HAND OFF SUMMARY

REASON AND OUTCOME OF HOSPITALIZATION:

A 22 year old male with no significant past medical history who presented on 11/3/17
due to fever, neck pain, and oral ulcers. Initial concern was for meningitis, patient
underwent LP that was unremarkable. He was started on vancomycin, ceftriaxone and
acyclovir, which was later de-escalated to doxycycline only. He was seen by infectious
disease who recommended immunological testing in additional to infectious disease
work up. Pertinent positive labs returned were elevated CRP, (+) ANA at 1:640, and
elevated ferritin. Infectious workup returned negative. Patient continued to spike fevers
throughout admission, however on day of discharge he was afebrile for >24h.
Rheumatology was consulted after ANA returned positive and started patient on
colchicine
 Continue colchicine 0.6mg BID
 PRN BMX oral solution for oral ulcers
 PRN acetaminophen for fevers

His blood work was unremarkable aside from thrombocytopenia which later resolved. He was seen by both ID
and rheumatology for infectious and immunological work up. Due to the positive ANA as below, patient was
thought to have a primary immunological disorder. He revealed during his hospital stay that his mother has a
history of C3 deficiency and is on plaquenil, and that he has Raynaud's syndrome in the cold weather. Suspicious
for Behcet's syndrome due to oral ulcers

Patient had an extensive infectious disease work up which included:

 LP (including fungal, HSV) - negative
 Blood cultures - negative
 Stool ova and parasites, salmonella - negative
 Atypical viral panel and flu - negative
 Culture of oral ulcer lesions and herpes simples DFA - negative
 Respiratory culture - negative
 Monospot test - negative
 Treponema pallidum Ab, VDLR - nonreactive, negative
 E. Chaffeensis IgG and IgM - negative
 Rocky Mountain Spotted fever IgG and IgM - negative
Pending infectious disease testing as above

Patient's immunological testing included:

 ANA - positive, 1:640 titre and nucleolar pattern
 IgG, IgM, IgA, C3, C4, anti-CCP - within normal limits
 HLA B27 - negative
 ACE - negative
 Pending immunological testing as above

Rheum suggested Check Anti DS DNA, anti ENA, SSA/B, HLA B27, C3, C4, humioral immunity profile
(given h/o low IgA)
Prednisone? If no infection
Behcets disease without joint pain
 Patient presented with a 1 month history of recurrent oral lesions. He had denied other lesions and
rashes. He had 2 large lesions on the inside vermillion border of lip on the right side. HSV testing
was negative. He had relief from BMX oral solution.

Pt is a 22 y.o.with no previous significant medical history who was hospitalized from 11/3 - 11/8/17 for
SIRS. He presented to the hospital with fever, neck pain, and oral ulcers. Initial concern was for
meningitis, but lumbar puncture was unremarkable. He was treated with antibiotics and infectious
disease consulted with negative workup. We consulted in the hospital. He was started on colchicine
twice per day. He did not receive any Prednisone while in the hospital.

3/15/2018
Here in the office today, the patient reports that he was at RGH 2-3 weeks ago with complaints of
fever, headache and neck pain. He was discharged after a few hours and put on a medrol Dosepak
which he finished. The patient notes that he has taken the colchicine throughout and today he feels
pretty well. He cannot pinpoint any one event which may have caused this most recent episode. He
denies having a recurrence of mouth sores, but the top of his mouth was "puffy and inflamed." As well,
he had a rash on his face. Other than some fatigue, however, he has had resolution of all these
symptoms. Patient did have a sinus infection recently for which he was put on Augmentin. Patient still
complains of bilateral lower back pain.

Since his last visit he has done generally well on colchicine 0.6 mg 2x daily.
He had one flare a few weeks ago with mouth ulcers, joint pain and skin rash
He was seen at the ED at RGH and a medrol Dosepak resolved his symptoms within a week.
He is feeling better now, but repeat blood work still shows a positive ANA and elevated LFT's.
I think Behcet's can be a working diagnosis.
He will continue colchicine 0.6 mg 2x daily and we gave him a medrol Dosepak to use as needed.
Will check blood work for autoimmune disease that affects liver and he will get monthly blood work
for this going forward.
We will see him in June to see how he makes out.
Patient also complains of lower back pain on a frequent basis particularly at night, we discussed going
on a non-steroidal and will start on diclofenac time release.

6/19/2018
Nicholas was seen accompanied by his mother today. Since his last visit he did decrease the
colchicine to 1 a day as it was bothering his stomach. He reports he is generally managing with his
current regimen. His knees and hands swell occasionally and he reports morning stiffness lasting
briefly about 10-15 minutes. He does have dry eye but is not had mouth ulcers or nasal
ulcerations. He did require 1 Medrol Dosepak since his last visit. He otherwise feels that he is
managing with his current regimen and is pleased.

12/10/2018
HPI: NIcholas is in today with his Mom. Since Thanksgiving he has felt poorly. He was tired and had headaches,
but no mouth sores. The symptoms worsened and developed fevers of 101.5, sore throat, joint aches and pains.
He started a medrol dose pack on 12/5 and felt better, but by the time he completed, the symptoms returned.
He called Friday looking to be seen, but no appointments available this day, so medrol dose pack was called into
pharmacy. By the following day, he felt much better, and he feels good today. He is on the 12 mg dose today. He
notes 3 similar episodes since September. He feels stress, or eating increased carbs triggers episodes
Started on Plaquenil
5/15/2019
Pt was last seen on 1-7-19. At tha time, he still noted morning stiffness with some schiness at the
hands, knees, and hips.
The pt has been feeling better on Plaquenil. He stopped it for about a month with recurrence of several
mouth sores. These resolved when he restarted Plaquenil. The pt has not used meloxicam and denies
sore joints currently
Behçet syndrome is a rare disease characterized by recurrent oral aphthae and any of several systemic
manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal disease,
neurologic disease, vascular disease, and arthritis. Most clinical manifestations of Behçet syndrome are
believed to be due to vasculitis.

●Behçet syndrome is more common (and often more severe) along the ancient silk road, which extends
from eastern Asia to the Mediterranean; it is most common in Turkey, while the prevalence is much
lower in North America and northern Europe. It typically affects young adults 20 to 40 years of age.
(See 'Epidemiology' above.)

●The common clinical feature in patients with Behçet syndrome is the presence of recurrent and
usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable
among different patients and populations.

●The greatest morbidity and mortality occur with ocular disease (affecting up to two-thirds of patients),
including uveitis and other changes; with vascular disease, including pulmonary artery aneurysms; and
with central nervous system disease, including focal parenchymal lesions, complications of vascular
thrombosis, and other abnormalities. Vasculitis in patients with Behçet syndrome is remarkable for its
ability to involve blood vessels of all sizes and to involve both arteries and veins (table 1).

●Cutaneous manifestations are common, which included acne, folliculitis, and erythema nodosum
lesions. Pathergy may be seen as an erythematous papular or pustular response to local skin injury.
Arthritis may be present which is characteristically intermittent, inflammatory but nonerosive,
asymmetric, and, usually, nondeforming; it often occurs during disease exacerbations and most
commonly affects the medium and large joints. A variety of gastrointestinal symptoms may also occur.
(See 'Cutaneous lesions' above and 'Arthritis' above and 'Gastrointestinal involvement' above.)

There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on
the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet
syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the
following clinical features:

•Recurrent genital aphthae

•Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal
vasculitis observed by an ophthalmologist)

•Skin lesions (including erythema nodosum, pseudo-vasculitis, papulopustular lesions, or acneiform

nodules consistent with Behçet syndrome)

•A positive pathergy test.

MKSAP:

Painful oral or genital ulcers

Inflammatory Eye disease our patient had photosensitivity
Pathergy popular or pustular response to local skin injury 48hrs after skin prick with sterile needle
Venous thrombosis of vena cava or dural sinusesc
Brain stem lesions, Aseptic meningitis; headache and diplopia
Inflammatory Arthritis
HLA B51***
Turkey, more in Men
Topical Steroids

Topical steroids for ulcers

Cholchicine to prevent ulcers
Plaquenil if chronic
Steroids if acute flare on cholchicine