Lyme arthritis of the pediatric lower extremity in the setting of polyarticular disease
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Abstract
Background
Lyme arthritis can be readily treated with use of oral antibiotics without any need for surgery. In Lyme-endemic areas, differentiating between Lyme arthritis and septic arthritis can be difficult. Laboratory testing for Lyme disease often results in a delay in diagnosis because many labs batch-test Lyme specimens only two times per week due to lack of equipment or increased expense. Delayed diagnosis can lead to unneeded surgery in cases in which the surgeon indicates the patient for a joint irrigation and debridement (I & D) for possible septic arthritis while waiting for Lyme serology results. The purpose of this study was to develop an algorithm for the treatment of patients with possible Lyme arthritis, with particular attention to poly-articular involvement.
Methods
Thirty-nine patients with poly-articular Lyme arthritis, including ankle involvement, were reviewed retrospectively. Patients were included if the ankle was involved, if they were less than 18 years of age, and had available laboratory information and a serologic diagnosis of Lyme disease.
Results
Only two patients had isolated ankle involvement; of those with poly-articular involvement, 34 patients had ankle/knee involvement. Nine patients presented with pain in the ankle with passive range of motion (PROM) (22 %); two (4.8 %) had refusal to bear weight, and 10 (24 %) had an antalgic gait. All patients had a positive Western blot. Ten patients had a peripheral white blood cell (WBC) count >12,500/mm3 , and 16 patients had an erythrocyte sedimentation rate (ESR) >40 mm/h.
Conclusion
Without immediate availability of Lyme serology, the decision to perform surgical drainage of a swollen joint in the setting of possible Lyme arthritis versus septic bacterial arthritis remains a clinical dilemma. Our data suggests that patients presenting with one or fewer Kocher criteria symptoms, poly-articular disease, and minimal pain with PROM have Lyme, rather than septic, arthritis. These patients can be treated with joint aspiration for cultures, appropriate antibiotics for Lyme disease, and careful serial exams while waiting for results of Lyme serology rather than immediate surgical I & D.
Keywords
Lyme arthritis Poly-articular involvement Clinical algorithmIntroduction
Lyme disease is an important consideration in the evaluation of the pediatric patient with a limp. In 60 % of patients who go untreated for Lyme disease, arthritis will be the hallmark feature, presenting several months after the initial tick bite [1]. Lyme disease is recognized as the most common tick-borne disease in the US; per the Centers for Disease Control (CDC), there were approximately 19,000 cases in 2006 [1]. The pediatric orthopaedist, rather than the pediatric rheumatologist, is often the first specialist to see a patient with Lyme arthritis because the patient often presents for an evaluation to rule out septic arthritis. This is of particular importance considering that Lyme arthritis responds to medical management with oral antibiotic administration. Rapid diagnosis of Lyme disease is often delayed, secondary to the delays in obtaining the results of serology testing. Unneeded I & D surgery may be performed in cases in which the patient’s presentation is consistent with septic arthritis or Lyme disease.
On clinical presentation of a patient with Lyme disease, the knee is involved in over 90 % of cases. Other joint involvement is less common. To our knowledge, there is limited description about Lyme arthritis of other joints (including the ankle) in the current literature. Furthermore, there are no reports in which the polyarticular nature of Lyme arthritis is used as a diagnostic criterion. The goal of this study is to review our experience with Lyme arthritis of the ankle in children, particularly in patients with poly-articular involvement, and to develop an algorithm for the treatment of children with possible Lyme arthritis.
Methods
This study was approved by the Penn State College of Medicine Institutional Review Board. This study, therefore, has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
CDC definition of Lyme Disease [12]
| Clinical case definition |
| Erythema migrans, or |
| At least one advanced manifestation, as defined below, and laboratory confirmation of infection |
| Advanced Manifestations (not including CV/CNS) |
| Musculoskeletal System |
| Recurrent, brief attacks (lasting weeks or months) of objective joint swelling in one or a few joints, sometimes followed by chronic arthritis in one or a few joints |
| Manifestations not considered criteria for diagnosis include chronic progressive arthritis, not preceded by brief attacks, and chronic symmetrical polyarthritis |
| Arthralgia, myalgia, or fibromyalgia syndromes alone are not criteria for musculoskeletal involvement |
| Laboratory criteria for diagnosis |
| Positive culture for B. burgdorferi from clinical specimen, or |
| Demonstration of diagnostic levels of IgM and IgG antibodies to the spirochetes in serum |
| Two-tier testing interpreted using established criteria, where: |
| Positive IgM is sufficient only when ≤30 days from system onset |
| Positive IgG is sufficient at any point during illness |
| Single tier IgG immunoblot seropositivity using established criteria, or |
| CSF antibody positive for B. burgdorferi by enzyme immunoassay (EIA) or Immunofluorescence Assay (IFA), when the titer is higher than it was in serum |
Representative joint involvement in 155 patients with Lyme disease. The knee was most frequently involved, with the ankle second most commonly involved
Results
One-hundred and fifty-five patients were found to have Lyme disease. The knee was most commonly affected, and the ankle was the second most commonly affected. The hip, the elbow, the wrist, and the shoulder were less frequently involved (Fig. 1).
Clinical presentation of 39 patients presenting with poly-articular lyme disease with ankle involvement
| Pt | Age | M/F | Joints involved | Pain with range of motion | Fever >38.5 °C | Refusal to bear weight |
|---|---|---|---|---|---|---|
| 1 | 17 | M | Ankles, knees, neck | No | No | No |
| 2 | 13 | F | L ankle | No | No | No |
| 3 | 8 | F | R ankle, R knee | Yes (R knee) | No | No |
| 4 | 7 | M | Ankles, hips | No | No | No |
| 5 | 8 | F | Ankles, L knee | No | No | No |
| 6 | 13 | M | Ankles, L knee, R wrist | No | No | No |
| 7 | 5 | M | L ankle, L knee, L hip, L knee, L shoulder | No | No | No (antalgic gait) |
| 8 | 7 | F | R ankle, knees, L hip | Yes (R ankle, knees) | No | No |
| 9 | 17 | F | Ankles, wrists | No | No | No |
| 10 | 14 | M | Ankles, knees | Yes (R ankle) | No | No (antalgic gait) |
| 11 | 12 | M | Ankles | No | No | No |
| 12 | 4 | M | L ankle, L knee | Yes (L knee) | No | No |
| 13 | 13 | M | R ankle, L knee, L wrist, neck | Yes (L knee) | No | No (antalgic gait) |
| 14 | 6 | F | Ankles, R hip | Yes (R hip, minimal) | No | Yes |
| 15 | 12 | M | Ankles, knees, back, neck, jaw | Yes (ankles) | No | No |
| 16 | 18 | F | Ankles, knees, elbows, wrists, MCPs | Yes (MCPs, R wrist, 4th and 5th toes) | No | No |
| 17 | 10 | F | Ankle, knees | Yes (L ankle, L knee) | No | No (antalgic gait) |
| 18 | 9 | M | Ankles, knees, wrists, R thumb | No | No | No |
| 19 | 8 | M | L ankle, L knee, L hip | Yes (L ankle, L knee, L hip, mild) | No | No |
| 20 | 9 | F | Ankles, knee, foot, elbows, neck | No | No | No |
| 21 | 12 | F | R ankle, R knee, R shoulder, R elbow, R wrist | Yes (R ankle, R knee, R wrist) | No | No |
| 22 | 5 | F | R ankle, R knee | No | No | No (antalgic gait) |
| 23 | 5 | M | R ankle, R knee, R hip | No | No | No (antalgic gait) |
| 24 | 10 | M | Ankle, shoulder, elbow, wrist, finger | Yes (R ankle) | No | No |
| 25 | 12 | M | L ankle, R knee, R elbow, neck | Yes (L ankle, R knee) | No | No |
| 26 | 6 | M | Ankle, R knee, R foot, elbow, wrist | Yes (R knee) | No | Yes |
| 27 | 7 | F | R ankle, knees, elbows, wrists | Yes (L shoulder, R wrist, R knee) | Yes | No |
| 28 | 2 | F | R ankle, knees, elbows | No | No | No |
| 29 | 11 | F | R ankle, R knee | Yes (R knee, mild) | No | No |
| 30 | 7 | F | L ankle, knees, L hip, L ankle | Yes (L knee) | Yes | No (antalgic gait) |
| 31 | 9 | M | Ankle, L elbow, wrists, neck | No | No | No |
| 32 | 12 | M | R ankle, R knee, L wrist | No | No | No |
| 33 | 6 | M | R ankle, R knee, hips | No | Yes | No (antalgic gait) |
| 34 | 12 | F | L ankle, knees, L midfoot | Yes (L ankle, R knee) | No | No (antalgic gait) |
| 35 | 7 | F | R ankle, R knee, R hip, R shoulder, R wrist, neck | No | No | No |
| 36 | 14 | M | L ankle, L knee, R elbow | Yes (R elbow, L knee) | No | No (antalgic gait) |
| 37 | 9 | F | Ankle, knee, shoulder, wrist | No | No | No |
| 38 | 9 | F | R ankle, R knee, R wrist, R thumb | No | No | No |
| 39 | 11 | F | Ankles, knees, elbows, L 5th finger | No | No | No |
Lyme laboratory results and treatment of 39 patients presenting with polyarticular lyme disease with ankle involvement
| Pt | Western blot (IgG) | ELISA | Antibiotic treatment |
|---|---|---|---|
| 1 | + | 3.25 | PO doxycycline |
| 2 | + | 1.65 | PO doxycycline |
| 3 | + | None | IV ceftriaxone, amoxicillin |
| 4 | + | None | Amoxicillin |
| 5 | + | None | Amoxicillin |
| 6 | + | None | Doxycycline |
| 7 | + | 5 | Amoxicillin |
| 8 | + | None | PO doxycycline |
| 9 | + | 2.25 | Cefuroxime |
| 10 | + | None | IV cefuroxime |
| 11 | + | None | PO doxycycline |
| 12 | + | None | Amoxicillin |
| 13 | + | None | PO doxycycline |
| 14 | + | None | Amoxicillin |
| 15 | + | 6 | IV ceftriaxone |
| 16 | + | None | IV ceftriaxone |
| 17 | + | 5.8 | Amoxicillin |
| 18 | + | None | PO doxycycline |
| 19 | + | None | IV ceftriaxone |
| 20 | + | 5 | Amoxicillin |
| 21 | + | 3.1 | PO doxycycline |
| 22 | + | None | Amoxicillin |
| 23 | + | None | Cefuroxamine |
| 24 | + | None | PO doxycycline |
| 25 | + | None | PO doxycycline |
| 26 | + | None | Amoxicillin |
| 27 | + | None | Amoxicillin |
| 28 | + | None | Cefuroxamine |
| 29 | + | 4.3 | PO doxycycline |
| 30 | + | None | Amoxicillin |
| 31 | + | None | PO doxycycline |
| 32 | + | None | PO doxycycline |
| 33 | + | None | Amoxicillin |
| 34 | + | 8.67 | PO doxycycline |
| 35 | + | 5.6 | Amoxicillin |
| 36 | + | None | Ceftriaxone |
| 37 | + | None | Cefuroxime |
| 38 | + | 8.2 | Amoxicillin |
| 39 | + | >5 | PO doxycycline |
Discussion
Lyme disease is a common cause of acute arthritis in children in endemic areas. Lyme arthritis responds readily to oral antibiotic management. The difficulty in the evaluation of Lyme arthritis is its clinical similarity to septic arthritis, especially when a patient presents with a single, swollen joint. Although serologic analysis is critical in identifying Lyme arthritis, obtaining these tests often results in a delay in diagnosis and sometimes unneeded surgical I & D. Therefore, the aim of this study was to develop a clinical algorithm to help differentiate Lyme arthritis from septic arthritis, with special attention given to ankle involvement and polyarticular involvement [2, 3].
Kocher identified four factors that could be used to distinguish septic arthritis from transient synovitis of the hip; these included fever >38.5, WBC >12, ESR >40, and an inability to bear weight on the affected leg. Kocher found that if 4/4 criteria were identified, the patient had close to a 99 % chance of having a septic process of the hip [4]. In contrast, if a patient had one out of four criteria, the likelihood of septic process of the hip decreased to 3 %.
Kocher criteria of 39 patients presenting with polyarticular lyme disease with ankle involvement
| Pt | Fever >38.5 °C | WBC (/mm3) | Refusal to bear weight | ESR (mm/h) | Kocher criteria |
|---|---|---|---|---|---|
| 1 | No | 10 | No | 8 | 0/4 |
| 2 | No | 12.8 | No | 25 | ¼ |
| 3 | No | 13.7 | No | 59 | 2/4 |
| 4 | No | n/a | No | 53 | 1/4 |
| 5 | No | 11.6 | No | 90 | 1/4 |
| 6 | No | WNL | No | WNL | 0/4 |
| 7 | No | n/a | No | 22 | 0/4 |
| 8 | No | 5 | No | 17 | 0/4 |
| 9 | No | 5.6 | No | 90 | 1/4 |
| 10 | No | 4.6 | No | 2 | 0/4 |
| 11 | No | n/a | No | n/a | 0/4 |
| 12 | No | 11 | No | 45 | 1/4 |
| 13 | No | WNL | No | WNL | 0/4 |
| 14 | No | 12.6 | Yes | 90 | 3/4 |
| 15 | No | 6.61 | No | n/a | 0/4 |
| 16 | No | WNL | No | n/a | 0/4 |
| 17 | No | WNL | No | 44 | 1/4 |
| 18 | No | 6.6 | No | 15 | 0/4 |
| 19 | No | 13 | No | 36 | 1/4 |
| 20 | No | 7.6 | No | 12 | 0/4 |
| 21 | No | WNL | No | 28 | 0/4 |
| 22 | No | 15.5 | No | 75 | 2/4 |
| 23 | No | n/a | No | n/a | 0/4 |
| 24 | No | n/a | No | n/a | 0/4 |
| 25 | No | 9 | No | 50 | 1/4 |
| 26 | No | 3.5 | Yes | 15 | 1/4 |
| 27 | Yes | 16.3 | No | 65 | 3/4 |
| 28 | No | 14.1 | No | 53 | 2/4 |
| 29 | No | 10.7 | No | 50 | 1/4 |
| 30 | Yes | 13.9 | No | 81 | 3/4 |
| 31 | No | n/a | No | 33 | 0/4 |
| 32 | No | n/a | No | n/a | 0/4 |
| 33 | Yes | 14.6 | No | 45 | 3/4 |
| 34 | No | 11.3 | No | 30 | 0/4 |
| 35 | No | 13.8 | No | 54 | 2/4 |
| 36 | No | 8.3 | No | 22 | 0/4 |
| 37 | No | 8 | No | 4 | 0/4 |
| 38 | No | 4.6 | No | 32 | 0/4 |
| 39 | No | 7.2 | No | n/a | 0/4 |
In the present study, we applied the Kocher criteria to patients with ankle and polyarticular involvement. We found that no patients had 4/4 Kocher criteria. Four patients had 3/4 Kocher criteria, and four patients had 2/4 Kocher criteria. Ten patients had 1/4 Kocher criteria, and 21 patients had 0/4 Kocher criteria. Although the Kocher criteria was developed for evaluating the pediatric hip, data from the present study suggest that these criteria are useful for differentiating Lyme from septic arthritis when the ankle and multiple joints are involved. In the current cohort of patients, the lack of Kocher criteria indicated the absence of a bacterial infectious process.
A recent multivariate analysis by Milewski in a Lyme-endemic area indicated that refusal to bear weight is the most predictive factor of septic arthritis [5]. Prior reports in the literature also suggested that refusal to bear weight is an important predictive factor of septic arthritis [6, 7]. This is consistent with the results of the present study. Furthermore, we concur with Culp that refusal to bear weight is rare with Lyme disease; in our study, only two patients (2/39, 5.1 %) with Lyme disease of the ankle refused to bear weight. However, these two patients did not have pain with passive range of motion, which indicated Lyme arthritis rather than septic arthritis. In the present study, the presence of pain with passive motion was seen in six patients and an antalgic gait was seen in 11 patients [8].
An algorithm to differentiate Lyme disease from septic arthritis may minimize the number of patients who undergo surgical debridement when the diagnosis is not clear. For example, in a 2011 study by Milewski, 40/123 (~24 %) cases of Lyme arthritis underwent operative debridement for presumed septic arthritis [5]. In our study, one patient (number 14) underwent surgical debridement, who was later found to have positive Lyme titers. This patient was subsequently managed with oral antibiotics. The low operative rate (2.5 %) in this study highlights the importance of the need to differentiate between Lyme and septic arthritis.
Williams reported that about 2/3 of patients with Lyme disease present with multiple joint involvement (an average of 2.4 joints affected) [9]. In addition, Williams reported that the ankle was the second most commonly affected joint, after the knee. We concur with Williams that the ankle was the second most commonly involved and report an even higher rate of polyarticular involvement of 94 %.
Comparative studies between Lyme disease and septic arthritis usually focus on a single joint involvement and often apply the Kocher criteria to monoarticular evaluation. In contrast, in the current study, we applied Kocher’s criteria to polyarticular Lyme disease, with emphasis on cases involving the ankle. The rate of isolated ankle involvement is uncommon. Our data indicate that only two (2/39) had isolated ankle involvement. Our data suggest that knee/ankle involvement was the most common combination of joints and was seen in 56 % of cases. The results of this study strongly suggest that this finding of polyarticular involvement indicates Lyme arthritis rather than septic arthritis. The sensitivity of polyarticular involvement related to Lyme disease was 97.4 %.
There are several salient points that our data affords for analysis. First, in the context of Kocher’s criteria, patients with Lyme disease rarely had an elevated temperature, an elevated ESR, an elevated WBC, or difficulty bearing weight. The lack of Kocher criteria was very suggestive of Lyme arthritis. Historical clues are unreliable, since only 17 % of our patients reported a tick bite and 31 % noted a rash. This lack of historical clues is consistent with previous reports in the literature [1].
We suggest the following algorithm to help differentiate between Lyme and septic arthritis. Patients with two or fewer Kocher criteria, polyarticular disease, an ability to bear weight, and minimal pain with passive range of motion are more likely to have Lyme disease and should be treated with appropriate antibiotics and careful follow-up while waiting for Lyme serology results. Patients with three or more Kocher criteria, monoarticular involvement, inability to bear weight, and pain with passive range of motion of the joint are more likely to have septic arthritis and should be treated with surgical I and D, cultures, and appropriate IV antibiotics, also while waiting for Lyme serology results.
Limitations include the retrospective design of the study from a single institution and the lack of a control group. This study is the first to evaluate the polyarticular nature of Lyme disease as a tool to help differentiate Lyme arthritis from septic arthritis. Although patients 14, 27, 30, and 33 demonstrated 3/4 Kocher criteria, the polyarticular nature of their presentation convinced the treating surgeon to manage the patients without surgery in three of four of these cases.
An additional limitation of this algorithm is its inability to differentiate Lyme arthritis from juvenile idiopathic arthritis, which can have a similar clinical presentation [10]. The mainstay of differentiating between Lyme and JIA is serologic testing. This is of particular importance in Lyme-endemic regions [10, 11].
Conclusion
- 1.
The ankle is the second most commonly involved joint in Lyme disease.
- 2.
In a Lyme-endemic area, polyarticular involvement indicates Lyme arthritis rather than septic arthritis.
- 3.
The Kocher criteria are helpful to differentiate Lyme arthritis from a septic joint.
- 4.
The development of a rapid Lyme test that can be performed in a few hours is needed.
Notes
Conflict of interest
The authors have no financial disclosures to report with regard to this study. The authors have full control of all primary data and agree to allow the journal to review the data if necessary.
Ethical standard
This study was approved by the Penn State College of Medicine Institutional Review Board (IRB). This study, therefore, has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Because of the retrospective nature of the study, the IRB at the investigating institution did not require a formal consent for inclusion of patients in this study. Patient data was garnered from an institutional database based on diagnostic codes for Lyme disease. All patient identifiers were removed prior to the preparation of this manuscript.
References
- 1.Smith BG, Cruz AI, Milewski MD, Shapiro ED (2011) Lyme disease and the orthopaedic implications of lyme arthritis. J Am Acad Orthop Surg 19:91–100PubMedCentralGoogle Scholar
- 2.Caird MS, Flynn JM, Leung YL, Millman JE, Dormans JG, D’Italia JP (2006) Factors distinguishing septic arthritis from transient synovitis of the hip in children: a prospective study. J Bone and Joint Surg (Am) 88:1251–1257CrossRefGoogle Scholar
- 3.Glotzbecker MP, Kocher MS, Sundel RP, Shore BJ, Spencer SA, Kasser JR (2011) Primary lyme arthritis of the pediatric hip. J Pediatric Orthop 31:787–790CrossRefGoogle Scholar
- 4.Kocher MS, Zurakowski D, Kasser JR (1999) Differentiating between septic arthritis and transient synovitis of the hip in children: an evidence-based clinical prediction algorithm. J Bone Joint Surg (Am) 81:1662–1670Google Scholar
- 5.Stans AA (2006) Osteomyelitis and septic arthritis. In Lovell and Winter’s Pediatric Orthopaedics, 6th edn. Lippincott-Wilkins, Philadelphia, pp 440–480Google Scholar
- 6.Milewski MD, Cruz AI, Miller CP, Peterson AT, Smith BG (2011) Lyme arthritis in children presenting with joint effusions. J Bone Joint Surg 93:252–260CrossRefGoogle Scholar
- 7.Willis AA, Widmann RF, Flynn JM et al (2003) Lyme arthritis presenting as acute septic arthritis in children. J Pediatric Orthop 23:114–118Google Scholar
- 8.Culp RW, Eichenfield AH, Davidson RS, Drummond DS, Christofersen MR, Goldsmith DP (1987) Lyme arthritis in children: an orthopaedic perspective. J Bone and Joint Surg (Am) 69:96–99Google Scholar
- 9.Williams CL, Strobino B, Lee A et al (1990) Lyme disease in childhood: clinical and epidemiologic features of ninety cases. J Pediatric Infectious Dis 9:10–14CrossRefGoogle Scholar
- 10.Boros C, Whitehead B (2010) Juvenile idiopathic arthritis. Aust Fam Physician 39((9):630–636Google Scholar
- 11.John J, Chandran L (2011) Arthritis in children & adolescents. Pediatrics in Rev 23(11):470–480CrossRefGoogle Scholar
- 12.Centers for Disease Control: Lyme Disease: (Borrelia burgdorferi.) Available at: http://wwwn.cdc.gov/nndss/script/casedef.aspx?CondYrID=752&DatePub=1/1/2011. Accessed March, 2014
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