she was seen in the Emergency Department for a cough with blood-tinged sputum and discharged on amoxicillin-clavulanate and azithromycin for community acquired pneumonia. Three days later, she presented to the hospital again for worsening hemoptysis and dyspnea and was admitted for acute hypoxic respiratory failure requiring intubation. PR3 antibody was positive, and she was extubated before transferring to our quaternary referral center for Rheumatology evaluation. The presenting labs were pertinent for: white blood cell count of 22 cells/mm3, hemoglobin of 12.8 g/L, creatinine of 5.2 mg/dL (baseline 0.9 mg/dL). Bilateral ground glass opacities were seen on the CT of the chest. The patient was on 5L of supplemental oxygen via nasal cannula and bilateral rales were auscultated on physical exam. Bronchoscopy was consistent with diffuse alveolar hemorrhage. Renal biopsy revealed pauci immune crescentic glomerulonephritis. Hemodialysis was started for
acute renal failure. A diagnosis of granulomatosis with polyangiitis was made and therapy was initiated with plasmapheresis, cyclophosphamide and rituximab. After 2 weeks of treatment, hemodialysis was discontinued, her creatinine improved close to baseline, and she was weaned to room air. She discharged and completed induction therapy outpatient with follow up in Rheumatology clinic. Discussion: This case demonstrates the severity of granulomatosis with polyangiitis and the importance of recognition of otomastoiditis as a precenting concern. This case also denotes the importance, particularly in young women, of early diagnosis and the potential for progression into diffuse alveolar hemorrhage. While combination induction therapy with both rituximab and cyclophosphamide remains controversial due to its adverse effects, this case displays its benefit in life threatening GPA with pulmonary involvement.
HEPATOPULMONARY SYNDROME (HPS) OUGHT TO BE CONSIDERED IN PATIENTS WITH CHRONIC LIVER DISEASE AND PERSISTENT HYPOXIA Amit Rajkarnikar, Jonathan B. Vignes; Louisiana State University, Lafayette, LA.
Introduction: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVDs) in the setting of liver disease or portal hypertension. Transthoracic contrast echocardiogram (TTCE) with agitated saline bubble study should be considered for patients with persistent hypoxia and chronic liver disease.
deferred for concerns of esophageal varices. A second bubble study showed bubbles clearly entering the left atrial cavity through pulmonary veins after 3 cardiac cycles. Bubbles appeared not to cross the atrial wall. This is most consistent with intrapulmonary shunt and with conjunction of underlying cirrhosis HPS was diagnosed. She was referred to a hepatologist. An arterial blood gas (ABG) revealed PaO2 of 61 mm Hg on room air at initial evaluation that corrected to 400 mm Hg with 100% FiO2. A repeat ABG two months later showed a PaO2 of 55 mm Hg on room air that improved to 348 on 100% FiO2. She met the criteria for moderate to severe Type 1 HPS. She is now listed for liver transplant. Discussion: Most patients with HPS present with symptoms and signs of chronic liver disease, that are neither sensitive nor specific for HPS. TTCE with agitated saline is the gold standard for diagnosing pulmonary vascular dilatation. Based on observational studies, liver transplantation demonstrates complete or near complete resolution
Case: 69-year-old female with nonalcoholic steatohepatitis related cirrhosis, splenic and
esophageal varices, chronic pancytopenia, type II diabetes mellitus, hyperlipidemia, and a remote history of a deep venous thrombosis presented with dyspnea on exertion and lightheadedness. Of note, the patient had two prior hospitalizations for atypical chest pain accompanied by shortness of breath in the span of 6 months. She underwent TTCE that demonstrated a positive bubble study with evidence of right-to-left shunt physiology, but undetermined if the bubbles were early versus late in their timing of the shunt. Transesophageal echocardiogram was
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