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Systemic Disease (Wolfe) > Autoimmune-- Inflammatory > Flashcards

Autoimmune-- Inflammatory Flashcards

1
Q

What are the classifications of uveitis?

A

acute v chronic, granulomatous v non-granulomatous, laterality and location

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2
Q

What are symptoms of acute uveitis?

A

pain (hallmark), photophobia, redness, sudden onset, self-limiting

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3
Q

What are signs of uveitis?

A

AC rxn, circumlimbal injection, synechiae, keratic precipitates, low IOP

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4
Q

Why might IOP be low in acute uveitis?

A

CB inflamed and non-functioning

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5
Q

What are symptoms of chronic uveitis?

A

no pain, minimal to no redness, ill-defined start, not self limiting

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6
Q

What are signs of chronic uveitis?

A

AC rxn, lack of circumlimbal redness, synechia, keratic precipitates (large mutton fat), elevated IOP

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7
Q

Why may IOP be elevated in chronic uveitis?

A

due to pupillary block, angle closure from PAS or mechanical obstruction of TB from inflammatory debris

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8
Q

What is recurrent?

A

a repeat episode of inflammation after complete resolution with or without treatment

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9
Q

What is recrudescent?

A

a rebound of the inflammation before complete resolution, usually occurring during the tapering of the steroid

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10
Q

What 2 cell types are involved in granulomatous inflammation?

A

epithelioid cells and macrophages

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11
Q

What 2 cells are involved in non-granulomatous uveitis?

A

lymphocytes and plasma cells

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12
Q

Which nodule can be either non or granulomatous?

A

koeppe nodule

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13
Q

What diseases are associated with granulomatous uveitis?

A

systemic conditions, autoimmune reactions and systemic infections aka sarcoid, TB, syphilis, lyme and HSV/VZV

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14
Q

What diseases are associated with non-granulomatous uveitis?

A

idiopathic, trauma, HLA-B27

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15
Q

What is anterior uveitis?

A

inflammation is limited to the anterior and posterior chamber

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16
Q

What is intermediate uveitis?

A

inflammation is mostly located near the peripheral retina, pars plana and vitreous

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17
Q

What is posterior uveitis?

A

inflammation of retina, choroid or both (chorioretinitis)

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18
Q

Ocular inflammatory conditions which may be in association with anterior uveitis?

A

keratitis, episcleritis, scleritis, lens-induced, post surgical

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19
Q

What are the top 3 etiologies of anterior (non-gran) uveitis?

A

idiopathic, HLA B27, ankylosing spondylitis

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20
Q

What are signs of intermediate uveitis?

A

snowbank, exudates at ora, vitreous snowballs, peripheral venous sheathing, candle wax dripping, cystoid macular edema

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21
Q

What are vitreous snowballs?

A

white and yellow colored aggregates of inflammatory cells that accumulate in the inferior vitreous

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22
Q

Why might cystoid macular edema occur in intermediate uveitis?

A

spillover of inflammation from the vitreous

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23
Q

What is the top differential dx for intermediate uveitis?

A

sarcoidosis

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24
Q

What does posterior uveitis often involve?

A

loss of vision and floaters, often associated with retinal vasculitis

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25
Q

What are differential diagnoses for posterior uveitis?

A

white dots syndromes, infectious, non-infectious

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26
Q

What are infectious causes of posterior uveitis?

A

TB, syphilis, toxoplasmosis, HSV/VZV/CMV, fungi

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27
Q

What are non-infectious causes of posterior uveitis?

A

idiopathic, sarcoidosis, MS, multifocal choroiditis, VKH syndrome, Behcet’s

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28
Q

What is panuveitis?

A

inflammation found in the anterior chamber, vitreous and retina/choroid

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29
Q

What are etiologies of panuveitis?

A

**sarcoid, syphilis, VKH, infectious endopthalmitis, behcet’s disease

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30
Q

What are characteristics of ocular sarcoidosis?

A

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

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31
Q

What can you expect with periphlebitis?

A

candlewax drippings and macro-aneurysms

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32
Q

What ocular manifestation of sarcoid can you biopsy?

A

conjunctival lesions

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33
Q

Describe lacrimal gland granulomas with sarcoid

A

S-shaped lid that is not tender to the touch and hard as a rock (contrasted with dacryoadenitis that is tender)

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34
Q

What are the 1st and 2nd great masqueraders?

A

syphilis and sarcoid

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35
Q

The most common lung finding in sarcoidosis is?

A

bilateral hilar adenopathy

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36
Q

What is the most common manifestation of sarcoidosis?

A

respiratory tract involvement (90%)

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37
Q

What percent of people develop permanent pulmonary abnormalities from sarcoid?

A

50% with 5-15% developing fibrosis of the parenchyma due to scarring of the granulomas

38
Q

What are the stages of lung involvement?

A

0=none // 1= bilateral hilar adenopathy // 2=pulmonary parenchyma involvement // 3= pulmonary infiltrates with fibrosis // 4= end stage lung disease

39
Q

What is the second most affected organ for sarcoid?

A

skin aka cutaneous involvement 9-37%

40
Q

Who gets more cutaneous involvement from sarcoid?

A

African Americans > Scandinavians

41
Q

T/F cutaneous sarcoid lesions usually resolve on their own

A

true, in under a month

42
Q

What are cutaneous sarcoid lesions?

A

erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, lupus pernio, violaceous

43
Q

What is erythema nodosum?

A

large, painful, hard nodule

44
Q

What is lupus pernio?

A

characteristic malar rash similar to lupus with red, inflamed hard area

45
Q

Which ethnicity has the highest cardiac involvement from sarcoid?

A

japanese

46
Q

What are signs of cardiac involvement of sarcoid?

A

conduction abnormalities (most common, ventricular arrythmias, fibrosis, granumola formation

47
Q

What is nervous system involvement of sarcoid?

A

neurosarcoidosis 10-25% of cases

48
Q

What are signs of nervous system involvement with sarcoid?

A

CN palsies, myelopathy, papilledema, palate dysfunction, hearing abnormalities, neuroendocrine changes, chronic meningitis, peripheral neuropathy, hypothalamic and pituitary abnormalities

49
Q

What cranial nerve palsy is most common with sarcoid?

A

CN VII potentially due to parotid involvement

50
Q

What are examples of endocrine involvement of sarcoid?

A

increased prolactin, increase in active metabolite of vitamin D, parotid enlargement

51
Q

What causes increased prolactin?

A

granuloma in the pituitary gland

52
Q

What is the cause and effect of an increase in 1, 25- dihydroxy Vitamin D?

A

occurs in granulomas, results in hypercalcemia and hypercalciuria

53
Q

How common is parotid gland enlargement?

A

5-10% usually bilateral with dry mouth

54
Q

What are gastrointestinal complications of sarcoid?

A

<1% symptomatic, mostly affects the stomach, + kidney, testicular and liver

55
Q

What is true of liver involvement of sarcoid?

A

20-30% have abnormal liver function tests and 70% have granulomas

56
Q

What is hematogenous involvement of sarcoid?

A

lymphopenia, anemia, leukopenia, thrombocytopenia, lymphadenopathy

57
Q

What is the lymphoadenopathy of hematogenous sarcoid?

A

occurs in 15% of cases, intrathoracic nodes (hilar) 75-90% of the time, peripheral is common, especially cervical

58
Q

What is musculoskeletal involvement of sarcoid?

A

5-15% of cases, arthritis, bone involvement and lofgren syndrome

59
Q

What is lofgren syndrome?

A

acute arthritis, bilateral hilar adenopathy and erythema nodosum; scandinavians > blacks

60
Q

Most common ocular manifestation of sarcoidosis?

A

bilateral granulomatous uveitis

61
Q

Most common systemic complaint of sarcoidosis?

A

fever, anorexia, arthralgia

62
Q

Most common pulmonary complaints of sarcoidosis?

A

dyspnea on exertion, cough, chest pain, hemoptysis (coughing up blood)

63
Q

When you have granulomas in the lung, what is in the epithelial tissue in the lung?

A

ACE – may be artificially low if patient is on ACE-inhibitor

64
Q

What is serum lysozyme?

A

prognostic (but not diagnostic) of sarcoidosis, serum lysozyme is excreted as the epithelial cells form the ball of inflammation

65
Q

More serum lysozyme=

A

more active granulomas

66
Q

What can be done to rule out TB?

A

TB skin test (PPD or mantoux), mycobacteria culture

67
Q

Why might you run a PCR of ocular fluid?

A

rule out herpes simplex or zoster

68
Q

What is radiologic testing of sarcoidosis?

A

chest x-ray, chest CT, PET scan

69
Q

What is a chest x-ray?

A

2D plane

70
Q

What is a chest CT?

A

3D layers

71
Q

What is PET scanning?

A

looks at metabolic activity of a lesion; SUV >7.0 high % positive carcinoma, 1.0-2.5 significant for cocci

72
Q

T/F there is no single diagnostic test specific for sarcoid

A

true

73
Q

What is sarcoid lab testing?

A

ACE, serum lysozyme, others (hypercalcemia, serum amyloid A, soluable interlukin receptor, glycoprotein KL-6)

74
Q

What is the only way to specifically and equivocally dx sarcoidosis?

A

sarcoid biopsy

75
Q

Where can you take a sarcoid biopsy?

A

conjunctiva, bronchial, peripheral lymph node, lacrimal gland

76
Q

What is the pathology of sarcoid biopsy?

A

macrophages mature to epitheliod cells, giant cells which are a conglomeration of epitheliod cells, noncaseating granuloma

77
Q

What is a caseating granuloma indicative of?

A

TB

78
Q

What does a sarcoid granuloma do?

A

damages tissue and organs due to the mass affect of granulomas on the anatomy and not to the release of mediators that damage cells

79
Q

What are 4 diagnostic criteria of sarcoidosis from the international workshop on ocular sarcoidosis?

A

definite ocular sarcoid, presumed ocular sarcoid, probable ocular sarcoid, possible ocular sarcoid

80
Q

What is definite ocular sarcoid?

A

biopsy supported with a compatible uveitis

81
Q

What is presumed ocular sarcoid?

A

bilateral hilar adenopathy on CXR with a compatible uveitis, no biopsy

82
Q

What is probably ocular sarcoid?

A

3 of the 7 ocular signs and 2 positive lab tests, biopsy not performed

83
Q

What is possible ocular sarcoid?

A

biopsy performed with negative result, 4 of the 7 ocular signs, tow positive labs

84
Q

What are seven signs of ocular sarcoidosis?

A

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

85
Q

What lab test should you do with a negative chest x-ray result for sarcoid?

A

chest CT

86
Q

What is the current corticosteroid protocol for systemic sarcoid disease?

A

40-80 mg prednisone daily for 8-12 weeks; gradual taper over a period of 6-12 months and monitor for steroid response

87
Q

What are corticosteroid-sparing agents?

A

cyclosporine A, methotrexate and anti-tumor necrosis factor alpha

88
Q

What is treatment of anterior uveitis?

A

topical steroid, pred forte for granulomatous; cycloplegics

89
Q

What is treatment of intermediate, posterior or panuveitis?

A

oral steroids, depot steroids

90
Q

What are depot steroids?

A

sub-conj or sub-tenon injection of kenalog, or iontophoresis

91
Q

What is iontophoresis?

A

electrical charge used to move pred acetate particles deeper into the eye

Systemic Disease (Wolfe) (10 decks)

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