Lyme Arthritis an Update for Clinical Practice
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CME REVIEW ARTICLE Lyme Arthritis An Update for Clinical Practice Katharine Christina Long, MD* and Keri Anne Cohn, MD, MPH, DTM&H† significantly swollen left knee that is mildly warm to the touch. Abstract: Lyme disease is the most common vector-borne illness in He has discomfort with range of motion but is not exquisitely ten- North America, with the majority of cases occurring in the Northeast and der. He ambulates with a limp. He has a temperature of 100.8°F. upper Midwest. Lyme arthritis is the most prevalent manifestation of late- The remainder of his vital signs and physical examination stage Lyme disease. Lyme arthritis typically presents as a monoarthritis is normal. or oligoarthritis in large joints such as the knee. Accompanying positive How do you proceed in evaluating this patient? What diagnos- 2-tier Lyme serologies or polymerase chain reaction from synovial fluid/ tic tests are warranted? What diagnostic tests are useful? How do tissue is considered diagnostic for patients from an endemic area. The you acutely differentiate between Lyme arthritis and a septic joint? mainstay of initial treatment is a prolonged course of oral antibiotics. What is the best initial treatment? What anticipatory guidance do Key Words: Lyme arthritis, Lyme disease, pediatric arthritis you provide the family? (Pediatr Emer Care 2018;34: 588–593) INTRODUCTION Lyme arthritis was first described as an epidemic of TARGET AUDIENCE oligoarticular arthritis in the 1970s. Steere et al1 published a series This continuing medical education activity is targeted at any of cases describing the culture-negative, serologic-negative arthri- health care provider who may encounter and diagnose pediatric tis afflicting a small Connecticut coastal town called Lyme. In patients presenting with Lyme arthritis. General pediatricians, 1982, Dr Burgdorfer2 was the first to isolate the infectious agent nurse practitioners, physician assistants, and health care providers a spirochete, which he named Borrelia burgdorferi. Lyme disease who practice in emergency departments or urgent cares may find is now considered the most common vector-borne illness in North this content beneficial to their practice. America and Europe.3 The geographical distribution of Lyme disease has been consistently expanding over the last few years, now predom- LEARNING OBJECTIVES inately throughout the Northeast and Minnesota and Wisconsin in the Midwest. In 2015, 95% of confirmed cases of Lyme disease After completion of this CME activity, the reader should be originated from 14 states (Fig. 1). better able to: The primary vector is the Ixodes tick. It is believed that the majority of human Lyme disease cases are transmitted by ticks 1. Describe the epidemiology of Lyme disease. while they are in their small and difficult-to-detect nymph stage. 2. Identify the common manifestation of Lyme arthritis and differ- An infected nymph must feed for at least 72 hours in order to entiate it from other similar disease presentations. transmit the disease because the spirochetes live in the midgut of 3. List available diagnostic and treatment options. the tick and migrate to the salivary glands only after the gut has become engorged.3,4 The transmission rate at 72 hours or longer is 25%.3 n 8-year-old boy presents to your emergency department with There is a distinct seasonality to the disease, with two thirds A a chief complaint of left knee swelling that started 3 days ago. of cases of early Lyme occurring in June, July, or August, al- The family and patient deny any history of trauma. The patient has though peak incidence is largely dependent on temperature and had a low-grade fever. The patient reports that he feels like his moisture.5,6 Because Lyme arthritis is a late manifestation that oc- “ ” knee is going to pop from the pressure. He denies any other con- curs weeks or months after the initial infection, presentation can stitutional symptoms. Because you are an astute historian, you dis- occur during any season. Unsurprisingly, Lyme disease is more of- cover that the patient visited his grandparents in Connecticut over ten diagnosed in people who spend a large proportion of time out- the summer, approximately 4 months ago. On physical examina- doors in endemic areas. tion, you see an alert and interactive young man with a PRESENTATION *Academic Chief of Emergency Department Education, Valley Children's Classically, there are 3 described phases of Lyme disease: Hospital, Clovis; and Clinical Instructor (Affiliate) of Emergency Depart- ment, Stanford University School of Medicine, Stanford, CA (Long); and early localized disease, early disseminated disease, and late dis- †Assistant Professor of Clinical Pediatrics, Perelman School of Medicine at ease. Although these entities are separated out for classification the University of Pennsylvania, Philadelphia, PA; and Director of Pediatric purposes, patients can present with overlapping symptoms. Early Emergency Medicine–Global Health Fellowship (Cohn), The Children's Hos- localized disease manifests as erythema migrans, a large singular pital of Philadelphia, Philadelphia, PA. The authors, faculty, and staff in a position to control the content of this CME annular skin rash that classically expands from the site of tick bite, activity and their spouses/life partners (if any) have disclosed that they have typically 3 to 30 days later. The rash is typically uniformly ery- no financial relationships with, or financial interest in, any commercial thematous or targetoid and can be associated with vesicular or ne- organizations pertaining to this educational activity. crotic areas in the center.4 Patients may or may not recall a tick bite Reprints: Katharine Christina Long, MD, Valley Children's Hospital, Madera, 1830 N Sanders, Clovis, CA 93619 (e-mail: [email protected]). on history. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. Multiple erythema migrans skin lesions are associated with ISSN: 0749-5161 early disseminated Lyme disease, which appears 3 to 5 weeks after 588 www.pec-online.com Pediatric Emergency Care • Volume 34, Number 8, August 2018 Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. Pediatric Emergency Care • Volume 34, Number 8, August 2018 Lyme Arthritis FIGURE 1. Reported cases of Lyme disease 2001 compared with 2015. States in 2015 with reported Lyme disease: Connecticut, Delaware, Maine, Maryland, Massachusetts, Minnesota, New Hampshire, New Jersey, New York, Pennsylvania, Rhode Island, Vermont, Virginia, and Wisconsin, taken from https://www.cdc.gov/lyme/stats/index.html. exposure. Other manifestations of early disseminated disease in- propose predictor models that have not yet been externally vali- clude systemic symptoms such as fever, headache, myalgias, dated. In Lyme-endemic areas, arthrocentesis may be cautiously and fatigue. Occasionally, there are neurological manifestations deferred, pending serology results in children 2 years or older with at this stage such as facial nerve palsies or Lyme meningitis. Lyme unilateral knee swelling who do not have a history of fever, have carditis with associated prolonged PR interval or complete heart no pain with short arc motion, and have a C-reactive protein of block can also occur. Late disease is associated primarily with less than 4.0 mg/L or have an erythrocyte sedimentation rate of Lyme arthritis and occurs weeks to months after exposure. Enceph- less than 40 mm/h and an absolute neutrophil count of less than alopathy and encephalitis are also part of late-stage disease presen- 10 Â 103 cells/μL, as they are unlikely to have septic arthritis.15,16 tation; however, these entities are extremely rare. Lyme arthritis typically presents as a monoarthritis or oligoarthritis primarily involving the knee.1,7–10 The knee tends Serologic Testing to be very swollen with limited range of motion. It can be ery- The immune system takes several weeks to develop antibod- thematous and warm.11 Pain varies, although Lyme arthritis tends ies after exposure to B. burgdorferi. In early-stage Lyme disease, it not to be exquisitely painful as is seen with septic joints, and chil- is well known that one can be seronegative even after clinically dren can usually still ambulate with a limp. Lyme arthritis can also manifesting signs of Lyme such as erythema migrans.17 However, manifest as an asymmetric oligoarthritis. In oligoarthritis, the knee by the time patients develop Lyme arthritis (late disease), immuno- is involved along with other large joints such as the hip, shoulder, globulin G for B. burgdorferi is positive and is considered diagnostic ankle, elbow, temporomandibular joint, and wrist. Occasionally, in a patient from an endemic area who presents with monoarthritis or the arthritis is accompanied by constitutional symptoms such as oligoarthritis.18 Immunoglobulin M may start to wane and can be fever and fatigue.1,10 Overall, the incidence of Lyme arthritis is de- negative by 30 days and may or may not be positive at the time of creasing, secondary to early recognition and treatment of Lyme serologic testing. Initial serologic testing is performed using an disease in patients. However, approximately 50% to 60% of pa- enzyme-linked immunosorbent assay screening, followed by a con- tients who are not treated in the early stages of Lyme disease will firmatory Western blot test. Lyme arthritis generates a robust immu- go on to develop Lyme arthritis.9 For patients with a diagnosis of nological response that subsequently seems to provide a protective