grand rounds shivani v. reddy, m.d. 3/6/2014 university of louisville department of ophthalmology...
TRANSCRIPT
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Grand Rounds
Shivani V. Reddy, M.D.3/6/2014
University of LouisvilleDepartment of Ophthalmology and
Visual Sciences
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Patient Presentation CC: blurry vision OU
HPI: 12 year old white male presents with 1 week history of blurry vision OU. He denies any pain, photophobia, flashes or scotomas.
He had a similar episode 4 months prior with associated pain and photophobia. Patient denies any associated malaise, nausea or flank pain during these episodes.
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HistoryPOHx and PMHx
1 year ago: patient presented to KCH with fever, malaise, flank pain
and 2 weeks of frothy urine
renal biopsy: tubular interstitial nephritis
treatment: prednisone 40 mg daily
4 months ago: patient developed blurry vision OU with pain and photophobia – diagnosed with acute anterior uveitis
treatment: topical Pred Forte, Cyclogyl
12 week course of methotrexate
FAM Hx none
ROS cushingoid features
MEDS prednisone 20 mg QOD, Cellcept 500mg PO BID
ALLERGIES NKDA
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VA cc P TTP
EOM: Full OU CVF: Full OU
20/20-3 ( -0.75 + 1.00 x 015) 4 3 mm
4 3 mm
16
16
no RAPD20/20 (-1.50 + 0.25 x
165)
Exam
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Ext WNL WNL
L/L WNL WNL
C/S 1+ injection 1+ injection
K few fine KP WNL
AC 2+Cell, 1+ Flare 1+ Cell, 1+ Flare
I/L posterior synechiae posterior synechiae
NO VITREOUS CELLS OU
OD OS
Anterior Segment
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Fundus Exam OD
2 + disc edema, normal macula, normal vesselsmultiple choroidal inflammatory foci on peripheral exam
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Fundus Exam
1 + disc edema with mild peripapillary NFL edema, normal macula, normal vessels. Multiple choroidal inflammatory foci (arrows)
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OCT
mild ILM gliosis, normal foveal contour
OS
OD
normal foveal contour
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FA/ICG
29 seconds: few areas of choroidal leakage on ICG
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FA/ICG
54 seconds: foci of choroidal hypofluorescence on ICG
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Summary
DDx:• TINU syndrome (tubular interstitial nephritis
and uveitis)• Infectious mononucleosis• Sarcoidosis • Other Infections (syphilis, herpesviridae) • Systemic lupus erythematosus
12 y/o WM with h/o tubular interstitial nephritis diagnosed 1 year prior and an episode of bilateral anterior uveitis 4 months prior presents with blurry vision OU while taking oral prednisone and Cellcept. Exam reveals anterior uveitis , optic disc edema and multiple foci of outer retinal and choroidal inflammation OD > OS.
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Treatment Given patient’s cushingoid features and long term prednisone use, prednisone dose was decreased to 10 mg every other day and Cellcept was increased to 1.5 gm daily
Follow-up pending
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TINU Syndrome A predominantly bilateral non-
granulomatous anterior uveitis found in a subset of patients with tubular interstitial nephritis (TIN)
First described in 1975 by Dobrin et.al.
relatively rare with reported incidence 1-2%
Uveitis occurs in approximately 65% of TIN patients
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TINU Syndrome Epidemiology
- young females (teens – 30)- younger age groups with more male
patients
No racial affinity
Some HLA associations with HLA-DQA1*01, HLA-DQB1*01 HLA-DQB1*05
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TINU Syndrome Pathogenesis not well understood
Autoantigen to both uveal and renal tubular cells (mCRP)
Inflammation is T- lymphocyte driven
Risk factors found in only 50% - antibiotics for URIs, NSAIDS, autoimmune diseases, Chlamydia and EBV concurrent infection
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Ocular Symptoms Patients present with anterior uveitis
symptoms (pain, redness, photophobia, decreased vision)
Other findings: optic nerve edema, retinal infiltrates, vitreous opacities
ocular symptoms follow TIN in 65%, are concurrent in 15%, precede by up to 2 months in 20%
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TIN Findings Increased serum creatinine
Abnormal urinalysis ( no definitive markers)- increased B2- microglobulin- urinary eosinophilia, pyuria,
hematuria- glucosuria
Renal biopsy for definitive diagnosis- Interstitial edema, mononuclear
infiltrate, eosinophils and noncaseating granulomas
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Interstitial edema, mononuclear infiltrate, eosinophils and noncaseating granulomas
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Treatment renal disease very responsive to
corticosteroids - typically treated for 3 to 6 months with slow taper
Uveitis treated with corticosteroids (topical and systemic)
Even though TIN typically is self limited, uveitis can be recurrent requiring immunomodulator therapy (IMT)• mycophenolate mofetil , methotrexate,
cyclosporine
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Prognosis
Long term complication are rare
Uveitis often persists longer than TIN but treatment rarely lasts over 1 year
Uveitis recurs in up to 40% of patients within a few months of therapy cessation but nephritis rarely recurs
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retrospective case series of 4 patients with TINU
Gender: 3 female, 1 male Age range: 10 -31 years
All patients presented with acute anterior uveitis. 3/4 patients with vitritis
2 patients presented with TIN before uveitic symptoms: Pt 1 – 4 months prior, Pt 2 - 3 years prior.
2 patients presented with TIN concurrent with uveitic symptoms
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10 year old caucasian female presenting with blurred vision,
floaters. Elevated ESR, creatinine. Anterior uveitis, vitritis and lesions
seen below
31 year old caucasion female with pain, redness, photophobia and floaters. Elevated creatinine.
Exam with anterior uveitis, vitritis chorioretinal lesions
11 year old half- asian male with concurrent TIN and pain, redness and
photophobia. Elevated creatinine. Exam with anterior uveitis, vitritis and punctate
chorioretinal lesion
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References 1. BSCS Section 9: Intraocular Inflammation and Uveitis2. BSCS Section 12: Retina and Vitreous3. Mandeville et al. The tubulointerstitial nephritis and uveitis
syndrome.Surv Ophth. 2001,46(3):195-208. 4. Suzuki H, Yoshioka K, Miyano M, et al. Tubulointerstitial
nephritis and uveitis (TINU) syndrome caused by the Chinese herb "Goreisan". Clin Exp Nephrol 2009; 13:73.
5. Mackensen et al Br J Ophth. 2011,95:971-976. 6. Birnbaum, et. al. Arch Ophthalmol. 2012;130(11):1389-
1394.7. Mandeville JT, Levinson RD, Holland GN. The
tubulointerstitial nephritis and uveitis syndrome. Surv Ophthalmol 2001; 46:195.
8. Reddy et al. HLA-DR, DQ class II DNA typing in pediatric panuveitis and tubulointerstitial nephritis and uveitis. Am J Ophthalmol. 2014 Mar;157(3):678-86
9. Ali A, Rosenbaum JT. TINU (tubulointerstitial nephritis uveitis) can be associated with chorioretinal scars.Ocul Immunol Inflamm. 2014 Jun;22(3):213-7.