Doctors in Spain Warn Against False Lyme Diagnoses in Children

In 2019, three medical societies in Spain issued a Position Statement regarding their concern about false positive Lyme disease diagnoses in children:

  • Asociación Española de Pediatría (AEP, Spanish Association of Pediatrics)
  • Sociedad Española de Reumatología Pediátrica (SERPE, Spanish Society of Pediatric Rheumatology)
  • Sociedad Española de Infectología Pediátrica (SEIP, Spanish Society of Pediatric Infectious Diseases)

Sadly, false information on the internet is encouraging diagnoses and treatment of children that can cause more harm then good. Additionally, Medical Child Abuse is a significant problem in the dangerous alternate universe of chronic Lyme quackery.

El documento original en español

A machine-assisted, unofficial translation of the Position Statement is below:

Position statement of the AEP, SERPE, and SEIP on the evidence-based diagnosis of Lyme disease

Sara Guillén Martína,, Esmeralda Núñez Cuadrosb, Alfredo Tagarro Garcíac, Cristina Calvo Reyd, representing the Spanish Society of Pediatric Rheumatology (SERPE) and the Spanish Society of Pediatric Infectious Diseases (SEIP)

  1. Hospital Universitario de Getafe, Getafe, Madrid, Spain
  2. Hospital Regional Universitario de Málaga, Málaga, Spain
  3. Hospital Infanta Sofía, Madrid, Spain
  4. Hospital Universitario La Paz, Madrid, Spain

The Spanish Society of Pediatric Rheumatology (SERPE) and the Spanish Society of Pediatric Infectious Diseases (SEIP) wish to communicate our concern about the erroneous diagnosis of Lyme disease that is being carried out in children with juvenile idiopathic arthritis.

In recent years, scientifically non-validated diagnostic tests have been marketed, which are causing children with arthritis of non-infectious etiology to receive antibiotics for a prolonged period of time. This trend may cause the appearance of sequelae at the articular level, and even more seriously, at the ocular level due to the development of chronic uveitis, which will limit the future life of these children.

Lyme disease is a multisystemic process caused by Borrelia burgdorferi sensu lato and transmitted by ticks. Within Borrelia burgdorferi sensu lato, 20 genospecies have been described, the most frequent in Spain being the B. garinii transmitted by the tick Ixodes ricinus1.

Most cases of this disease are diagnosed in the northern half of the Iberian Peninsula. An incidence of 3-5 cases/100,000 population per year is estimated in the areas with the highest incidence, such as La Rioja, most of them in their early localized form.

The clinical aspects are divided into 3 stages (table 1). Joint involvement occurs in Europe in 3-25% of patients. It is mono-articular or oligo-articular with little inflammatory component, unlike cases in the USA, and typically affects knees for a period of months or years, without systemic symptoms2-4. In published series in northern Spain, the prevalence of joint manifestations in children under 15 years of age was exceptional, corresponding to 5.9% of the total number of patients with arthritis.

Table 1.

Clinical classification of Lyme disease

Early Localized phase – Stage IMigratory erythema or benign lymphadenosis cutis with or without lymphadenopathy 
Early disseminated phase – Stage IIMultiple migratory erythema and/or neurological, cardiac or acute joint manifestations 
Chronic* phase – Stage IIIAcrodermatitis chronica atrophicans, tertiary neuroborreliosis, or persistent or recurrent arthritis (at least 6 months)

Diagnosis is made by serology, which can be negative in the early phase. Culture is very laborious, has little sensitivity, is easily contaminated and is only available in reference centers. PCR in liquids or tissues can be used as a complementary diagnostic tool, although in synovial tissue samples in patients with arthritis, it can have a sensitivity of up to 80%2-4.

Private laboratories offer a multitude of non-validated diagnostic tests (Table 2), without quality criteria, that attempt to demonstrate infection with B. burgdorferi1.

In order to confront this trend, scientific societies have developed guidelines with strict diagnostic criteria, most of them recognizing as valid diagnostic tests the performance of 2 consecutive serologies:

  • first using enzyme-linked immunosorbent assays (ELISA) or immunofluorescence (IFA), which are not very specific and present a risk of cross-reactivity, but their sensitivity is very high, and
  • later confirmation using Immunoblot or Western blot2-4.

Table 2.

Non-validated diagnostic tests for the diagnosis of Lyme disease

Centers for Disease Control and Prevention (CDC) listing of diagnostic tests not recommended for the diagnosis of Lyme disease:

  • Capture assays for antigens in urine
  • Culture, immunofluorescence staining, or cell sorting of cell wall-deficient or cystic forms of B. burgdorferi
  • Lymphocyte transformation tests (ELISPOT)
  • Quantitative CD57 lymphocyte assays
  • “Reverse Western blots”
  • In-house criteria for interpretation of immunoblots
  • Measurements of antibodies in joint fluid (synovial fluid)
  • IgM or IgG tests without a previous ELISA/EIA/IFA

In conclusion, due to the low incidence of joint involvement due to Lyme disease in Spain, its diagnosis will be made when the following criteria are met:

  • epidemiological criteria (having been in a Borrelia transmission zone and checking the tick bite; this is not obligatory),
  • compatible clinical criteria, and
  • microbiological diagnosis in 2 steps with different and validated serological techniques.

For all these reasons, the scientific societies involved recommend the diagnosis and follow-up of patients with suspected Lyme disease in centers specializing in pediatric infectious disease.

In addition, in cases with joint involvement, differential diagnosis with arthritis of other etiologies more frequent in our environment should be carried out, estimating the appropriate treatment by the specialist in paediatric rheumatology, if the infectious cause is ruled out.

* LymeScience Note

Medical experts in North America have abandoned the term “chronic Lyme” because of confusion. However, some European and older texts use the term “chronic” to refer to “Late” Lyme disease, which can occur after months or years of untreated infection, if the body fails to clear it.

“Chronic Lyme arthritis” also may refer to arthritic symptoms that occur before or after treatment. As used herein, the medical societies are referring to Late Lyme disease and not pseudoscientific diagnoses.

For more on the stages of Lyme infection, see the 2016 review by Steere and colleagues.

Bibliography

[1] A. Portillo, S. Santibáñez, J.A. Oteo. Enfermedad de Lyme. Enferm infecc Microbiol Clin, 32 (2014), pp. S37-S42

[2] P. Brouqui, F. Bacellar, G. Baranton, R.J. Birtles, A. Bjoërsdorff, J.R. Blanco, ESCMID Study Group on Coxiella, Anaplasma Rickettsia and Bartonella; European Network for Surveillance of Tick-Borne Diseases, et al. Guidelines for the diagnosis of tick-borne disease in Europe. Clin Microbiol Infect, 10 (2004), pp. 1108-1132.

[3] G.P. Wormser, R.J. Dattwyler, E.D. Shapiro, J.J. Halperin, A.C. Steere, M.S. Klempner, et al. The clinical assessment, treatment, and prevention of Lyme disease, human granulocytic anaplasmosis, and babesiosis: Clinical practice guidelines by the Infectious Diseases Society of America. Clin Infect Dis, 43 (2006), pp. 1089-1134.

[4] M. Cruickshank, N. O’Flynn, S.N. Faust, Guideline Committee. Lyme disease: Summary of NICE guidance. BMJ, 361 (2018), pp. k1261