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TITILE: Ocular Sequelae in a Patient Diagnosed with Sarcoidosis

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TITILE: Ocular Sequelae in a Patient Diagnosed with Sarcoidosis TITILE: ACE Testing: When Sarcoidosis Calls ABSTRACT: Sarcoidosis is an inflammatory condition which predominantly affects the lungs, skin, and eyes. Anterior uveitis is a common ocular manifestation of the condition that has the potential to cause sight threatening consequences. I. CASE HISTORY -Patient demographics – 61 y/o AA female -Chief complaint- bilateral redness x 3 weeks, accompanied by pain OS only -Ocular history - corneal gutatta, visually insignificant cataracts -Medical History - osteoporosis, depression, chronic insomnia -Medications - alendronate, cetirizine, fluoxetine, zolpidem -Other salient information: - patient was told she had ‘systemic inflammation’ several months ago - went away over time with vitamin D supplements II. PERTINENT FINDINGS -Anterior segment at first visit: OD: tr/1 cells OS: 2+ cells with mild flare with focal area of posterior synechiae OS @ 1:00 -Posterior segment within normal limits at first visit -Lab tests ordered: CBC, ESR, CRP, RF, ANA, ACE, lysozyme, RPR, TB-gold, HLA-B27, lyme Of those, ACE and lysozymes returned elevated -Radiology study: chest x-ray results: mild bilateral prominence of both hila -Pt sent to pulmonologist, where she was diagnosed with sarcoidosis -Patient returned 2 months later reporting a ‘spider web’ in vision OS for the past 2 weeks, and then sudden vision loss -Newly noted intermediate uveitis with macular edema OD, mild optic nerve head edema OU -Case discussed with ophthalmology and pt started on 60mg oral prednisone -MRI of brain and orbits did not reveal ONH or chiasmal enhancement or intracranial/orbit granulomas -carotid/echocardiograms – negative -Sent to ophthalmology and diagnosed with bilateral sarcoid uveitis – anterior and intermediate, now with sarcoid- related atypical optic neuritis OS III. Differential Diagnosis -Anterior uveitis: Sarcoidosis, Tuberculosis, Ankylosing spondylitis, Syphilis, Psoriatic arthritis, Behcet's, Lyme, Lupus, Herpes -Uveitis with pulmonary disease: Sarcoidosis, Lyme disease, Lymphoma -Optic nerve edema: Papilledema, Optic neuritis, Non-arteritic or Arteritic anterior ischemic optic neuropathy, Central retinal artery occlusion -Uveitis with optic nerve involvement: Multiple sclerosis, infectious (Syphilis, Lyme), Neoplastic (carcinomatous meningitis) -Based on all laboratory and radiologic studies, sarcoidosis is presumed etiology for this patient's signs and symptoms IV. Diagnosis and discussion -Sarcoidosis = systemic non caseating epitheliod cell granuloma -most commonly affects the lungs, eyes, skin, and lymph nodes -severe cases can lead to organ failure -Patient demographics: -women are slightly more likely -typical age range: usually 20-40 -African Americans: more likely to have more severe or recurrent cases, and more likely to have pulmonary involvement -(+) family history carries an increased risk -Causes: idiopathic, although genetic predisposition is likely and may be caused by exposure to bacteria, viruses, certain antigens – dust, chemicals -Diagnosis: made by tissue biopsy of noncaseating granulomas -Further labs/tests: ACE levels, lysozyme levels, chest x-ray, chest computerized tomography (CT), gallium scintillogray, pulmonary tests, measures of serum and calcium levels -High ACE in Sarcoid, HIV, DM, histoplasmosis, hyperthyroid, lymphoma, TB -Normal ACE levels does not rule out sarcoid as they may be low at earlier stages -Lysozymes elevated in sarcoidosis -CBC and calcium levels may also be elevated -Patient symptoms -vary depending on affected organs -may present gradually or acute/transient -may be asymptomatic and are inadvertently diagnosed after an unrelated chest x-ray is performed -general symptoms include fatigue, decreased weight, enlarged lymph nodes, fever -Lung involvement: most commonly affected organ -chronic dry cough, shortness of breath, chest pain, wheezing -hilar and mediastinal lymph nodes commonly affected -Skin Involvement: erythema nodosum -generally tender, warm, red/purple bumps often at shins/ankles but also nose, cheeks, ears -Heart granulomas can develop resulting in abnormal heart rhythms -Joint disease -Renal insufficiency resulting in hypercalcemia -Ocular Involvement -occurs in 25-50% of cases -may occur in early stages, with systemic manifestations present, in asymptomatic patients, years before systemic involvement -Anterior Segment: -eyelid nodules -conjunctival granulomas -conjunctival/lacrimal gland infiltration – LG infiltration can decrease tear productivity resulting in dry eye -Band keratopathy – in the presence of increased Ca+ levels -Anterior uveitis – anterior chamber reaction, KPs, posterior synechiea, cataract -Posterior Segment: -Intermediate or posterior uveitis, cystoid macular edema, candle wax droppings, punched out lesions, periphlebitis, retinitis, exudative retinal detachment -multifocal choroiditis -neuroretinitis -Retinal vascular – narrowed, tortuous, exudation, macroanuerysms V. Treatment, management -topical steroids/cycloplegics, periocular steroids for anterior uveitis -subtenon steroid deposit, intravitreal steroids for intermediate uveitis -systemic corticosteroids for unresolving cases -anti-immune drugs – methotrexate -Organ transplant in severe cases -This patient: anterior uveitis initially treated with topical steroids and cycloplegic, but switched to oral prednisone (60mg) and then intravenous steroids (IV solumedrol: 1 gram x 3 days) when there was no improvement. -Patient's ocular manifestations are currently managed with maintenance topical therapy (Pred forte once a day). Systemic disease is managed with Cellcept. Her vision has returned to 20/20 OD, 20/30- OS. VI. Conclusion Sarcoidosis is an inflammatory condition characterized by the prominence of systemic granulomas. Diagnosis is aided by the utilization of chest x-rays and lab testing, specifically ACE and lysozyme levels. Oftentimes, treatment is not necessary as patients may have the condition and be asymptomatic. Uveitis is the most common ocular manifestation of the condition and, in most cases, may be successfully treated with the use of topical steroids and cycloplegia. In more severe cases, intermediate or posterior uveitis can develop and result in more sight threatening conditions such as cystoid macular edema and optic nerve head edema. In these cases, patients may benefit from systemic steroids and/or immunosuppressants. .
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