Systemic Flashcards
Reactive arthritis
Reactive arthritis is associated with two types of infection: (1) post-dysentery caused by Gram-negative bacteria (e.g. Shigella, Salmonella, Yersinia, Campylobacter); and (2) post-venereal (e.g. caused by Chlamydia).
The classic triad of reactive arthritis are: urethritis, polyarthritis, and conjunctivitis. The medical student way to remember this is: “can’t see, can’t pee, can’t climb a tree”.
A mucopurulent and papillary conjunctivitis is the most common ocular manifestation of reactive arthritis. This conjunctivitis can be associated with a mucocutaneous discharge but without a swollen preauricular node. Other ocular signs include iritis, a punctate epithelial keratitis, and anterior stromal infiltrates.
Other systemic signs include “keratoderma blennorrhagicum” (i.e. papules, vesicles, or pustules on the palms and soles), “circinate balanitis” (non-painful penis rash), sacroiliitis, plantar fasciitis, and Achilles tendonitis. Reactive arthritis can also be associated with life-threatening conditions such as cardiac conduction defects, pericarditis, and aortic insufficiency. In fact, some believe that Christopher Columbus died from cardiac complications from reactive arthritis.
More than 75% of individuals with reactive arthritis are HLA-B27 positive. Interestingly, reactive arthritis is much more common in AIDS patients. The true cause of this increased incidence is currently unknown.
Heerfordt-Waldenstrom
Heerfordt-Waldenstrom syndrome is a type of sarcoidosis syndrome characterized by uveitis, parotiditis, fever, and facial nerve palsy.
Other syndrome in sarcoid: Lofgren = legs =triad of erythema nodosum, b/L hilar adenopathy, arthritis
Behcet’s
Behcet’s pts typically have more prominent non-ocular manifestations than ocular ones. Systemic manifestations include recurrent oral ulcers (MC sign), epididymitis, skins lesions (e.g. erythema nodosum), CNS involvement, and arthritis, cardiac (myositis). The anterior segment is usually more prominently involved in Behcet’s disease and includes a severe uveitis often with hypopyon formation. Posterior segment signs include widespread occlusive vasculitis, retinal necrosis, ischemic optic neuropathy, and severe vitritis. This disease is associated with HLA-B51. HLA-A29 is associated with birdshot choroidopathy; HLA-DR2 is associated with pars planitis; and HLA-B27 is associated with the various spondylarthropathies (e.g. ankylosing spondylitis).
Morbidity: eyes (2/3), vascular dz (1/3), CNS dz (10-20%).
Corticosteroids - mainstay for initial Rx of acute BD, but most pts eventually become resistant to steroid therapy. Starting dose 1.5 mg/kg/day with gradual taper.
Start immunosuppression with immunomodulating agents: azathioprine, cyclopsorine, infliximab.
Hypopyon with or without fibrin
Behcet’s - less likely to have fibrin
HLA B27 - more likely to have fibrin
Subglottic stenosis
Wegener’s
Tracheal collapse or h/o tracheostomy
polyarteritis nodosa
Phlebitis
Sarcoidosis, MS, Birdshot, Eales
Arteritis
ARN, PAN, SLE, Susac (retinoscochleocerebral angiopathy)
Both arteritis and phlebitis
Behcet and toxoplasmosis
Retinitis: focal vs. multifocal
Focal = toxoplasmosis Multifocal = HSV/VZV, candidiasis
keratoderma blenorrhagicum
“psoriasis” on palms/soles - seen in Reiters
brown aseptic abscesses
rash on palms/soles in adults
syphilis (secondary/maculopapular) and Reiters (keratoderma blenorrhagicum)
Photophobia
2/2 inflamed iris-ciliary body contracting –> pain 2/2 consensual pupillary response
Myopia and hyeropia in uveitis
Myopia from cataract
CME causing hyeropia
Child
JIA
toxoplasmosis
toxocariasis
pars planitis
Young adult
Anklyosing spondylitis
pars planitis
Fuchs
Adult > 50 yo
Birdshot
serpiginuous
masquerade
Male in uveitis
most things female (MEWDS, PIC/MCP, JRA)
Male = ankylosing spondylitis, Behcets (but incomplete M = F), PAN.
Nodules in uveitis
sarcoidosis, lepropsy, RA
rash on palms/soles in kids
Kawasaki
Erythema nodosum
red nodules on lower EXTENSOR surfaces. Panniculitis - inflammation of subactue fatty tissue. delayed hypersensitivity CUBS Crohns, Coccidomycosis, cat scratch Ulcerative colitis Behcet Sarcoid
livedo reticularis
dilation of capillaries and venules 2/2 blood stasis or vascular changes. Worse in cold temperatures. RSPD PAN SLE dermatomyositis RA
Myalgias
PAN
Tendonitis
Reiter’s syndrome (Achilles’ tendon)
Arthritis
JRA Behcet's (but not as big of a feature) Lyme dz RA = inflammed synovium Reiters (seronegative arthropathy = inflammed tendon @ insertion; PAIR)
sarcroilitis
anklyosing spondylitis, IBD
enthesitis
inflammation @ site of ligament or tendon insertion = HLA-B27 assoc/arthropathies
sausage digits/dactylitis =
psoriatic arthropathy
sausage digits
psoriatic arthritis
Peripheral neuropathy
PAN
Headache
VKH
Aseptic meningitis
Behcet’s
saddle nose deformity
syphilis, Wegener’s, relapsing polychondritis
Relapsing polychondritis
Inflammed pinna/auricular chondritis (80%)
Abs to collagen (also present in sclera)
episcleritis (40%) and scleritis (15%)
nasal chondritis - saddle nose deformity
vestibular dysfxn
Oral ulcers/aphthous stomatitis
Behcet’s and Reieters
SOB
Sarcoidosis, Wegener’s, TB?
diarrhea
Crohn’s, Whipple dz, ulc colitis
abdominal pain from ischemia
PAN
orchitis
PAN
Renal problems
PAN = protenuria, vascular nephropathy, renal vessel aneurysms
Wegener’s/intersitital nephritis = hematuria, casts
Wegener’s = glomerulonephritis
TINU
Animals
Deer = lyme dz DUSN - racoons and dogs pigeons - cryptococcus mosquitos - wnv black flies = onchocerciasis
KPs - active vs. inactive
active = creamy white inactive = pigmented or glassy comment on shape = stellate KPs in Fuchs, herpes, toxoplasmosi comment on shape (lower half of cornea) classically diffuse (limbus to limbus) in fuchs, sarcoid, herpes, CMV
Hypopyon
HLA-B27, endophthalmitis, corneal ulcer, Behcet’s, Rifabutin, malingnacy, “silicone oil”
Heterochromia
siderosis bulbi (darker iris)
fuchs’
waardernburg’s syndrome = white forelock, broad nasal bridge
congenital horner’s (lighter)
snowball vs snowbank
snowball = cell/proteinaceous debris (sarcoidosis) snowbank = fibroglial tissue and cells leaking from peripheral phelbitis. located inferiorly along pars planitis
Swollen ON and retina
MCC of swollen ON and retina = CRVO (also think malignant HtN)
cat scratch
DUSN (early)
IRVAN = young healthy (mostly female), retinal arteritis OU with numerous aneurysmal dilations of the retina and ON head arterioles, neuroretinitis, uveitis)
Visual loss 2/2 exudative maculopathy and NV sequelae of retinal ischemia
Focal choroid lesions and vitreous cell
Vitreous cells = toxocariasis
No vitreous cell = serpiginous, tumor
Multifocal choroidal lesions and vitreous cell
Multifocal
vitreous cell = most white dots, VKH, SO
no vitreous cell: OHS, PIC, Krill
Panuveitis
MC b/L, granulomatous, and NOT idiopathic
Sarcoidosis Behcets VKH/SO syphilis TB
Granulomatous uveitis
sarcoidosis - discrete TB VKH/SO - diffuse syphilis lens induced uveitis - zonal (phacoanaphylactic) IOFB
Acute uveitis
HLA-B27 Behcet Lyme ARN MEWDS APMPPE
Chronic
JRA serpiginious birdshot pars planitis intraocular lymphoma post-usrgical uveitis sarcoidosis
MC uveitis
Anterior (usually idopathic) > panuveitis> posterior (usually infectious) > intermediate (usually idiopathic)
Anterior uveitis
Idiopathic HLA-B27 Ankylosing sponydlitis Reiters IBD psoriasis Fuchs' JRA sarcoidosis
Intermediate uveitis
pars planitis Lyme MS sarcoidosis PCNSL
posterior uveitis - retinitis causes
Focal = toxoplasmosis, cystericercosis
Multifocal = HSV/VZV, syphilis, sarcoidosis, candidiasis
posterior uveitis - choroiditis
Focal choroiditis
- vitreous cells: toxocariasis
- no vitreous cells: serpiginous, tumor
Multifocal choroiditis
- vitreous cells: white dot cells, VKH/SO
- no vitreous cells: OHS, PIC, Krill
ANCA
c-ANCA = Wegener's, Cytoplasmic staining, Ab directed to proteinase 3 p-ANCA = perinuclear staining, Ab to myeloperoxidase (MPO), crescentic glomerulonephritis (strong assoc), PAN (moderate assoc), Churg-Strauss (moderate)
serology syphilis
RPR & VDRL - if positive, indicate active dz. Nml with treatment
FtA-ABS: stay positive for life.
Serology lyme
Two step test:
1) ELISA or IFA 1st and if positive
2) confirm with western blot/PCR
May be FALSE positive: syphilis, HIV, EBV, RA, SLE
Bartonella serology
B. henselae Ab in cat scratch or parinaud’s
IgG antibody detection with IFA (indirect fluorescent Ab)
Toxoplasmosis
negative titers R/O toxoplasmosis, but otherwise not very helpful.
Acute infection indicators: 4 fold increase in IgG titers. Positive IgM or IGA. Titers do not correlate with ocular dz 2/2 low serum levels.
HLA
Class 1 = on all nucleated cells. HLA A, B, C. antigen presenting platform for CD8 (suppressor T cells).
Class 2 = macrophages/dendritic cells. HLA-DR, -DP, DQ. palfor for CD4 cells
HLA -DR4
SO/VKH and JRA (also DW2)
HLA-B7, -DR2
MS/POHS
HLA - B44
retinal vasculitis
Pathergy test
Behcets
LP
syphilis, lyme, VKH
CXR
TB, sarcoid, thymoma, wegners
Gallium or PET scan
sacroidosis
EKG
LHON, Kearn-sayre syndrome, ankylosing spondylitis
T lymphocyte modulators
cyclosporine (SE HTN): pars planitis, Behcet, VKH
Antimetabolites
MTX (JRA), mycophenolate
Alkylating agents
Cyclophosphamide (heme cystitis, sterility). Wegener,s behcets, VKH/SO, relapsing polychondritis
Biologic response modifiers
Etanercept (?JRA)
Infliximab (Behcet)
surgical considerations
wait 3 mo of quiescence for CE/IOL and 6 mo for PK
Stellate KP
Fuchs heterochromic iridocyclitis
herpes
toxoplasmosis
fuchs
thought to be 2/2 rubella reactivation
intermediate uveitis
lyme, pars planitis, MS, sarcoid, PCSNL
posterior uveitis
infection vs. white dot
panuveitis
sarcoid, behcet’s, VKH/SO, syphilis, TB
Ankylosing spondylitis
aortic insufficiency, cardiomegaly, conduction defects, colitis, apical fibrosis of the lungs
Eye findings: anterior uveitis, episcleritis, scleritis
Rx: physical therapy and NSAIDS
Reiters
conjunctivitis, urethritis, arthritis
Assoc/w/: chlamydia, ureaplasma, urealyticum, yersinia, shigella, salmonella
3 major or 2 major + 2 minor
Major: urethritis, polyarthritis, conjunctivitis, keratoderma blenorrhagicum (psoriatic rash on palms/soles)
Minor: plantar fascitis/Achilles tendinitis, circinate balanitis, painless mouth ulcers, prostatitis, cystitis, sacroilitis, tendinitis, recent diarrhea, iritis/keratitis
Fuchs
develop lighter iris if depigmentation of posterior pigment layer
darker iris if stromal atrophy in blue iridis reveal pigment layer
JRA PAUCIarticular
RF-, ANA+, pauciarticular/oligoarticular (< 16 yo
late onset - HLA-B27 boys, sudden u/L self-limited duration anterior uveitis
JRA - POLYarticular
true RA, adolescent females(eyes: dryness/scleritis rarely iritis)
Still’s
high fever, HSP, adenopathy, leukocytitis, iritis rare
Kawasaki
< 5 yo
5/6 criteria: fever, b/L conjunctivitis, mild bilateral NONgranulomatous uveitis, rash, cervical adenopathy, oral lesions (fissures, strawberry tongue), lesions of extremities (redness of palms/soles)
Rx: aspirin, IVIG,
systemic steroids CONTRAINDICATED 2/2 risk of coronary artery aneurysm.
phacoantigenic glaucoma
acute penetration lens injury. Granulomatous rxn (type 3) s/p latent period rexposure to lens protein through surgery or trauma
uveitis with HYPOTONY initially. Later 2ndary glaucoma may develop.
Histology: PMNS at site of ruptured capsule surrounded by epitheloid and multinucleated giant cells
pars planitis
75% bilateral, 90% of intermediate uveitis, 25% of uveitis in kids,
We are about pars planitis 2/2 CME
HLA-DR15, MS
mild flare with few KPs, anterior vitritis, peripheral retinal periphelbitis, hyperemic disc, no synchiae.
Px: usually burns out in 5-15 years
Lyme dz
Borrelia burgdorfei 2/2 tick (Ixodes dammini in eastern USA or Ixodes pacificus western US)
Lyme = MC vector borne dz in USA
Dx test: lyme titer, IFA or ELISA followed by western blot
Rx: tetracycline, erythromycin or pencillin
For neuro-ophthalmic lyme disease IV: ceftriaxone or penicillin
Lyme stage 1
Stage 1: during 1st month erythema migrans, FOLLICULAR CONJUNCTIVITIS, fever, malaise, myalgia
Lyme stage 2
1-4 mo s/p infection with following manifestations.
NEED TO GET AN LP IF YOU ARE THINKING YOU ARE LOOKING AT STAGE 2
Lyme uveitis = CNS involvement; LP
ocular - keratitis, iritis (GRANULOMATOUS), intermediate uveitis, vitritis, optic neuritis, panophthalmitis
neurological: 30-40% patients, 7th NP, encephaltiis, meningitis
MSK - arthritis, tendonitis, joint effusions
Lyme stage 3
over 5 months - keratitis, atrophic skin changes, chronic arthritis, adult respiratory distress syndrome, neuropsych
Toxoplasmosis
oocyst (soil form)
tachyzoite (active infectious form)
bradyzoite (latent form) - thousands of them in a cyst
Rx:
triple therapy: pyrimethamine + sulfadiazine + prednisone + folinic acid (? + clinda)
Bactrim DS + prednisone
?chronic bactrim DS for suppression
Toxocariasis
2/2 ingestion of contaminated soil
o. Ocular larva migrans: u/L solitary lesion does not complete life cycle (worm not in stool)
1) Endophthalmitis form - lots of inflammation
2) posterior granuloma form = organism encased in tissue in back of eye. does best b/c encased in the back of the eye. Can’t cause RD or generate a lot of inflammation
3) Granuloma off to the side - classic strand that goes to posterior pole/ON
o. Visceral larva migrans, fever, LA, HSP, pneumonitis, eosinophili, no ocular inv
Dx: can do AC tap for eosinophils, ELISA for toxocara Ab titers from blood or AC
Rx: topical steroids and cycloplegics, PPV for RD. Thiabendazole for systemic toxocariasis but may make ocular dz worse.
POHS
yeast (yeast form is which causes dz/problem) and filamentous fungi
eastern/midwestern USA with N. european descent
HLA-B7, DR2
Triad: PPA, multiple punched out CR, CNV. NO VITRITIS
N. european descent
Birdshot, PXE, POHS
CMV
CD4 < 50
Different types: frosted branch, brush border, HIV retinopathy, cheese pizza
ARN
usually incompetent, 2/3 u/L
PAINFUL, mutton fat KP, likes arteries, mild iritis, pale disc edema, within 2 mo the necrotic retina sloughs, salt/pepper pigmentation
Rx: acyclovir IV, ganciclovir, corticosteroids, ASA
watch other eye, may develop ARN within 4 weeks
75% develop RD
PORN
ARN in AIDS, painless with minimal intraocular inflammation
multiple discrete peripheral or central areas of retinal opacification
vasculitis not prominent
Poor Px, 2/3 NLP
Rx: foscarnet + ganciclovir
subacute sclerosing panencephalitis
rare wild-type measles. Usually unvaccinated kids 8 year s/p primary infection
Si/Sx: nonspecific maculopathy, focal retinitis, disc swelling, minimal vitritis
Systemic findings: personality change, seizures, dementia, death
eye findings may precede neuro findings by weeks to years.
No Rx
Candidiasis
10% endophthalmitis from candidemia
Si/Sx: anterior uveitis, retinal hemorrhage, perivascular heathing, chorioretinitis with fluffy white lesions, vitreous abscess, subretinal abscess
Rx: amphotericin B (intravenous or intravitreal), fluconazole, voriconazole
pneumocystitis choroiditis
people with HIV/AIDS used to get this if they weren’t on bactrim
cysticercosis
taneia solium or saginata
“balloon in eye” = intravitreal (encapsulated worm)
harder to ID if subretinal
PPV Rx
Leprosy
mycobacterium leprae
likes COLD areas: skin, nerves, nasal mucosa, eyes
keratitis, scleritis, GRANULOMATOUS iridocyclitis, hypotony, phthisis, many others
Rx: dapsone, rifampin
TB
likes highly oxygenated areas (apex of lungs), choroid = multifocal choroiditis
Rx with dapsone
Leprosy, relapsing polychondritis, OCP, brown recluse spider bites
DUSN
dog hookworm (ancylostoma caninum) and raccoon (Baylisascaris procynosis)
Diffuse u/L subacute neuroretintiis/u/L wipe out syndrome
1st phase = postequatorial multiple evanescent grayish white dots with vitritis, retinal vasculitis
2nd phase: ON atrophy, extensive pigment dispersion, arteriolar atttenuation (look like RP)
Onchocerciasis
2nd MCC blindness in Africa
Blackfly vector and intermediate host
sub-cutaneous nodules
dead microfilariae cause AC rxn
Si/Sx: microfilaria swimming in AC, keratitis, iridocyclitis, chorioretinitis, RPE dispersion, CR atrophy, ON atrophy
Rx: ivermectin (macrolytic lactone) - single PO dose of 150 ug/kg, repeated annualy x 10 years
Whipple’s dz
Tropheryma whippelii
malabs, diarrhea, migratory polyarthritis
neuro-ophtho: nerve palsy, ophthalmoplegia, nystagmus
rarely: anterior uveitis, vitritis
Rx: bactrim possibly for 1 year
P. acnes
ANAEROBIC gram positive rod
sequered in capsular bag s/p cataract surgery
chronic GRANULOMATOUS uveitis with fibrin/hypopyon
Rx: intravitreal vanc and cephalosporin + IOL and capsule removal.
Ophthalmomyiasis
ocular invasion of fly larvae into AC, posterior segment, or subretinal (tracks of atrophied RPE)
death of organism causes inflammation
granulomatous uveitis,
including syphilis, Lyme disease, tuberculosis, Behcet’s disease, VKH/SO
CNV MC in which white dot syndrome?
A: MCP
MEWDS is an acute onset syndrome characterized by multiple, small gray-white dots at the level of the deep retina and RPE in the posterior pole. In some patients, a transient foveal granularity develops that is pathognomonic of this condition. Patients typically present with a unilateral decrease in vision (80%). It is more common in women in the second to fifth decades. Treatment is unnecessary as the fundus exam and vision typically improves spontaneous over 2-6 weeks. Rarely patients can have persistent visual field defects. CNV does not occur.
Multifocal choroiditis and panuveitis syndrome is a bilateral disease that predominantly affects women between the second and sixth decades. Patients present with bilateral vitritis, disruption of the peripapillary RPE and multifocal choroiditis. The multiple yellow choroidal lesions later evolve into chorioretinal scars similar to “histo spots.” Presenting symptoms include floaters, decreased vision, photopsia and visual field defects (e.g., enlarged blind spot). CNV occurs in approximately 20% of affected eyes and is the leading cause of vision loss in these patients. Local or systemic corticosteroids and/or immunomodulating agents are usually necessary to control inflammation after infectious etiologies are ruled out. CNV is treated with anti-VEGF therapy.
Birdshot retinochoroidopathy (vitiliginous chorioretinitis) is characterized by vitritis (100%), variable degrees of disc edema, vascular sheathing, and characteristic yellow, ovoid, “birdshot” chorioretinal lesions that are most numerous in the nasal retina. Patients present with floaters, blurred vision and peripheral photopsia. Vision loss is most commonly the result of cystoid macular edema (CME) in up to 30% of patients. CNV can occur but is rare. HLA-A29 is positive in 90% of patients. Immunomodulation is frequently needed.
Serpiginous choroidopathy is a recurrent inflammatory disease of the choroid that causes a serpiginous (pseudopodial) or geographic pattern of scarring in the posterior fundus. Acute lesions have a geographic zone of gray-yellow discoloration of the RPE which spreads from the disc. These areas then become atrophic over weeks to months and new lesions can occur elsewhere or contiguous with the areas of atrophy. The fellow eye may become involved months or years later. Dense scotomata corresponding to the involved areas develop. CNV occurs only rarely at the margin of an area of chorioretinal atrophy.
Note: CNV development is also very common in punctate inner choroiditis (PIC), with an incidence of up to 30% in areas of chorioretinal scarring.
