Autoimmune-- Collagen-Vascular Flashcards
List autoimmune collagen-vascular diseases
rheumatoid arthritis, juvenile rheumatoid arthritis, systemic lupus erythematosus, sjogren’s, ankylosing spondylitis, reactive arthritis, scleroderma
List vascular autoimmune diseases
granulomatosis with polyangiitis (wegener’s), temporal arteritis
What is the most common ocular manifestation of autoimmune disease?
dry eye
What is a collagen-vascular disease?
development of an immune response by the organisms against the host tissue
What is the etiology of collagen-vascular disease?
idiopathic or secondary to trauma, infection, or exposure to environmental antigens or cross-reactivity between medications
What happens in a collagen-vascular disease?
deposition of fibrin and diffuse inflammatory damage to connective tissue and vascular system (targets collagen and elastin) disseminated throughout the body
What is the etiology of rheumatoid arthritis?
unknown, environmental or viral factors may initiate disease in susceptible individuals
What is the demographic of RA?
W>M 25-50 year onset
What is the MOA of RA?
production of abnormal IgG causes autoantibody production (rheumatoid factors RF then attack the IgG); process attracts inflammatory cells to synovial fluid causing red, painful, swollen joints
What is testing for RA?
80% of patients are positive for rheumatoid factor
What are systemic manifestations of RA?
gradual onset over weeks to months affecting hands and feet first; other organs affected include heart, lungs, CNS, skin and GI tract; physical impairment and deformity affects quality of life
What are early systemic symptoms of RA?
fatigue, anorexia, morning stiffness, multi-joint involvement, symmetric joint swelling with associated warmth, swelling, tenderness, pain
What are rheumatoid nodules?
cutaneous nodules– firm, round, subcutaneous masses on the extensor surface of the forearms or near affected joint
What are ocular manifestations of RA?
DRY EYE, episcleritis, anterior scleritis, scleromalacia perforans, posterior scleritis, uveitis, glaucoma
What is scleromalacia perforans?
painless thinning of the sclera in RA patients
What are s/s of posterior scleritis?
pain, decreased VA, redness, diplopia, proptosis, choroidal folds, retinal striae, dis edema, macular edema, RD; important to look for injection of sclera deep into fornix
What is the sign of posterior scleritis on B scan?
T sign, depicts fluid behind globe
Does every case of scleritis necessitate a systemic work up?
yes, high prevalence for systemic disease w/ scleritis
What is keratopathy secondary to RA?
sclerosing keratitis, acute stromal keratitis, furrowing, sterile peripheral ulcerative keratitis
What is sclerosing keratitis?
associated with anterior scleritis at level of stroma, peripheral infiltrates, vascularization, lipid deposition, scarring, grayish opacification of stroma
What is sterile peripheral ulcerative keratitis?
secondary to scleral inflammation, leads to thinning and perforation; systemic treatment required
T/F PUK in the course of systemic connective tissue disorder may reflect the presence of a potentially lethal systemic vasculitis?
true
RA diagnosis
(+)RF 80%, (+)ANA 20%, elevated ESR, RA confirmed by synovial fluid analysis, X-rays
What is (+) RF?
not specific for RA, (+) with Lupus, Sjogren, sarcoid, scleroderma, polymyositis, infectious disease
What is prognostic for the course and severity of RA?
elevated ESR (and CRP)
What do x-rays show?
narrowed joint spaces, loss of cartilage; more helpful later in the disease
What is RA medical treatment?
NSAIDS and analgesics, oral and intra-articular glucocorticoids, and DMARDS
What are NSAIDS and analgesics for in RA tx?
control pain and inflammation, GI upset (rx w/ H2 blocker), platelet and liver dysfunction
Describe oral and intra-articular glucocorticoids for RA tx?
5-10 mg daily, weight gain, DM, osteoporosis, infection, cataract, IOP
What is DMARDS?
disease modifying rheumatic drugs
What are examples of DMARDS?
hydroxychloroquine (plaquenil), gold salts, methotrexate, TNF inhibitors
What is HCQ?
antimalarial, 1-6 months for maximum therapeutics
HCQ ocular side effects
whorl keratopathy, toxic maculopathy
What are gold salts?
IM or oral, 3-6 months to reach maximum therapeutic benefit
What are gold salt ocular side effects?
corneal or conjunctival deposits, lens deposits, K ulcer, iritis, conjunctivitis
What is methotrexate?
IM or oral, immunosuppressive agent/chemotherapy drug, patients should also take folic acid supplements
What are methotrexate ocular side effects?
punctate keratitis, periorbital edema, blepharitis, conjunctival hyperemia, photophobia
What are TNF inhibitors?
subcutaneous injections 2x/week; remicade, humira, enbrel
What are ocular side effects of TNF inhibitors?
optic neuritis
HCQ AAO screening guidelines risk factors for toxic retinopathy
HCQ >5.0 mg/kg real weight and CQ >2.3 mg/kg real weight; duration of use > 5 years; subnormal GFR; concomitant tamoxifen use; macular disease
HCQ AAO screening guideline clinical examination techniques
automated VF (24-2 and 10-2 in Asian population due to maculopathy being further out), spectral domain OCT
What is no longer recommended for HCQ clinical examination?
fundus exam, time-domain OCT, fluorescein angiography, full-field ERG, amsler, color testing, EOG
What is the most common connective tissue disease in children?
juvenile rheumatoid arthritis
What is the most common etiology of uveitis in children?
juvenile rheumatoid arthritis
What are the 3 forms of JRA (JIA)?
Pauciarticular, polyarticular, systemic
What is pauciarticular JIA?
1-4 joints affected
What is polyarticular JIA?
5 or greater joints
What is systemic JIA?
Still’s disease, high spiking fevers (104+), lymphadenopathy, evanescent (transient) salmon-colored rash
What is onset of JIA?
all forms <5 years old, no complaint of pain but avoids limb use
JIA etiology
girls>boys 4:1; precipitating factors: trauma, URI, hereditary components; most RF (-)
What is the most common form of JIA?
60% of all JIA is pauciarticular
What form of JIA is most likely to develop uveitis?
pauciarticular
What are 3 JRA ocular manifestations?
cataract, band keratopathy, glaucoma
Cataracts and JIA
50% of eyes with chronic anterior uveitis secondary to steroid use and inflammation
Band keratopathy and JIA
77% of eyes with chronic anterior uveitis, not calcium related, VA loss when visual axis affected
Glaucoma and JIA
15-45% with uveitis, secondary to steroid use and inflammation
T/F treatment of JIA is systemic steroids
false, oral steroids should be avoided in children with JIA related uveitis because these drugs have overwhelming effect on bone growth
What is systemic treatment of JIA?
intra-articular prednisolone or triamcinolone, physical therapy, NSAIDS, DMARDS
What is systemic lupus erythematosus?
idiopathic, chronic, multi-system inflammatory disease characterized by pathogenic antibodies that attack cells and tissues throughout the body; exacerbations and remissions
Etiology of systemic lupus erythematosus
genetic predisposition, environmental influence triggers immune response
Dx of autoimmune lupus
(+) ANA in 90%
What are forms of lupus?
auto-immune, discoid lupus erythematosus, drug induced
What is discoid lupus erythematosus?
dermatologic condition (cutaneous form) (-) ANA
What is drug induced lupus?
(+) ANA, chlorpromazine, hydralazine, phenytoin, isoniazid, methyldopa, quinidine
T/F ocular severity of lupus relates to systemic severity
true, if you see ocular signs, you can assume they are not doing well
Demographics of lupus
females 90% > males, AA and hispanics most effected, 1st-4th decades of life
Age of onset for lupus?
average age 30 years old
What cancers does lupus increase the risk of?
lymphoma and cervical cancer
What is the most common presenting symptom of lupus?
non-erosive, non-deforming, symmetrical arthropathy
What 3 systemic side effects of lupus were emphasized?
discoid lesions, butterfly rash, and raynuad’s phenomenon
What are constitutional side effects of lupus?
90%; fever, fatigue, weight loss
What are musculoskeletal side effects of lupus?
90%; arthritis, myalgia, arthralgia, joint deformities
What are cutaneous lesion side effects of lupus?
80%; discoid lupus erythematosus, butterfly rash (40%), alopecia, photosensitivity, oral ulcers
What are hematologic side effects of lupus?
80%; anemia, vasculitis, thrombosis (15%) venous, arterial; associated with antiphospholipid syndrome
What are neurologic side effects of lupus?
50%; HA, anxiety, depression, mood swings, Bell’s palsy
What are cardiac side effects of lupus?
50%; accelerated atherosclerosis, carditis
What are pulmonary side effects of lupus?
pleural effusions
What are renal disease side effects of lupus?
50%; proteinuria, nephrotic syndrome, renal failure
What are GI side effects of lupus?
45%; mild pain, diarrhea, abnormal liver enzymes, colitis
What percent fetal loss is associated with lupus?
30%
SLE lab testing
(+) ANA 95%, anti-native double stranded DNA very specific for SLE
Rheumatology criteria for SLE
4 of 11 symptoms (none ocular); anemia, pericarditis, diarrhea, cognitive dysfunction, vascular thrombosis, musculoskeletal arthralgia and myalgia, proteinuria, malar rash
Why might you see madarosis in SLE?
madarosis secondary to scarring of the hair follicle from the discoid lesion
SLE corneal effects
k-sicca, stromal infiltrates, ulcer, vascularization, SPK, pannus, furrowing
SLE conjunctival effects
nonspecific conjunctivitis: bulbar and palpebral
SLE sclera effects
scleritis, diffuse or nodular
SLE uvea effects
non-granulomatous uveitis, choroidopathy causes damage to overlying RPE and can lead to serous detachment of RPE
SLE periorbital effects
skin lesions: discoid lupus, edema
SLE EOM effects
myositis
SLE vitreal effects
hemorrhage from NVD, NVE
SLE retina effects
CWS, vasculitis induced proliferative retinopathy > hemorrhages > R; vascular occlusion arterial >venous
T/F 88% of those with lupus retinopathy have active systemic disease
true, retinopathy parallels systemic disease activity
SLE ON effects
optic neuritis, AION, atrophy
SLE neuro-ophthalmic effects
CN palsies (diplopia), transient ptosis, pupil abnormalities, VF loss, cortical blindness, INO, nystagmus, papilledema
SLE tx
rest, avoidance of stress and UV light, NSAIDS, HCQ, corticosteroids, cytotoxic agents
What are examples of cytotoxic agents (immunosuppressants)?
azathioprine, mycophenolate mofetil, cyclosporine, methotrexate, leflunomide, cyclophosphamide, chlorambucil, nitrogen mustard
What is management for SLE?
refer to rheumatologist
What is the prognosis of SLE?
if only cutaneous and musculoskeletal: good; if brain, lungs, hearts, or kidney: poor
What is the survival rate of SLE?
10 years 90%; >20 years 63-75%
What is cause of death with SLE?
cardiovascular disease, infection, renal failure
T/F organismal invasion is more likely to cause a granulomatous uveitis
true
What is sjogren’s syndrome?
autoimmune disorder with progressive loss of lacrimal and salivary exocrine glands
What is the demographic for sjogren’s?
women age 50, no race or ethnic predilection
What is primary sjogren’s?
more aggressive form of disease process, must rule out lymphoma (5%)
What is sjogren’s secondary to?
RA, SLE, scleroderma, polymyositis
What are oral manifestations of sjogren’s?
painful fissures in corners of mouth/tongue, periodontal disease, oral yeast infections, aphagia, enlarged parotid glands “chipmunk cheeks”
What are systemic manifestations (xerosis)?
skin, gynecological complications
What sjogren’s systemic manifestations were emphasized?
Raynaud’s phenomenon, trigeminal nerve involvement (facial pain and numbness), joint pain, myalgia, fatigue, and high risk for lymphoma
What are minor systemic manifestations of sjogren’s?
respiratory tract: nosebleeds, alteration of smell and taste, difficulty speaking, chronic cough, bronchitis; GI: chronic gastritis, reduced exocrine secretion in the GI tract can lead to gastritis and pancreatitis; kidney and liver: interstitial inflammation of kidney
What are ocular manifestations of sjogren’s?
aqueous layer of tear film diminished, mucous strands/filamentary keratitis, injection, corneal opacification, pannus, ulceration
Ocular diagnosis of sjogren’s
K sicca, (+) lip biopsy confirming lymphocytes, confirmed connective tissue or autoimmune disorder
K sicca in sjogren’s
schirmer test or phenol red thread test, decreased TBUT, decreased tear meniscus, corneal staining
What does NaFl “stain”?
devoid epithelium, dryness
What does Rose Bengal stain?
devitalized cells
What are sjogren’s antibody tests?
antiSS-A (RO), antiSS-B (LA), and antiSS-C; detect antinuclear antibodies and confrims diagnosis
What blood testing is completed for sjogren’s?
check ANA, RF, and ESR
Tx of oral sjogren’s symptoms
saliva substitutes, dental hygiene, rinses, anti-fungal tablets and creams, frequent liquid consumption, humidifiers, nasal sprays, oral pilocarpine, cevimeline
What is cevimeline for?
stimulates exocrine glands
Tx of systemic sjogren’s symptoms
skin creams, NSAIDS for myalgia and arthralgia, systemic steroids
Tx of ocular sjogren’s symptoms
reduce symptoms, prevent complications, copious ATs, topical cyclosporin A, punctal occlusion, oral flax seed oil and omega 3 fish oil capsules, lateral tarsorrhaphy, moisture goggles, spectacle side shields
What is filamentary keratitis tx?
lubrication, NaCl drops and ung, acetylcysteine 10% to break up mucous, debride filaments, bandage CL
What does topical cyclosporinA (Restasis) do?
stimulates tear production by suppressing inflammation
What are examples of spondyloarthropaties?
ankylosing spondylitis, psoriatic arthritis, reiter’s syndrome, inflammatory bowel disease
What is consistent between spondyloarthropaties?
inflammatory disorders (-) RF and (+) HLA-b27 involving the central or axial skeleton and resulting in non-granulomatous anterior uveitis
What is the demographic for ankylosing spondylitis?
young men
What ocular involvement is present before systemic ankylosing spondylitis symptoms (initial presentation of disease)?
acute, unilateral non-granulomatous uveitis
T/F non-granulomatous uveitis is related to systemic severity of ankylosing spondylitis
false, not related to systemic severity of disease, completely resolves in between occurrences
What are signs of acute, unilateral non-granulomatous uveitis?
painful and confined to the AC, a lot of fibrin in AC, pupil may become occluded with a fibrous membrane, hypopyon
What is ankylosing spondylitis characterized by?
inflammation of distal joints and central skeleton; produces lesions at the site of the attachment of the ligament to bone (most commonly around the pelvis and spine); lower back pain
Why is early diagnosis of ankylosing spondylitis important?
early dx and tx is essential to prevent permanent spinal deformity aka bamboo spine
How is ankylosing spondylitis diagnosed?
(+) family history, (+) HLA-B27, (-) RF and ANA, elevated EST and C-reactive protein, elevated alkaline phosphatase, X-ray reveals sacroilitis and other joint involvement
What is ankylosing spondylitis systemic treatment?
relieve pain and stiffness via exercise and physical therapy, oral NSAIDS (indomethacin + H2 blocker), glucocorticoid injection, antirheumatic drugs
What is ankylosing spondylitis ocular treatment?
topical steroids and cycloplegics, topical phenylephrine for breaking PS, monitor for side effects like cataracts of increased IOP, educate pt on s/s of uveitis and FU q6-12 months
What is psoriatic arthritis?
psoriatic skin and nail disease with arthritis of the spine
What is psoriasis?
mild to moderate itching, discrete erythematous lesions with scaling and flaking; nail involvement parallels arthritis severity
What is nail involvement in psoriatic arthritis?
discolored, thickened, cracked, pitted nails
What are the demographics of psoriatic arthritis?
males=females, peak age of onset is 4th or 5th decade; periods of exacerbations and remissions
What is the diagnosis of psoriatic arthritis?
(-) RF (-) ANA (+) HLA, clinical s/s, (+) family history,
What are ocular complications of psoriatic arthritis?
conjunctiviits and uveitis
What is tx of conjunctivitis from psoriatic arthritis?
supportive therapy, cold compresses, vasoconstricots, lubricants
What is tx of uveitis from psoriatic arthritis?
cycloplegics, topical steroids
What is systemic treatment for psoriatic arthritis?
antirheumatic meds, topical retinoid gels/corticosteroid creams and UV light therapy for skin lesions, steroid injections for synovitis
What is reactive arthritis?
acute arthritis associated with an antecedent infection ex: chlamydial; characterized by distal joint inflammation (large weight bearing joings)
What is the reactive arthritis triad?
arthritis, urethritis, conjunctivitis; if all 3 are not present= incomplete form of the disease
The majority of reactive arthritis infections follow which 2 types of infection?
sexually acquired infection usually chlamydia or enteric infection usually salmonella
What percent of reactive arthritis from STI causes ocular involvement and what is the demographic?
50% w/ males 5x > females
What percent of reactive arthritis from enteric infection causes ocular involvement and what is the demographic?
70% w/ males = females
Which is a more common reactive arthritis infection, enteric or STI?
enteric
What are ocular concerns of reactive arthritis?
unilateral or bilateral conjunctivitis and acute nongranulomatous uveitis
Describe the unilateral or bilateral conjunctivitis from reactive arthritis
initial symptom, develops within days of systemic infection; non-infectious and self limiting (non-purulent w/ SPK)
Describe the acute nongranulomatous uveitis from reactive arthritis
recurrent, unilateral, KPs lead to synechia
What is the diagnosis of reactive arthritis?
history and physical exam, (+) HLA-B27 75% of the time, elevated ESR and CRP, (-) ANA, CBC w/ mild anemia, radiographic confirmation of joint involvement
What is systemic treatment of reactive arthritis?
pain relief, exercise encouraged, NSAIDS, glucocorticoids avoided unless pt is confined to bed, if chlamydial then oral azithromycin
What is ocular treatment of reactive arthritis?
vasoconstictors/lubricant for conjunctivitis, topical steroids/cycloplegic/systemic steroids for uveitis, topical fluoroquinolone for keratitis or corneal ulceration, manage secondary glaucoma
Scleroderma
read in textbook
What is giant cell arteritis/temporal arteritis?
disease of the elderly average onset 70 years old
What is systemic (autoimmune) vasculitis?
obliterative vasculitis, arterial walls become thickened by granulomatous deposition and giant cell formation results in luminal narrowing and ischemia of downstream tissues w/ predilection for cranial arteries
What is vision loss from giant cell?
AION, ophthalmic artery involvement, severe and permanent, unilateral to bilateral in 1-10 days if no treatment 50-75%, TIAs with preceding photopsia and HA
What is polymyalgia rheumatica (PMR)?
aching and stiffness in torso and proximal limbs, exclusive to the elderly, occurs with GCA, 15-30% of people with PMR get GCA
GCA and PMR demographics
caucasians, females > males; about 50% of those with GCA get PMR
What is the clinical presentation of GCA?
elderly patient with sudden LOV and HA + fever, malaise, night sweats, weight loss, anorexia, severe HA, neck pain, jaw claudication, lack of temporal pulse in 40%; 20% with no symptoms
What are ocular manifestations of GCA?
amarosis fugax, AION, AAION, APD, altitudinal VF defect, CRAO, CN palsy, ischemia of A or P seg
What is AAION?
sudden, painless, permanent vision loss, edematous disc, splinter hemes, CWS, (+) APD, altitudinal VF defect
What does A seg ischemia lead to?
K edema, iritis, hypotony, cataract
What does P seg ischemia lead to?
neovascular glaucoma
What is the diagnosis of GCA?
age, s/s, elevated EST and C-reactive protein
T/F you should wait for diagnostic evidence to treat GCA?
false, treat prior to results in enough clinical evidence
What is a temporal artery biopsy for?
confirming GCA diagnosis, serial sectioning of the vessel so that inflamed areas are not missed; assess 25 mm length, 15% are biopsy negative
What is normal ESR?
male age/2 and female (age +10)/2
What is 100% sensitive for GCA?
CRP >2.45 mg/dL
What is treatment for GCA?
refer to ER STAT; goal is to preserve vision in the fellow eye with oral or IV corticosteroids to decrease arterial inflammation and increase blood flow
How are GCA and GPA (Wegner’s) different?
GCA= large to medium artery while GPA= small artery and/or vein
