M. Khalil 1 , R. Granieri 1 ; 1 University of Pittsburgh, Pittsburgh, PA (Tracking ID #73745) LEARNING OBJECTIVES: 1. To recognize ascites as a complication of eosinophilic gastroenteritis (EG) 2. To diagnose and treat EG. CASE INFORMATION: A 50 year-old female with no significant PMH presented with a 2 month history of epigastric pain that was associated with nausea and decreased appetite. She reported the pain to be constant and exacerbated with food, but mildly relieved with a bowel movement. She denied any vomiting, melena, hematochezia, fever, chills, recent travel, change in diet or alcohol abuse. Two weeks prior to admission, she developed non-bloody, watery diarrhea, increased abdominal girth, and 12 lb. weight gain, despite the poor appetite. Outpatient evaluation with abdominal ultrasound was unremarkable and an upper GI series revealed dysmotility of the lower esophagus with tertiary contractions. Treatment with rabeprazole provided minimal relief. Physical examination upon admission was remarkable for a mildly distended and diffusely tender abdomen with positive shifting dullness but no rebound, guarding or organomegaly. The remainder of physical exam was normal including heme negative stool. Lab work was significant for WBC of 14,000 with 30% eosinophils. Liver function tests were normal. Serum albumin was 2.4 and ascitic fluid analysis showed: pH 7.31, albumin 1.8, negative cytology, and WBC of 4700 with 96% eosinophils. Stool culture and sensitivity, ova and parasites were negative. Further workup with abdominal ultrasound with Doppler showed patent vessel and normal direction of flow. Upper endoscopy revealed erythema of the antrum. Colonoscopy was normal. CT scan of the abdomen and pelvis showed diffusè`L akes of pus'' small bowel thickening without fat stranding and moderate ascites. Trans-vaginal ultrasound was negative for ovarian mass. Bone marrow biopsy was normal. The patient refused surgical bowel biopsy and diagnosis of EG was established on the basis of exclusion. She was started on oral dose of prednisone for 6 weeks and discharged to home. Follow up visit and blood work showed 0% eosinophils, resolution of ascites, resolution ofabdominal pain, and good appetite. IMPLICATIONS/DISCUSSION: EG is an uncommon disorder characterized by gastrointestinal symptoms, blood eosinophilia, and eosinophilic infiltration of the gastrointestinal wall. The signs and symptoms of EG are related to the layer and extent of the bowel involved with eosinophilic infiltration. Patients with subserosal infiltration may develop eosinophilc ascites. The diagnosis of EG is confirmed by a full thickness biopsy of the bowel in the absence of intestinal parasitic infection. Treatment of EG is empiric because no prospective, randomized clinical trials are available to guide treatment. Prednisone (20 to 40 mg/day) is recommended for two weeks and should be tapered over the next two weeks. Improvement usually occurs within two weeks of therapy. Despite the rarity of EG, it should be suspected in a patient with any GI symptoms associated with peripheral eosinophilia because this treatable disease can disguise itself as the irritable bowel syndrome. LEARNING OBJECTIVES: 1) Recognize that pulmonary hypertension is not uncommon patients with SLE; 2) Recognize that primary pulmonary hypertension in SLE patients often presents earlier than pulmonary hypertension from a secondary cause. CASE INFORMATION: A 21-year-old woman was transferred to our hospital for management of her pulmonary hypertension (PHT) and systemic lupus erythematosus (SLE). She initially presented a year ago with progressive cough and pleuritic-type chest pain. 8 months prior to admission, she underwent an echocardiogram which revealed a pulmonary artery pressure (PAP) of 27 mm Hg. Four months later, she was admitted to a community hospital for congestive heart failure and was found to have positive anti-Smith, antiphospholipidand anti-nuclear antibodies, and was diagnosed with SLE. Her symptoms gradually progressed to dyspnea on exertion, paroxysmal nocturnal dyspnea and Raynaud's phenomenon. She was subsequently readmitted to that hospital 2 weeks prior to transfer to us for worsening of her symptoms. There, she had a generalized seizure and was initiated on plasmapheresis treatment. Examination revealed a thin, anxious lady in mild dyspnea and an obvious malar rash. Cardiac auscultation revealed a loud and palpable P2, and a II/VI systolic murmur at the tricuspid area. She had a non-tender hepatomegaly and mild bilateral pedal edema. Echocardiogram revealed a PAP of 76 mm Hg, with evidence of right heart enlargement and mild tricuspid regurgitation. A chest CT scan with angiography and a ventilation-perfusion scan showed no signs of a pulmonary embolism. A right heart catheterization was done as well showing moderate PHT and a moderate response to epoprostenol challenge. Workup for lupus cerebritis was negative. She was initiated on prednisone and Cytoxan for SLE. Plasmapheresis was discontinued. Diltiazem, Valsartan and Warfarin were initiated to control her pulmonary hypertension. She tolerated the medications well and was discharged with outpatient follow-up, with the consideration of using epoprostenol in the future should her condition worsen. IMPLICATIONS/DISCUSSION: Prevalence of PHT in SLE ranges from 5% to 14%. A third of SLE patients have positive antiphospholipid antibodies, although the significance of this in the development of PHT is unknown. In one study of SLE patients, 5.9% were found to have pulmonary hypertension, of which 48% of them had no identifiable cause, the rest found to have a secondary cause. For the subgroup with secondary hypertension, 50% of them were due to valvular abnormalities. The primary pulmonary hypertension tended to present far earlier after diagnosis of the SLE compared to secondary PHT, 8.8 versus 43.2 months. Raynaud's phenomenon in secondary hypertension is associated with a more severe pulmonary artery pressure. These patients seem to respond to epoprostenol therapy although there have been documented cases of fatal thrombocytopenia. year-old female with rheumatoid arthritis, hypertension, and seizure disorder presented with two days of hematuria. She denied fevers, dysuria, urinary frequency, headaches, visual changes, or cough. Her last seizure was two weeks prior to presentation. Medications included gabapentin, phenytoin, methotrexate, and amlodipine. There was no fever and her physical examination was otherwise negative for lymphadenopathy, hepatosplenomegaly or petechiae/purpura. Initial workup revealed a platelet count of 7,000, H/H of 7.3/24, WBC of 8.2, LDH of 900, and Cr of 3.5. Peripheral smear demonstrated schistocytosis. The patient was diagnosed with TTP-HUS and began daily plasmapheresis and prednisone, during which her platelet count improved to 130,000 and her LDH normalized. She was discharged to continue outpatient pheresis but was readmitted one week later with fever (1038 F) and a platelet count of 78,000. She was begun on plasmapheresis with cryoprecipitate-poor plasma, vincristine, and steroids. She responded well; her pheresis was decreased to every other day and prednisone was tapered. She was discharged with a platelet count of 398,000 to continue outpatient pheresis and vincristine, which were discontinued after one month. One month later, the patient had a platelet count of 70,000, LDH of 1,186, and schistocytes on peripheral smear. Plasmapheresis and prednisone were restarted, and a splenectomy was performed. Discharge platelet count was 199,000. Two weeks later, her platelet count was 57,000. Pheresis and vincristine were restarted with good response. IMPLICATIONS/DISCUSSION: TTP and HUS are diseases with abnormalities in multiple systems. Classically, TTP is described as having more severe neurologic disease, whereas HUS has more of a renal component. Clinically, however, patients are described as having TTP-HUS because most present with the same symptoms, have identical pathologic changes, and receive the same treatment. The classic pentad of thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, renal abnormalities and fever is present in few patients. The diagnosis is made primarily by the presence of thrombocytopenia and microangiopathic hemolytic anemia. The etiology is a deficiency of von Willebrand factor (vWF) cleaving protease, which results in the accumulation of unusually large vWF multimers, causing platelet aggregation. Causes of TTP-HUS include E. coli 0157:H7 and medications such as ticlopidine, cyclosporine, and quinine. Most adult cases, however, are idiopathic. Treatment is plasmapheresis, the mechanism of which is not completely understood. 10 to 20% of patients have an incomplete response; steroids, vincristine or IVIG can be added in these cases, but are not alternatives to pheresis. Some studies show improvement with splenectomy, especially in conjunction with pheresis and steroids. 30 to 50% of patients have exacerbations of thrombocytopenia and hemolysis when pheresis is tapered. Relapse is defined as recurrence after 30 symptom-free days without treatment. Both are treated with reinstitution of daily pheresis. It is essential that physicians involved in the care of the patient with TTP-HUS recognize that many patients may have worsening disease with tapering of pheresis and to be aware of alternate life-saving treatments if this occurs. year-old female presented with 4 weeks of malaise, a dry cough, and recent high fevers. She also had night sweats for the 6 months since she started hormone replacement therapy. She had no pets and no recent travel history. Though exposed to tuberculosis as a child, she had never been tested or treated for it. She had begun minocycline just over a month earlier for rosacea and was on loratidine for seasonal allergies. She did not drink alcohol and quit smoking 5 years prior. Physical exam was notable for high fever, tachypnea, pulse oximetry of 96% on room air, inspiratory crackles at the right base and dullness at the left base. Labwork: WBC 15.2 with 84% neutrophils; blood eosinophilia increased over several days to 15%. ESR was 70. Chest radiograph showed small bilateral effusions, no cardiomegaly, and bilateral, peripheral, apical alveolar infiltrates. PPD was negative. Her fevers persisted and the left pleural effusion increased on intial therapy for community-acquired pneumonia. The effusion was transudative, with 1755 WBCs, 12% neutrophils and 10% eosinophils. A bronchoalveolar lavage (BAL) and transbronchial biopsies were performed; BAL fluid contained 63% eosinophils and biopsies revealed focal aggregates of eosinophils around the airways, consistent with eosinophilic pneumonia. Oral prednisone was started and both symptoms and eosinophilia resolved 48 hours after her first steroid dose. IMPLICATIONS/DISCUSSION: EP is characterized by eosinophilic pulmonary infiltrates. It presents as an acute febrile illness with dyspnea, night sweats, weight loss, dry cough, crackles or wheezing on lung exam, increasing blood eosinophilia and bilateral, peripheral patchy infiltrates on chest radiograph. The ESR is often elevated. Etiologies include common medications, including minocycline, parasitic infections, HIV, and fungal infections. A high index of suspicion, confirmed by BAL fluid eosinophilia and diffuse eosinophilic infiltration of the lung on biopsy, leads to the diagnosis. The response to steroids is dramatic, as was seen in our patient, with symptoms, eosinophilia, and lung infiltrates decreasing within 48 hours. Treatment is continued for 3 months to prevent relapses, which respond to re-intitiation of steroids.