4. CT disorders & Vasculitides Flashcards
(120 cards)
1
Q
what is a characteristic sign for dermatomyositis
& what are the rashes that may present
A
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”
2
Q
how is the respiratory tract involved in granulomatosis w/ polyangiitis (aka wegener’s granulomatosis)
A
90% nasal involvement –> saddle nose/crusting/bleeding/obstruction
erosive sinus dz
CXR - inflitrate, nodules (Asx nodule –> alveolar hemorrhage) and cavitary lesions
3
Q
what labs should be considered for SLE
A
Antibodies to dsDNA, Smith (Sm), Ro/SS-A, La/SS-B, histones
urinalysis
CBC, ESR, ANA, complement levels, antiphospholipid Ab
4
Q
what are symptoms that accompany kawasaki dz
will this cause morbidity?
A
fever, LAD, Rash, “strawberry tongue”
morbidity from coronary** involvement (**anyeurysm or MI)–> could be yrs later!
5
Q
When treating SLE pts w/ corticosteriods, you have to monitor….
A
monitor for avascular necrosis of bone (hips & knees)
monitor for osteoporosis
6
Q
Does lupus-like syndrome/drug-induced cause renal/neurological symptoms?
A
NO!
7
Q
what serology test will confirm lcSSc
A
(+) anti-centromere
8
Q
When DM is diagnosed, start looking for…
A
occult malignancy (CBC, CMP, UA, age appropriate screening)
look for: NHL, cervical, breast, lung, pancreatic, stomach, colorectal, prostate, ovarian, & multple myeloma
9
Q
what inflammatory response is responsible for SLE
A
type III hypersensitivity
10
Q
what population is kawasaki dz (mucocutaeous LN syndrome) most common
A
<5 yo
Male
highest incidence: Japan
11
Q
DDx for Proximal M. weakness……….
A
12
Q
where does anti-GBM (goodpasture syndrome) occur
A
glomerular capillaries
pul capillaries (–> pul hemorrhage –> death if not treated)
13
Q
what are common features of SLE
A
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
14
Q
How does polymyalgia rheumatica present?
A
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”
15
Q
what does the Schirmer test check for?
A
keratoconjunctivitis sicca - if foreign body sensation produced tears
16
Q
what population does inclusion body myositis (IBM) present most
& how does it present?
A
>40-50 yo
MALE!
CAUCASIAN!
-weakness: finger flexion or quad weakness
17
Q
what is the gold standard for diagnosing PAH w/ IcSSc
A
Righ heart cath = elevated pul A pressure
(but can also use 2D echo)
18
Q
what is the cause of mortality in SLE years later after Dx
A
accelerated atherosclerosis - bc chronic inflam –> become cause of death
5x greater chance of MI compared to general pop
thromboembolic events - freq cause of mortality
19
Q
Why shouldn’t you give glucocorticoids to dcSSc pts
A
bc it can induce renal crisis
20
Q
DDx for attached figure…
A
= cotton wool spots
SLE/APS
HTN, Diabetes
ischemia, infxn, embolic idiopathic
*and more!
21
Q
what are treatments for sjorgens
A
symptomatic =
Regular eye Dr/dentist visits
Dry eyes - artificial tears, lubricating ointments, etc
Xerostomia- freq sip of water; sugarless candy
Arthalgia: hydroxychloroquine
22
Q
Which autoimmune/vasculitides disorders are multisystem
A
SLE
Scleroderma
Behcet Syndrome
23
Q
which type of SSc has an increased incidence of bronchoalveolar carcinoma
A
dcSSc & lcSSc
24
Q
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?
A
= inflammatory myopathies
symmetrical (bilateral) proximal Ms - cant stand or climb
myalgia, weakness, typical dermal features (DM)
(look for CK and aldolase to be elevated)
25
DDx for thrombosis..
APS
Protein C or S def
Anti-thrombin def
Factor V Leiden def
HIT
Sepsis
Systemic vasculitis
DIC
TTP
26
what is libman-sacks endocarditis (SLE)
immune deposits collecting on valves
non-infectious
27
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"
28
Which type of SSc presents w/ CREST syndrome?
what does CREST stand for?
limited (IcSSc)
=Calcinosis cutis
=Raynaud's
=Esophageal dysmotility
=Sclerodactyly
=Telangiectasia
29
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
30
What are the main treatments for SLE
avoid sun exposure/wear sunscreen
NSAIDs
glucocorticoids (topical/systemic)
Hydroxychloroquine
31
How are Sulfa antibiotics related to SLE
sulfa causes SLE flare
32
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\*
33
what is type three antiphospholipid Ab
directed at serum cofactor beta-2glycoprotein I (**Beta2GPI)**
\*Ab should be measured on 2 occasions 12 wks apart\*
34
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
35
how do you diagnose and treat IgAV (HSP)
Dx = biopsy w/ IgA deposits
Tx= supportive & glucocorticoids
36
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_
37
what serology tests will confirm dcSSc
(+) anti-Scl 70 (aka Anti-DNA topoisomerase I)
& (+) anti-RNA polymerase III
38
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
39
What is detected in indirect immunoflourescene w/ autoimmune dz present?
What are the limitations
(+) ANA (anti-nuclear Ab)
NOT SPECIFIC
40
what genetic/environmental factors may contribute to SLE
sex hormone/x-linked
UV light
virus (EBV)
41
what is a DDx for the attached picture
DLE
Tinea infxn (ring worm)
Psoriasis
Morphea (localized scleroderma)
42
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)
43
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**
44
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**
45
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)
46
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_
47
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
48
what are the systemic vasculitides associated w/ variable vessel size
eosinophilc granulomatosis w/ polyangiitis
behcet syndrome
49
what is the treatment for APS?
Anticoagulation - continued indefinitely
(direct oral anticoag = DOAC)
(low MW heparin - Vit K antagonist)
50
what vessels/organs does the eosinophilic granulomatosis w/ polyangiitis (EGPA) (aka Churg-Strauss syndrome) affect?
_small/medium vessels_
**respiratory tract** & other organs
51
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!
52
How does IgAV (HSP) present?
more common in **kids**
tetrad
1. **_palpable purpura:_** _no thrombocytopenia!!_
2. arthritis/arthralgia
3. abd pain
4. renal dz: glomerulonephritis
53
what are the 6 types of cardiac events that 50% of SSc pts will present with
myocardial fibrosis
cardiomyopathy
pericarditis
myocarditis
pericardial effusion
arrhythmia
54
what is the triad of symptoms related to small vessel involvement in behcet syndrome
**_\*recurrent mouth ulcers_**
**genital ulcers**
**eye inflams**
55
what are characteristics of primary Raynaud
_benign_
_symmetric_
**exaggerated physiologic response to cold/emotion**
Female, 15-30 yo
56
What population readily presents w/ inflammatory myopathies
Female
African American
57
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
58
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
59
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!
60
what serology results will present w/ lupus-like syndrome/drug-induced
(+) ANA
**(+) Anti-histone Ab (95%)**
61
how do you Dx IcSSc
PE
serology **(+) Anti-centromere**
EGD/barium swallow (for esophageal probs)
echo/right heart cath (for Pul HTN)
62
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**
63
how does SSc affect the MSK system
**carpel tunnel syndrome**
tendon friction rub
fibrosis & adhesion of tendon sheaths
64
What are the characteristics of discoid lupus erythematosus (DLE)
commonly presents on the head
= **well-defined inflamm plaques --\> evolve into atrophic, disfiguring scars**
65
What population does scleroderma (SSc) present in most
30-60 yo
female
66
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
67
what aspect of SSc will cause an increase risk in esophageal adenoCA
barrett esophagus
68
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**_
69
how is raynauds episodic
Pallor (vasoconstiction) --\> cyanosis (ischemia) --\> erythema (reperfusion)
\*thumb rarely affected
70
what population does Sjogrens present in
Female
mid 50s (postmenopausal)
71
Thyroid fibrosis from SSc can cause...
hypothyroid
72
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
73
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\*
74
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_
75
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)
76
how do you diagnose DM
biopsy: perimysial/perivascular inflam **; perifascicular atrophy**
## Footnote
**increase CK & aldolase**
**(+) Anti-Jo1**
77
Which type of SSc has the worst prognosis
dcSSc
78
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
79
what are the phases of dcSSc?
inflam edematous phase --\> fibrotic phase
skin induration, hypo/hyperpigment --\> loss of body hair/impaired sweating
fibrotic joints --\> stiffness
80
how do you Dx and Tx Behcet syndrome
Dx: Hx/PE & **Serology: HLA-B51**
Tx: low dose glucocorticoids
81
what are the medium vessel systemic vasculitides
thromboangiitis obliterans (aka beurger dz)
polyarteritis nodosa
kawasaki dz
82
Sjrogen's has a strong association w....
(malignancy)
B cell NHL (MALToma)
83
What population & vessels does polyarteritis nodosa affect the most?
**M** ; ppl w/ **HBV**
(bc associated w/ HBV)
**medium vessels/segmental**
84
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?!)
85
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)
86
in general, what population is more susceptible to autoimmune dz's
female
minority populations (rhematologic dz)
87
at what ratio is ANA normal/neg?
when is it (+)/clincally significant
\< 1:40 = normal/neg
higher the ratio more clinically significant (+) = 1:160
88
what are the small vessel systemic vasculitides
1. IgAV (aka henoch-schonlein purupura)
2. Anti-GMB (aka goodpasture)
3. Granulmatosis w/ polyangiitis (wegener)
89
what are sicca symptoms
=due to destruction from inflam response
1. dry eyes
2. dry mouth: **increased incidence of oral infxn (candida) & dental caries**
3. vaginal dryness
4. tracheo-bronchial dryness
5. **_parotid_** & other major salivory glands enlarged
90
how do you Dx and Tx thrombangiitis Obliterans (Buerger Dz)
Dx: _Angiograph- **"corkscrew" appearance**_
Tx: **_STOP SMOKING!_**
91
what are the hallmarks of EGPA (Churg-Strauss Syndrome)
**_asthma + eosinophilia**_ --\> vasculitis w/ _**granulomas_**
92
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
93
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)
94
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
95
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
96
what is DDx for pulmonary-renal syndrome
97
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\*\*\*\*\*
98
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_**
99
what population is SLE prevalent in
Female
African American & Hispanic
(presents heterogenous - vary pt to pt)
100
what malignancies are pts w/ SLE more susceptible to?
lymphoma,
lung CA
cervical CA
101
what is DLE
a varient of **cutaneous lupus**
**independent or manifestation of SLE**
102
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**
103
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)**
104
what are the phases of EGPA (Churg-strauss syndrome) & their respective symptoms
1. _prodrome phase_: allergic dz - months-yrs
2. _Eosinophilia-tissue infiltration phase:_ **eosinophilia** & tissue infiltrations **(extravascular**) - lung/GI/other)
3. _vasculitis phase:_ **systemic necrotizing** heart, lung, nerves, skin **& Palpable purpura**
105
what population does Takayaxu Arteritis most likely occur
\< 40 yo
Female (teen/young women)
Asia
106
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
107
what are treatments for DM/PM
**_glucocorticoids!_**
methotrexate, azathiprine, cyclophosphamide, cyclosporine, IVIG, mycopenolate mofetil, rituximab, hydroxychloroquine
108
how do you diagnose raynaud?
**_Nailfold capillaroscopy_**
normal in primary
secondary = **distorted w/ widened and irregular loops, dilated lumen & areas of vascular dropout**
109
how does neonatal lupus present
rashes, thrombocytopenia, hemolytic anemia and arthritis
**_Permanent complete heart block_**- found during utero, birth or neonate
110
what is the difference btn primary and secondary Anti-Phospholipid Ab Syndrome (APS)
primary - pts w/o SLE
secondary = 1/3 pt w/ SLE
111
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
112
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_**
113
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**
114
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_**
115
what are the large vessel systemic vasculitides
takayazu arteritis
giant cell arteritis (aka temporal arteritis)
116
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
117
How do you Dx and Tx Polymyalgia Rhematica
everything is normal EXCEPT **_elevated ESR and CRP_**
Tx: glucocorticoids
118
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)
119
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)
120
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)
