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Posterior Seg: Inflammation I (Pars Planitis and Sarcoidosis)
Terms in this set (30)
This is a chronic inflammatory syndrome which the signs of inflammation are most pronounced in the vitreous (Vitritis). Many names have been given to this condition including intermediate uveitis, cyclitis, vitritis. This term is used to refer to -idiopathic- intermediate uveitis. This can be also secondary to sarcoidosis, syphilis, and Lyme disease
a) Pars Planitis
Pars Planitis: Is common, 10-15% of all uveitis. Occurs primarily in what demographics? Seldom seen before the age of 5 or after the age of 30. About 70-80% of cases are bilateral or unilateral? No race or sex predilectation.
a) Young adults and children
a) Major symptoms are _______ and _____ vision resulting from cells in vitreous and/or CME. Usually no ___, ____ or ______.
b) Anterior Segment: Externally the eyes of patients with pars planitis do not appear inflamed. The conjunctive and sclera are with or without injection? The AC may be clear or show only mild cells and flare.
c) Posterior Segment: Most signs and symptoms are in the vitreous cavity where there are ______ opacities, cells and debris. The vitreous gel is syneretic and shows varying amount of fibrin cells and strands. _____ opacities are mostly preretinal and concentrated ______.
The hallmark of pars planitis is the presence of what?
Initially exudates may appear as discontinuous yellow-white clumps, they increase in number and size until they coalesce to form a fluffy white exudate over the inferior peripheral retina and pars plana. This membrane has been termed what?
a) Floaters, hazy
b) Pain, redness, or photophobia
e) Snowball, inferiorly
f) Exudates in the inferior vitreous base
a) Posterior Segment: ______ frequently occurs, and is most common cause of what? Peripapillary retinal edema may be present. ERM formation is not uncommon. A patchy periphlebitis may be present in the retinal periphery. _______ of the pars plana exudate (Snowbank) is not uncommon, and may result in VH.
b) Vision decrease
a) FA: Diffuse leakage from the peripheral retinal venules and capillaries, without any predilectation for the inferior retinal vasculature. Suggests that snowbank formation inferiorly is most likely due to _______ phenomenon. Is the inferior peripheral retina the source of inflammation in pars planitis? _________ is localized to region of snowbanking. What is used for evaluation of CME and ERMs?
Natural History of Pars Planitis:
Prolonged Smoldering: ______% of all cases have a chronic smoldering course. Many patients will suffer one more more episodes of ________ of the inflammation.
b) Benign Course: Self-limiting course characterized by gradual improvement without a single episode of ______ of the low-grade activity. 10% of cases have this pattern.
Natural History of Pars Planitis:
a) Visual Prognosis: Over 70% have 20/50 or better. 20/200 or worse in 6% of cases.
What is the most common cause of irreversible vision loss?
What is the most common complication of pars planitis (42%)?
CME is the second most common complication (28%). Cataracts are caused by inflammation and steroid use to clear inflammation.
a) Macular damage (CME or ERM)
Diagnosis of Pars Planitis: The diagnosis is based completely on what two things? The presence of pars plana exudate (snowbank) helps make diagnosis, but is it required? Patients without snowbanking may represent an ____ stage or _____ form of the disease. Consider pars planitis in any patient who has a generally __ External eye with ______ And debris in the vitreous cavity especially in the presence of an ______ over the inferior pars plana
a) History and Clinical Findings
c) Earlier, milder
d) Quiet, cells, exudates
Diagnosis of Pars Planitis (DDx):
a) ________: vitritis with or without snowanks can occur in ocular version. The prevalence of patient with vitritis has been reported between 2 and 10%
b) Intraocular _____ may masquerade as this vitritis. Retina would show subretinal mass. Should be suspected in _____ patients that do not do what?
c) Elderly, steroid therapy
Diagnosis of Pars PLanitis (Diagnostic Tests):
T/F. There is no specific laboratory test to establish a diagnosis of pars planitis.
b) There are certain tests to rule out specific conditiosn that mimic pars planitis. Such as sarcoid (serum lysozyme, ACE, Chest x-ray), TB (skin test, chest x-ray), Syphilis (FTA-ABS) and Lyme (ELISA).
Treatment of Pars Planitis: Treatment is indicated if vision is _____ or worse (due to CME) or vision better than 20/40 with severe ______. Are topical steroids of any value? ______ ______ steroid injection is effective in the treatment of CME and in reducing the vitritis (80-90% success rate in improve VA; 75% reducing the vitritis). The _______ (fluocinolone acetonide) intravitreal implant helpful for those patients who require frequent injections
Treatment of Pars Planitis: What may be beneficial for cases of CME or persistent intraocular inflammation that fails to respond to corticosteroids? CME may take months to resolve after vitrectomy and often requires intensive steroid therapy after surgery. Removal of inflammatory debris may decrease stimulus for CME. ERM peel can also be accomplished during vitrectomy.
This is a chronic idiopathic multisystem granulomatous disorder through to result from an exaggerated cellular immune response to a variety of self antigens or non-self antigens. Ocular involvement is common, occurring in 15-25% of patients with sarcoidosis. Must be considered as a DDx in almost any type of ocular _________, because can affect any part of the _____ or _______.
c) Eye, adnexa
Epidemiology of sarcoidosis: occurs worldwide but is predominant in certain ethnic and racial groups. In USA sarcoid is 3x higher in blacks than whites. Women have a slightly higher incidence. The disease affects all age groups but its rare in children, more common in adults. Almost 75% of cases occur in patients under the age of ______.
Clinical Features of Sarcoidosis:
Is characterized by the formation of ________ ________ in affected organs.
The disease affects predominantly the ____ and thoracic lymph nodes, skin and eyes. The lungs are most frequently affected with pulmonary involvement in 90% of patients. Frequently, the disease is _____ and discovered by chest x-ray performed for unrelated causes.
a) Non-caseating granulomas
Clinical Features of Sarcoidosis:
a) Pulmonary sarcoid: bilateral hilar ______ can be detected on a chest radiograph.
b) Extrapulmonary lesions: lymph node involvement is common. Lymph node _____ is often used for confirmation of the diagnosis. Skin lesions are common and include sarcoid granulomas and erythema nodosum. ________ occurs in about 5% of cases and includes cranial nerve palsies, papilledema, peripheral neuropathy, meningitis, and space-occupying cerebral lesions.
*Diagnosis of Sarcoidosis: In the absence of the known _______ agent, the diagnosis remains mainly a diagnosis of _______.
T/F. There are no definitive diagnostic serological or radiological tests for sarcoidosis.
Because of lack of specificity, no laboratory or radiological test is _________ for sarcoidosis. The presence of ________-____ granulomas on tissue biopsy with compatible clinical features is considered as proof of diagnosis of sarcoidosis*
Diagnosis of Sarcoidosis: The single best test for the evaluation of patients with suspected sarcoidosis is the ________ _-_ since it is abnormal in 90% of patients with his disease. However, and abnormal chest film does not unequivocally establish diagnosis. Chest x-ray usually shows ______ ________ in combination with interstitial _______ disorder
a) Chest x-ray
b) Hilar adenopathy
*Dx and Tx of Sarcoidosis:
No single lab test is pathopnomonic for sarcoidosis. _____________ is important (sarcoidal granulmoas produce ACE; Serum ACE levels reflect disease activity). ______ _____ produced by macrophages and epitheliod cells may be more sensitive but less specific than ACE, suggesting a diagnosis of sarcoidosis. The mainstay of treatment is what?*
a) Serum angiotensin Converting Enzyme (ACE)
b) Serum lysozyme
c) Cortiocosteroid treatment
Ocular findings in Sarcoidosis: cular disease may be the initial manifestation in sarcoidosis and may progress to severe visual impairment and even blindness. Ocular involvement manifests in 25-60% of patients with systemic sarcoid.
The most common manifestations are what two things?
Ocular sarcoid is usually bilateral.
a) Uveitis (30-70%)
b) Conjunctival Nodule (40%)
Ocular Findings of Sarcoidosis (Lids and Conjunctiva): ______ ___________ involvement is common and usually asymptomatic. Large granuloma leading to diplopia or severe KCS may develop.
T/F. Eyelid and conjunctival granulomas are very common.
The association of sarcoidosis with autoimmune thyroid disease is well known (Grave's disease).
a) Lacrimal gland
Ocular Findings of Sarcoidosis (Uveitis)
a) Uveitis is a frequent and early feature of sarcoidosis. More than ______ of uveitis cases manifested within 1 year after the onset of systemic disease. Uveitis preceded the non-ocular signs of sarcoidosis in about 30%.
b) Anterior uveitis: May be symptomatic or have "silent uveitis".
This is uveitis without symptoms of typical pain, photophobia, and tearing
b) Silent Uveitis
Ocular Findings of Sarcoidosis:
a) _____-_____ ____ _ or small granulomatous KPs are common.
b) ____/______ _____ granulomatous of the iris may develop, especially in chronic sarcoid. These are small nodules on the pupillary border. Busacca are usually larger located in mid periphery.
c) ______ _____ ___: may lead to corneal band keratopathy, secondary cataract, glaucoma and CME.
Mutton-fat keratic precipitates
Chronic Anterior Uveitis
Ocular Findings of Sarcoidosis: Clinical picture resembling pars planitis, including _______ and _____. Intermediate uveitis may precede more severe posterior segment changes. Differentiation from idiopathic par planitis and other types of intermediate uveitis is difficult.
Ocular Findings (Posterior Segment): _____ _____ is the second most common ocular finding of ocular sarcoid (after anterior uveitis), sin in 10%-17% of patients with ocular sarcoid. Tends to occur where? Perivascular sheathing is common. More severe periphlebitis with heavy sheathing is known as what? _______ ____ may be small and numerous or large and solitary (resembling a tumor).
a) Retinal periphlebitis
c) Candle Wax Drippings
d) Choroidal Granulomas
Ocular Findings (Posterior Segment):
Major source of vision loss is what?
Neovascularization NVE and NVD may occur. ______ _____ involvment in 1-7% of patients with ocular sarcoid. Optic nerve granulomas, sarcoid optic neuropathy, and papilledema. It has been reported that patients with posterior sarcoid uveitis have a higher risk of what?
b) Optic Nerve
c) CNS involvement
Ocular Findings Sarcoid (Neuro-Ophthalmic Findings): Present in 12% of sarcoid patients.
What is the most common manifestation of CNS involvement?
Others include ON disease, chiasmal syndrome, cranial nerve palsies, motility disorders, encephalopathy, and disorders of the hypothalamus and pituitary gland.
a) Facial nerve palsy
Diagnosis of Ocular Sarcoid: The laboratory investigations that provide value in the diagnosis of ocular sarcoidosis. Negative PPD skin test. Elevated serum _____ levels. Elevated serum ______. __________ revealing hilar adenopathy.
c) Chest x-ray
Diagnosis of Ocular Sarcoid: four levels of certainty for the diagnosis of ocular sarcoidosis:
a) Definite ocular sarcoid: via ___-supported with compatible uveitis.
b) Presumed ocular sarcoid: positive _____ _____ with a compatible uveitis.
c) Probable sarcoid: Negative chest x-ray with three signs and two positive lab tests
d) Possible ocular sarcoid: negative biopsy but at least four intraocularsigns and two positive lab tests.
T/F. Not a single test that can prove this without a doubt can only make a case for it.
b) Chest x-ray
Treatment of Ocular Sarcoid:
What is the mainstay of therapy for ocular sarcoidosis?
Posterior segment lesions generally require systemic steroids. CME, neo, and granulomas all respond to systemic anti-inflammatory treatment. For refractory disease, low dose methotrexate may be effective.
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