4. CT disorders & Vasculitides Flashcards
what is a characteristic sign for dermatomyositis
& what are the rashes that may present
weakness w/o sensory symptoms
Skin lesions:
- Gottron’s patches/papules -raised violaceous lesions over dorsal DIP, PIP and MCP joint
- Heliotrope rash: periobital edema, purple suffusion over eyelid
- Periungual erythema
- V-neck erythema: poikiloderma: “shawl sign”
how is the respiratory tract involved in granulomatosis w/ polyangiitis (aka wegener’s granulomatosis)
90% nasal involvement –> saddle nose/crusting/bleeding/obstruction
erosive sinus dz
CXR - inflitrate, nodules (Asx nodule –> alveolar hemorrhage) and cavitary lesions
what labs should be considered for SLE
Antibodies to dsDNA, Smith (Sm), Ro/SS-A, La/SS-B, histones
urinalysis
CBC, ESR, ANA, complement levels, antiphospholipid Ab
what are symptoms that accompany kawasaki dz
will this cause morbidity?
fever, LAD, Rash, “strawberry tongue”
morbidity from coronary** involvement (**anyeurysm or MI)–> could be yrs later!
When treating SLE pts w/ corticosteriods, you have to monitor….
monitor for avascular necrosis of bone (hips & knees)
monitor for osteoporosis
Does lupus-like syndrome/drug-induced cause renal/neurological symptoms?
NO!
what serology test will confirm lcSSc
(+) anti-centromere
When DM is diagnosed, start looking for…
occult malignancy (CBC, CMP, UA, age appropriate screening)
look for: NHL, cervical, breast, lung, pancreatic, stomach, colorectal, prostate, ovarian, & multple myeloma
what inflammatory response is responsible for SLE
type III hypersensitivity
what population is kawasaki dz (mucocutaeous LN syndrome) most common
<5 yo
Male
highest incidence: Japan
DDx for Proximal M. weakness……….
where does anti-GBM (goodpasture syndrome) occur
glomerular capillaries
pul capillaries (–> pul hemorrhage –> death if not treated)
what are common features of SLE
Cutaneous: Malar “butterfly” rash, photosensitivity
Arthritis: inflam, symmetric, non-erosive
Hematologic: venous or atrial thrombosis
Cardiopulm: pericarditis, libman-sacks endocarditis, increased risk for MI bc accelerated atherosclerosis
Nephritis
Neurological: seizures, psychosis
How does polymyalgia rheumatica present?
Stiffness, soreness, & M. Pain
-proximal (shoulder, neck, pelvic girdle), severe, symmetrical, Morning –> Daylong
Feel weak bc of pain (subjective)
“I have trouble combing my hair and putting a coat on”
what does the Schirmer test check for?
keratoconjunctivitis sicca - if foreign body sensation produced tears
what population does inclusion body myositis (IBM) present most
& how does it present?
>40-50 yo
MALE!
CAUCASIAN!
-weakness: finger flexion or quad weakness
what is the gold standard for diagnosing PAH w/ IcSSc
Righ heart cath = elevated pul A pressure
(but can also use 2D echo)
what is the cause of mortality in SLE years later after Dx
accelerated atherosclerosis - bc chronic inflam –> become cause of death
5x greater chance of MI compared to general pop
thromboembolic events - freq cause of mortality
Why shouldn’t you give glucocorticoids to dcSSc pts
bc it can induce renal crisis
DDx for attached figure…
= cotton wool spots
SLE/APS
HTN, Diabetes
ischemia, infxn, embolic idiopathic
*and more!
what are treatments for sjorgens
symptomatic =
Regular eye Dr/dentist visits
Dry eyes - artificial tears, lubricating ointments, etc
Xerostomia- freq sip of water; sugarless candy
Arthalgia: hydroxychloroquine
Which autoimmune/vasculitides disorders are multisystem
SLE
Scleroderma
Behcet Syndrome
which type of SSc has an increased incidence of bronchoalveolar carcinoma
dcSSc & lcSSc
if a pt states they have difficulty rising from a chair/bathtub or climbing stairs - what should you add to you DDx?
What else would they present with?
= inflammatory myopathies
symmetrical (bilateral) proximal Ms - cant stand or climb
myalgia, weakness, typical dermal features (DM)
(look for CK and aldolase to be elevated)
DDx for thrombosis..
APS
Protein C or S def
Anti-thrombin def
Factor V Leiden def
HIT
Sepsis
Systemic vasculitis
DIC
TTP
what is libman-sacks endocarditis (SLE)
immune deposits collecting on valves
non-infectious
what drugs can cause lupus-like syndrome/drug-induced
(rmr: this syndrome promotes demethylation of DNA)
meds = Methyldopa, TNF inhibitors,Quinidine, Isoniazid, Chlorpromazine, Hydralazine, Procainamide, Minocycline
“My Tipsy Queen Is Crushing Heroin Past Midnight”
Which type of SSc presents w/ CREST syndrome?
what does CREST stand for?
limited (IcSSc)
=Calcinosis cutis
=Raynaud’s
=Esophageal dysmotility
=Sclerodactyly
=Telangiectasia
how would you dx and treat polyarteritis nodosa?
Dx: Hx/PE –>
Biopsy : inflitation/destruction of blood vessels by inflam cells –> fibrinoid necrosis, NO granulomas
Angiogram: micro-aneurysm
Serology: check for hep B (HBsAg, HBsAb, HBeAg)
Tx: glucocorticoids
What are the main treatments for SLE
avoid sun exposure/wear sunscreen
NSAIDs
glucocorticoids (topical/systemic)
Hydroxychloroquine
How are Sulfa antibiotics related to SLE
sulfa causes SLE flare
what is type two antiphospholipid Ab and how do you test for it?
lupus anticoagulant
-risk factor for venous & arterial thrombosis & miscarriage
= prolonged aPTT
test = dilute russel viper venom test (DRVVT) - if pt has Ab - then adding this will corect the timing of aPTT
*Ab should be measured on 2 occasions 12 wks apart*
what is type three antiphospholipid Ab
directed at serum cofactor beta-2glycoprotein I (Beta2GPI)
*Ab should be measured on 2 occasions 12 wks apart*
What are characteristics of localized SSc?
population?
symptoms?
children
discreet areas of discolored skin induration
NO Raynauds & NOT systemic
patches = morphea
coalesced patches = generalized morphea
how do you diagnose and treat IgAV (HSP)
Dx = biopsy w/ IgA deposits
Tx= supportive & glucocorticoids
what diagnostic results will specifically help Dx sjorgens
Polyclonal hypergammaglobulinemia
(+) anti Ro (SSA)- may lead to newborn complete heart block
(+) anti La (SSB) - never present w/o Ro
lip biopsy- lymphoid foci in accessory salivary gland = essential
what serology tests will confirm dcSSc
(+) anti-Scl 70 (aka Anti-DNA topoisomerase I)
& (+) anti-RNA polymerase III
What type of SSc would you add to your DDx if you heard fine crackles (rales)
how would you diagnosis this
=interstitial lung dz
= dcSSc
(also present w/ chronic dry cough, dyspnea)
Dx by pul fxn test (PFT) and lung CT
What is detected in indirect immunoflourescene w/ autoimmune dz present?
What are the limitations
(+) ANA (anti-nuclear Ab)
NOT SPECIFIC
what genetic/environmental factors may contribute to SLE
sex hormone/x-linked
UV light
virus (EBV)
what is a DDx for the attached picture
DLE
Tinea infxn (ring worm)
Psoriasis
Morphea (localized scleroderma)
What are symptoms that present w/ diffuse SSc (dcSSc)
(that dont present in lcSSc)
=systemic
(both) diffuse involvement - include proximal extremities & trunk
early & progressive internal organ involved: interstitial lung dz & renal crisis (may see hemolytic anemia)
what are the multisystems affected by behcet sydrome
MSK: large joint arthalgia
Neuro: mimic MS
GI: ulcers thru-out tract (can occus in distal ileum/cecum)
Pathergy- pustules at site of sterile needle prick
How is polymyositis (PM) different form dermatomyositis (DM)?
what are Dx factors for polymyositis?
NO skin changes (unlike DM)
Dx =
biopsy - endomysial inflam w/ invasion of non-necrotic M. fibers w/o features of other Dx
Labs: elevated creatine kinase
serology: anti Jo-1
if a pt w/ dcSSc comes in w/ abrupt onset of malignant HTN, hemolytic anemia and progressive renal insufficiency, what would your diagnosis be
renal crisis
*this is why you pay attn to BP in these pts, but it is a heralding feature of renal dz)
(typically happens w/i 4 yrs of onset)
What drugs do not work or should be avoided in sjorgens
glucocorticoids - not effective for sicca symptoms
AVOID atropinic drugs and decongestants
no immunomodulatory drug proven efficacious
What are skin changes associated with limited and diffuse SSc
hypo/hyperpigment
dry/itchy = bc glands obliterated by fibrosis
masklike facies, perioral furrowing
microstomia
atrophic skin –> ulcerations
telangiectasias
Ca2+ deposits
Raynauds phenomenon
what are the systemic vasculitides associated w/ variable vessel size
eosinophilc granulomatosis w/ polyangiitis
behcet syndrome
what is the triad of symptoms related to small vessel involvement in behcet syndrome
*recurrent mouth ulcers
genital ulcers
eye inflams
what are characteristics of primary Raynaud
benign
symmetric
exaggerated physiologic response to cold/emotion
Female, 15-30 yo
What are the characteristics of discoid lupus erythematosus (DLE)
commonly presents on the head
= well-defined inflamm plaques –> evolve into atrophic, disfiguring scars
How do you Dx and Tx GCA/TA
DX: elevated ESR (>50 mm)
serlogy: HLA-DR4
temporal A biopsy (gold standard!) - segmental granulomatous vasculitis w/ multinucleated giant cells
Tx: **** Start glucocorticoids BEFORE biopsy! - prevent blindness
what is DDx for pulmonary-renal syndrome
How does Takayasu Arteritis present
(large vessels (aorta & branches)) =chronic/relapsing
“pulseless dz” - obliterate UE peripheral pulse; collaterals = limb loss from ischemia (rare)
50% pul involvement
Renal artery stenosis (see on renal doppler US) –> HTN & Retinopathy
*aortic complications: dilation, regurgitation, aneurysm, rupture
What are the hallmarks of granulomatosis w/ polyangiitis (Wegener’s granulomatosis)
what population does it present?
granulomatous inflammation
necrotizing vasculitis
segmental glomerulonephritis (hematuria, RBCs, proteinuria)
=MALE! >40 yo
(also present w/ venous thrombitic events, hearing loss, orbital masses, ulcerative keratitis/scleritis/episcleritis/conjunctivitis)
