case presentation 2010 fois meeting - uveitis · manisha mehta, md luis alonso gonzalez-gonzalez,...

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CASE PRESENTATION Sana S. Siddique¹´², C. Stephen Foster ¹´² 1 Massachusetts Eye Research and Surgery Institution (MERSI), Cambridge, MA 2 Ocular Immunology and Uveitis Foundation

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Page 1: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

CASE PRESENTATION

Sana S. Siddique¹´², C. Stephen Foster ¹´²1Massachusetts Eye Research and Surgery Institution (MERSI), Cambridge, MA2 Ocular Immunology and Uveitis Foundation

Page 2: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

History of Presenting Illness

54 year old Caucasian female with recurrent unilateral redness and irritation OS

4-5 episodes over 4 years treated with Tobradex

No workup, systemic treatment, or IMT specifically for ocular symptoms

1 month of redness and irritation prior to initial presentation at MERSI

Page 3: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Past Medical History

Cutaneous pemphigus vulgarisDiagnosed by skin biopsy in 2004Extensive esophageal involvement initiallyNumerous treatments: multiple courses of oral Prednisone CellCept, Rituxan and IV-Ig (since 2005)

Currently in remission on Rituxan and IV-Ig

DepressionParoxetine

Recurrent genital herpesSystemic Acyclovir

Page 4: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Initial Examination

VA sc 20/20 OD, 20/25 OS

Pupils 4>2 OU, no RAPD

CVF full OU

EOM full OU

IOP 20mmHg OU

Page 5: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:
Page 6: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Exam OD OS

Lids WNL WNL

Conjunctiva/Sclera White & quiet Diffuse 1+ injection with 3+ superficial& deep vessel injection superiorly witha central white opacity

Cornea Clear Clear

Anterior Chamber Deep and quiet Deep and quiet

Iris Round and reactive

Round and reactive

Lens PSC PSC

Page 7: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

PLAN??Serologies

Conjunctival & Scleral biopsy for Immunohistochemical staining

Page 8: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Laboratory Studies

HLA typing

ACE

Lysozyme

ESR, CRP

C3, C4, CH50,

RF

Properdin factor 3

Bartonella henselae & quintana IgG/IgM

Hepatitis B

ANCA

Antibody testing: SSA

SSB

SS-DNA

DS-DNA

Smith

Page 9: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Laboratory Studies

IgE : 298 (<114 normal)

ANA : positive Titer 1:160

Pattern homogenous

C1Q complexes: 8.1 (<4.4 normal)

C3D complexes: 18 (<8 normal)

Page 10: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Intraoperative Findings

Page 11: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Conjunctival Biopsy: H&E

Page 12: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Conjunctival Biopsy: PAS

Page 13: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Direct IF staining of conjunctival biopsy with IgG

Page 14: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Conjunctival Biopsy: Direct IF

Page 15: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Direct IF staining of conjunctival biopsy with anti-HSV IgG

Page 16: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Conjunctival Biopsy – HSV IgG

Page 17: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Direct IF staining of conjunctival biopsy with anti-VZV antibody (negative control)

Page 18: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:
Page 19: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Diagnosis??

Ocular Pemphigus Vulgaris complicated by HSV

Page 20: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Treatment

Acyclovir was replaced with Valacyclovir

Ocular inflammation has been quiet for the past 10 months while on therapy with IV-Ig infusions and valacyclovir, without topical steroids

Page 21: Case Presentation 2010 FOIS Meeting - Uveitis · Manisha Mehta, MD Luis Alonso Gonzalez-Gonzalez, MD Thank You. Title: Case Presentation 2010 FOIS Meeting Author: sana Created Date:

Acknowledgements

C.Stephen Foster, MD, FACS, FACR

Quan Nguyen, MD

Mark Dacey, MD

Manisha Mehta, MD

Luis Alonso Gonzalez-Gonzalez, MD

Thank You