Emergency Medicine Curriculum Utilizing the Flipped Classroom Method: Infectious Disease and Immunology

Author(s): Ostro, Benjamin; King, Andrew; Branditz, Lauren; Martin, Daniel; Bachmann, Daniel; Panchal, Ashish; Barrie, Michael


Problem identification, general and targeted needs assessment:
Traditional lecture-based didactics may not be the most effective or preferred method for emergency medicine resident education. 6 Previously, we used a traditional lecture format in our residency curriculum despite overwhelming evidence favoring a more hands-on, "flipped classroom" approach. 10,11 From the perspective of resident learners, the chance to remain fully engaged through the asking of questions developed from personal experiences, in addition to also learning from the experiences of others, provides a manner of learning that makes a topic more difficult to forget. 5 Infectious disease and immunology emergencies are identified as an important aspect of an emergency medicine curriculum as outlined in the ABEM Model EM curriculum. 8 This topic makes up a significant portion of the content that will appear on the emergency medicine intraining exam. Because of this, we decided that infectious disease and immunology emergencies warranted a dedicated content block within our flipped classroom curriculum. We grouped immunologic emergencies, such as anaphylaxis, transplant-related emergencies, and collagen vascular disease with infectious disease to create the immunology content block.
Both educators and learners benefit from an interactive and collaborative classroom, leading to the creation and implementation of this proposed curricular model at our emergency medicine residency program. 12 This weekly small group curriculum has now replaced three hours of traditional lecture-based didactics. Learners divide into small groups of about 20 participants. Each group is led a faculty facilitator, with the option for senior residents to facilitate discussion. Since implementation, residents and educators are engaging in new, valuable flipped classroom learning communities. Through the curriculum, we continually seek to foster self-directed learning and increased collaboration between resident learners and education faculty members. This ensures that resident time will be maximized and learning will be more efficient and effective, therefore providing a potential positive impact on patient care and physician wellness. Currently, minimal flipped classroom curricular materials dedicated to the core content of emergency medicine exist.

Goals of the curriculum:
We aim to teach the presentation and management of infectious disease and immunology emergencies through the creation of a flipped classroom design. The topics include sepsis, sexually transmitted infections, tropical diseases, angioedema and anaphylaxis, transplant-related emergencies, and collagen vascular diseases. This unique, innovative curriculum utilizes resources chosen by education faculty and resident learners, study questions, real-life experiences, and small group discussions in place of traditional lectures. In doing so, a goal of the curriculum is to encourage self-directed learning, improve understanding and knowledge retention, and improve the educational experience of our residents.

Objectives of the curriculum:
Each chapter within our curriculum has individual objectives as outlined in the appendices; however, educational objectives for the overall curriculum include: 1. Sepsis a. Define both systemic inflammatory response syndrome (SIRS) and quick sepsis related organ failure assessment (qSOFA) and how they are used to screen patients with possible sepsis. b. Understand the most recent Surviving Sepsis guidelines. c. List the most common causes of sepsis. d. Review the workup and diagnosis of sepsis.

Evaluation and Feedback:
This curriculum was literature-based and specifically designed to maximize active learning using the flipped classroom learning model. We overcame initial challenges and skepticism from both educators and learners to execute a successful, novel curricular model. Both resident learners and faculty educators have provided an overwhelming amount of positive feedback. Additionally, a survey was administered to each resident prior to initiation of the curricular innovation, and repeated at the conclusion of the first 18-month cycle. Learners and educators were enthusiastic about the conference structure and expressed a preference for it rather than the previous, lecturebased didactics. More recently during the second 18-month cycle of the flipped classroom curriculum, students were surveyed on their perceived quality of instruction of the various program components. In comparing varying conference activities over the last year, a majority of residents (60.9%) preferred small group discussions to formal grand rounds lectures. This curriculum has been delivered to two cohorts of learners, the content having been delivered twice in three years with about 50 residents per cycle. On the most recent iteration, residents evaluated the teaching methods as effective, with an average rating of more than 4.6 out of 5 (4 being agree, 5 being strongly agree). The curriculum is critically evaluated and updated by education faculty members in order to ensure educational material remains current and consistent with the emergency medicine core content.

Objectives
By the end of this small group session, the learner will be able to: 1. Define both systemic inflammatory response syndrome (SIRS) and quick sepsis-related organ failure assessment (qSOFA) and how they are used to screen patients with possible sepsis. 2. Understand the most recent Surviving Sepsis guidelines. 3. List the most common causes of sepsis. 4. Review the workup and diagnosis of sepsis. 5. Discuss the management of a patient with suspected sepsis and common complications.

Question Prompts:
1. Describe your assessment of this patient? What commonly used screening criteria exist for sepsis?
a. This patient presents with signs and symptoms consistent with sepsis due to urinary tract infection. The markedly elevated lactate and hypotension are concerning for septic shock. b. In the emergency department, identifying patients with sepsis can be extremely difficult.
Systemic inflammatory response syndrome (SIRS) criteria were developed over twenty years ago and can be present in a variety of conditions that are not sepsis. For example, patients with ankle fractures may be tachycardic and tachypneic due to pain. c. Systemic inflammatory response syndrome is diagnosed when a patient has two or more of the following: repeat chest X-ray shows diffuse infiltrates and you notice bruising of the skin, oozing from IV sites, and pink tinged sputum.
Question Prompts: 1. What potential complications are occurring in this patient? What would be your diagnostic strategy to verify these? a. This patient may be developing disseminated intravascular coagulation (DIC), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy leading to hemorrhage. b. Diagnostic tests for DIC include a complete blood count (CBC) with smear, lactate dehydrogenase (LDH) (elevated), liver function tests (increased bilirubin), coagulation studies (elevated), fibrin degradation products (elevated), d-dimer (elevated), and fibrinogen (decreased). c. The patient's shortness of breath may be a sign of pulmonary edema from excessive fluid administration or acute respiratory distress syndrome (ARDS). Acute respiratory distress syndrome is an acute inflammatory process within the lungs leading to increased pulmonary vascular permeability. The chest X-ray will show diffuse infiltrates. 2. How would you treat these complications in the ED? a. Patients with ARDS will be hypoxic and often require mechanical ventilation. Noninvasive ventilation is not as helpful as mechanical ventilation. During mechanical ventilation low tidal volume ventilation is recommended. The use of positive end-expiratory pressure (PEEP) can be employed according to the low tidal volume (6-8mL/kg ideal body weight) ventilation strategy described in the ARDSnet protocol. 4 For patients who fail the low tidal volume ventilation strategy, prolonged ventilation can be considered. b. Regarding DIC, the treatment is aimed toward treating the underlying cause. In the case of sepsis, this is the administration of appropriate antibiotics and source control as well as aggressive hydration. Patients with platelet counts less than 50,000 x 10 3 /mm 3 should be given platelet transfusions if there is serious bleeding or the need for urgent/emergent surgery. Patients with platelet counts of less than 10,000 x 10 3 /mm 3 should be given platelets because of the risk of spontaneous bleeding. Consider the use of fresh frozen plasma in the setting of serious bleeding and significantly prolonged prothrombin time (PT) and partial thromboplastin time (PTT) or a fibrinogen level less than 50mg/dL. Cryoprecipitate can also be considered because this provides a good source of fibrinogen with less volume than fresh frozen plasma.

Objectives
By the end of this small group session, the learner will be able to: 1. Discuss the most common sexually transmitted infections (STIs) managed in the emergency department (ED). 2. Describe the appropriate workup, treatment, and disposition of patients with cervicitis and pelvic inflammatory disease (PID). 3. Discuss the state specific guidelines for mandatory health department reporting of STIs. 4. Discuss the evaluation and management of meningitis in patients with human immunodeficiency virus (HIV). 5. Review acquired immunodeficiency syndrome (AIDS) defining illnesses.

Case Studies
Case 1: A 19-year-old female presents to the emergency department for lower abdominal pain. She reports pain in the left lower quadrant ongoing for several days associated with vaginal discharge. She is sexually active with males and does not routinely use barrier protection. On exam, the patient has left lower quadrant tenderness. External genitalia exam reveals shallow, tender erythematous ulcerations along the labia. Pelvic exam reveals purulent discharge from the cervical os. She has exquisite cervical motion tenderness and also tenderness of the left adnexa.
Question Prompts: 1. What testing is needed for this patient?
a. In patients with suspected pelvic inflammatory disease (PID) with unilateral adnexal tenderness, consider a transvaginal ultrasound to evaluate for evidence of tubo-ovarian abscess (TOA). b. The ulcers may be swabbed as well and sent for herpes simplex virus (HSV) culture. c. Urinalysis and pregnancy test should also be ordered. d. In most practice settings, it is also be appropriate to screen for sexually transmitted diseases such gonorrhea, chlamydia and trichomonas. Common testing also includes screening for Gardnerella vaginalis (bacteria thought to be pathogenic in bacterial vaginosis) and candida. Routine testing for bacterial vaginosis can be deferred to an outpatient basis unless the patient is symptomatic on presentation. e. Below is a wet mount image of clue cells diagnostic of bacterial vaginosis: Ostro  PO/intravenous (IV) 100mg BID PLUS either cefotetan 2g IV or cefoxitin 2g IV BID. An alternative regimen is clindamycin 900mg IV three times daily (TID) AND gentamycin 5mg/kg IV daily. Consider adding metronidazole for trichomonas coverage. b. Patients with isolated cervicitis should be treated with drug regimens effective against both gonorrhea and chlamydia. Treat with ceftriaxone 250mg IM once PLUS azithromycin 1g PO once. An alternative regimen is ceftriaxone 250mg IM once plus doxycycline 100mg PO BID x 7 days. Patients should abstain from sexual intercourse for seven days after treatment AND until resolution of symptoms. c. In the case of severe penicillin/cephalosporin allergy, patients can be treated with azithromycin 2g PO once. These patients should be re-evaluated seven days after treatment to ensure resolution of symptoms. Additionally, a test of cure should be performed three weeks after completion of treatment. Nucleic acid amplification tests remain positive in the presence of dead organisms and should not be used earlier than three weeks due to risk of false positive results. d. In general, pregnant patients with PID should be admitted to the hospital and started on IV antibiotics. Doxycycline is category D for pregnancy and should be avoided. Consider alternative regimen such as clindamycin and gentamycin in consultation with obstetrics and gynecology (OB/gyn). e. Of note, men who present with urethritis, epididymitis, or orchitis with suspected sexually transmitted infection should be treated similarly. Epididymitis may be due to enteric bacteria so also consider levofloxacin 500 mg orally once a day for 10 days (often older patients). Trimethoprim-sulfamethoxazole is an alternative for patients allergic to quinolones or with a history of tendon rupture. 3. What are indications for admission? a. Tubo-ovarian abscess b. Pelvic inflammatory disease in setting of pregnancy i. Pelvic inflammatory disease during pregnancy occurs most commonly within the first 12 weeks before a mucus plug develops which acts as a barrier to ascending infection. ii. Pregnant women with suspected PID are high risk for maternal morbidity and preterm delivery. These patients should be admitted and given parenteral antibiotics. c. Nausea, vomiting, fever, failure of outpatient therapy.

While in the ED her Affirm (rapid polymerase chain reaction [PCR] antigen test) is positive for
Gardnerella vaginalis. Is this considered a sexually transmitted infection? What are the treatment options? What are the specific risks in pregnant women? a. Bacterial vaginosis (BV) is the most common vaginal infection in women ages 15-44. Sexual activity likely contributes to BV. While BV is not considered an STI, having BV can increase your chances of getting an STI. Bacterial vaginosis rarely affects women who have never had sex. Recommended bacterial vaginosis treatment for symptomatic women is metronidazole 500 mg orally twice daily x 7 days, metronidazole gel 0.5% intravaginally once daily x 5 days, or clindamycin cream 2% intravaginally once each night x 7 days. b. Pregnant women with BV are more likely to deliver prematurely (early) or with low birth weight as compared to women who do not have BV while pregnant. 5. What is the differential for the patient's painful vaginal lesions? What is the most likely diagnosis?

DIDACTICS AND HANDS-ON CURRICULUM
What is the most appropriate treatment?
The most likely diagnosis is genital herpes simplex virus (HSV). Alternative etiologies include syphilis (painless), chancroid (painful), lymphogranuloma venereum (painless), and granuloma inguinale (painless). a. All patients with first episode of HSV need to be treated with acyclovir or valacyclovir. The most common treatment recommendation is acyclovir 400 mg orally TID x 7-10 days or valacyclovir 1 g orally BID x 7-10 days. Patients with severe disease or complications (eg, disseminated infection, pneumonitis, or hepatitis) or central nervous system (eg, meningoencephalitis) need hospitalization and IV treatment. 6. The patient is discharged with the appropriate antibiotic regimen. Two days later her cervical swab returns positive for gonorrhea. Is this a reportable disease? What sexually transmitted infections must be reported to the health department? a. Sexually transmitted diseases should be reported in accordance with state and local statutory requirements. b. In Ohio, HIV/AIDS, hepatitis (A, B, C, D, E), syphilis, gonorrhea and chlamydia are all reportable. Ohio's reportable disease list is found at: http://www.odh.ohio.gov/reportablediseases. There are other diseases on the list which are not sexually transmitted. c. Instructors should review their own states requirements to discuss during the session.
Case 2: A 28-year-old incarcerated male presents to the ED for evaluation of "flu-like symptoms." He reports having a nonproductive cough for about one week, fever, headache, neck pain, nausea, and vomiting. The prison guards report he has seemed confused over the last 24 hours. Past medical history is significant for HIV with an unknown recent CD4 count. The patient admits he has not seen his infectious disease specialist for more than a year. On exam, your patient is ill appearing. He opens eyes to talk to you, but complains of photophobia. He will not turn his head because it increases pain in his neck and back.

Question Prompts:
1. What is your primary concern in terms of diagnosis?
a. This patient presents with signs and symptoms concerning for meningoencephalitis. 1. What factors in the patient's history raise your concern for possible travel-related illness? What other questions or historical features are useful when assessing fever in the returning traveler? a. The features of this presentation that are concerning are the travel to a malaria-endemic area (sub-Saharan Africa), the questionable compliance with malaria prophylaxis, and the mosquito bites. Other considerations for this patient (or any returning traveler with fever) should include: immunization status, immunocompetence, diet history while traveling, sick contacts including any treatment in local healthcare facilities, fresh water exposure, sexual encounters, animal bites or animal exposure, and potential bodily fluid exposures (eg, tattoos). b. Keep in mind that illness in a returning traveler does not necessarily represent tropical disease. Clinicians should consider typical domestic causes of fever and other constitutional symptoms such as pneumonia, urinary tract infection (UTI), and soft tissue infections. Also stay mindful of the global disease patterns for worldwide pathogens such as influenza. The peak incidence rates vary from region to region resulting in the ability for a patient to present with a common disease at an uncommon time for that region. c. While returning travelers may present with a spectrum of symptoms, fever is by far the most common symptom and also the symptom most consistently seen with acute lifethreatening illnesses. d. The differential for travel-related illness includes the following: malaria, dengue fever, enteric fever, Zika, Ebola, chikungunya, and Middle East respiratory syndrome coronavirus (MERS-CoV)-just to mention a few. 2. What travel-related illness is this patient's presentation most concerning for and what is the mechanism of transmission for this disease? What are the diagnostic and therapeutic considerations for this disease? a. While the presentation is somewhat nonspecific (as travel-related diseases often are), the main concern based on the locale traveled to is malaria. Specifically, the provider should be concerned for Falciparum malaria given the presence of Plasmodium Falciparum in this area. Malaria is a protozoan infection that is transmitted by the Anopheles mosquito. This mosquito tends to be most active at dusk. Typically, the incubation period for P. Falciparum is 7-14 days although it can extend up to 6 weeks. b. The diagnostic test of choice for malaria is a peripheral blood smear with presence of parasites. This test should be performed three times to improve sensitivity. Other screening labs include: complete blood count (CBC), basic metabolic panel, liver function tests (LFTs), coagulation studies, blood culture, and lactate. More specific testing should be guided based on the other symptoms and concern for specific end-organ dysfunction. World Health Organization (WHO) criteria for severe malaria include one or more of the following: prostration, impaired consciousness, respiratory distress, multiple convulsions, circulatory collapse, pulmonary edema, abnormal bleeding, jaundice, hemoglobinuria, severe anemia (hemoglobin less than 7g/dL or hematocrit less than 20% in adults, less than 5g/dL or less than 15% in children less than 12 years), hypoglycemia, acidosis, or elevated lactate. c. Management is comprised by initial resuscitation, appropriate anti-malarial medication, and screening/treatment of end-organ dysfunction. Most patients, like this one, do not require intervention for airway, breathing and circulation (ABCs), though some patients will have a severe presentation with their malaria. The adequate chemotherapy depends on the suspected strain of Plasmodium and the local resistance pattern. For this patient's travel pattern, artesunate is the preferred parenteral antimalarial drug over quinidine for first-line therapy. For oral therapy, atovaquone-proguanil is preferred over chloroquine for Falciparum due to resistance patterns. Chloroquine is still appropriate for non-Falciparum malaria. Chemoprophylaxis with anti-malarial medication and reduction of mosquito exposure are cornerstones of disease prevention for malaria. d. Complications of severe malaria are varied and can include the following: cerebral malaria, acute respiratory distress syndrome (ARDS), anemia, renal dysfunction, jaundice, blackwater fever (massive hemoglobinuria causing dark urine), hypoglycemia, shock, lactic acidosis, gastrointestinal (GI) symptoms, and disseminated intravascular coagulation. The specific complications should be treated aggressively in addition to initiation of the antimalarial medication. e. Chikungunya, a mosquito-borne infection caused by the Chikungunya virus (CHIKV), may present similarly with fever, joint pain, headache, and rash. The infection is typically selflimited with symptoms resolving within a week. However, occasionally patients experience joint pain for months. Treatment is supportive. f. Over the last five years there have been several outbreaks of Ebola first in West Africa and more recently in the Democratic Republic of Congo. While the World Health Organization declared the most recent outbreak over in July 2018, Ebola should still be considered for any traveler from these areas presenting with hemorrhagic fever.

DIDACTICS AND HANDS-ON CURRICULUM
Case 2: A 28-year-old male with past medical history of gastroesophageal reflux disease presents to the ED with complaint of fever and diarrhea. He has noticed fever over the past two weeks to 102°F. He has two days of loose stool, which is described as "pea soup." Review of systems is additionally positive for mild abdominal pain, malaise, headache, and arthralgias. His travel history includes returning from a two-week trip to India one month ago. His vitals are temp 101°F, blood pressure (BP) 100/60mmHg, heart rate (HR) 70/min, respiratory rate (RR) 16/min, and oxygen saturation (O2sat) 98% on room air. His abdomen is slightly distended but soft and without signs of peritonitis.
Question Prompts: 1. What type of travel-related illness is of highest concern? Describe the natural course of this disease process. a. The primary etiology for this presentation is enteric fever, which is caused by infection with Salmonella species S. typhi or S. paratyphi. This disease accounts for approximately 20% of travel-related acute life-threatening fever. The distribution is widespread across Central and South Americas, Africa, and Asia-though it is highest on the Indian subcontinent. This is also a disease that can affect pediatric patients more severely. The incubation period is 10-20 days with an illness pattern that follows four one-week phases. The first week includes fever with nonspecific symptoms of headache, congestion, cough, and malaise. The second week shows progression to apathy, higher fevers with relative bradycardia, abdominal distention, and "rose spot" rash. In severe cases the toxicity continues to rise into the third week with tachypnea, diffuse 'pea soup' diarrhea, weakness, and mental status changes. The fourth week shows resolution and improvement in these symptoms. b. The other travel-related illness that presents with diarrhea is traveler's diarrhea. This entity typically does not include fever though, and it is much more self-limited and benign. Transmission is via fecal-oral route, but the most common pathogen involved in this disease is Enterotoxigenic E. coli. Some other pathogens include Campylobacter, Shigella, and Giardia. This disease process has a much shorter incubation period than enteric fever and overall illness timeframe. Most cases are self-limiting and management is largely symptomatic with loperamide and oral hydration. Antibiotic therapy with three to five days of ciprofloxacin can be considered for more prolonged cases (greater than 10-14 days), or in severe cases in patients with fever, abnormal vital signs, evidence of dehydration, or electrolyte derangements.
i. Patients with HAE have deficiency in either the functionality or quantity of C1 esterase inhibitor (C1-INH) which is responsible for inhibiting conversion of kallikrein to bradykinin. Lack of C1-INH leads to increased bradykinin levels and, in turn, angioedema. ii. Angiotensin I converting enzyme (ACE) is responsible for breakdown of bradykinin.
Patients taking ACE inhibitors are thus prone to developing high levels of bradykinin which can cause angioedema. 2. What is the appropriate initial management and diagnostic workup in this patient?
a. The initial management of patients with angioedema is focused on the need for airway intervention. Patients who demonstrate evidence of laryngeal edema should be intubated early for airway protection. Airway involvement may be ascertained on a clinical basis by evaluating for dysphonia, difficulty tolerating oral secretions, and stridor. These patients should be intubated emergently. Alternatively, in equivocal cases, the airway should be visualized either by laryngoscopy or nasopharyngoscopy with all equipment available for intubation. b. In this case, the patient's airway is patent and he does not have any signs of laryngeal involvement and does not require any immediate interventions. In more equivocal cases, nasopharyngoscopy can be used to evaluate for edema of the glottis structures. c. Diagnosis of angioedema in the emergency department is based on clinical presentation, history, and physical examination. Diagnostic testing aimed at determining the exact cause of the angioedema is beyond the means of the emergency department and laboratory testing may take several days to result. Furthermore, there is no definitive diagnostic test for ACE inhibitor-induced angioedema. If there is a suspicion for HAE, serum C4 and CI-INH levels are often ordered as a screening test followed by C1-INH levels for confirmation. (See Case 2) 3. What is the appropriate treatment for this patient? Should he be intubated for airway protection?
a. The Ishoo staging of angioedema helps predict which patients will require airway intervention based on the location and extent of the edema. c. Pharmacologic treatment of ACE inhibitor-induced angioedema in the ED has two phases: management of acute episodes and short-term prophylaxis. i. The first step is discontinuation of the offending agent which in this case is lisinopril.

Stage
ii. Unlike histaminergic angioedema such as anaphylaxis, when treating bradykininmediated angioedema, antihistamines, steroids and epinephrine have limited, if any, utility. However, due to their low cost and minimal side effects, it is often protocol to administer H1 blockers, H2 blockers, and steroids. iii. Fresh frozen plasma (FFP) (class II recommendation): Although the data is limited and of poor quality, FFP is often given to patients with angioedema. In theory, administration of FFP will replete the patient's stores of C1-INH which in turn prevents further production of bradykinin. a. Disposition should be made on the basis of the clinical presentation and likelihood for needing airway intervention. This can best be predicted with the Ishoo staging system (see above). Patients with isolated lip/face swelling or soft palatal swelling may be placed in observation. Conversely, those with lingual edema should all be admitted to the ICU for close airway monitoring. b. Patients admitted placed in observation will be routinely watched for at least 4-6 hours after peak clinical edema before discharge. c. For this patient, his swelling is limited to his lip and thus he would be an appropriate candidate for observation. 5. You're ready to discharge the patient. You have counseled the patient on discontinuation of his lisinopril but you ask yourself, is it safe to start him on an angiotensin receptor blocker (ARB)? a. The use of ARBs after an episode of ACE inhibitor-induced angioedema is controversial. A meta-analysis in 2008 showed that the risk of angioedema on an ARB after confirmed ACE inhibitor-induced angioedema is 3.5%-9.4%. Because there are many treatment options for hypertension, many would avoid ARBs in patients with a history of ACE inhibitor-induced angioedema. Case 2: A 22-year-old female with no known past medical history presents with a chief complaint of hoarse voice, tongue swelling, and difficulty breathing. She states that her symptoms started a few hours prior to arrival. She denies any obvious precipitating factors. She was not eating or taking medications prior to the onset of her symptoms and she has no known allergies. She notes she has had similar albeit milder episodes in the past, but they resolved spontaneously and she never sought medical attention. When asked about family history she says that her mother has had throat swelling several times requiring intubation. Review of systems is remarkable for colicky abdominal pain. She denies pain, rash, pruritus, chest pain, fever, and chills. On physical exam she is in moderate respiratory distress with tachypnea, dysphonia, and mild stridor. Oropharyngeal exam reveals significant lingual edema and swelling of the posterior structures. Her vitals are T 98.6°F, HR 110/min, RR 25/min, BP 110/60mmHg, O2sat 95% on RA.

DIDACTICS AND HANDS-ON CURRICULUM
Question Prompts: 1. What is the most likely diagnosis? What is a likely explanation of her abdominal pain? a. This patient is most likely suffering from HAE. The key to this case is eliciting a family history. b. Hereditary angioedema is most often inherited in autosomal dominant fashion. c. There are three types of HAE. The differences are beyond the scope of emergency medicine practice. d. Just as in anaphylaxis, angioedema can affect the gastrointestinal mucosa and can produce abdominal pain and rarely bowel obstruction when severe. 2. What is the most appropriate management of this patient? What diagnostic tests should be ordered? a. In any case of angioedema there should be a low threshold to establish a definitive airway. This patient demonstrates dysphonia, stridor, and tachypnea suggesting that she is suffering from angioedema involving the larynx. The most appropriate next step in management is emergent intubation for airway protection. b. When preparing to intubate this patient it is crucial that you identify predictors of difficulty with both bag valve mask ventilation (BVM) and direct laryngoscopy. This patient will be predicted to be difficult to bag due to obstruction from lingual edema. Because this patient cannot be safely rescued with BVM, paralysis and rapid sequence intubation are contraindicated. This patient's lingual edema will make direct laryngoscopy very difficult. Although video laryngoscopy is an option, it may be difficult to physically fit the blade into the mouth. The most appropriate method of intubation in this case is fiberoptic nasotracheal intubation. The patient should be sat upright and sedated with an agent such as ketamine. A paralytic should be available for use if the cords are adducted. It is vital that a double set up be available at the bedside in case emergent surgical airway is necessary. Of note, laryngeal mask airways (LMAs) or other extraglottic devices may not be effective in cases of severe lingual edema. It should be cautioned that any physical irritation of the airway during evaluation or intubation may increase the edema present and result in further airway compromise. i. These patients require high doses of systemic steroids (0.5 to 1.0 g/day of methylprednisolone) often given in combination with other immunosuppressive agents. A number of other immunosuppressive agents have also been used for refractory disease including rituximab and belimumab. Immunosuppressive agents are also given for frequent flares. These medications should only be initiated by an ED physician after consultation with rheumatology. 2. Discuss the work up and differential for this patient's chest pain.

DIDACTICS AND HANDS-ON CURRICULUM
a. Systemic lupus erythematosus patients are at risk for thromboembolic disease especially those with antiphospholipid antibodies. Therefore, pulmonary embolism (PE) and acute coronary syndrome must be in the differential. Serositis including pleuritis and pericarditis should be considered and should be suspected if there is a new pleural effusion. Pericarditis is felt to be the most common cardiac manifestation but myocarditis is also possible and therefore a cardiac echo should be considered. As with any case of pericarditis, a bedside ultrasound should be performed to rule out pericardial effusion which can accumulate rapidly and progress to cardiac tamponade. b. The risk of coronary artery disease (CAD) is at least doubled in SLE patients and particularly in young females. Autopsy series in young SLE patients show that up to half had significant atherosclerosis. If initial work up is negative, provocative testing for CAD should be considered. c. Systemic lupus erythematosus patients are at high risk of developing infection from the immunosuppression associated with the disease and with the treatment so pneumonia should also be considered. d. Emergency department work up for this patient should include ECG (looking for tachycardia, ST-segment changes, right heart strain and conduction system changes), chest X-ray, troponin, complete blood count, acute inflammatory markers, chemistry to assess renal disease. Although D-dimer can be considered, these patients are often high risk for PE rather than moderate or low risk and therefore may need a computed tomography angiography (CTA). In addition, CT can be more sensitive to pick up pulmonary infiltrates. 3. What in this patient's history can explain her pancytopenia?
a. Systemic lupus erythematosus can cause pancytopenia, leukopenia or thrombocytopenia. Infections can also cause bone marrow suppression. Since the patient was recently started on methotrexate, if the patient has not been taking folate with this medication, worsening anemia and thrombocytopenia can also be caused by folate deficiency. Case 2: A 44-year-old female with a history of dermatomyositis (DM) presents with a chief complaint of cough, wheezing, and increased weakness. She explains that the diagnosis of DM was made several months ago after "muscle tests" and lab tests and her presentation of an unusual rash with papules and proximal muscle weakness, creating problems climbing up stairs and standing from sitting positions. She is now taking prednisone 5 mg per day and azathioprine 100 mg per day and has been on this regimen for the last two months. On her exam she is weaker than normal and has wheezes on lung exam. Temperature is 99.0°F with otherwise unremarkable vital signs. Her labs and chest X-ray are negative.

DIDACTICS AND HANDS-ON CURRICULUM
Question Prompts: 1. Discuss the usual presentation and treatment of dermatomyositis. a. Most patients present during ages 40 to 50 with muscle weakness (90%), and muscle pain (50%) and the skin manifestations may precede the muscle symptoms. Deltoids and hip flexors are the most common muscles involved. Classic skin manifestations include Gottron's papules, which are erythematous to violaceous papules that occur symmetrically over the extensor (dorsal) aspects of the metacarpophalangeal (MCP) and interphalangeal (IP) joints. In addition, these lesions may involve the skin between the MCP and IP joints, particularly when the eruption is prominent. Gottron's papules often have associated scale and may ulcerate. When scaling is present, the lesions may mimic psoriasis or lichen planus. The heliotrope eruption is an erythematous to violaceous eruption on the upper eyelids, sometimes accompanied by eyelid edema. Patients may have midfacial erythema that can mimic the malar erythema seen in SLE. In contrast to those with SLE, patients with DM will often have involvement of the nasolabial fold. b. The mainstay of treatment includes glucocorticoid therapy. Ideally, normalization of enzymes and complete recovery of muscle strength should occur before glucocorticoids are tapered. Continuation of high-dose glucocorticoids for more than six weeks may cause steroid myopathy and decline in strength. Steroid sparing medications include azathioprine. c. Selected patients with severe life-threatening weakness or patients with severe dysphagia at risk for aspiration may benefit from the addition of intravenous immune globulin (IVIG) to initial treatment with glucocorticoids. Intravenous immune globulin may have a more rapid onset of action than glucocorticoids, but prolonged treatment is limited by difficulty of administration, cost, and potential toxicity. Intravenous immune globulin also has a role in the treatment of resistant and recurrent cases. 2. What is the most likely diagnosis? How might these symptoms be related to her diagnosis of DM? a. Patients with DM suffer from weakness of the striated muscle of the upper third of the esophagus. This can result in severe GERD which can lead to severe aspiration and chronic pulmonary disease. These symptoms occurring despite prednisone and azathioprine suggests refractory disease and not recurrent disease. Rituximab and IVIG should be considered if the disease becomes refractory. Gastrointestinal and pulmonary disease along with increased weakness could mean refractory disease requiring admission for IVIG. In addition, rapidly progressive interstitial lung disease in the setting of DM can also be lethal. Case 3: A 43-year-old male with ankylosing spondylitis presents with a chief complaint of eye redness, pain, and photophobia. He states that his symptoms were gradual in onset and have been progressive over the last week. He denies trauma to the eye or any precipitating event. He denies fevers, headaches, nausea, vomiting, and eye drainage. He is on infliximab but takes no other medications. His vital signs are all within normal limits.

DIDACTICS AND HANDS-ON CURRICULUM
Question Prompts: 1. Discuss the general approach to ankylosing spondylitis (AS) and the treatment. a. Ankylosing spondylitis is a chronic inflammatory disease manifested by back pain and progressive spinal stiffness. Goals of treatment are to eliminate symptoms and prevent complications of spinal disease such as flexion contractures and dorsal kyphosis, and to minimize extraspinal and extraarticular manifestations and comorbidities. b. All patients with AS show significant changes in the plain radiographs of the sacroiliac joints in the form of erosions or fusion. In advanced disease, plain radiographs of the spine will reveal "bamboo spine" with virtually complete fusion of the vertebral column. At that stage there is usually fusion of the sacroiliac joints. c. Pharmacotherapy includes one or more of the following: nonsteroidal anti-inflammatory drugs (NSAIDs), sulfasalazine, and anti-tumor necrosis factor (anti-TNF) agents. Unlike other collagen vascular diseases, systemic glucocorticoids have a limited role, but intraarticular injections may be helpful to some patients. 2. What is the likely diagnosis and treatment for the eye findings in this patient? a. The most likely diagnosis here is anterior uveitis. Roughly 30%-40% of patients with AS will develop anterior uveitis in their lifetime. b. Although the most likely diagnosis here is anterior uveitis, infectious causes must be ruled out and ophthalmology consultation or at least very close ophthalmology follow-up is indicated. c. Anterior uveitis may produce pain, redness, photophobia (and consensual photophobia).
The redness is primarily noted at the limbus (the junction between the cornea and the sclera). Such patients can have a constricted pupil, pain, and cells and flare on exam. d. Noninfectious causes of anterior uveitis are generally treated with topical glucocorticoids such as prednisolone acetate (1 percent). A mydriatic agent can relieve pain due to spasm of the muscles controlling the pupil and will also help to prevent the formation of posterior synechiae that may interfere with the function of the pupil.

Small Group Evaluation
The moderator demonstrated adequate knowledge of subject.

5) Strongly
Agree 4) Agree 3) Slightly Agree 2) Disagree 1) Strongly Disagree The moderator's facilitation of the conference facilitated my learning.

5) Strongly Agree 4) Agree 3) Slightly Agree 2) Disagree 1) Strongly Disagree
The overall discussion was relevant to the stated topic(s).

Preparation -was faculty well prepared?
Needs Improvement Effective Exemplary

Engaged residents -Encouraged discussion and actively participated, demonstrated enthusiasm?
Needs Improvement Effective Exemplary Strengths: Areas for Improvement: Reviewer Recommendations: Resident Facilitator Evaluation

Preparation -was the resident facilitator well prepared?
Needs Improvement Effective Exemplary