Paradoxical response in ocular bartonellosis
© The Author(s) 2011
Received: 30 September 2011
Accepted: 20 October 2011
Published: 5 November 2011
Cat scratch disease is an infectious disease caused by the bacteria Bartonella henselae  and is transmitted by cat scratches or exposure to cat saliva. The spectrum of its ocular manifestations includes Parinaud's oculoglandular syndrome, iritis, vitritis, neuroretinitis, panuveitis, retinitis appearing as small white intraretinal infiltrates, and choroidal white lesions [1, 2]. B. henselae is reported to be the most common cause of neuroretinitis; typically characterized by optic disk edema in association with a partial or complete macular star. The majority of infected patients presenting with neuroretinitis reported in the literature are treated with antibiotics. In some cases steroids were added. However several reports showed that even patients who did not receive treatment had an excellent visual outcome . We describe here a paradoxical response to treatment in a young female presenting with bilateral neuroretinitis and inflammatory optic disk lesions.
We herein describe the development of paradoxical response to treatment in a patient with serologically confirmed ocular bartonellosis. The patient developed central scotoma in one eye 4 days following the institution of anti-Bartonella and steroid therapy and following clinical improvement. We speculate that the initiation of the medical therapy led to a further compromise of the optic nerve function consequent to the overwhelming inflammatory reaction that sequentially settled following the use of high-dose steroid therapy.
Paradoxical response following initiation of therapy is also known as Jarisch–Herxheimer reaction (JHR) and both Jarisch and Herxheimer observed this reaction in patients suffering from syphilis who were being treated with mercury. It is now described in association with a variety of microbial infections, such as borreliosis, trichinellosis, Q fever, leptospiral infections, brucellosis, typhoid fever, and myalgic encephalomyelitis. It is classically described as being a systemic inflammatory response syndrome characterized by fever, chills, headache, myalgia, and exacerbation of skin lesions. No systemic involvement occurred in our patient; however, this reaction was limited to the deterioration in visual functions. Cheung and Chee reported on the occurrence of ocular disease (panuveitis and retinitis) with no systemic involvement following the institution of anti-tuberculous therapy for biopsy-proven tuberculous cervical lymphadenitis . Prompt improvement was achieved with the addition of oral steroids. Fathilah and Choo described worsening of ocular disease following the institution of therapy for ocular syphilis . To our knowledge, this is the first report of a paradoxical reaction occurring in a patient with ocular bartonellosis.
The pathophysiologic features of JHR are hypothesized to be a consequence of endotoxin reaction; however, recent studies showed that JHR is preceded by a cytokine surge with elevation of serum levels of tumor necrosis factor (TNFα), interleukin-6, and interleukin-8 . Fekade et al. were successful in showing that JHR can be suppressed by anti-TNFα antibodies . The favorable response to corticosteroids in patients with JHR  may result from transcriptional downregulation of TNFα, IFNɤ, and IL6 .
Our patient showed remarkable improvement in visual function following the institution of high-dose steroid therapy. We speculate that oral steroids that were initially instituted were insufficiently effective in obliterating the inflammatory cascade associated with the ocular infection, and only following the use of high-dose steroids was the paradoxical reaction reversed and persistent improvement consequently ensued.
Paradoxical response has been described in association with a variety of microbial infections, such as borreliosis, trichinellosis, Q fever, leptospiral infections, brucellosis, and typhoid fever. To our knowledge, this is the first report of a paradoxical reaction occurring in a patient with ocular bartonellosis. High-dose steroids were needed in order to suppress the overwhelming inflammation associated with the response to treatment.
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