Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is Vasculitis

A

Collection of autoimmune diseases that cause inflammation of the blood vessels
Inflammation leads to damage (Impaired flow, vessel leakage, hemorrhage, orang damage, death)

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

Vasculitis General findings

A

Fever, malaise/fatigue, weight loss
Labs: leukocytosis, thrombocytosis, decreased albumin, anemia, elevation of CRP or ESR
(Small Vessel) kidney involvement

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

Small Vessel Casculitis Sign and Symptoms

A 
Scleritis
Palpable purpura
Glomerulonephritis
Inflammatory arthritis
Diffuse hemorrhage
Mononeuritis multiplex CNS (nerve blood supply issues)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Small Vessel Vasculitis ANCA (anti neutrophilic cytoplasmic antibody) associated
C-ANCA (cytoplastic staining), Serine protease 3 (PR3)
P-ANCA (perinuclear staining), Myeloperoxidase (MPO)
Atypical ANCA - neither c/p staining

What are the associated diseases?

A

Granulomatosis with polyangiitis (GPA, aka Wegener’s Granulomatosis)
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA aka Churg-Strauss)

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

Granulomatosis with polyangiitis signs and symptoms

A

C-ANCA/PR3 > -PANCA/MPO
ENT & Raspatory disease common - otitis, tracheal stenosis
Eyes - 30-60% orbital inflammation, episcleritis, scleritis, uveitis, lacrimal duct obstruction, retinal artery thrombosis.
Renal disease (esp. w/ pregnancy)
Nervous system - mononeuritis complex (foot drop. facial paralysis), pachymeninges, CN neuropathies, pituitary/hypophysis
Skin - purpura, Ulcers
MSK - arthralgia, myalgia, inflammatory arthritis
Saddle nose

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

Microscopic Polyangiitis (MPA)

A

Silent Kidney punch
P-ANCA > C-ANCA/PR3
All have rapidly progressive Glomerulonephritis
similar to GPA - except much less ENT involvement
Diffuse alveolar hemorrhage, no nodular/granulomatous lung disease
GI tract may be involved

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

Eosinophilic granulomatosis with polyangiitis (EGPA)

A

Like GPA + asthma/eosinophils, more and allergic “flavor”
Lungs with nodular lesions
Adult-onset asthma - severe, steroid dependent
mononeuritis multiplex
much less renal disease
peripheral eosinophilia, melts away with steroids

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

How to diagnose Small Vessel Vasculitis

A

Clinical syndrome
Labs: evidence of inflammation, renal disease, positive ANCAs - EGPA peripheral eosinophilia
Biopsy: renal biopsy showing immune GN, skin, lung, sinuses, sural nerve
Imaging: CXR, CT of lungs maybe sinuses

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

Small Vessel Vasculitis (ANCA+)treatments

A

Induction: IV methylprednisolone (1 G daily for 3 days -> 1mg/kg PA), RITUXIMAB, (for severe), Cyclophosphamide
Maintenance: RITUXIMAB&raquo_space; others: Azathioprine, Methotrexate, Mycophenolate

EGPA: above plus anti-IL-5 (Mepolizumab)

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

Henoch Schoenlein Purpura Symptoms

A 
Most common children 3-10 
M>F 2:1
Upper respiratory infection
Immune complexes again IgA deposit in blood vessels
Purpuric rash
Abdominal pain/blood, diarrhea
inflammatory arthritis/arthralgias
Glomerulonephritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Henoch Schoenlein Purpura diagnosis

A

Clinical symptoms, biopsy of rash or kidney: Immune-florescence would show IgA deposition
Elevation of blood IgA in 50%
Resolves without treatment in 94% of kids, 84% of adults

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

Henoch Schoenlein Purpura treatment

A

Symptoms: NSAIDs, Acetaminophen, oral steroids
Rash: treat with dapsone
Severe kidney disease: IVIG, Cyclophosphamide, Azathioprine

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

Anti-GBM disease aka Goodpasture’s disease

A

Antibodies directed against glomerular basement membrane
Typically presents with glomerulonephritis and diffuse alveolar hemorrhage = pulmonary renal syndrome
more common in Caucasians, also some specific ethnic groups
Age 20-30 or 60-70
may be a result of environment and genetics (cocaine, infections, smoking, metal dust)

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

Anti-GBM disease diagnostics

A

Blood anti-GBM antibodies
chest imaging and potentially bronchoalveolar lavage
renal biopsy - showing linear IgG staining pattern on immunofluorescence

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

anti_GBM treatment

A

plasma exchange
high dose steroids
Cyclophosphamide

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

Other diseases associated with small vessel vasculitis

A

RA
SLE
Sjogren’s
Treatment: steroids, rituximab and/or Cyclophosphamide

17
Q

Medium Vessel Vasculitis Signs and Symptoms

A 
Livedo rash
Ulcerative skin lesions
Mononerutis multiplex
Coronary involvement
GI and renal involvement
18
Q

Polyarteritis Nodosa

A

(Med Vessel)
Can be skin limited - livedo rash, ulcerativ skin lesions, nodular lesions, purpura - deep tissue biopsy to see med size vessels

OR

systemic:
Oset 40-60 - skin +
fever, weight loss, night sweats, fatigue
peripheral nerves
renal infarcts
mylagias, arthralgias
Abdominal pain
(least common) Stroke, seizure, heart failure, MI, retinal hemorrhage, potic ischemia, orchitits/testicular pain
19
Q

Poly arteritis Nodosa diagnosis

A

Labs: elevated ESR/CRP
CBC: anemia, thrombocytosis, leukocytosis
CMP elevated creatinine, LFT abnormalities, low albumin

May be associated with Hep B

20
Q

Poly nodosa diagnosis

A

Differential: segmental arterial mediolysis, fibromuscular dysplasia, Ehlers-Danlos, pseudoxanthoma elasticum

Dx on presentation, angiogram or CT radiogram showing typical vasculitis changes in vessels. Biopsy often shows fibrinoid necrosis and inflammatory infiltration of vessel wall

21
Q

Polyarteritis Nodosa treatment

A

Severe: pulse steroids, Pred 1mg/kg. Cyclophosphamide for induction, azathioprine, or methotrexate form maintenance
Mild-moderate: Prednisone tapered for 6-12 months, Methotrexate or Azathioprine
HepB associated: Pred 60mg/day for a week, taper 50% day till off, Plasma exchange, HepB antiviral treatment

22
Q

Kawasaki disease demographics

A

Medium Vessel Vasculitis
Peak incidence 9-12 months, 80% <5 yo
Male>Female 2:1 (Japaneses 17x)
Prolonged, high fevers with conjunctivitis, desquamative rash, strawberry tongue
Can cause aneurysm/pseudoaneurysm affecting the Heart

23
Q

Kawasaki Disease Key symptoms

A

Conjunctivitis
Rashed with cracked and red lips
Strawberry tongu
Desquamation (skin peeling)

24
Q

Kawasaki disease complete diagnosis

A

Polymorphous rash, oropharyngreal mucous membrane change, conjunctival injection, extremity changes, lymphadenopathy
Supportive labs: ESR & CRP
Suspected diagnosis: EKG, Echo, discuss with pediatric cardiology

25
Q

Kawasaki disease treatment

A

Acute: IVIG 2g/kg & high dose aspirin 80-100mg/kg/day; once fever resolves, decrease aspririn, risk of aneurysms controlled with corticosteroid, warfarin
Treatment failures: repeat second IVIG course, IV solumedrol, infliximab
Serial echocardiograms: At 1, 2, 6, 12 weeks then every 5 years

26
Q

Large vessel vasculitis

A

Aortic arch disease in Takayasu

Giant cell arteritis

27
Q

Giant Cell Arteritis Overview

A

Most common vasculitis for people over 50

Caucasian, F>M

28
Q

Giant Cell Arteritis Sympoms

A

Constitutional Symptoms: fever, weight loss (15-20%)
Cranial: Jaw claudication, headache, scalp pain/tenderness
Eye: vision changes (blurry, double, loss, optic neuritis/atrophy) - loss in up to 15%
CV: unequal BP (aortic involvement) bruits
Resp: cough
MSK: PMR symptoms (shoulder/hip girdle stiffness)

29
Q

Gian Cell Arteritis Labs

A

Nonspecific: Elevated ESR & CRP, CBC of anemia and thrombocytosis, CMP elevated alk phos
Cranial disease - TA US showing characteristic findings of temporal artery biopsy
Extracranial disease - PET, MRA, CTA

30
Q

Giant Cell Arteritis Differential

A 
Takayasu
Bechet's
IgG4 RD
Heritable CTD
Lymphoma

Jaw claudication amyloidosis
Zoster

31
Q

GCA Treatment

A

High dose steroids (IV if threatened vision, otherwise 1mg/kg daily for 1 month bolowed by lsow taper),
Low dose aspirin
Tocilizumab
Methotrexate
Monitoring - ESR/CRP, imaging, lipids, LFTs, PLTs. on Tocilizumab

32
Q

Takayasu Arteritis overview

A

young onset (~25)
F»M 8:1
Asian ethnicities
Hypertension - unequal BPs (>10 systolic) take BP in all 4 extremities

33
Q

Takayasu Arteritis Phases

A

Phase 1: fevers, arthralgias, weight loss
Phase 2: vessel pain, tenderness
Phase 3: fibrotic, bruits and ischemic symptoms (limb claudication, lightheadedness/syncompe)

34
Q

Takayasu arteritis Labs

A

Nonspecific - ESR & CRP elevation (or normal in 30%)

Imaging: MRA preferred, CTGA, FDG-PET

35
Q

Takayasu Arteritis treatment

A 
high dose steroids  - Prednisone 30 mg BID with slow taper
Methotrexate, Azathioprine
Maybe anti-TNF before IL6 etc.
Relapse is common
Surgery not indicated
36
Q

Bechet’s overview

A

M:F 1:1
Mean onset 30
more common in ethnicities of the silk road
HLA–B51 association outside US more severe course

37
Q

Bechet’s clinical syndrome

A

Recurrent painful mouth/genital ulcers
May have inflammatory eye disease - anterior/posterior uveitis, retinal vasculitis
Skin manifestations - pseudofolliculitis, papulopustorlar lesions, E nodosum

38
Q

Bechet’s differential

A

Differential: Crohn’s disease, recurrent aphthous stomatitis, reactive arthritis, lupus and others

MSK (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions