Vasculitis Flashcards

1
Q

Vasculitis
define
general symptoms

A

Vasculitis
- inflammation of the vasculature/blood vessels
- body perceives these vessel cells as invaders, produces antibodies against these protiens: creates damange to the vessel walls
- in addition, increased WBC increase antibodies, causing a clotting cascaude of fibrinogen & decreases the size of the lumen= ischemia

arteries, veins, small, mediaum and large

Symptoms of all vasculitis
- fatigue/malaise
- fever
- weight loss
- myalgia, arthralgia
- night sweats
- specific symptoms depend on the size and location of teh vessel affected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Vasculitis
Classifcations: Large

A

Large Vessel Vasculiti s
- narrowing lumen = decreased blood flow = end organ ischemia or infarction
- PE: bruits or thrills due to increased constricted and force trying to make it through

Types
- giant cell arteritis/ PMR
- takayasu arteritis
- behcet’s syndrome

Symptoms
- limb caaudication
- asymmetric BP
- absent pulses
- aortic dilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Vasculitis: Medium Vessel Classification

A

Medium Vessel Vasculitis
- inflammation of the blood vessel, so the blood gets backed up flowing through it
- this puts pressure on the vessel prior to the localized inflammation = ballooning outwards = ANYEURYSUM

Types
- Polyarteritis nodosa
- Kawasaki disease

Symptoms
- cutaenous nodules
- ulcers
- livedo reticularis
- digital gangrene
- microanyuresums

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Vasculitis: Small Vessel Classification

A

Small Vessel
- the small vessels are damaged: so they will completely break since they’re like one cell thick = blood leakage
- this leak is within organ system = leading to bleed and inflammation of the organ

ANCA Related
- microscopic polyangiitis
- wegeners
- churg-strauss (eosinophliic)

Immune Complex
- Henoch-Schonlein Purpura
- Cryoglobulinemic Vasculitis
- Anti-Cttq vasculitis

Symptoms
- purpura
- lesions
- hives
- glomerulonephrisis
- alvolar hemrrhage
- splinter hemorrhages
- uveitis
- episclerisis/scleritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

secondary causes of vasculitis to occur

A

Infection
- menginococcal infection = inflammation of vessels = burst (purpura) or occulde (ischemia)
- stretococcal infection = erythema nodosum
- viral infection Hep B/C too

Underlying Disease
- cancer
- autoimmune diseases: RA, SLE, IBD

Cold Injury
- frost bite
- cryglobulinemia

Drugs
- antithryoid meds
- hydralazine
- minicycline
- anx.
- anticonvusants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Takayasu Arteritis (pulseless disease or aortic arch syndrome)

Etiology
Symptoms

A

Etiology
- inflammation of the aorta
- rare, femal and young

Symptoms
- systemic: fever, WL, NS, malaise, pain (can be early and go away)
- bruits, absent or reduced pulses
- claudication arms, legs, sping, gut
- ischemic heart disease, HF
- pulmonary HTN
- HA and amaurosis fugux (temp. blind) (NOT stroke)
- BP variability by limbs
- HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Takayasu Arteritis (pulseless disease or aortic arch syndrome)

Diagnosis
Treatment

A

Diagnosis
- imaging angiography: MR or CT

Treatment
surgery or steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Giant Cell Arteritis
etiology
symptoms

A

Etiology
- a disease of the large to medium vessels
- eldery
- systemic pan-arteritis: typically the temporal artery, carotid
- LOTS of pt. on imaging have large vessel inflammation
- realted on spectrum to polymyalgia rheumatica

Symptoms
- temporal HA (severe)
- scalp tenderness (hurts to shower)
- jaw claudication
- throat pain & systemic non-specific symptoms

PE
- can have pulseless, tender, enlarged or noducalr temporal artery

Additional Symptoms
- asymmetric pulses: if aorta involved, regugr, bruits at clavicles
- vision changes!! amerosis fugaux, diplopia, sudden blindness
- confusion, depression, tingling tongue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Giant Cell Arteritis
diagnosis
treatment

A

Diagnosis
- ESR!!!! GET THE ESR!!!!
- confirmatory with temporal artery biopsy

Treatment
- urgernt administration of high dose steroids: prednisone
- IV methylpred. if there is vision loss
- taper after 1 month & use ESR as aguide
- low dose asprin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Polymyalgia Rheumatica (PMR)
etiology
symptoms
diagnosis
treatment

A

Etiology
- same spectrum of diseases as giant cell arteritis
- cytokines in the blood/arteries
- older women!!!

Symptoms
- girdle joint pain and stiffness: shoulders and pelvis bilaterally
- wont be WEAK: but will be sore
- systemic symptoms
- can come before the symptoms of ginat cell arteritis

Diagnosis
- can be clinical
- ESR may be high

Treatment
- low-dose steroids: taper after 1 month to a much much lower dose for 1 year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Behcet’s Syndrome
Etiology
Symptoms

classic
arthritis
eyes
CNS
GI

A

Etiology
- vasculitis in large, medium and small vessels
- assocaited with HLA-b51 gene

Symptoms
- Behcet’s = UCLERS!!
- ulcers in the mough, toungue and cheecks
- gnenital ulcers
- skin lesions: tender erythemadous papular (look like erythemia nodosum) that ulcerate

pathergy: when you needle stick them, they form a pustule at the prick site

Arthritis
- knees and ankles
- no joint erosins: xray looks ok

Eyes
- posterior uveitis (retina)
- anterior uveitis : red eye, photophobia, ** hypophon (WBC!!)**
- treat eye issues with dialtion and steroids

CNS
- sterile meningitis: PL shows lymphcytic pleocytosis

GI
- ulcers in ileum and cecum

Pulmonary
- pulm. artery anyuresum & hemorraage

Hematology
- increase clotting: DVT, PE and clot risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Behchet’s
Diagnosis
Treatment

A

Diangosis
- elevated ESR/CRP
- no ab. to show
- HLA B51
- clincial

Treatment
-for mucoccutaneous = colchicine
- for severe symptoms = corticosteroids: prednisone
- for occular/CNS = infliximab, cyclosporines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Polyarteritis Nodosa (PAN)
- etiology (everywhere but)
- symptoms

A

Etiology
- a medium vessel disease: causing necorsis to the arteries
- impacts everywhere except lungs
- commonly impacts the kidneys
- RISK FACTOR: Hep B (or familial adenosine gene mut.)

Symptoms: depend on what organ is invovled
- general: fever, weigh tlss
- infarction or ischmia to the organ invovled
- kidney: renal artery stenosis, anyureusm or HTN
- brain: stroke
- heart: MI, myocarditis
- live r
- skin: gangrene, purpura, nodules
- PNS: pain in nerves, sensory motor loss
- testes: pain/infarct

Additional Symptoms
- myalgias, neuropathy and arthralgias
- mononueritis multiplex: foot drop
- GI: pain with eating, appendicitis, perfoated bowel, cholecystisi
- Skin: skin ulcers at the malleoli
- gangree, livedo reticularis & nodules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Polyarteritis nodosa (PAN)
diagnosis
treatment

A

Diagnosis
- tissue biopsy at the site of the effected area (skin, nerve, organ)
- angiography: to see the small anyuresums/dilations in teh arteries

Treatment
- high dose steroids: if criticall ill IV methylpred.
- cyclophosphamide

treat the Hep N with antivirals and plasmapharesis and prednisone

survival good if they get treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Buerger’s Disease
etiology
symptoms

A

Buerger’s Disease
Etiolgoy
- vasculitis with inflammation specific to the hands and feet
- thromboangiitis oblierans is the other name
- caused mostly by smoking: triggers autoimmune reaction against healthy tissue
- men, smokes

Symptoms
- pain in hands and feet
- red, enlarged cord veins in hhands
- sores that dont heal
- leg pain, numbeness, tingling, burining
- gangrene if untreated
- usuall 2+ limbs affected
- can see decreased pulses
- worsened sx. with stress or cold expsoure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Buerger’s Disease
Diagnosis
Treatment

A

Diagnosis
- rule out raymauds!
- angiogram or arteriogram of UE and LE
- doppler US
- skin bx. can be done but they dont heal well so not often done

Treatment
- STOP SMOKING
- no cure: symptomatic
- massage to increased warmth, avoid cold

17
Q

Henoch-Schonlein Purpura (HSP)
most commony HYPERSENSITIVTY svasculitis

Etiology
Symptoms

A

Etiology
- hypersensitivity: a leukocytoclasic vasculitis with IgA deposition
- very common in kids

Symptoms

Arthritis
- knees and ankles

Palpabale Purpura
- lower extremities (butt, legs, feet)

Hematuria
- renal invovlement

Abd. Pain
- in kids this is common, vasculitis of teh abd. tract and GI bleeding can occur

18
Q

Henoch-Schonlein Purpura (HSP)
symptoms as they differ by adults to kids

A

Kids
- large joint arthritis: knees, ankles
- edema
- purpura on the limbs
- abd. pain
- not the kidneys

Adults
- small joint arthritis: wrists, MCP, PIP, MTP
- no edema or GI invovlement often
- purpura on limbs and torso
- kidney involvement
- often familail

19
Q

Henoch-Schonlein Purpura (HSP)
Diagnosisi
Treatement

A

Diagnosis
- kidney Biopsy: see segmental glomerulonephritis with creseant and mesangial deposition of IgA in the BM

Treatment
- adults: this will porsb become CKD and have chronic skin disease
- adults = steroids: prednisone, azathioprine, mycophenolate

Kids: steroids not helpful, symptomatic

20
Q

Cryoglobulinemia
etiology
symptoms

A

Etiology
- cold antibodies in the blood
- immune complex mediated: small vessel vasculitis (hemorrahges)
- most commonly assocaited with Hep C
- RH + IgG = complex = vasculitis

Symptoms
- palpabale purpura that reoccur
- periphearl neuropathy
- proliferative glomeularnephritis
- digital gangere
- abd. pain

meltz triad: purpura, arthraliga and weakness

21
Q

Cryoglobulinemia
diagnosis (TESTS!!!)

A

Diagnosis
- abnormal LFTs
- + serum cryoglobulins
- + RF
- low C4

- clincial dx. based on situation

Treatment
- antivirals for Hep C
- if severe: steroids + cyclophosphamide OR rituximab

22
Q

Wegener Granulmatosis
(granulomatosis with polyangiitis)
etiology

A

Etiology
- C-ANCA: anti-neutrophil cytoplasmic antibody disease
vasculitis of small arteries, arterioles and capillaries
- necrotizing granulomatous: getting lesions of the upper and lower respiratory tracts
- glomerulonephritis

Symptoms
- URI symptoms: sinusitis, OM, etc. : can have ENT deformities like saddle nose from perforated nasal septum
- renal: proteinuria and hematuria
- LRI symptoms: cough, dyspnea, hemoptysis

Additional Symptoms
- migratory arthritis of large joints
- eye: ulilater ptosis, scleritis, uveritis, keratitis
- purpura/skin lesions
- neuropathy/parasthesias
- systemic fever, WL and fatigue
- increased VTE risk

23
Q

Wegener Granulmatosis
(granulomatosis with polyangiitis)

Diagnosis

A

Diangosis
- CBC: anemia, leukocytosis, thrombocytosis
- elevated CRP/ESR
- Urine: proteinuria & RBC with RBC casts and mayeb WBC
- C-ANCA autoantibodies : most will have these
-

CT chest: granulomatous disease
- masses, cavities etc. = plueral effusions and hilar adenopathy

Tissu Bx.
lung biopsy is preferred to find granulomaous inflammation

Kideny Bx.
- segemental necrotizing cresents

can get nasal biopsy

24
Q

Wegener Granulmatosis
(granulomatosis with polyangiitis)

treatment

A

Treatment

induction therapy
methotrexate = mild

cyclophosphamide + steroids OR rituximab + steroids = severe

Maitenance
- methotrexate (not in renal failure)
- rituximab
- azathioprine

end up on dialysis

25
Q

Microscopic Polyangitis
etiology
symptoms

A

Microscopic Polyangitis
Etiolgoy
- P-ANCA
- Pauci-immune nongranulomatous necotrtizing vasculitis
- affects small vessels:capillaries
- glomerulonephritis and pulmonary capillaritis
- rarely = med induced

Symptoms
-purpura
cutaneous vasculitis:ulcers, splinter hemorrhages, vasciulobullous lesions
interstitail lung fiberosis: pulmonary hemorrhage = hempotysis
- mononeuritis multiplex

26
Q

Microscopic Polyangitis
Diagnosis
Treatment

A

Diagnosis
- GET A P-ANCA
- Esr/CRp
- RBC casts, proteinuria and hemoturia
- Renal biopsy: to see necotritizng

REMEBER THESE HAVE NO GRANULMOA!! just ruputres capillaires leading to bleeding in skin and kidney and lungs

Treatment
induction

methotrexate = mild
cyclophosphamide, rituximab = severe

maitence
- methotrexate, azathioprine, rituximab

27
Q

Churg-Strauss Immune Complex
etiology
symptoms
diagnosis
treatemnt

A

Etiology
- eosinophilic granulomatous with polyangitis

SYmptoms
- think asthma: common in these pt.
- eosinophilia rich granulomas in blood
- in lungs!!!
- peripheral neruopathy

Diagnosis
- blood eosinophila level
- CXR or CT lungs
- Biopsy of lungs!!!

Treatment
- palliative
- steroids or immunosup.

28
Q

Raynaud’s Phenomenon
etiology
stages/Symptoms
risk factors

A

Etiology
- primary: unknown just response of the arterioles to colr or stress
- secondary: undelying rheumatic disease (scleroderma MC)

Phases
- well-demarkated excessive vasoconstriction: fingers turn white the blue (spares thumb)
- excessive voasdilation = fingers turn red and burn and thorbbing pain

can be symmetric (unilateral more if its secondary)
if see nail fold chagnes: think scleroderma related

Risks
- smoking
- chemical exposure
- autoimmune conditions
- injury/trauma
- meds

29
Q

Raynauds Phenomenon
diagnosis
treatement

A

Diangosis
- ab. to rule out secondary causes
- look at capillaries on nail bed to see if its secondary!!

Treatement
- lifestyle modifications
- mittnes, lotions, avoid vasoconstrictions (decongestants),smoking
- etc.

Medication Treatemnet = nifedipine CCB
can do surgery sympathectomy (cerival or digital)