A 57 year-old man living in the Northeastern U.S. with hepatitis C and rheumatoid arthritis, neither treated, underwent surgery for retinal detachment. 1 week post-operatively he developed severe frontal headache, arthalgias, myalgias, malaise, anorexia, fever/chills, and night sweats. Seen urgently, his PCP noted an acutely ill male with severe pain on palpation of the neck/spine, and blanching erythematous patches on the trunk, back, and thighs. He was admitted. Relevant investigations included: (1) white cell count 17,500/uL, creatinine 1.1 mg/dL, lactic acid 3.4 mmol/L; (2) Chest X-ray with bibasilar increased markings; (3) Head/facial CT with left ophthalmic abnormalities, likely post-procedural, including posterior chamber air, hyperdense vitreous fluid, and anterior scleral enhancement; (4) Right upper quadrant ultrasound with cirrhosis. Differential diagnosis included pneumonia, meningitis, diskitis/osteomyelitis, endocarditis, tickborne illnesses (Lyme, Babesiosis, Rickettsioses), viral infections (CMV, EBV, HIV), Still’s, lupus, and vasculitis. The appropriate infectious/rheumatologic serum studies were sent. Ceftriaxone, Azithromycin, plus Doxycycline were started. Approximately 18 hours after presentation, tachycardia, hypotension, white cell count 27,100/uL with 55% band forms, creatinine 2.1 mg/dL, and lactic acid 5.4 mmol/L raise concern for severe sepsis. Symptoms remained stable. Antibiotics were broadened to Ceftazidime, Ampicillin, Vancomycin, and Doxycycline. An expanded evaluation was performed including brain and cervical spine MRI, lumbar puncture, plus transthoracic echocardiogram. The following morning Lyme IgM antibodies returned strongly positive, allowing final diagnosis of early Lyme with Jarish-Herxheimer Reaction (JHR).
Discussion: JHR is a transient inflammatory reaction triggered by antibiotic therapy for spirochete infections. JHR is described in syphilis, Lyme, other tickborne illness, and leptospirosis. The exact pathogenesis of JHR remains unclear. There are reports describing JHR in other bacterial, fungal, and protozoal infections, suggesting JHR is not organism specific, but related to organism load and the individual host immune system. JHR presents abruptly within hours of 1st antibiotic dose with fever/chills, malaise, myalgias, flushing, tachypnea, hypotension, and worsening cutaneous lesions. The incidence of JHR in early Lyme is estimated to be 15%. JHR is usually self-limited to 24 hours so treatment is supportive; antibiotics should be continued. This case highlights the profound laboratory abnormalities noted in JHR. Our patient’s underlying autoimmune disease and cirrhosis-associated immune dysfunction may have predisposed him to an intense JHR.
Physicians must be acutely aware of JHR when treating early Lyme, consider host predisposition to JHR, then balance the risk/benefit of supportive care for JHR alone before instituting a broad diagnostic evaluation and treatment for alternate etiologies of sepsis.