The Case Files
Welcome to the Case Files!
The Case Files is an anecdotal collection of emergency medicine cases to enable physicians and researchers to find clinically important information on unusual conditions.
Case reports should focus on:
- Unusual side effects or adverse interactions.
- Unusual presentations of a disease.
- Presentations of new and emerging diseases, including new street drugs.
- Findings that shed new light on a disease or an adverse effect.
Comment on a case or submit your own case following the instructions in the Submissions box to the right.
Tuesday, October 01, 2013
By Scott Goldstein, DO
I recently saw an 87-year-old African American woman with past medical history of rheumatoid arthritis on prednisone, iron, and potassium complaining of facial swelling over one or two days. She denied any history of diabetes mellitus, nausea and vomiting, headache, tooth extraction, trauma, or similar past episodes. She does admit to subman dibular swelling with pain, fever (up to 101°F at home), and anterior neck fullness. This started a “couple of days ago,” and her son was worried enough to bring her to the ED.
Upon arrival, she was in mild distress, hypertensive at 151/84 mm Hg, heart rate was 94 bpm, temperature was 101.8°F, and nonhypoxic at 98% on room air. She was sitting upright in mild distress. Her airway was obscured by her protruding tongue (see photograph), but she denied any trouble breathing. Breath sounds were equal, and her blood pressure was mildly elevated. Her pharynx could not be visualized secondary to current habitus. Her tongue was elevated and protruding with a firm intralingular base. Her neck was warm, swollen, and firm. The patient's only other complaint was 2+ pitting edema of both ankles, which she stated was a chronic problem.
The patient's chemistries, CBC, and coagulation studies were normal. ENT evaluated the patient, and agreed with a presumed diagnosis of Ludwig's angina from the physical exam. Anesthesia nasal-intubated the patient, clindamycin and metronidazole were given, and she was transported to the ICU.
The next day, a CT scan read by the attending radiologist revealed sialolithiasis of the submandibular gland, which excluded the diagnosis of Ludwig's angina. Sialolithiasis are salivary gland stones, and are found in about one percent of the population. Up to 95 percent of the stones are located in the submandibular glands. In patients with mild to moderate disease, stones can be confirmed by palpation or purulent discharge from the glandular duct with massage. This method also can be therapeutic if the obstruction is milked out of the duct at the time of diagnosis. Plain films and CT scans can be used to confirm ductal stones and obstructions. (Textbook of Family Practice. W.B. Saunders Co., 2002: 468.)
Stone formation is thought to arise from precipitation of crystalline material around an organic source, like bacteria or epithelial cells. (Otolaryngology: Head & Neck Surgery. Mosby, 2004: 1307; 4123.) Because one theory on formation is around an organic and possibly bacterial source, antibiotics to cover penicillinase-resistant organisms should be given. Symptomatic relief can be achieved by moist heat applied to the affected area, and the judicial use of sialogogues. The most common sialogogues used are usually citric, which helps promote salivary production.
Spaces in the peripharyngeal area can be invaded by external bacteria leading to various infections, including peritonsillar abscess, Ludwig's angina, retropharyngeal abscess, Lemierre's syndrome, osteomyelitis, uvulitits, lymphadenitis, and parotitis, to just name a few.
The mainstay of treatment is antibiotics and airway management, not incision and drainage. Surgical management may be reserved for severe cases and cases not responding to antibiotics. All cases should have an emergent ENT consult.
If conservative and symptomatic treatment fails or obvious active infection is present, stone removal is a viable option. There are many different methods to remove a salivary gland stone, the preferred being transoral stone removal under local anesthesia. This method is preferred over more invasive attempts because it has a higher chance of preserving the gland and causes less scarring of the ductal system. (Arch Otolaryngol Head Neck Surg 2001;127:432.) Some of the more novel approaches that are coming to the forefront are extracorporeal shock wave lithotripsy (ESWL) (Brit Dent J 2002:193:89) and interventional radiological approaches using stone retrieval catheters. (Dentomaxillofac Radiol 1996;25:242.) Some of the newer approaches, like the use of retrieval catheters, might soon become the preferred method of stone removal because they are not surgical and cause less postoperative damage to the ductal system.
Life-threatening diseases need to be considered and treated early in the evaluation of a patient. Even if the final diagnosis does not match the initial diagnosis, the presumed life threat needs to be treated. This patient presented with many of the symptoms of Ludwig's angina, which is potentially fatal. The final diagnosis is not considered a life-threatening disease, but the real possibility of airway obstruction made it a possibly devastating disease. The patient refused surgery to remove the obstructions, and left the hospital on oral antibiotics and ENT follow-up.
Thursday, September 26, 2013
By Andrew Park, DO, MPH, & Jeremy Hunter, DO
A 6-month-old boy was brought to the emergency department by his parents for seizure activity just prior to arrival. They reported that the baby had been feverish for two days, and was evaluated in a different ED. He was given Tylenol there.
He was eventually diagnosed with a viral syndrome and then discharged home. The parents reported that he had been febrile but otherwise normal since being discharged from the ED, but he began shaking his arms and appeared to be having a seizure approximately five minutes before arrival at our ED. The parents denied any previous seizure activity.
The child was placed in a treatment room for immediate evaluation of persistent seizure activity. His rectal temperature was 38.7°C on presentation, and physical exam revealed an inconsolable male infant with hypertonic upper and lower extremities. The patient’s arms were flexed approximately 45 degrees at the elbow, his fontanelle was bulging, and his head was rotated approximately 30 degrees to the left. The remainder of the physical examination was unremarkable except for mild frontal bossing.
IV access was attempted but proved difficult. During IV attempts, the patient’s mental status returned to normal, according to the parents, though they said he less active than usual. Seizure activity ceased. An intraosseous line was placed and sample obtained for laboratory analysis. A full septic workup was performed including lumbar puncture to confirm suspected meningitis because of the baby’s fever, prolonged seizure activity, and bulging fontanelle upon arrival.
Vancomycin, ceftriaxone, and dexamethasone were administered in the ED while awaiting results from laboratory. Laboratory results revealed WBC count 6.5, Hgb 10.5, Na 133, K 4.3, Cl 101, HCO3 19, glucose 126, albumin 4.4, calcium 4.5 (9.0-11.0), ionized calcium 0.56 (1.0-1.3), and alk phos 1085(95-327). CSF studies were negative for infectious process and revealed normal glucose and protein levels, negative gram stain, and 0 WBCs.
Further questioning of the parents revealed that the parents were vegan, and the patient was being breastfed by his mother. The patient received no formula or supplementation. The patient was dark-skinned, and had been brought to the ED during the late winter when he would have very limited access to direct sun exposure.
The patient was being transferred to the pediatric service as the laboratory results were returning. IV calcium was administered upon arrival to the PICU. The patient’s further workup revealed a 25-OH vitamin D level <4.0 (normal 30-80). Radiographs of bilateral wrists and knees were obtained that revealed bilateral metaphyseal flaring of distal femurs, proximal tibias, as well as bilateral metaphyseal flaring of radiuses and ulnas consistent with rickets. The patient was continued on calcium and vitamin D supplementation with normalization of calcium levels and decreasing alkaline phosphatase levels, and he had no more seizures.
Nutritional deficiency rickets is relatively rare in the United States. Weisberg et al found 166 U.S. cases of rickets in children under 18 between 1986 and 2003. (Am J Clin Nutr 2004;80[Supply 6]:1697S.) Most cases of rickets occur in African-American children that are breastfed. (Am J Clin Nutr 2004;80[Supply 6]:1697S.) Rickets seems to be reemerging, likely related to an increase in breastfed infants and the lack of education to breastfeeding mothers about vitamin D supplementation. (Pediatrics; 2008;122:1142.)
Vitamin D3 functions with parathyroid hormone (PTH) to maintain intracellular and extracellular calcium concentrations. If an individual’s vitamin D levels are not adequate, intestinal absorption of calcium is inadequate, which then leads to bone resorption to increase calcium levels. As bone resorption continues, parathyroid hormone and alkaline phosphatase levels will be high, calcium levels may be normal or low, and eventually bony changes consistent with resorption will become evident. Progression of the disease will lead to signs and symptoms of hypocalcaemia late in the disease process, including prolonged QT intervals, tetany, and seizures.
Vitamin D can be obtained enterally or via a photochemical reaction with ultraviolet light-B waves in the skin. Enteral sources of vitamin D include some fatty acids in fish and foods fortified with vitamin D. Human breast milk has very low levels of vitamin D. The photochemical reaction in the skin that produces vitamin D is between UV-B rays and 7-dehydrocholesterol. Melanin competes with 7-dehydrocholesterol for UV-B waves, which is why dark-skinned individuals are more likely to develop hypovitaminosis D when compared with lighter skinned individuals. (Am J Clin Nutr 2004;80[Supply 6]:1697S.) The American Academy of Pediatrics now recommends a minimum daily intake of 400 IU of vitamin D beginning soon after birth. (Pediatrics 2008;122:1142.)
Rickets may present in many different ways, but is typically discovered in the ED once the signs and symptoms of hypocalcaemia develop, including seizure, neuromuscular irritability, or tetany. (Pediatrics 2006; 118(5):2226.) The differential diagnosis for infantile seizure is broad and includes common etiologies such as infectious, metabolic derangements, trauma, toxic ingestions, drug withdrawal, and familial seizure disorder.
Our patient had all the key risk factors to place him at risk of hypocalcaemia, but was initially confusing because of a recent illness and presence of a fever. Sorting out these key features and risk-stratifying to determine what the patient is most at risk for is what we do best as emergency physicians. It is important for the emergency physician to obtain a detailed history and recognize patients at increased risk for developing rickets. Vitamin D-deficient rickets is a relatively rare disease in the United States, but one that seems to be increasing because of the increased number of breastfed infants with suboptimal or no vitamin D supplementation. (Pediatrics 2006;118:2226; 2008;122:1142.)
The growth plate in rickets is thickened and disorganized, with a large zone of hypertrophic cartilage cells. An irregular perforation of the cartilage plate by osteoclasts appears because there is little calcified cartilage. The woven bone on the surface of some of the primary trabeculae is unmineralized and will be easily fractured. Such microfractures often lead to hemorrhage at the interface between the plate and the metaphysis.
Dr. Park (pictured) is an assistant professor of emergency medicine at the University of Kansas Medical Center. Dr. Hunter is a third-year emergency medicine resident there.
Wednesday, August 21, 2013
By Punam Patel, DO, & Steven Sattler, DO
A 62-year-old Caucasian woman presented to the emergency department with palpitations that had started that morning. She had no history of similar symptoms and denied medications, allergies, and alcohol or drug use. She had quit smoking cigarettes 13 years earlier.
She initially denied all other symptoms, but staff overheard the patient and spouse complaining of headaches. They reported that they had been awakening with headaches and fatigue since moving into a new home a week earlier.
Vital signs upon arrival were respiration, 20 breaths per minute; heart rate, 156 beats per minute; blood pressure, 113/78 mm Hg, and temperature 97F orally. Physical exam showed a well-appearing woman in no acute distress with a rapid irregularly irregular heartbeat. The rest of the physical exam was normal.
An electrocardiogram showed AF with rapid ventricular response, a PVC, and a prolonged QTc. (Figure 1.) Laboratory results including complete blood count, basic metabolic panel, cardiac enzymes, and TSH were unremarkable. The COHb level was 22.2% of total hemoglobin. A chest radiograph was normal. The patient was started on high-flow oxygen therapy. The heart rate was controlled with Diltiazem 15 mg IVP followed by Diltiazem 90 mg PO.
The patient’s symptoms resolved with a repeat electrocardiogram that displayed normal sinus rhythm. (Figure 2.) A hyperbaric therapy-equipped institution was consulted, but we decided not to transfer her because her symptoms had resolved. The fire department was notified of the case to ensure a safe return home.
Cardiology was consulted, and an echocardiogram was normal. The patient was monitored in a telemetry unit overnight with no further episodes of AF. The patient was discharged on Diltiazem CD 120 mg PO daily and scheduled to follow up with her cardiologist. Diltiazem was discontinued shortly after discharge with no recurrence of AF. No signs of delayed neurological sequelae were reported, and the CO culprit was a faulty furnace.
Carbon monoxide intoxication is the leading cause of unintentional poisoning deaths in the United States. CO is a colorless, odorless, tasteless but highly toxic gas byproduct from incomplete combustion of hydrocarbon fuels. (J Neurol Sci 2007;262:122.) Lacking a unique clinical vignette, CO poisoning is a challenging diagnosis that is often missed.
CO when inhaled is readily absorbed from the lungs to the bloodstream forming a tight but slowly reversible complex with hemoglobin (Hb) known as carboxyhemoglobin (COHb). (Toxicology 2000;145:1.) CO easily displaces oxygen because the affinity of hemoglobin for CO is 210 times its affinity for oxygen. The reduced oxygen-carrying capacity results in tissue hypoxia and impaired cellular oxidative metabolism. The organs with the highest demand for oxygen such as the brain and the heart are more vulnerable to injury.
Clinical manifestations of CO poisoning range from mild to severe. Early symptoms include fatigue, headache, dizziness, confusion, nausea, and vomiting. Moderate symptoms include ataxia, tachypnea, palpitations, and chest pain. Hypotension, cardiac arrhythmias, myocardial ischemia, respiratory depression, seizures, and coma indicate severe intoxication.
The clinical symptoms of CO poisoning are often nonspecific, and can mimic a variety of common disorders. The severity ranges from mild flu-like symptoms to coma and death. (J Neurol Sci 2007;262:122.) A high index of suspicion and detailed history are essential to make this diagnosis. A delayed or incorrect diagnosis can mean poor outcomes for the patient and cohabitants.
The clinical spectrum of cardiac involvement in patients with carbon monoxide intoxication is broad, and may encompass cardiomyopathy, unstable angina, myocardial infarction, arrhythmias, heart failure, myocardial stunning, cardiogenic shock, and sudden death. (Clin Biochem 2012;45[16-17]:1278.) Chronic exposure to CO can induce myocardial injury and fibrosis while acute exposure is associated with arrhythmias that may lead to sudden death. (J Mol Cell Cardiol 2012;52:359.)
Myocardial injury is attributed to tissue hypoxia from hemoglobin’s extremely high affinity for CO, but this does not explain the cause of arrhythmias because COHb levels do not correlate to electrocardiogram changes. (J Mol Cell Cardiol 2012;52:359.) QT dispersion and variability, which reflect ventricular repolarization, and P-wave dispersion as a marker of sinoatrial conduction, are pathologically increased with CO exposure. (Respir Physiol Neurobiol 2012;184:204.) These electrocardiographic disturbances are directly linked to increased susceptibility of AF, premature ventricular contractions, and lower threshold for developing ventricular fibrillation observed after CO poisoning. (Respir Physiol Neurobiol 2012;184:204.) A detailed investigation of the effects of CO on ion channel activity is needed. (J Mol Cell Cardiol 2012;52:359.)
Carbon monoxide is eliminated through the lungs; its typical half-life is three to four hours. High-flow 100% oxygen therapy reduces the half-life to 30 to 60 minutes and hyperbaric oxygen therapy at 2.5 atm to 15 to 23 minutes. (Clin Biochem 2012;45[16-17]:1278.) The initial mainstay treatment for carbon monoxide toxicity is 100% oxygen at normal barometric pressure. HBO is recommended for severe poisoning regardless of CO level.
The specific requirements for normobaric versus hyperbaric treatment and their outcomes is a continued topic of debate. (Toxicology 2000;145:1.) A clinical policy statement by the American College of Emergency Physicians concluded that HBO is a treatment option but cannot be mandated; they failed to identify clinical variables where HBO would prove beneficial or harmful. (Ann Emerg Med 2008;51:138.) A Cochrane review concluded that existing evidence does not show that HBO decreases the incidence of adverse neurological sequelae. (Cochrane Database Syst Rev 2011 Apr 13:CD002041.) Pregnancy was an exclusion criterion in most randomized trials, but should always be considered because of potential benefit for mother and fetus. (Crit Care Clin 2012;28:537.)
This case illustrates the high index of suspicion required to link a new arrhythmia to CO poisoning. The complaint that led to the diagnosis of CO poisoning was initially missed in our case, and it could have easily resulted in misdiagnosis. Arrhythmias are commonly referred to as an effect of CO, but reports of CO-induced AF with resolution after oxygen therapy are limited.
Dr. Patel is a second-year emergency medicine resident, and Dr. Sattler is an emergency physician and the associate research director at Good Samaritan Hospital Medical Center, West Islip, NY.
Figure 1. Electrocardiogram showing atrial fibrillation on presentation to the ED.
Figure 2. Electrocardiogram showing normal sinus rhythm 22 hours after first presentation.
Tuesday, July 16, 2013
By Omer Richman, DO, and David Levy, DO
Pelvic angiography demonstrating right external iliac artery aneurysm with active extravasation of contrast into the jejunum.
A 54-year-old woman presented to the emergency department twice within one week for melena, hematemesis, and weakness. Between these ED visits, the patient underwent a colonoscopy, upper endoscopy, and wireless capsule endoscopy as an outpatient, which showed a non-bleeding arteriovenous malformation in the jejunum but were otherwise non-diagnostic.
The patient had a past medical history of end-stage renal disease secondary to type I diabetes mellitus, B-cell lymphoma, and lymphangioleiomyomatosis. She had a past surgical history of simultaneous pancreas-kidney transplant in 1989 (both grafts subsequently failed), living unrelated renal transplant in 2006, and pancreatic transplant in 2008, the latter of which failed four months prior to presentation. She denied a prior history of gastrointestinal bleeding, nausea, vomiting, diarrhea, and constipation. She was taking insulin, tacrolimus, and prednisone, and denied non-steroidal anti-inflammatory use.
Her vital signs on her second visit were blood pressure 112/53 mm Hg, heart rate 87 beats per minute, respiratory rate 20 breaths per minute, and temperature 37.5°C (99.5°F). Physical examination revealed a patient who was awake, alert, and oriented to person, place, and time with no conjunctival pallor. Her heart had a regular rate and rhythm without murmurs, rubs, or gallops. She had clear lungs bilaterally, and her abdomen was soft, mildly distended, and diffusely tender without rebound or guarding, with normoactive bowel sounds and no organomegaly. Digital rectal examination revealed normal rectal tone, no external hemorrhoids or fissures, and guaiac positive stool that was not grossly bloody or melanotic.
The patient received supportive care, and was admitted to the medical floor for symptomatic gastrointestinal bleeding. She had additional episodes of melena on the medical floor with dropping hematocrit, and was transferred to the medical intensive care unit.
The patient had an episode of massive hematochezia during her stay in the ICU, after which she became profoundly hypotensive with systolic blood pressure in the 40s and loss of consciousness, and she was intubated. Laboratory samples sent during this episode revealed hemoglobin and hematocrit of 4.4 mg/dL and 13.1%, respectively. Massive transfusion protocol was initiated, and a STAT bedside esophagogastroduodenoscopy was performed, which was non-diagnostic. She was taken to interventional radiology for an angiogram, which demonstrated an aneurysm of the right external iliac artery with extravasation of contrast into the jejunum. (Figure.)
Two stent-grafts were placed with no further filling of the aneurysm on completion arteriogram. The patient’s blood pressure rose and eventually stabilized in the 110s/50s over the next several hours. Her hospital course was later complicated by acute tubular necrosis and oliguria requiring hemodialysis, disseminated intravascular coagulopathy, and febrile episodes, but she was ultimately extubated and made a full recovery.
This case of upper and lower gastrointestinal bleeding was caused by arterioenteric fistula formation in a simultaneous pancreas-kidney (SPK) transplant patient who later underwent repeat kidney and pancreas transplants. Initial complication rates for this procedure were very high, but outcomes have improved dramatically with technical advances, the introduction of cyclosporine, and the surgery being performed more frequently. (Am Surg 2004;70:529.) That means emergency physicians are more likely to encounter these patients and the complications associated with their operations.
Bleeding from an arterioenteric fistula is seen more often as a long-term complication with timing intervals ranging from 60 days to 10 years post-transplantation. (Am Surg 2004;70:529; Transplant Proc 1994;26:2292; Transplantation 2001;72:966 and 1997;63:903; Arch Surg 1989;124:660.) It is a rare complication, but is associated with a high mortality rate. (Cardiovasc Intervent Radiol 2008;31:542.) These patients can exsanguinate quickly, so consideration should be given to immediate intervention. Angiography with embolization has been used to treat gastrointestinal bleeding since the 1970s, but recent literature has shown that endovascular stent-graft placement should be considered as a valuable alternative in hemodynamically unstable patients. (Cardiovasc Intervent Radiol 2007;30:313; Transplantation 2001;72:336.)
Gastrointestinal bleeding is a common presenting complaint to the emergency department that may be associated with significant morbidity and mortality. Colonoscopy and endoscopy are often used to evaluate gastrointestinal bleeding, but have a low yield in particularly brisk bleeds. Likewise, nuclear medicine studies have been used in localization of the bleeding vessel in patients with a history of vascular reconstruction, but have the same limitation with high rates of bleeding and are not appropriate for a hemodynamically unstable patient. (J Med Case Rep 2010;4:357.) The next step would be conventional angiography or surgical intervention when these modalities cannot be utilized or are non-diagnostic.
Special consideration should be given to a patient who has had vascular reconstructive surgery. A rare but potentially fatal source of gastrointestinal hemorrhage in such a patient is an arterioenteric fistula. Massive bleeding in such patients is often preceded by a small sentinel bleed or intermittent bleeding for years prior. (Am Surg 2004;70:529; J Med Case Rep 2010;4:357.) Tien et al examined the efficiency of angiography as the initial diagnostic and therapeutic modality for patients with a sentinel bleed after pancreaticoduodenectomy (PD). (Ann Surg Oncol 2008;15:1855.) Twenty of 283 patients (7%) in the study group who underwent PD between July 2002 and June 2007 had a sentinel bleed.
Seven of these 20 (35%) had pseudoaneurysm detected on angiography, and all were successfully embolized. Compared with a historical control group of 311 PD patients who did not undergo angiography after a sentinel bleed, the number of hemodynamically unstable patients, units of transfused packed red blood cells, and mortalities related to bleeding were significantly lower in the study group. Immediate and accurate diagnosis of sentinel bleed has the potential to prevent massive hemorrhage. It may be prudent to strongly consider angiography as the initial management for select patients with even relatively benign-appearing gastrointestinal bleeding. This does not, however, preclude the need to involve a surgical consultant because there is no guarantee that angiography will be successful in identifying the source or controlling the hemorrhage.
Not enough data are available to unequivocally recommend angiography as first-line management in transplant patients with gastrointestinal bleeding, but it is an important tool at the emergency physician’s disposal. Patients with a prior transplant history who present to the emergency department with gastrointestinal bleeding, regardless of the severity, should warrant a high index of suspicion for arterioenteric fistula because a missed or delayed diagnosis may rapidly become fatal.
A literature search revealed only a few other cases of arterioenteric fistulas after SPK transplant, only one in the emergency medicine literature. (Ann Emerg Med 2003;42:587.) Our case typifies the need to have a high index of suspicion for a rare cause of a common emergency department complaint.
Dr. Richman is a second-year emergency medicine resident and Dr. Levy the director of the emergency medicine residency program at Good Samaritan Hospital in West Islip, NY.
Monday, April 29, 2013
By Alice Chao, MD, and Ayesha Khan, MD, MPH
A 44-year-old previously healthy woman presented to the emergency department with chest pain and shortness of breath. The patient reported a two-month history of polyarthralgias and rash. She initially noted swelling and pain of her ankles that progressed to her knees, wrists, and shoulders.
The patient also reported a non-pruritic rash with erythematous borders on her extremities that would come and go (Figure 1), nodules in her skin, and intermittent fevers.
She denied any insect bites or recent travel, and had no personal or family history of autoimmune disorders. She reported a sore throat two weeks prior to onset of symptoms that was not treated. The patient was found to be in sinus tachycardia (Figure 2), and she complained of chest pressure and difficulty breathing.
She continued to have persistent tachycardia despite fluid hydration and pain control. A CT angiogram of the chest was negative for pulmonary embolism. The patient was admitted for persistent tachycardia and further workup of recent symptoms that were concerning for acute rheumatic fever. Laboratory tests revealed a high antistreptolysin O titer. Throat culture was positive for Streptococcus pyogenes.
She also had elevated ESR and CRP. An echocardiogram was done during the hospitalization, which revealed mild thickening of the mitral valve and aortic valve with mild mitral and aortic regurgitation. Echo also revealed minimal pericardial effusion. The treating physicians felt the patient met the criteria for acute rheumatic fever (major criteria: polyarthritis, carditis/valvulitis, erythema marginatum [Figure 3], subcutaneous nodules; minor criteria: fever, arthralgia, elevated ESR and CRP, prolonged PR interval).
The patient was started on penicillin 250 mg four times a day for two weeks, followed by 250 mg BID indefinitely for prophylaxis. She was also discharged on prednisone 40 mg daily and ibuprofen 800 mg TID for symptomatic relief. The patient's arthralgias and rash improved after treatment was initiated, and she is being followed by rheumatology and cardiology as an outpatient. Acute rheumatic fever (ARF) is a delayed sequela of group A streptococcus (GAS) pharyngeal infection.
A latent period of two to three weeks precedes the first signs of ARF. ARF and subsequent rheumatic heart disease affect almost 20 million people in the developing world, and are the leading cause of cardiovascular death in patients under age 50. (Lancet Infect Dis 2005;5:685.) The mean incidence is 19 per 100,000 population.
A much lower incidence of ARF is reported in the United States and developed countries, likely because of improved hygienic standard and routine antibiotic treatment of acute pharyngitis. The incidence of ARF in developed countries has been as low as two to 14 cases per 100,000, though recent local outbreaks have occurred. (Circulation 2009;119:1541; New Engl J Med 1987;316:421; Pediatr Infect Dis J 1990;9:97.)
T. Duckett Jones, MD, first described and published diagnostic criteria for acute rheumatic fever in 1944. (JAMA 1944;126:481.) The American Heart Association subsequently established guidelines for diagnosing rheumatic fever (See Table 1 at end of article). (JAMA 1992; 268:2069.)
A diagnosis of acute rheumatic fever requires the presence of two major manifestations or of one major and two minor manifestations with supporting evidence of a preceding group A streptococcal infection, according to the Jones criteria. Our patient had four major criteria (polyarthritis, carditis, erythema marginatum, and subcutaneous nodules) and four minor criteria (fever, arthralgia, elevated ESR and CRP, and prolonged PR interval). She also had elevated antistreptolysin O titers and a positive throat culture for group A streptococcus, both supporting a prior GAS infection.
The three major treatment goals of acute rheumatic fever are relief of symptoms, eradication of GAS, and prophylaxis against future GAS infection to prevent recurrent cardiac disease. Aspirin or other antiinflammtory agents are the mainstay of symptomatic management because of ARF, including relief of discomfort related to arthritis and fever.
Oral penicillin V is the drug of choice for eradication of GAS pharyngitis. Amoxicillin and cephalexin are also acceptable treatments. Approved alternatives for patients with potential severe hypersensitivity to beta-lactam antibiotics are azithromycin, clarithromycin, and clindamycin. (Circulation 2009;119:1541.)
Prevention of recurrent GAS pharyngitis is the most effective method to limit progression of rheumatic heart disease because patients who have had rheumatic fever are at high risk for a recurrent attack. This secondary prevention requires continuous antibiotic prophylaxis, the duration of which depends on the risk of recurrence. Patients who have had rheumatic carditis are at relatively high risk for recurrence with cardiac involvement progressing with each recurrence.
Prophylaxis in these patients should continue until the patient reaches age 21 or for a total of 10 years (whichever is longer). Our 44-year-old patient, who is older than the typically affected population (ages 5–15), will need prophylaxis for several years. The antibiotic of choice for prophylaxis is IM penicillin G every four weeks or PO penicillin V twice daily. (Circulation 2009;119:1541.)
Recent resurgence of rheumatic fever in the United States after years of decline suggests increases in undiagnosed and untreated cases of streptococcus pharyngitis. Rheumatic heart disease, a preventable illness, can lead to devastating complications such as cardiomyopathy, congestive heart failure, or complete heart block. It is important for physicians to remain diligent in diagnosing and treating cases of confirmed or suspected streptococcal pharyngitis.
Dr. Chao is a second-year resident in the Stanford/Kaiser
Emergency Medicine Residency Program. Dr. Khan is
a clinical instructor in emergency medicine at Stanford
Table 1. Guidelines for Diagnosing an Initial Attack of Rheumatic Fever
Elevated acute phase reactants (ESR, CRP)
Prolonged PR interval
Supporting Evidence of Antecedent Group A Streptococcal Infection
Positive throat culture of rapid streptococcal antigen test
Elevated or rising streptococcal antibody titer
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