Joint manifestations of Lyme borreliosis JOINT MANIFESTATIONS OF L YME BORRELIOSIS P. Herzer ABSTRACT Musculoskeletal involvement is a common feature of Lyme Borreliosis. Migratory pain in muscle, joints, or entheses occur early in the illness. Weeks to months later, untreated patients may begin to have intermittent or chronic mono- or oligoarticular arthritis, primarily affecting the knee. Only a small percentage of these patients develop chronic joint involvement. In patients with acrodermatitis chronica atrophicans, distinctive signs of joint and bone involvement can be found beneath the skin lesions. KEY WORDS Lyme BoTTeliosis, arthritis, joint and bone involvement INTRODUCTION Lyme arthritis (LA) was first described in North America by Steere et al. in 1976 as a previously unrecognized rheumatic disorder (1,2). Soon it tumed out that LA was only one expression of a multisystem inflammatory disease (3), the dermatological and neurological manifestations of which had been described as separate entities in Europe in the early 1900s (4-6). Thus, it was believed for a long tirne that arthritis was a special American feature of Lyme Borreliosis (LB). In the meantime, reports on LA frorn nearly all European countries (reviewed in ref. 7) have confirmed previous lines of reasoning that the presumed continental difference regarding arthritis merely reflected the lack of awareness of LA in Europe (8). acta dennatovenerologica A.P A. Vol 5, 96, No 3-4 INCIDENCE OF JOINT MANIFESTATIONS In a study of 55 patients in North America who had not received antibiotics for relief of erytherna migrans (EM), ten patients (18%) experienced arthralgias and 28 patients (62%) developed true arthritis with its onset as long as two years after suffering from EM (9). However, only one out of 16 patients in Sweden developed arthritis after spontaneous healing of EM (10); the follow-up period for this study was not reported. Moreover, by the tirne that EM and neurological manifestations of LB come to be treated more readily with antibi6tics, such cases of „classic LA" with preceding extra- articular manifestations will be increasingly rare. 143 Joint manifestations of Lyme borreliosis Nevertheless, LA may occur as the sole manifestation of LB. Serological stuclies in our patients with otherwise unclassified monoarticular or oligoarticular arthritis suggest that LA without extra-articular manifestations of LB preponderates over „classic LA" (11-13). the early disseminated infection (Stage 2) of LB (9). Arthralgias usually affect multiple joints in a migratory pattern. Particular episodes last from hours to days and are separated by days to months of remission (2,3,7,9,11-13). The duration of arthralgias in patients who were not treated for EM ranged from 1 month to 6 years (mean 3.1 years) (9) . Attacks of arthralgias were also reported to occur as long as 10 years after the spontaneous resolution of LA (12,14). CLINICAL FEATURES A variety of musculoskeletal manifestations may occur in LB, inclucling arthralgias, myalgias, true arthritis, tenclinitis and bursitis. ARTHRITIS ARTHRALGIAS Attacks of pain in joints, entheses, tendons, muscles, bones or the spine are characteristic of Frank arthritis may occur within a few days to two years after the onset of the illness, on the average of 3 to 6 months (9,13). LA often begins at Table. Musculoskeletal features of Lyme Borreliosis: mimicry of rheumatic entities. Arthralgias/Myalgias Acute Monarticular Arthritis Migratory Arthritis Intermittent Arthritis Pauciarticular Arthritis ACA *-associated joint deformities *A CA acrodermatitis chronica atrophicans 144 Fibromyalgia Polymyalgia rheurnatica Osteoarthritis Gout, Pseudogout Septic Arthritis Sarcoid Arthritis entities, Viral Arthritis ( e.g. Rubella, Parvovirus B19) Gonococcal Arthritis Rheurnatic Fever Whipple's Disease Intermittent Hydarthrosis Palindromic Rheurnatism Seronegative Spondyloarthropathies, e.g. Reactive Arthritis Psoriatic Arthritis Enteropathic Arthritis Juvenile Oligoarthritis (HLA-B27 positive) Juvenile Oligoarthritis (ANA positive) Early Rheumatoid Arthritis Systemic Lupus Erythematosus Osteoarthritis Hallux valgus, Hammertoes Rheumatoid arthritis acta dermatovenerologica A .P A. Vol 5, 96, No 3-4 Joint manifestations of Lyme bo1Teliosis the same extremity that was affected by the tick bite or EM (12). Typically, there is an intermittent mono- or oligo- articular arthritis in large joints which can be migratory from tirne to tirne. The knee is the most common joint affected (2,3,7,9,13,14). In many instances affected knees are extremely swollen, moderately warm, but often not particularly painful. Baker's cysts develop in many cases and may rupture early. Attacks of arthritis affecting other joints, however, may be very painful and may resemble gout or septic arthritis (11,15). Evanescent diffuse swelling in the hand and sausage digits have been noted in a few patients. Moreover, heel swelling, suggesting enthesopathy, may occur (11). Some European authors claim that sacroiliitis, Reiter's syndrome and ankylosing spondylitis may also be caused by infection with Borrelia burgdorferi (Bb) (16-19), which however is still questionable (9,13,20,21). Episodes of LA last from a few days to a few months. Patients can have an isolated attack or multiple recurrences over many years. In the majority of patients LA has a self-limited course (9). However, LA becomes chronic in about 10% of patients. In cases of chronic LA, radiographic findings of erosive arthritis may be detected (12,13,22). Studies from North America suggest that there is a genetic susceptibility to developing chronic LA; e.g., HLA DR 2 and HLA DR 4 have been faund to be significantly increased in patients with chronic joint involvement (23). However, a study of German patients with LA did not provide evidence of an HLA-related risk of developing LA or chronic joint manifestations (12,24). JOINT AND BONE ABNORMALITIES IN ASSOCIATION WITH ACRODERMATITIS CHRONICA ATROPHICANS Distinctive signs of joint and bone involvement beneath the skin lesions may be faund in long- standing acrodermatitis chronica atrophicans (ACA). In one study, 11 out of 50 patients with ACA had luxations or subluxations of finger or toe joints (25). Since radiographs did not show erosive lesions, these joint defarmities were referred to as an ACA- associated Jaccoud-like arthropathy (12). Moreover, periosteal thickening of bones, suggesting perios~itis, was faund radiographically under cutaneous les1ons of ACA in a few cases (25). DIAGNOSTIC CRITERIA None of the rheumatic features of LB, per se, is absolutely pathognomonic; LA can mimic a number of other diseases ( see Table) and vice versa. The coincidence with typical extra-articular manifestations of LB, in particular with EM, is the most reliable diagnostic criterion. However, LA may be the sole manifestation of LB. In LA the possibility to cultivate or demonstrate Bb is very slim. Polymerase chain reaction (PCR) far diagnostic testing may be useful (26), however, this new technique is still not recommended far routine purposes. Thus, laboratory confirmation is generally limited to serological testing. However, serology involves pitfalls due to matters of test standardization and the occurrence of specific IgG- antibodies from remote asymptomatic infections. Thus, it is most important to emphasize that serological findings must be interpreted within the context of the clinical picture and the respective differential diagnoses. The undue readiness to believe in the diagnostic significance of a positive Lyme test would lead to the widespread overdiagnosis of LA REFERENCES l. Steere AC, Malawista SE, Snydman DR, Andiman WA. A cluster of arthritis in children and adults in Lyme, Connecticut. Arthritis Rheum 1976; 19: 824. 2. Steere AC, Malawista SE, Snydman DR et al. Lyme arthritis. An epidemic of oligoarticular arthritis in children and adults in three Connecticut commu- nities. Arthritis Rheum 1977; 20: 7-17. 3. Steere AC, Malawista SE, Hardin JA et al. Erythema chronicum migrans and Lyme arthritis. acta de1Tnatovenerologica A.P.A. Vol 5, 96, No 3-4 The enlarging clinical spectrum. Ann Intern Med 1977; 86: 685-98. 4. Afzelius A. Verhandlungen der dermatologischen Gesellschaft zu Stockholm. Sitzung vom 28. Oktober 1909. Arch Derm Syph (Berlin) 1910; 101: 404. 5. Garin C, Bujadoux HC. Paralysie par les tiques. J Med Lyon 1922; 71: 765-67. 6. Bannwarth A. Chronische lymphozytare Meningitis, 145 Joint manifestations of Lyme borreliosis entziindliche Polyneuritis und „Rheumatismus". Ein Beitrag zum Problem „Allergie u. Neivensystem" in zwei Teilen. Arch Psychiatr Neivenkr 1941; 113: 284-376. 7. Herzer P. Joint manifestations. In: Weber K, Burgdorfer W (eds.). Aspects of Lyme Borreliosis. Berlin Heidelberg New York: Springer 1993; 168-84. 8. Herzer P. Lyme arthritis in Europe: comparisons with reports from North America. Ann Rheum Dis 1988; 47: 789-90. 9. Steere AC, Schoen RT, Taylor E. The clinical evolution of Lyme arthritis. Ann Intern Med 1987; 107: 725-31. 10. Asbrink E, Olsson I. Clinical manifestations of .. , erythema chronicum migrans Afzelius in 161 patients. Acta Derm Venereol (Stockh) 1985; 65: 43-52. 11. Herzer P, Wilske B. Lyme arthritis in Germany. Zbl Bakt Hyg A 1986; 263: 268-74. 12. Herzer P. Lyme Borreliose. Epidemiologie, Atiologie, Diagnostik, Klinik und Therapie. Darmstadt: Steinkopff 1989. 13. Herzer P. Joint manifestations of Lyme Borreliosis in Europe. Scand J Infect Dis 1991; Suppl. 77: 55- 63. 14. Szer IS, Taylor E, Steere AC. The long-term course of Lyme arthritis in children. N Engl J Med 1991; 325: 159-63. 15. Jacobs JC, Stevens M, Duray PH. Lyme disease simulating septic arthritis. JAMA 1986; 256: 1138- 39. 16. Valesova M, Trnasvsky K. Joint manifestations of Lyme Borreliosis in Czechoslovakian patients. Z Rheumatol 1990; 49: 192-96. 17. Weyand CM, Goronzy JJ. Immune response to Borrelia burgdorferi in patients with reactive arthritis. Arthritis Rheum 1989; 32: 1057-64. 18. Kinigadner U, Mur E, Most J et al. Borrelia infection as a possible cause of HLA-B27 negative sacroiliitis. J Rheumatol 1991; 18: 484-85. 19. Prohaska E, Kristoferitsch W, Stanek G. Spinal involvement in Lyme Borreliosis. Zbl Bakt Hyg A 1989; Suppl 18: 261-62. 20. Blaauw I, Nohlmanns L, Peeters A et al. 1s there any evidence far an association between ankylosing spondylitis and Borrelia burgdorferi infection? J Rheumatol 1992; 19: 579-81. 21. Cimmino MA, Accordo S, Sambri V. Western blotting far the diagnosis of Borrelia burgdorferi infection in patients with sacroiliitis. J Rheumatol 1992; 19: 833-34. 22. Lawson JP, Steere AC. Lyme arthritis: radiologic findings. Radiology 1985; 154: 37-43. 23. Steere AC, Dwyer E, Winchester R. Association of chronic Lyme arthritis with HLA-DR 4 and HLA-DR 2 alleles. N Engl J Med 1990; 323: 219- 23. 24. Herzer P, Schewe S, Scholz S, Albert E. HLA- antigens in late Lyme Borreliosis (Lyme arthritis and acrodermatitis chronica atrophicans ). IVth International Conference on Lyme Borreliosis (Stockholm) 1990, A 98 (abstr.). 25. Hovmark A, Asbrink E, Olsson I. Joint and bone involvement in Swedish patients with Ixodes ricinus-borne borrelia infection. Zbl Bakt Hyg A 1986; 263: 275-84. 26. Nocton JJ, Dressler F, Rutledge BJ et al. Detection of Borrelia burgdorferi DNA with polymerase chain reaction in synovial fluid from patients with Lyme arthritis. N Engl J Med 1994; 330: 229-34. AUTHOR'S ADDRESS Peter Herzer, MD, professor, Tal 6 (am Marienplatz), D-80331 Munich, Germany 146 acta dennatovenerologica A.P A. Vol 5, 96, No 3-4