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 treatment for APS?
Anticoagulation - continued indefinitely
(direct oral anticoag = DOAC)
(low MW heparin - Vit K antagonist)
what vessels/organs does the eosinophilic granulomatosis w/ polyangiitis (EGPA) (aka Churg-Strauss syndrome) affect?
small/medium vessels
respiratory tract & other organs
How do you dx and tx EGPA (churg strauss)
Dx: CBC w/ diff- look for high eosinophils
Serology: ANCA (+) typically MPO-ANCA (pANCA)
Imaging: CXR or lung CT
Lung biopsy: granulomas & vascular changes eosinophils in tissue
Tx: glucocorticoids & NO smoking!
How does IgAV (HSP) present?
more common in kids
tetrad
- palpable purpura: no thrombocytopenia!!
- arthritis/arthralgia
- abd pain
- renal dz: glomerulonephritis
what are the 6 types of cardiac events that 50% of SSc pts will present with
myocardial fibrosis
cardiomyopathy
pericarditis
myocarditis
pericardial effusion
arrhythmia
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 population readily presents w/ inflammatory myopathies
Female
African American
what are the serology results for SLE
(+) ANA - nonspecific
(+) anti-ds DNA - correlate w/ dz activity (use to help monitor pt thru treatment)
(+) Sm- does NOT correlate w/ dz activity
decreased C3 or C4 - increased consumption; suggest dz activity
What may cause mortality in the early years after diagnosis of SLE
increased infection (esp opportunistic organisms)
active SLE mainly due to kidney/CNS dz
besides CREST syndrome, what else is may present in limited (IcSSc)
how does this affect prognosis?
pulmonary HTN (SOB), digital ischemia
still has good prognosis!
what serology results will present w/ lupus-like syndrome/drug-induced
(+) ANA
(+) Anti-histone Ab (95%)
how do you Dx IcSSc
PE
serology (+) Anti-centromere
EGD/barium swallow (for esophageal probs)
echo/right heart cath (for Pul HTN)
why can IcSSc be misdiagnosed as SLE
IcSSC is indolent ( delayed onset & slow progression)
*typically Dx in advanced dz- pt may present w/ arthralgia & (+) ANA
how does SSc affect the MSK system
carpel tunnel syndrome
tendon friction rub
fibrosis & adhesion of tendon sheaths
What are the characteristics of discoid lupus erythematosus (DLE)
commonly presents on the head
= well-defined inflamm plaques –> evolve into atrophic, disfiguring scars
What population does scleroderma (SSc) present in most
30-60 yo
female
how do you dx and tx takayasu arteritis
Dx: MRI or CT angiograph (long smooth tapered stenosis of aorta)
Biopsy: granuloma w/ some giant cells, fibrosis in chronic stage
Tx: glucocorticoids
what aspect of SSc will cause an increase risk in esophageal adenoCA
barrett esophagus
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
how is raynauds episodic
Pallor (vasoconstiction) –> cyanosis (ischemia) –> erythema (reperfusion)
*thumb rarely affected
what population does Sjogrens present in
Female
mid 50s (postmenopausal)
Thyroid fibrosis from SSc can cause…
hypothyroid
what are the measures taken to manage/prevent SLE
minimize conventional risk factors for atherosclerosis (hypercholesterolemia, HTN, obesity, inactivity)
avoid smoking
flu vaccine every yr
pneumococcal vaccination ever 5 yrs
preventive cancer screening - bc increased risk for malignancy
Which antiphospholipid Ab will cause a false (+) for syphilis and why?
type 1
bc anti-cardiolipin Abs (non-treponemal tests - RPR & VRDL)
*Ab should be measured on 2 occasions 12 wks apart*
what are hallmarks of SSc
thickening & hardening of skin
microangiopathy & fibrosis of skin and viseral organs
obliteration of eccrine sweat glands and sebaceous glands –> dry itchy skin
what are symptoms that present w/ dcSSc
soft tissue swelling, erythema, pruritus
fatigue, stiffness, malaise
arthralgia, M. weakness, carpal tunnel
Raynaud (but later than IcSSc)
how do you diagnose DM
biopsy: perimysial/perivascular inflam ; perifascicular atrophy
increase CK & aldolase
(+) Anti-Jo1
Which type of SSc has the worst prognosis
dcSSc
What is the purpose of treatment for SSc?
What are some examples
=supportive only - improve quality of life (no therapy available to alfter dz course)
-educate, Ca2+ channel blocker, ACE inhibitor, Anti-reflux, cyclophospamide, phophodiesterase type 5 inhibitor
what are the phases of dcSSc?
inflam edematous phase –> fibrotic phase
skin induration, hypo/hyperpigment –> loss of body hair/impaired sweating
fibrotic joints –> stiffness
how do you Dx and Tx Behcet syndrome
Dx: Hx/PE & Serology: HLA-B51
Tx: low dose glucocorticoids
what are the medium vessel systemic vasculitides
thromboangiitis obliterans (aka beurger dz)
polyarteritis nodosa
kawasaki dz
Sjrogen’s has a strong association w….
(malignancy)
B cell NHL (MALToma)
What population & vessels does polyarteritis nodosa affect the most?
M ; ppl w/ HBV
(bc associated w/ HBV)
medium vessels/segmental
how do you Dx/Tx Kawasaki Dz
Dx: Clinical
Tx:IVIG w/i 10 days of sxs
high dose ASA (aspirin in ped pt, whaaaat?!)
How do you Dx and Tx granulomatosis w/ polyangiitis (wegener’s)
Dx: serology = (+) ANCA (C-ANCA aka PR3-ANCA)
biopsy= vessel changes w/ granulomas
Tx: NO smoking
cylcophosphamide, high dose glucocorticoid, rituximab, methotrexate (if renal fxn normal)
in general, what population is more susceptible to autoimmune dz’s
female
minority populations (rhematologic dz)
at what ratio is ANA normal/neg?
when is it (+)/clincally significant
< 1:40 = normal/neg
higher the ratio more clinically significant (+) = 1:160
what are the small vessel systemic vasculitides
- IgAV (aka henoch-schonlein purupura)
- Anti-GMB (aka goodpasture)
- Granulmatosis w/ polyangiitis (wegener)
what are sicca symptoms
=due to destruction from inflam response
- dry eyes
- dry mouth: increased incidence of oral infxn (candida) & dental caries
- vaginal dryness
- tracheo-bronchial dryness
- parotid & other major salivory glands enlarged
how do you Dx and Tx thrombangiitis Obliterans (Buerger Dz)
Dx: Angiograph- “corkscrew” appearance
Tx: STOP SMOKING!
what are the hallmarks of EGPA (Churg-Strauss Syndrome)
asthma + eosinophilia** –> vasculitis w/ **granulomas
how do you manage/treat raynauds
wear gloves, stay warm (coat, hat, etc)
lotion
stop smoking
limit/stop symapthomimetic drugs
Ca2+ channel blocers
secondary raynaud: treat underlying cause; surgery
What do you add to your DDx if serum creatine kinase and aldolase are elevated but ESR and CRP are normal…
what other diagnostic findings could you look for?
inflammatory myopathies
EMG & M. biopsy - characteristic findings
typical dermal features (Dermatomyositis - pt 7-15 or 30-60 yo)
what serology results will be present in mothers’ w/ neonatal lupus affected child
how can this be prevented
Anti-Ro (SSA) Ab or LA (SSB)
(possible in sjogren pts too)
-perform sonogram of fetus 1-2 wks starting 16 wk gestation if mom is (+) Ro-Ab
how do you Dx DLE
& how do you treat it
Dx = clinical exam & biopsy (histology)
Tx = photoprotection + topical anti-inflam/systemic antimalarial drugs *early treatment to prevent scarring
what is DDx for pulmonary-renal syndrome
what are the symptoms that present with polyarteritis nodosa
Skin: livedo reticularis, SubQ nodules, ulcers, digital gangrene
Peripheral N.: vasculitis neuropathy, monomeuritis multiplex (foot drop)
Renal: renin mediated HTN, renal infarct
Cardiac: newly acquired HTN-secondary to renal cause, CHF, MI
constituitional: fever, mailase, Wt loss
*****LUNGS are SPARED*****
what are characterisitics of secondary raynaud
secondary to something!
occurs in CTD, hematologic and endocrine conditions, use of beta-blockers or CA drugs (cisplatin and bleomycin)
>30 y/o
UNILATERAL
More severe –> ischemia
what population is SLE prevalent in
Female
African American & Hispanic
(presents heterogenous - vary pt to pt)
what malignancies are pts w/ SLE more susceptible to?
lymphoma,
lung CA
cervical CA
what is DLE
a varient of cutaneous lupus
independent or manifestation of SLE
what population is Behcet syndrome most commonly found?
and what vessels does it affect
Turkey, Asia, Mid East
variable vessel size:
=large vessels –> aneurysms (venous involvement –> DVT)
=small vessels
How can be GI system be affected by SSc
malnutrion (fat, protein, B12, D def)
xerostomia
esophagus - GERD, dysphagia, barrett’s esophagus
gastropareisis
gastric antral vascular ectasia (GAVE syndrome)
chronic diarrhea bc bacterial overgrowth
pseudo-obstruction
primary biliary cirrhosis/cholangitis (anti-mito ab)
what are the phases of EGPA (Churg-strauss syndrome) & their respective symptoms
- prodrome phase: allergic dz - months-yrs
- Eosinophilia-tissue infiltration phase: eosinophilia & tissue infiltrations (extravascular) - lung/GI/other)
- vasculitis phase: systemic necrotizing heart, lung, nerves, skin & Palpable purpura
what population does Takayaxu Arteritis most likely occur
< 40 yo
Female (teen/young women)
Asia
where and how does GCA (aka TA) present
large vessels - cranial A & Aortic arch
=HA, Jaw claudication (pain/fatigue w/ chewing), visual abnormalities (amaurosis fugax or diplopia)
associated w/ PMR
what are treatments for DM/PM
glucocorticoids!
methotrexate, azathiprine, cyclophosphamide, cyclosporine, IVIG, mycopenolate mofetil, rituximab, hydroxychloroquine
how do you diagnose raynaud?
Nailfold capillaroscopy
normal in primary
secondary = distorted w/ widened and irregular loops, dilated lumen & areas of vascular dropout
how does neonatal lupus present
rashes, thrombocytopenia, hemolytic anemia and arthritis
Permanent complete heart block- found during utero, birth or neonate
what is the difference btn primary and secondary Anti-Phospholipid Ab Syndrome (APS)
primary - pts w/o SLE
secondary = 1/3 pt w/ SLE
How does pericarditis present
what makes it better/worse
substernal, constant, crushing or sharp chest pain - worse w/ cough/deep inspiration (pleuritic)
worse when supine, better w/ sitting upright/leaning forward
hear pericardial friction rub
diffuse ST elevation on ECG
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 is similar about
Giant cell arteritis (GCA) (aka temporal arteritis (TA))
&
Polymyalgia rheumatica (PMR)
freq co-exist
F, > 40-50 yo, Caucasian
constitutional symp w/ normal WBC
elevated ESR and CRP
how do you diagnose and treat inclusion body myositis
dx: endomysial inflam, rimmed vacuoles, invasion of non-necrotic M. fibers
labs = mild elevation/normal creatine kinase
Serology= anti-cN1A autoAb
Tx = refractory to treatment, supportive
what are the large vessel systemic vasculitides
takayazu arteritis
giant cell arteritis (aka temporal arteritis)
how does the presentation of SSc vary in Caucasian pts vs African American pts
difference in how multisystems are affected
Caucasian - present with Secondary Raynauds phenomenon first
African American: present w/ skin hypo/hyperpigmentation first
How do you Dx and Tx Polymyalgia Rhematica
everything is normal EXCEPT elevated ESR and CRP
Tx: glucocorticoids
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)
who is most susceptible to Thrombangiitis Obliterans (aka Buerger Dz)
what are the associated symptoms
*ONLY occurs in smokes (often =M (<35 yo))
dilated medium vessels –> proximal
thrombosis –> loss of digits –> hand/feet (internal organ dx = rare)
how do you diagnose and treat Anti-GBM (goodpasture syndrome)
Dx: biopsy renal : depostion of Anti-basement membrane Ab
UA (protein/blood)
Tx: plasmapheresis - remove Abs; glucocorticoids, cyclophosphamide (dialysis sometimes)
