Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences

Subjective CC/HPI: 31F presents with right eye pain, upper lid edema/tenderness and copious discharge OD x 2 days. She has mild/moderate pain with EOM, but no diplopia. She denies any vision changes, fever, or congestion.

History POH: none PMH: none Eye Meds: none Meds/Allergies: none, NKDA Social Hx: no pets, no travel

Objective OD OS OD OS VA: 20/2520/20 Pupils: 5->25->2, no rAPD IOP:2118 EOM:full OU

Objective PLE: OD E/L/L:ST erythema/edema C/S1+ injection, mild temporal chemosis KClear ACD&Q I/LWNL VitWNL DFE:all WNL OU

Clinical photos

Assessment 31F with moderate/severe unilateral superotemporal orbital pain x 2 days with copious watery discharge. 31F with moderate/severe unilateral superotemporal orbital pain x 2 days with copious watery discharge. CT scan showing inflammation/hyperintensity of the right lacrimal gland CT scan showing inflammation/hyperintensity of the right lacrimal gland Dx: Acute Dacryoadenitis

Laboratory CBC: 14.1 CBC: BMP: WNL BMP: WNL ESR/CRP: 7/9.4 ESR/CRP: 7/9.4 EBV: IgG positive, IgM positive EBV: IgG positive, IgM positive

Laboratory CBC: 14.1 CBC: BMP: WNL BMP: WNL ESR/CRP: 7/9.4 ESR/CRP: 7/9.4 EBV: IgG positive, IgM positive EBV: IgG positive, IgM positive 9

Treatment One dose IV Vanc and Ceftriaxone in ED One dose IV Vanc and Ceftriaxone in ED Sent home on po Keflex x 5 days Sent home on po Keflex x 5 days Warm compresses PRN Warm compresses PRN

Background Self-limited condition Self-limited condition Inflammatory enlargement of the lacrimal gland Inflammatory enlargement of the lacrimal gland Pathophysiology poorly understood but thought to be due to ascension from the conjunctiva Pathophysiology poorly understood but thought to be due to ascension from the conjunctiva 1/10K ophtho patients 1/10K ophtho patients Acute & Chronic forms Acute & Chronic forms

Forms of Dacryoadenitis Acute Acute Unilateral severe pain, redness, and pressure in ST quadrant Unilateral severe pain, redness, and pressure in ST quadrant Rapid onset Rapid onset Chronic Chronic Unilateral or bilateral Unilateral or bilateral Painless enlargement of lacrimal gland > 1 month Painless enlargement of lacrimal gland > 1 month More common More common

Etiology Infectious Infectious Viral: Mumps, Epstein-Barr Virus, HZV, Mononucleosis Viral: Mumps, Epstein-Barr Virus, HZV, Mononucleosis Bacterial: Staph aureus, N. gonorrhoeae, Syphilis, Chlamydia, TB Bacterial: Staph aureus, N. gonorrhoeae, Syphilis, Chlamydia, TB Inflammatory Inflammatory Sarcoidosis, Grave’s Dz, Sjögren’s, IgG-4 related disease, benign lymphoproliferative lesions Sarcoidosis, Grave’s Dz, Sjögren’s, IgG-4 related disease, benign lymphoproliferative lesions

Exam Gland is often prolapsed, enlarged, and tender Gland is often prolapsed, enlarged, and tender Chemosis Chemosis Injection Injection Mucopurulent discharge Mucopurulent discharge Eyelid edema/erythema Eyelid edema/erythema Submandibular adenopathy Submandibular adenopathy Mild ophthalmoplegia Mild ophthalmoplegia

Treatment Viral: supportive measures (e.g. warm compresses, oral NSAIDs) Viral: supportive measures (e.g. warm compresses, oral NSAIDs) Bacterial: oral Cephalosporin such as Keflex Bacterial: oral Cephalosporin such as Keflex Inflammatory: treat underlying disease Inflammatory: treat underlying disease Consider biopsy if refractory to treatment of underlying disease Consider biopsy if refractory to treatment of underlying disease

Research Retrospective case series Retrospective case series 8 patients with IgG-4 related disease of salivary and lacrimal glands 8 patients with IgG-4 related disease of salivary and lacrimal glands Performed EBV FISH analysis on biopsied tissues Performed EBV FISH analysis on biopsied tissues Found a positive correlation Found a positive correlation between IgG-4 concentrations between IgG-4 concentrations and EBV load and EBV load EBV viral load may have EBV viral load may have prognostic value in these pts prognostic value in these pts

References 1. Kanski JJ. Acute dacryoadenitis. Clinical Ophthalmology: A Systemic Approach 6 th edition. Butterworth, Heineman, Elsevier; 2014: BCSC 7 Orbit, Eyelids, and Lacrimal System:2014. pp Rhem MN, Wilhelmus KR, Jones DB. Epstein-barr virus dacryoadenitis. AM J Ophthlmol 2000;129: Boruchoff SA, Boruchoff SE. Infections of the lacrimal system. Infect Dis Clin North Am. Dec 1992;6(4):