Autoimmune-- Inflammatory Flashcards
What are the classifications of uveitis?
acute v chronic, granulomatous v non-granulomatous, laterality and location
What are symptoms of acute uveitis?
pain (hallmark), photophobia, redness, sudden onset, self-limiting
What are signs of uveitis?
AC rxn, circumlimbal injection, synechiae, keratic precipitates, low IOP
Why might IOP be low in acute uveitis?
CB inflamed and non-functioning
What are symptoms of chronic uveitis?
no pain, minimal to no redness, ill-defined start, not self limiting
What are signs of chronic uveitis?
AC rxn, lack of circumlimbal redness, synechia, keratic precipitates (large mutton fat), elevated IOP
Why may IOP be elevated in chronic uveitis?
due to pupillary block, angle closure from PAS or mechanical obstruction of TB from inflammatory debris
What is recurrent?
a repeat episode of inflammation after complete resolution with or without treatment
What is recrudescent?
a rebound of the inflammation before complete resolution, usually occurring during the tapering of the steroid
What 2 cell types are involved in granulomatous inflammation?
epithelioid cells and macrophages
What 2 cells are involved in non-granulomatous uveitis?
lymphocytes and plasma cells
Which nodule can be either non or granulomatous?
koeppe nodule
What diseases are associated with granulomatous uveitis?
systemic conditions, autoimmune reactions and systemic infections aka sarcoid, TB, syphilis, lyme and HSV/VZV
What diseases are associated with non-granulomatous uveitis?
idiopathic, trauma, HLA-B27
What is anterior uveitis?
inflammation is limited to the anterior and posterior chamber
What is intermediate uveitis?
inflammation is mostly located near the peripheral retina, pars plana and vitreous
What is posterior uveitis?
inflammation of retina, choroid or both (chorioretinitis)
Ocular inflammatory conditions which may be in association with anterior uveitis?
keratitis, episcleritis, scleritis, lens-induced, post surgical
What are the top 3 etiologies of anterior (non-gran) uveitis?
idiopathic, HLA B27, ankylosing spondylitis
What are signs of intermediate uveitis?
snowbank, exudates at ora, vitreous snowballs, peripheral venous sheathing, candle wax dripping, cystoid macular edema
What are vitreous snowballs?
white and yellow colored aggregates of inflammatory cells that accumulate in the inferior vitreous
Why might cystoid macular edema occur in intermediate uveitis?
spillover of inflammation from the vitreous
What is the top differential dx for intermediate uveitis?
sarcoidosis
What does posterior uveitis often involve?
loss of vision and floaters, often associated with retinal vasculitis
What are differential diagnoses for posterior uveitis?
white dots syndromes, infectious, non-infectious
What are infectious causes of posterior uveitis?
TB, syphilis, toxoplasmosis, HSV/VZV/CMV, fungi
What are non-infectious causes of posterior uveitis?
idiopathic, sarcoidosis, MS, multifocal choroiditis, VKH syndrome, Behcet’s
What is panuveitis?
inflammation found in the anterior chamber, vitreous and retina/choroid
What are etiologies of panuveitis?
**sarcoid, syphilis, VKH, infectious endopthalmitis, behcet’s disease
What are characteristics of ocular sarcoidosis?
granulomatous iritis, PAS, PS, vitreous opacities, bilateral, chorioretinal lesions, periphlebitis, optic disc nodule, choroidal nodule, conjunctival lesion, scleral plaque, lacrimal gland granulomas, disc edema, cystoid macular edema
What can you expect with periphlebitis?
candlewax drippings and macro-aneurysms
What ocular manifestation of sarcoid can you biopsy?
conjunctival lesions
Describe lacrimal gland granulomas with sarcoid
S-shaped lid that is not tender to the touch and hard as a rock (contrasted with dacryoadenitis that is tender)
What are the 1st and 2nd great masqueraders?
syphilis and sarcoid
The most common lung finding in sarcoidosis is?
bilateral hilar adenopathy
What is the most common manifestation of sarcoidosis?
respiratory tract involvement (90%)
What percent of people develop permanent pulmonary abnormalities from sarcoid?
50% with 5-15% developing fibrosis of the parenchyma due to scarring of the granulomas
What are the stages of lung involvement?
0=none // 1= bilateral hilar adenopathy // 2=pulmonary parenchyma involvement // 3= pulmonary infiltrates with fibrosis // 4= end stage lung disease
What is the second most affected organ for sarcoid?
skin aka cutaneous involvement 9-37%
Who gets more cutaneous involvement from sarcoid?
African Americans > Scandinavians
T/F cutaneous sarcoid lesions usually resolve on their own
true, in under a month
What are cutaneous sarcoid lesions?
erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, lupus pernio, violaceous
What is erythema nodosum?
large, painful, hard nodule
What is lupus pernio?
characteristic malar rash similar to lupus with red, inflamed hard area
Which ethnicity has the highest cardiac involvement from sarcoid?
japanese
What are signs of cardiac involvement of sarcoid?
conduction abnormalities (most common, ventricular arrythmias, fibrosis, granumola formation
What is nervous system involvement of sarcoid?
neurosarcoidosis 10-25% of cases
What are signs of nervous system involvement with sarcoid?
CN palsies, myelopathy, papilledema, palate dysfunction, hearing abnormalities, neuroendocrine changes, chronic meningitis, peripheral neuropathy, hypothalamic and pituitary abnormalities
What cranial nerve palsy is most common with sarcoid?
CN VII potentially due to parotid involvement
What are examples of endocrine involvement of sarcoid?
increased prolactin, increase in active metabolite of vitamin D, parotid enlargement
What causes increased prolactin?
granuloma in the pituitary gland
What is the cause and effect of an increase in 1, 25- dihydroxy Vitamin D?
occurs in granulomas, results in hypercalcemia and hypercalciuria
How common is parotid gland enlargement?
5-10% usually bilateral with dry mouth
What are gastrointestinal complications of sarcoid?
<1% symptomatic, mostly affects the stomach, + kidney, testicular and liver
What is true of liver involvement of sarcoid?
20-30% have abnormal liver function tests and 70% have granulomas
What is hematogenous involvement of sarcoid?
lymphopenia, anemia, leukopenia, thrombocytopenia, lymphadenopathy
What is the lymphoadenopathy of hematogenous sarcoid?
occurs in 15% of cases, intrathoracic nodes (hilar) 75-90% of the time, peripheral is common, especially cervical
What is musculoskeletal involvement of sarcoid?
5-15% of cases, arthritis, bone involvement and lofgren syndrome
What is lofgren syndrome?
acute arthritis, bilateral hilar adenopathy and erythema nodosum; scandinavians > blacks
Most common ocular manifestation of sarcoidosis?
bilateral granulomatous uveitis
Most common systemic complaint of sarcoidosis?
fever, anorexia, arthralgia
Most common pulmonary complaints of sarcoidosis?
dyspnea on exertion, cough, chest pain, hemoptysis (coughing up blood)
When you have granulomas in the lung, what is in the epithelial tissue in the lung?
ACE – may be artificially low if patient is on ACE-inhibitor
What is serum lysozyme?
prognostic (but not diagnostic) of sarcoidosis, serum lysozyme is excreted as the epithelial cells form the ball of inflammation
More serum lysozyme=
more active granulomas
What can be done to rule out TB?
TB skin test (PPD or mantoux), mycobacteria culture
Why might you run a PCR of ocular fluid?
rule out herpes simplex or zoster
What is radiologic testing of sarcoidosis?
chest x-ray, chest CT, PET scan
What is a chest x-ray?
2D plane
What is a chest CT?
3D layers
What is PET scanning?
looks at metabolic activity of a lesion; SUV >7.0 high % positive carcinoma, 1.0-2.5 significant for cocci
T/F there is no single diagnostic test specific for sarcoid
true
What is sarcoid lab testing?
ACE, serum lysozyme, others (hypercalcemia, serum amyloid A, soluable interlukin receptor, glycoprotein KL-6)
What is the only way to specifically and equivocally dx sarcoidosis?
sarcoid biopsy
Where can you take a sarcoid biopsy?
conjunctiva, bronchial, peripheral lymph node, lacrimal gland
What is the pathology of sarcoid biopsy?
macrophages mature to epitheliod cells, giant cells which are a conglomeration of epitheliod cells, noncaseating granuloma
What is a caseating granuloma indicative of?
TB
What does a sarcoid granuloma do?
damages tissue and organs due to the mass affect of granulomas on the anatomy and not to the release of mediators that damage cells
What are 4 diagnostic criteria of sarcoidosis from the international workshop on ocular sarcoidosis?
definite ocular sarcoid, presumed ocular sarcoid, probable ocular sarcoid, possible ocular sarcoid
What is definite ocular sarcoid?
biopsy supported with a compatible uveitis
What is presumed ocular sarcoid?
bilateral hilar adenopathy on CXR with a compatible uveitis, no biopsy
What is probably ocular sarcoid?
3 of the 7 ocular signs and 2 positive lab tests, biopsy not performed
What is possible ocular sarcoid?
biopsy performed with negative result, 4 of the 7 ocular signs, tow positive labs
What are seven signs of ocular sarcoidosis?
mutton fat KPs/small granulomatous KPs and/or iris nodules, TM nodules and/or PAS, vitreous opacities displaying snowball, multiple chorioretinal peripheral lesions, nodular and/or segmental periphlebitis (+/-) candlewax dripping and/ore retinal macroaneurysm in an inflammed eye, optic disc nodule/granuloma, bilaterality
What lab test should you do with a negative chest x-ray result for sarcoid?
chest CT
What is the current corticosteroid protocol for systemic sarcoid disease?
40-80 mg prednisone daily for 8-12 weeks; gradual taper over a period of 6-12 months and monitor for steroid response
What are corticosteroid-sparing agents?
cyclosporine A, methotrexate and anti-tumor necrosis factor alpha
What is treatment of anterior uveitis?
topical steroid, pred forte for granulomatous; cycloplegics
What is treatment of intermediate, posterior or panuveitis?
oral steroids, depot steroids
What are depot steroids?
sub-conj or sub-tenon injection of kenalog, or iontophoresis
What is iontophoresis?
electrical charge used to move pred acetate particles deeper into the eye
