in this setting, is not yet reflected in cardiology guidelines. To reduce the risk of thromboembolism, anticoagulation trials have been done but have not conclusively shown benefit over risk. We present a case of left middle cerebral artery (MCA) stroke after de novo HF in a patient with sinus rhythm, who was previously not on anticoagulation. Case: A 57-year-old male presented to our hospital with worsening shortness of breath for 3 days. He had signs of hypervolemia consistent with an acute heart failure exacerbation. Pertinent physical exam findings included lower extremity edema, rales, and jugular venous distension. He also had fatigue and exertional dyspnea. During his hospital course, he underwent imaging studies that revealed cardiomegaly, absence of pulmonary embolism, and a severely reduced ejection fraction. During his acute heart failure admission, he developed a left MCA stroke suspected to be of cardioembolic etiology and was successfully treated with tissue plasminogen activator (tPA). Conclusion: According to the most recent AHA/ACC cardiovascular disease guidelines, anticoagulation is not indicated in systolic heart failure patients with sinus rhythm. However, our case is rare because the patient had no underlying risk factors for thromboembolism and significant past medical history. He developed new-onset nonischemic cardiomyopathy complicated by a stroke. Therefore, we suggest the need for prophylactic anticoagulation should be assessed on an individual basis, with the assistance of shared decision making, especially when the ejection fraction is acutely and severely reduced.

Differential diagnosis and treatment of edema of lower extremities

The article gives a definition, classification and mechanisms for the development of edema (increased hydrostatic pressure, increased capillary permeability, decreased colloidal osmotic pressure). The main diseases accompanied by edematous syndrome are considered, diagnostic search algorithms are given. The clinic and treatment of edema of the lower extremities due to lymphatic and venous insufficiency are separately discussed. The data of a clinical study of the effectiveness of drugs based on diosmin and hesperidin in the treatment of chronic venous insufficiency are presented.

The Effects of Manual Lymphatic Drainage and Kinesiotaping on Lower Extremity Edema

The Effects of Manual Lymphatic Drainage and Kinesiotaping on Lower Extremity Edema

A Latin American Patient With a Left Ventricular Pseudoaneurysm Presenting With Progressive Dyspnea and Palpitations

A 71-year-old male presented with a 6-month history of intermittent palpitations. He also mentioned progressive orthopnea, bilateral lower extremity edema, and dyspnea on exertion. He recently moved from Santa Cruz, Bolivia, to the United States. He did not report any history of chest pain, visual changes, or syncope.

Great saphenous vein stump: a risk factor for superficial/deep venous thrombosis and an indication for prophylactic anticoagulation? - a retrospective analysis

ABSTRACTBackground: Great saphenous vein (GSV) grafts are used for coronary artery bypass surgeries, but the remaining stump of the GSV may be the nidus for superficial and deep vein thrombosis. This study aims to determine the risk of thrombosis in the GSV stump in patients who developed lower extremity swelling following coronary artery bypass graft (CABG).Methods: We conducted a single-center retrospective analysis at Abington Jefferson Hospital of 100 patients who underwent CABG with GSV. Patients were monitored via follow-up for seven days for the development of saphenous vein thrombosis without any prophylactic anticoagulation for venous thrombosis. Risk factors including age, diabetes, hypertension, smoking, familial thrombophilia’s, family history of thrombosis, malignancy, and confounding factor-like early mobilization that may potentially alter the results were recorded.Results: The mean age of included patients was 70 years, and 65% of participants were men, 35% were women. Fourteen percent of the patients developed pain, swelling and edema in a leg where the graft was taken. We included patients aged >50 years with coronary artery disease who underwent CABG with SVG and developed lower extremity symptoms concerning for thrombosis. These patients underwent duplex ultrasound for possible GSV stump thrombosis. Any patients with coronary artery disease but no CABG or no lower extremity edema were excluded from the study. We found no saphenous vein thrombosis in the stump of the GSV in patients with clinical symptoms of thrombosis in their lower extremities based on duplex imaging.Conclusion: Based on our findings, the postoperative risk of developing thrombosis at the GSV stump and its extension to the deep veins is low and does not warrant prophylactic anticoagulation for venous thromboembolism. However, we recommend that further prospective studies with larger samples for an extended duration are warranted for better assessment of the risk of venous thrombosis in the GSV stump with minimal confounding factors.

70-Year-Old Man With Fatigue, Shortness of Breath, and Bilateral Lower Extremity Edema

A 70-year-old man presented for evaluation of 5 months of fatigue, shortness of breath, and bilateral lower extremity edema. His medical history was notable for coronary artery disease with coronary artery bypass grafting 2 years previously, nicotine dependence (>30 pack-years), type 2 diabetes mellitus, hypothyroidism, and obesity (body mass index, 30 kg/m2). Medications included furosemide, spironolactone, aspirin, atorvastatin, and levothyroxine. Review of systems revealed dyspnea with exertion (New York Heart Association, class III-IV; Eastern Cooperative Oncology Group score, 4), orthostatic hypotension, bloating, early satiety with a 9-kg unintentional weight loss over 4 months, lower extremity edema, and discoloration around the left eye.

Evaluation of losartan plus hydrochlorothiazide combination therapy against amlodipine monotherapy in patients of hypertension

Background: Microalbuminuria has been shown to predict cardiovascular disease (CVD) in patients with hypertension. Recently the FDC of losartan and hydrochlorothiazide (HCTZ) has been reported to be effective for achieving a target BP level and also improvement in cardiovascular prognosis. The present study was conducted to compare effect of losartan plus hydrochlorothiazide combination therapy and high dose amlodipine monotherapy on blood pressure and microalbuminuria.Methods: Total 184 patients with hypertension were randomly allocated to two groups. The patients in group 1 received Amlodipine 5 mg orally for first 4 weeks. The patients from group 2 received losartan 50 mg orally for first 4 weeks. Patients in group 1 were titrated to amlodipine 10 mg orally for next 4 weeks. The patients in group 2 were titrated to FDC of losartan (50 mg) plus HCTZ (12.5 mg) for next 4 weeks. Follow–up visits were scheduled at 4 weeks and 8 weeks. Pulse rate, sSBP and sDBP were estimated at each follow–up. Microalbuminuria was estimated at 8 weeks.Results: There was no significant difference in mean change in sSBP, sDBP and pulse rate between two treatment groups (p&gt;0.05). There was greater reduction in microalbuminuria in group 2 patients (p&lt;0.0001). The adverse effects such as flushing and lower extremity oedema were significantly more in amlodipine group (p&lt;0.05).Conclusions: Losartan plus HCTZ has similar effect on BP, better safety profile and superior effect on microalbuminuria level reduction.

Antihypertensive Effect of Switching to a Fixed Perindopril/Amlodipine Combination in Patients Ineffectively Treated by Free Sartan-Containing Combinations. Results of the AVANGARD Study

In the multicenter open uncontrolled observational program AVANGARD 203 doctors in 53 cities of the Russian Federation included 658 patients who had not achieved target BP on therapy with two drugs, one of which was sartan (sartan with diuretic, calcium antagonist, β-blocker, or moxonidine in 49%, 33%, 17%, and 1% of cases, respectively). This therapy was replaced with a fixed combination of perindopril arginine/amlodipine. Duration of observation was 3 months. On therapy with perindopril arginine/amlodipine, BP decreased 159.9±8.5/92.1±7.4 to 125.8±7.1/77.4±5.5 mm Hg. Target BP &lt;140/90 mm Hg was achieved in 93.5% of patients (office measurement); target BP &lt;135/85 mm Hg - in 83.5% of patients (home measurement). Mean 24-hour BP variability decreased from 4.4±2.9/3.0±2.0 to 3.0±2.2/2.2±1.7 mm Hg (p&lt;0.01). Number of patients complaining of headache decreased by 2.9 times, dizziness - by 2.8 times, fatigue - by 2.3 times, irritability - by 3.0 times, sleep disturbances - by 2.3 times, dyspnea - by 3.8 times, palpitations - by 2.7 times, angina pectoris attacks - by 4.6 times. Dose of perindopril arginine/amlodipine was 10/5 mg in 36.6%, and 10/10 mg in 28.3% of cases, respectively. Number ofparticipants who dropped out ofthe study prematurely was 11 (1.6%) (1 because of adverse event). Adverse events were observed in 4 more patients (2 [0.14%] - edema of lower extremities, and 2 [0.14%] -cough), but they did not require the withdrawal of therapy. In case of ineffective combination therapy containing sartans, transfer of patients to a fixed combination of perindopril and amlodipine should be considered.

Man With Bilateral Leg Swelling.

A 52-year-old man without known medical history presented with painful, progressive, bilateral lower extremity edema over a two-week period. An abdominal exam noted a firm left upper quadrant mass. Emergency department (ED) point-of-care ultrasound (POCUS) revealed a hyperechoic, heterogeneous structure in the inferior vena cava that was determined to represent a tumor thrombus extending from a primary renal cell carcinoma. This case demonstrates how POCUS was valuable in rapidly diagnosing this rare cause of lower extremity edema and its usefulness in directing the initial ED management of this patient.

78-Year-Old Woman With Intermittent Chest Pain and Palpitations

78-Year-Old Woman With Intermittent Chest Pain and Palpitations

METHICILLIN-RESISTANT STAPHYLOCOCCUS AUREUS-INDUCED PERICARDITIS AND PERICARDIAL TAMPONADE IN AIDS PATIENT: PRESENTATION, PROGRESSION, AND MANAGEMENT

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: We report a case of 46-year old woman with untreated AIDS (CD4 count 12), and recurrent neck abscesses, who presented with a neck abscess, and developed acute pericarditis, which resulted in pericardial effusion and tamponade. Blood, purulent drainage from the abscess, and pericardial fluid cultures were positive for methicillin resistant staphylococcus aureus (MRSA). The patient was successfully treated with 6 weeks of IV Vancomycin, and pericardial drainage. CASE PRESENTATION: The patient is a 46 year old woman with past medical history of AIDS (Most recent CD4 count 12) who was not on anti-retroviral therapy due to social reasons with recurrent skin/soft tissue infections, who presented with fever and chest pain of 1 week duration. She also reported a recurrent anterior neck mass. On hospital admission, her chest pain was constant for the past week, and is worse with movement and deep inspiration. She states the pain is under her left breast and radiates towards her left shoulder. She denies shortness of breath, or lower extremity edema. DISCUSSION: MRSA Pericarditis is a very rare life-threatening infection, with only a few cases reported in literature [1] [2] [3]. Mortality rates for purulent pericarditis were reported to be as high as 40% in the treated population. In immunosuppressed patients or following thoracic surgery, Staphylococcus aureus (30%) and fungi (20%) are more common [10]. CONCLUSIONS: Management of MRSA pericarditis was done through pericardial drain, with some cases treated with pericardial window (Class I recommendation for pericardial window, and Class IIa for subxiphoid pericardiotomy by 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology). Our case was managed by pericardial drainage alone, and although she had recurrence of pericardial effusion, pericardiotomy was felt to be very risky given her AIDS with CD4 count Reference #1: 1) Kurahara, Y. and Kawaguchi, T. Cardiac Tamponade with Community-acquired Methicillin-resistant Staphylococcus aureus Pericarditis. Intern Med. 2013;52(15):1753. Reference #2: 2)Hussam, M., Ragai, M., Iman, M. and Zakaria, A. Community-acquired methicillin-resistant Staphylococcus aureus pericarditis presenting as cardiac tamponade. South Med J. 2010 Aug;103(8):834-6 Reference #3: 3)Oizumi, H., Ichinokawa, H., Hoshino, H., Shitara, J. and Suzuki, K. Pericardial Window for Methicillin-Resistant Staphylococcus aureus Pericarditis. Ann Thorac Surg. 2019 Jan;107(1):e27-e29. DISCLOSURES: No relevant relationships by Laith Ali, source=Web Response No relevant relationships by Brian Cuneo, source=Web Response No relevant relationships by Amre Ghazzal, source=Web Response No relevant relationships by Tariq Sallam, source=Web Response

2475 Polycystic Liver Disease Causing Inferior Vena Cava Compression and Venous Thromboembolism

INTRODUCTION: Polycystic Liver Disease (PLD) is a rare disorder that can be diagnosed in patients with greater than 10 fluid filled-cysts identified on imaging. PLD is associated with two genetic disorders autosomal dominant polycystic liver disease and autosomal dominant polycystic kidney disease. Since PLD is uncommon, it is difficult to study both the natural history of the disease and treatment modalities. We present a case of a massive hepatic cyst in a 95-year-old patient with polycystic liver disease that led to inferior vena cava compression and venous thromboembolism. CASE DESCRIPTION/METHODS: A 95-year-old male with past medical history of colon cancer status-post resection twenty-five years ago, with no subsequent recurrence and hemorrhagic hepatic cyst, two months prior, presented for evaluation of abdominal distension, lower extremity edema and shortness-of-breath. Physical exam was remarkable for a firm distended abdomen with hepatomegaly and bilateral lower extremity pitting edema. Laboratory evaluation showed a hemoglobin of 8.6 mg/dL, with a baseline hemoglobin of 8.0 mg/dL on prior admission. An abdominal CT scan showed numerous hepatic cysts with an enlarging right hepatic lobe cyst, measuring 25 × 25 × 30 cm, with interval resolution of blood products within the cyst, and an incidentally noted pulmonary embolism in the right lower lobe. The size of the cyst led to compression of the inferior vena cava and displacement of the right kidney. Ultrasound revealed that it was multiseptated in nature. Image-guided drainage and catheter placement was performed, revealing 5.7 liters of clear brown fluid. Cytologic evaluation revealed no evidence of malignancy and aerobic and anaerobic cultures showed no growth. A duplex ultrasound of the lower extremities exhibited acute thrombus of the left deep femoral vein and sluggish flow throughout all vessels. Given the patient's prior hemorrhage, anticoagulation was contraindicated and an inferior vena cava filter was placed. At the time of filter placement, the cyst had decreased in size and sclerotherapy was performed. The patient felt substantial improvement in his dyspnea and abdominal distension and was discharged home with plans for sclerotherapy as an outpatient. DISCUSSION: PLD is a rare disorder which makes the natural history difficult to define. Our case further clarifies the association of PLD with external compression of the inferior vena cava. It adds data to the literature by illustrating the complication of venous thromboembolism.

ACUTE PULMONARY GRAFT VS HOST DISEASE RESPONDING TO IBRUTINIB THERAPY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Acute graft versus host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (HSCT) usually involves the liver, skin and gut and lung involvement is rare. We report a case of acute GVHD with respiratory failure developing 18 months post allogeneic HSCT that was successfully treated with Ibrutinib. CASE PRESENTATION: A 58-year old male with relapsed small lymphocytic lymphoma (SLL) following matched, sibling-donor allogeneic HSCT complicated by chronic GVHD presented with acute onset shortness of breath following completion of slow corticosteroid taper. He presented 18 months post-transplant with a one-week history of progressive dyspnea, anorexia, weight loss, diarrhea, lower extremity edema, dizziness and lethargy. Dyspnea rapidly progressed to acute hypoxemic respiratory failure requiring high-flow oxygen therapy (HFOT). CT chest progressed from right middle and lower lobe ground glass opacities on admission to bilateral multi-lobar parenchymal opacities (Figure 1). Oxygen requirements progressed in spite of broad-spectrum antibacterial and antifungal therapy; he underwent bronchoscopy with transbronchial biopsy that showed chronic interstitial inflammatory infiltrate with scattered T cells, with no evidence of SLL/CLL (Figure 2). Microbiological testing, including bronchoalveolar lavage fluid cultures, was negative. The diagnosis of acute GVHD was made and the patient was started on high-dose IV methylprednisolone on hospital day (HD) 9. After 5 days, he remained on HFOT; he received tocilizumab and was started on ibrutinib on HD 14. By HD 18, his pulmonary status had significantly improved. By HD 22, he was off supplemental oxygen, and by HD 23, his chest CT demonstrated resolution of lung opacities. DISCUSSION: Non- infectious lung complications (NILC) is a nonspecific term that includes pulmonary edema, diffuse alveolar haemorrhage, idiopathic pneumonia syndrome, post-transplant lymphoproliferative disorder and many other pulmonary complications that can occur in patients post HSCT (1). Acute GVHD is one of the complications responsible for severe morbidity and often, mortality. As per the 2014 NIH consensus criteria, diagnosis of acute GVHD depends on clinical manifestations, not to the temporal relationship with transplantation (2). The clinical course of acute GVHD typically mimics steroid-responsive cryptogenic organizing pneumonia; however in certain cases, it may be refractory to even high doses of steroids. Our patient’s clinical improvement following initiation of immunomodulatory therapies including tocilizumab and ibrutinib, provides support for using these novel agents. CONCLUSIONS: Ibrutinib is a tyrosine kinase inhibitor that has been shown to benefit patients with chronic GVHD (3). Our case highlights its use with a positive outcome in acute GVHD refractory to steroids. Reference #1: Pena E, Souza CA, Escuissato DL, Gomes MM, Allan D, Tay J, et al. Noninfectious pulmonary complications after hematopoietic stem cell transplantation: practical approach to imaging diagnosis. Radiographics. 2014;34(3):663-83. Reference #2: Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group report. Biol Blood Marrow Transplant. 2015;21(3):389-401.e1. Reference #3: Miklos D, Cutler CS, Arora M, Waller EK, Jagasia M, Pusic I, et al. Ibrutinib for chronic graft-versus-host disease after failure of prior therapy. Blood. 2017;130(21):2243-50. DISCLOSURES: No relevant relationships by Divyansh Bajaj, source=Web Response No relevant relationships by Nancy Hardy, source=Web Response No relevant relationships by Van Holden, source=Web Response No relevant relationships by Shreya Kanth, source=Web Response No relevant relationships by Akshay Kohli, source=Web Response No relevant relationships by Laura Malone, source=Web Response Consultant relationship with Medtronic Please note: $1001 - $5000 Added 03/17/2019 by Edward Pickering, source=Web Response, value=Consulting fee Consultant relationship with Medtronic Please note: $1001 - $5000 Added 03/13/2019 by Ashutosh Sachdeva, source=Web Response, value=Consulting fee Unrestricted education grant relationship with Olympus Please note: $1001 - $5000 Added 03/13/2019 by Ashutosh Sachdeva, source=Web Response, value=Grant/Research Support

2326 Primary Squamous Cell Carcinoma of the Liver: A Rarity

INTRODUCTION: Primary liver squamous cell carcinoma (PLSCC) is a very rare entity with less than 40 reported cases since 1970. PLSCC has been known for late diagnosis, high malignancy rate, and poor prognosis of less than 1 year. The exact pathogenesis of PLSCC is unclear, though various theories exist. CASE DESCRIPTION/METHODS: A 72-year-old male with a history of NASH cirrhosis and hepatocellular carcinoma (HCC) status post right hepatic lobectomy 11 years prior and microwave ablation of a presumed recurrent HCC mass just 5 months prior, presented with a 2-month history of progressive fatigue, shortness of breath, lower extremity edema, and abdominal pain. He was hypotensive with an initial blood pressure of 90/54mmHg and had a distended abdomen without evidence of jaundice, encephalopathy, or acholic stools. Labs were notable for WBC 16.9 × 10^3/uL, lactic acid of 4.5mmol/L, and alpha-fetoprotein marker of 1.9ng/mL. CT Abdominal/Pelvis revealed a large burden of diffuse intrahepatic lesions spanning all lobes—findings compatible with HCC—and multiple bilateral kidney simple cysts. Patient underwent an ultrasound guided biopsy of the largest hepatic lesion measuring 2.3 × 2.0 × 2.8 cm. Unfortunately, his condition rapidly worsened with significant elevation in liver enzymes and lactic acid and he passed away. Pathology results resulted shortly after his passing and showed a partially necrotic high grade carcinoma favoring squamous cell carcinoma. It was strongly positive for immunomarkers: CK5, CK7, p40, HSP70 (non-specific), but negative for GATA3, CK20, glypican 3, Hep Par1, S100 protein. DISCUSSION: In this case, the patient was thought to have HCC, but its aggressive clinical course was highly unexpected. Data on the diagnosis of PLSCC is limited and the mechanism is uncertain, though it has a strong association with hepatic cysts, stones, and cirrhosis. Hepatic cysts were not clearly visualized on imaging, though studies do show a positive correlation between the incidence of kidney and hepatic cysts. As this patient had multiple benign bilateral kidney cysts, we suspect that hepatic cysts could have also been present which makes PLSCC very likely given the lack of evidence of a different primary source. A liver carcinoma with such an aggressive clinical course should increase clinical suspicion for PLSCC, however further understanding of the pathogenesis of this disease needs to be investigated.

2443 Dealing With DILI: When the Diagnosis Is Not Always Clear Cut

INTRODUCTION: Drug induced liver injury (DILI) is a rare, mostly reversible cause of liver failure (ALF); however, in rare cases DILI can unmask or trigger underlying chronic liver disease. We present a case of DILI with two novel findings: one, the patient consumed tea extract not yet cited on the LiverTox registry, and two, the patient's DILI likely triggered autoimmune hepatitis (AIH), an occurrence noted only a few times in the literature. CASE DESCRIPTION/METHODS: Patient is a 43-year-old female with no medical history who presented with acute onset jaundice and dark urine. She endorsed a “tea cleanse” which included dandelion roots, milk thistle and barks one month prior to admission. Her jaundice persisted despite cessation of the herbal tea intake. Liver function tests (LFTs) showed AST 2553, ALT 3022, alkaline phosphatase 135, total bilirubin 23.8, direct bilirubin 15.5, INR 1.8 and MELD 25. MRCP was consistent with acute hepatitis. ALF workup was negative except for elevated IgG1 and IgG4. Liver biopsy revealed panlobular mixed inflammatory cell infiltrate and necrosis but absent fibrosis, concerning for DILI, viral infection or AIH. Patient was managed supportively for DILI with downtrending LFTs. However, her LFTs worsened with total bilirubin 20 and INR 2.1. She developed ascites and lower extremity edema. The patient received corticosteroids for AIH despite negative ANA, anti-smooth muscle and anti-LKM antibodies with marked improvement in synthetic liver function. DISCUSSION: Although acetaminophen is the most common offending drug associated with DILI (40-50% of ALF cases annually), herbal or dietary supplements are not an uncommon cause, seen in 11-12% of cases per year. Accurate diagnosis of DILI can be made with clinical history, exclusion of other etiologies of ALF on laboratory evaluation, and with liver biopsy. The database LiverTox documents etiologies of DILI. “Be Well Teas,” consumed by our patient, yields no results but should be included to prevent future DILI cases. Further, despite biopsy consistent with DILI, rebound of total bilirubin and INR in our patient demanded further investigation. Likely the patient had pre-existent AIH triggered by DILI given her improvement with immunosuppression, cited previously in only in a few case reports. Concurrent, underlying AIH should be considered with relapse of liver dysfunction despite cessation of the inciting drug.

IMPROVEMENT IN PULMONARY HYPERTENSION SECONDARY TO HEREDITARY HEMORRHAGIC TELANGIECTASIA FOLLOWING BEVACIZUMAB THERAPY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by multiple vascular malformations, including telangiectasias and arteriovenous malformations (AVM) in the brain, lung and liver. When hepatic AVMs occur, high output cardiac failure can result, ultimately leading to acute cor pulmonale. We discuss a patient case with high output cardiac failure, due to hepatic AVMs. CASE PRESENTATION: This is a 57 year old female with confirmed HHT (ACVRL1 gene mutation) presented to pulmonary clinic for dyspnea. She was recently hospitalized due to dyspnea, lower extremity edema and recurrent epistaxis. Chest radiography with enlarged cardiac silhouette with pulmonary vascular congestion, chest angiography and V/Q were negative for acute pulmonary embolism. Echocardiography showed ejection fraction of 60-65% with severe tricuspid regurgitation, and severe pulmonary arterial hypertension with estimated right ventricular systolic pressures of 82mmHg. Outpatient pulmonary function test with FEV1 of 1.83 which was 64% of predicted, with FEV1/FVC 74% of predicted along with reduced six minute walk test of 356 meters, with elevated right heart pressures (Table 1). She was started on intravenous bevacizumab every three months for maintenance therapy. Repeat right heart catheterization after 6 months with significant improvement (Table 2). Repeat six minute walk test was 494 meters. Upon follow up, she reported significant improvement in epistaxis, dyspnea and lower extremity swelling. DISCUSSION: Due to this patient’s extensive hepatic AVM, she developed high output cardiac failure and severe pulmonary hypertension. In patients with HHT, hepatic AVMs are considered the most prevalent visceral AVM, although more than 90% can be clinically silent. Patients with hepatic AVMs can develop pulmonary hypertension along with high output cardiac failure. Interestingly, these patients are usually carriers of the ACVRL mutations [1], such as in our patient. Given the rarity of this disease, only few studies suggest treatments for HHT, including VEGF inhibitor, bevacizumab. In 2009, a French open label phase 2 single arm trial was conducted in 25 patients with severe HHT with symptomatic liver involvement. A significant decrease of high cardiac index had been demonstrated in 17 of 24 patients. Initially, mean CI was 5.01 L/min/m2, with a significant decrease after three months of treatment, with a mean CI at 4.2 L/min/m2 [2]. Due to significant improvement in our patient's respiratory symptoms and pulmonary hypertension, as noted on repeat right heart catheterization, intravenous bevacizumab should be considered as treatment for patients with HHT. CONCLUSIONS: In patients with hereditary hemorrhagic telangiectasias, it is imperative to consider intravenous bevacizumab, especially in patients with pulmonary hypertension. Reference #1: Sopeña, B., Pérez-Rodríguez, M. T., Portela, D., Rivera, A., Freire, M., & Martínez-Vázquez, C. (2013). High prevalence of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. European journal of internal medicine, 24(3), e30-e34. Reference #2: Dupuis-Girod S, Ginon I, Saurin J-C, Marion D, Guillot E, Decullier E, et al. Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 2012; 307: 948–955. https://doi.org/10.1001/jama.2012.250 PMID: 22396517 DISCLOSURES: No relevant relationships by Bindu Akkanti, source=Web Response No relevant relationships by NEAL GEORGE, source=Web Response no disclosure on file for Juneja Harinder; no disclosure on file for Victor Machicao; No relevant relationships by Jennifer Sunny, source=Web Response

2757 Gastric Amyloid: The Patient Who Could Not Eat at AL

INTRODUCTION: Gastroparesis is defined as delayed gastric emptying in the absence of mechanical obstruction. Diabetes and medication side effects are common causes. AL amyloidosis is a systemic disease due to extracellular fibril deposition that can affect multiple organs. Gastro-intestinal (GI) involvement is less common than in other organ systems but can present in many different ways, including gastroparesis. CASE DESCRIPTION/METHODS: A 77 year old non-diabetic man from Mississippi presented with a one year history of intermittent abdominal distention, nausea and bloating. In addition, he had increasing dyspnea on exertion and lower extremity edema. Outside work-up consisted of multiple imaging studies demonstrating marked gastric distention as well as delayed gastric emptying (Figures 1 and 2). Outside colonoscopy was unrevealing and EGD was negative except for a distal esophageal ulcer and focal active duodenitis. He was started on a PPI and trialed on an alternating regimen of metoclopramide and erythromycin, without symptomatic relief. He was referred to our hospital for further diagnostic work-up. Repeat colonoscopy showed congested distal ileal mucosa and friable ascending colonic mucosa. Biopsies from both sites revealed amyloid deposits by Congo red stain (Figure 3). Serum protein electrophoresis noted a monoclonal IgG lambda peak with M-spike of 1.5 g/dL and bone marrow biopsy revealed approximately 70% of marrow cellularity consisting of plasma cells. Echocardiogram confirmed severely increased concentric left ventricular wall thickening consistent with cardiac amyloidosis. Interestingly, neither fat pad biopsy nor bone marrow biopsy showed presence of amyloid. The patient was diagnosed with myeloma and AL amyloidosis with gastro-intestinal and cardiac involvement. DISCUSSION: This case highlights the broad differential for gastric distention and dysmotility. When the ‘usual suspects’ such as diabetes, medication, post-surgery and neurological disease have been ruled out, rarer causes like an infiltrative process should be considered. Common manifestations of GI tract amyloidosis include GI bleeding, malabsorption, protein-losing enteropathy and chronic dysmotility. The diagnosis of amyloidosis is based on positive tissue diagnosis. While the combination of a fat pad and a bone marrow biopsy has a high sensitivity (89%) for diagnosing AL amyloidosis, negative results do not rule out amyloid elsewhere and tissue sampling from the affected organ site is essential, as demonstrated in this case.

3048 New Onset Ascites Related to Peritoneal Carcinomatosis

INTRODUCTION: Peritoneal carcinomatosis (PC) is a metastatic feature in several gastrointestinal malignancies. As one of the most common presenting symptoms is new onset ascites, understanding the workup and typical findings of malignant ascites is crucial for appropriate and time sensitive diagnosis and treatment of such malignancies. CASE DESCRIPTION/METHODS: A 50-year-old female with past medical history significant for hypertension and alcohol abuse presented to the hospital with complaint of intermittent weakness and progressive abdominal fullness for one month. Her exam was notable for subconjunctival palor, abdominal ascites with generalized tenderness, and lower extremity edema. Labs showed leukocytosis and microcytic anemia, with hemoglobin of 5.9. CT abdomen/pelvis revealed cardiomegaly, moderate volume ascites, and diffuse body wall edema. Iron studies were obtained (low iron, low-normal ferritin) and the patient was transfused. A diagnostic paracentesis was completed with resultant SAAG of 0.6. Cytology revealed benign appearing mesothelial cells and histiocytes. Due to the patient's lack of insurance, GI was consulted for inpatient evaluation of iron deficiency. Colonoscopy showed a large (&gt;10 cm) ulcerated lesion in the sigmoid colon with pathology consistent with moderately-differentiated adenocarcinoma. Repeat CT was obtained for staging purposes, showing diffuse peritoneal carcinomatosis, distal colon lesion, L4 vertebral sclerosis, and large volume ascites. The patient was discharged with plans to follow up with GI and oncology to discuss therapeutic options. DISCUSSION: Peritoneal carcinomatosis is defined as the dissemination of cancer cells throughout the periteoneum. About 8% of patients with colorectal cancer present with PC; most commonly with nonspecific symptoms such as fatigue, abdominal pain/fullness, and bowel obstruction. Since new onset ascites is common to PC, it is important to identify malignant ascitic fluid. Malignant ascites related to peritoneal carcinomatosis is mainly caused by subphrenic vessel obstruction and accounts for 7% of ascites cases. A diagnostic paracentesis is indicated, with typical fluid findings of WBC &gt;500, SAAG &lt;1.1, and total protein &gt;2.5. Fluid cytology is important, however sensitivity is affected by the number and quality of specimens used, making the overall sensitivity only about 60-70%. After a diagnosis of malignant ascites is made, the patient should be counseled and referred for evaluation of available therapeutic options.

LESSONS LEARNED FROM TRIPLE COMBINATION THERAPY IN PULMONARY ARTERIAL HYPERTENSION

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Current guidelines published by Chest in 2019 suggest that patients who present with low or intermediate risk pulmonary arterial hypertension (PAH) should be initiated on oral therapy with endothelin receptor antagonists (ERAs) and phosphodiesterase-5 inhibitors (PDE-5i) or soluble guanylate cyclase stimulators (sGCs) (1). They suggest using triple combination therapy including subcutaneous or IV prostacyclin analogues in high-risk patients (1). The following is a series of three patients with PAH who dramatically improved on triple combination therapy. CASE PRESENTATION: A 49 year old male with Sjogrens disease and associated PAH (APAH) was found to have clinical improvement on an ERA, PDE-5i, and prostacyclin analogue combination. With the switch of the PDE-5i to a sGCs based on the Respite trial and switch of ERA choice within the class due to side effect (nasal congestion and lower extremity edema), the patient had near normalization of his echocardiogram and right heart catherization (RHC) findings as well as an increase in his 6 minute walk distance (6MWD) (2) (see Table 1, Patient 1). Similarly, a 75 year-old female with scleroderma APAH was treated with an ERA, PDE-5 inhibitor, and a prostacyclin analogue. Significant hemodynamic improvement was noted when ERA choice was changed within its class and sildenafil was changed to a SGCs (2) (See Table 1, Patient 2). Finally, a 44 year-old female with PAH secondary to HIV was noted to have improvement in 6MWD and decrease in mean pulmonary artery pressure and pulmonary vascular resistance with the addition of an ERA and PDE-5 inhibitor to IV epropostenol. Interestingly, when IV epropostenol was switched to subcutaneous (SC) trepostinil due to recurrent line infections, the patient’s hemodynamics worsened. When the patient developed angioedema to ambrisentan, she was switched to macitentan with subsequent improvement in 6MWD and hemodynamics (see Table 1, Patient 3). DISCUSSION: Classically, it is considered of limited efficacy to switch ERAs within the class and there is limited information in added benefit of switching PDE-5 inhibitors to Riociguat, a sGC stimulator. In each of these patient cases, changes were made in medication regimens due to progression of disease or medication side effects and patterns of clinical improvement were subsequently noted. This suggests that by using triple drug combination therapy, including parental prostacyclines, and switching medications within the ERA class and between nitric oxide pathway agents (PDE-5 inhibitors to sGC stimulators), physicians may further optimize a patient’s clinical status by hemodynamic parameters and 6MWD. CONCLUSIONS: Triple combination therapy may lead to dramatic clinical and hemodynamic improvement. Further studies need to be performed to determine if triple therapy might lead to better long-term survival. Reference #1: Klinger, J. R. (2019). Therapy for Pulmonary Arterial Hypertension in Adults. Chest Journal, 155(3), 565-586. doi:https://doi.org/10.1016/j.chest.2018.11.030 Reference #2: Hoeper, M. M., Corris, P. A., Klinger, J. R., Langleben, D., Naeije, R., Simonneau, G., & Benza, R. L. (2016). RESPITE: Riociguat in pulmonary arterial hypertension patients with an inadequate response to phosphodiesterase type 5 inhibitors. 4.3 Pulmonary Circulation and Pulmonary Vascular Diseases. https://doi.org/10.1183/13993003.congress-2016.oa263 DISCLOSURES: No relevant relationships by Sheeja Schuster, source=Web Response Consultant relationship with Actelion Please note: $5001 - $20000 Added 03/15/2019 by Roxana Sulica, source=Web Response, value=Consulting fee Consultant relationship with Bayer Please note: $5001 - $20000 Added 03/15/2019 by Roxana Sulica, source=Web Response, value=Consulting fee Consultant relationship with United Therapeutics Please note: $5001 - $20000 Added 03/15/2019 by Roxana Sulica, source=Web Response, value=Consulting fee research relationship with Reata Please note: $5001 - $20000 Added 03/15/2019 by Roxana Sulica, source=Web Response, value=insitution was paid for resear