A 58 Year Old Woman with Vision Loss, Headaches, and Oral Ulcers.pdf
n e w e ng l a n d j o u r na l
minished substantially and the RPR titer had
decreased to 1:8.
The patient’s boyfriend of 7 years underwent
syphilis testing; the test was positive, with an
RPR titer of 1:128. He and the patient both reported having no other sexual partners. The
source and timing of the patient’s infection
remained uncertain. Her low RPR titer at diagnosis was attributed to profound immunosuppression, including treatment with rituximab 3 to
4 months before the syphilis testing, although
there is limited information in the literature regarding the effect of the various immunosuppressants this patient had received on the ability
to mount an RPR titer.12
Dr. Rif kin: After this patient’s hospitalization
and treatment with antibiotic agents for ocular
syphilis, she returned to the uveitis clinic. The
rash and retinitis had resolved, but the scleritis
and iritis remained active, as did the mouth ulcers. She was treated with topical glucocorticoids,
and she again received infliximab, given concerns about underlying Behçet’s disease.
Six weeks later, retinal detachment developed
in the right eye, and the patient underwent surgery. Four months later, her retina detached
again, and she had a second surgery. Her infliximab dose was increased, and the scleritis became inactive.
One year after admission, the patient’s vision
1. Okada AA, Goto H, Ohno S, Mochi
zuki M. Multicenter study of infliximab
for refractory uveoretinitis in Behçet disease. Arch Ophthalmol 2012;130:592-8.
2. de Menthon M, Lavalley MP, Maldini
C, Guillevin L, Mahr A. HLA-B51/B5 and
the risk of Behçet’s disease: a systematic
review and meta-analysis of case-control
genetic association studies. Arthritis
3. Smith JR, Rosenbaum JT. Neurological concomitants of uveitis. Br J Ophthalmol 2004;88:1498-9.
4. Smith JR, Mackensen F, Rosenbaum
JT. Therapy insight: scleritis and its relationship to systemic autoimmune disease.
Nat Clin Pract Rheumatol 2007;3:219-26.
5. Patel DV, Horne A, House M, Reid IR,
McGhee CN. The incidence of acute anterior uveitis after intravenous zoledronate.
6. Conrady CD, Larochelle M, Pecen P,
Palestine A, Shakoor A, Singh A. Checkpoint inhibitor-induced uveitis: a case se-
m e dic i n e
is now reduced to counting fingers peripherally
in the right eye and is 20/20 in the left eye, with
normal intraocular pressures. Her scleritis is
inactive, and she has chronic mild iritis in the
right eye. Her retina is scarred but remains attached under silicone oil. She is receiving ongoing treatment with topical glucocorticoids, oral
prednisone, and an infliximab infusion. The
most recent CSF analysis was notable for a total
protein level of 83 mg per deciliter (as compared
with 160 mg per deciliter at diagnosis) and a
nucleated-cell count of 3 per cubic millimeter (as
compared with 64 per cubic millimeter at diagnosis), findings that suggest the patient had an
adequate response to antimicrobial therapy.
Fina l Di agnosis
This case was presented at the postgraduate course “Advances
in Rheumatology,” directed by Dr. John H. Stone.
Dr. Rosenbaum reports receiving consulting fees from AbbVie,
Gilead, Janssen, Roche, Novartis, Eyevensys, Regeneron, and
UCB and grant support, paid to his institution, from Pfizer; and
Dr. Rifkin, receiving fees for serving as an investigator from
AbbVie, Santen, EyeGate, Clearside Biomedical, and Aldeyra
Therapeutics. No other potential conflict of interest relevant to
this article was reported.
Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.
We thank Drs. Nicole Fett, Kevin White, Sirichai Pasadhika,
Akshay Thomas, and Sruthi Arepalli for valuable advice and input in the development of the differential diagnosis.
ries. Graefes Arch Clin Exp Ophthalmol
7. Touitou V, LeHoang P, Bodaghi B. Primary CNS lymphoma. Curr Opin Ophthalmol 2015;26:526-33.
8. Case Records of the Massachusetts
General Hospital (Case 33-2002). N Engl J
9. Furtado JM, Arantes TE, Nascimento
H, et al. Clinical manifestations and ophthalmic outcomes of ocular syphilis at a
time of re-emergence of the systemic infection. Sci Rep 2018;8:12071.
10. Hook EW. Syphilis. Lancet 2017;389:
11. Tran TH, Cassoux N, Bodaghi B,
Fardeau C, Caumes E, Lehoang P. Syph
ilitic uveitis in patients infected with
human immunodeficiency virus. Graefes
Arch Clin Exp Ophthalmol 2005;
12. Amaratunge BC, Camuglia JE, Hall
AJ. Syphilitic uveitis: a review of clinical
manifestations and treatment outcomes
of syphilitic uveitis in human immunodeficiency virus-positive and negative
patients. Clin Exp Ophthalmol 2010;38:
13. Davis JL. Ocular syphilis. Curr Opin
14. Gass JD, Braunstein RA, Chenoweth
RG. Acute syphilitic posterior placoid
chorioretinitis. Ophthalmology 1990;97:
15. Wickremasinghe S, Ling C, Stawell R,
Yeoh J, Hall A, Zamir E. Syphilitic punctate inner retinitis in immunocompetent
gay men. Ophthalmology 2009;116:1195200.
16. Westeneng AC, Rothova A, de Boer
JH, de Groot-Mijnes JD. Infectious uveitis
in immunocompromised patients and the
diagnostic value of polymerase chain reaction and Goldmann-Witmer coefficient
in aqueous analysis. Am J Ophthalmol
17. Jabs DA, Forman M, Enger C, Jackson
JB. Comparison of cytomegalovirus loads
n engl j med 380;11 nejm.org March 14, 2019
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