Can Lyme disease trigger an autoimmune disease?

Adult-onset Still’s disease (AOSD) is often thought to be an autoimmune disease, but is, in fact, a systemic auto-inflammatory condition, believed to be caused by an over-reactive immune response to an infection, such as Lyme disease. As Cimmino points out, both diseases share several clinical characteristics.¹

Still’s disease can cause “a triad of high fever, salmon-colored nodular rash and arthritis and/or arthralgia,” explains Ocon in the British Medical Journal.² Lyme disease can also present with fevers, atypical rashes and arthritis and/or arthralgias.

Both conditions were first identified in children. Still’s disease was initially considered a severe version of juvenile idiopathic arthritis (JIA), while Lyme disease symptoms were originally attributed to juvenile rheumatoid arthritis (JRA).

Lyme disease and autoimmune diseases

A growing number of studies indicate that Lyme disease may trigger an autoimmune response in some individuals or symptoms may mimic an autoimmune disease.

Cross et al. describe the case of a Lyme disease patient with persistent neuropsychiatric symptoms who developed an autoimmune disorder, known as Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).³ The patient recovered following treatment with intravenous immunoglobulin (IVIG).

Investigators report the case of an elderly woman whose Lyme disease symptoms mimicked dermatomyositis (DM), a serious autoimmune disease which typically requires immunosuppressive therapy. The patient was eventually diagnosed with Lyme disease and recovered completely with antibiotic treatment.

The authors suggest, “[Lyme disease] could be a great mimicker of other autoimmune diseases like DM.”

Case Presentation: Still’s disease

A 61-year-old man presented with a “complaint of intermittent spiking fevers, night sweats, generalised malaise, as well as a history of erythematous circular rashes on his right upper extremity,” writes Ocon.²

He was diagnosed clinically with Lyme disease and received two 10-day courses of doxycycline, but continued to suffer from fevers, chest pressure, a dry cough, along with malaise and arthralgia.

The man was subsequently diagnosed with Still’s disease, as he met the criteria with a fever greater than 102.2°F for at least 1 week; a characteristic rash; a white cell count of at least 10,000; lymphadenopathy, and elevated liver transaminases.

He was treated successfully with intravenous steroids and anakinra (a humanised interleukin-1 receptor antagonist), which is used to modulate the immune system.

Author’s Takeaway:

“For the first time, we describe a case of AOSD precipitated by Lyme disease.”

“Lyme disease is a rare trigger of adult-onset Still’s disease, likely mediated via immune system inflammatory activation.”

“AOSD presented with a rare manifestation of haemorrhagic pericarditis and tamponade.”

“We believe that the immunological response to Lyme disease may have triggered AOSD via a hyper-activated immune system.”

UPDATED: July 1, 2021