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(Ch24) Vasculitis Flashcards

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1
Q

Epidemiology of vasculitis ?

A

All ages
Ave age adults–> 47 and kids–> 7
F>M

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2
Q

Most common location of vasculitis and why?

A

dependent areas (e.g. LE) due to gravity

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3
Q

Time period from event to onset of vasculitis ?

A

7-10 days

systemic sx precedes skin (avg 6months)

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4
Q

What are the 2 main mechanisms/pathophysiology of vasculitis?

A

1.Immune complex medicated
2. ANCA-mediated (neutrophils rather than by immune complexes hence “pauci immune”)

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5
Q

What is the mechanism of immune complex mediated vasculitis ?

A

Binding of Ab to circulating Ag e.g. meds, infections or Ca →Immune complex deposition in postcapillary venules→↑complement →↑adhesion molecule.

  1. Complement split products (C3a and C5a) →mast cell degranulation and neutrophil chemotaxis.
  2. MAC C5–C9 on endothelium→↑clotting cascade→↑thrombosis, inflammation & angiogenesis
  3. Mast cell degranulation→↑vascular dilation & permeability.
  4. Neutrophils → proteolytic enzymes i.e. collagenases & elastases + oxygen radicals → vessel wall damage
  • ↑E-selectin, P-selectin, ICAM-1, VCAM-1, PECAM-1 on endothelium + adhesion molecules on WBC e.g. P-selectin glycoprotein ligand-1, LFA-1, Mac-1 →↑cell migration
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6
Q

What is the mechanism of ANCA mediated vasculitis ?
P-ANCA=MPO
C-ANCA=PR3

A

Activation by TNF-α → intracellular proteins e.g. proteinase 3, myeloperoxidase expressed on neutrophils surface→↑adhesion to vessels → Neutrophils release ROS + toxic mediators + c-ANCA against PR3/ p-ANCA against MPO formation→ vessel damage

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7
Q

What is the most common cause of vasculitis?

A
  1. Idiopathic (50%)
  2. Autiommune dis (15-20%)
  3. Infections (15-20%)
  4. Drugs (10%)
  5. Neoplasms (5%) (mostly heme except IgA –> lungs ca)
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8
Q

Mention 5 common drugs to trigger vsculitis

A

BON TM:

B-lactams abx
OCP
NSAIDs
Thizide
MTX

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9
Q

List 10 casueses of vasculitis
VASCULITIS

A

VASCULITIS
V- viral (Hep C, Hep B, HIV)
A- autoimmune (SLE, RA, Sjogren’s, Henoch-Schonlein purpura)
S- staph, strep infections
C- cryoglobulinemia (e.g. from Hep C, RA)
U- ulcerative colitis, crohn’s
L- lymphoproliferative disorders (esp. hairy cell leukemia)
I- infective- endocarditis, meningococcemia
T- thiazides, NSAIDS
I- immune complex (e.g. from endocarditis)
S- Septra, b-lactam antibiotics

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10
Q

Mention classification of vasculitis based on vessels size

A

1. Large vessel:
* Takaysu
* Temporal arteritis

2. Medium vessel:
* PAN

3. Mixed medium and small:
* Granulomatosis with polyangitis
* Microscopic polyangitis
* Esinophilic granulomatosis with polyangitis

4. Small vessl:
* CSVV
* HSP
* Acute Hemorrhagic Edema of Infancy
* Erythema elevatum diutinum
* Cryog II and III
* Urticarial vasculitis

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11
Q

Mention skin manifestation of vasculitis based on vessels size

A

1. Large vessel:
Ulceration and necrosis

2. Medium vessel:
SebQ nodules
Livedo reticularis
Ulcers
Digital necrosis

3. Mixed medium and small:
both medium and small manifestations

4. Small vessl:
Palpable purpura
Petechia
Erythema

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12
Q

Mention risk factors for systemic sx in vasculitits

FAP (FP=Full blown)

A

FAP
1. fever
2. Absence of pain
3. Paresthesias

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13
Q

Predictors of chronicity (>4w) in vasculitits

aCaf= C=chronic

A

ACAF
1. Arthralgia
2. cryoglobulinemia
3. Absence of fever

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14
Q

Demographic MC affected by HSP and during what time of the year

A

MC vasculitis in kids esp boys< 10 Yrs

↑Winter

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15
Q

HSP MC As/w and when is the onset of sx

A

Mostly 1-2 wks following URTI

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16
Q

Which Abs found in HSP

A

IgA1 deposits

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17
Q

What is the tetrad of HSP

A

tetrad of
Palpable purpura
Arthritis
Abdo pain
Hematuria

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18
Q

What is the most common skin manifestation of HSP

A

Palpable purpura (100%)

can be targetoid lesions & foci of necrosis esp adults.

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19
Q

What is the MC neoplasm As/w HSP/ IgA in adults

A

** lung Ca** in adults

vs non-IgA CSVV a/w hematologic CA

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20
Q

What is the MC systemic dis/associtation with IgA vasculitis

A

MC Arthritis 75%
Others:
* GI Sx 50-75%:Intussusception + perforation
Diarrhea is more in adults
* Renal Sx (50%): 3% kids & 30% adults▸ CKD
* Fever
* Orchitis +/- lung sx

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21
Q

Risk factors for nephritis in HSP/IgA vasculitis

A

Risk for nephritis:
1. Male >10y at onset
2. Severe GI sx
3. Joint sx
4. persistent or recurrent purpura
5. ?Purpura in trunk/UE
5. Labs (↑Wbc,↑plt,↑ASO,↓C3)
6. HLA-B35
7. Path: ↓eosino in adults, PV C3 not IgA + papillary edema

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22
Q

Predictors of relapse in HSP

A
  1. Age >30 Yrs
  2. Underlying systemic disorder
  3. Persistent purpura >1 month
  4. Abdominal pain
  5. Hematuria
  6. Absence of IgM of DIF
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23
Q

Predictors of Poor prognosis in HSP/IgA vasculitis

A
  1. Adult onset
  2. Renal failure at Dx
  3. Nephrotic syndrome
  4. HTN
  5. ↓Factor XIII
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24
Q

which gene mutation As/w ↓GI Sx in HSP

A

✗ ICAM-1469 K/E variant

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25
Which tx reduce **duration** and **purpura recurrence** in HSP
Dapsone + colchicine
26
Which sx of HSP can be relieved by SCS
SCS ↓Arthritis, abdominal pain & GI Sx ↓Duration of purpura **NOT recurrence** 🚫 ** Prevent renal sequelae**
27
Small vessel vasculitis tx: Q1:1st line tx of HSP, AHEI and CSVV Q2:1st tx for UV/HUVS Q3:Which vasculitis 1st line tx is CS+ritux and antivrial Q4: EED 1st line tx
1. **HSP, AHEI and CSVV** supportive anti H NSAIDS Leg Elevation etc SCS is not effective in AHEI 2. **HUVS/UV** Anti-H, indomethacin Daposne, SCS, Pentoxyfylline 3. **Cryoglobulinemia II and III** 4. Dapsone , ILK, NSAIDs
28
Age of onset of AHEI and time of the year?
↑♂ < 2Yrs ↑Winter sx 1-2 post trigger
29
Mention few possible triggers of AHEI
URTI (viral or bacterial, CoxA, adeno, strep) > diarrheal illness > UTI Acetaminophen, TMP/SMX & Abx
30
Sx and skin maifestation of AHEI
baby looks well Abrupt, ASx, Arcuate, polycyclic or **targetoid with dusky center ** sys: Fever ~45% **Tender, non- pitting edema** Face, ears, limbs & scrotum **Characteristic**
31
Most common associations of Urticarial vasculitis
Idiopathic is MC but A/w: AI-CTD esp **Sjögren + SLE** Serum sickness, Cryoglobulinemia Infection; HCV/HBV & EBV Medications Malignancies; Hematologic
32
which demographic is HUVS is exclusive to?
80% Normocomplementemic; benign **Hypocomplementemic exclusively in♀.**
33
Normo vs hypo complementic vasculitis
Normo--> skin limited Hypo --> systemic (HUVS)
34
Urticarial vasculitis vs Urticaria
Vasculitis: Last > 24 Hr A/w burning & pain vs itchyiness Resolve w/ PIH
35
MC systemic sx of HUVS
MC Arthralgias; arthritis in HUVS but also may have Severe COPD in smoker
36
Hypocomplementemic urticarial vasculitis syndrome (HUVS) diagnostic criteria
2 Majors + ≥2 minors **Majors**: 1. Urticaria for 6 months 2. Hypocomplements **Minors**: 1. Bx proved vasculitis 2. Arthralgia or arthritis 50% 3. ⚑ Uveitis or episcleritis 4. Glomerulonephritis 10% 5. Recurrent abdominal pain 6. +ve C1q precipitin test & ↓C1q
37
Labs for Urticarial vasculitis
Labs for UV: C3/C4 ESR, ANA (rare dsDNA, Sm) anti-C1q precipitin C1q levels
38
What are labs results for HUVS and UV
**UV labs**:↑ESR, ↓ C3 + ↓C4 & +ve ANA **Lack anti-dsDNA & anti-sm ** vs SLE **HUVS**: same labs + +ve anti-C1q precipitin &↓C1q
39
HUVS vs SLE
HUVS: Eye Sx COPD angioedema Lack anti-dsDNA & anti-sm
40
What is the MC As/w EED vasculitis
HIV
41
Mention 2 malignancies Asw EED
IgA monoclonal gammopathy hairy cell leukemia others AsW: Autimmune dis infections
42
EED presentation
Symmetric ASx red papules+/- doughy fibrosis **On acral + periarticular esp extensor elbow** systemic is Extremely rare except ocular finidings **mainstay rx Dapsone**
43
Characteristic hisopath finiding of EED
Intracellular lipidosis Classic finiding of late stage
44
sybtypes of cryoglobulinemia and which is cold induced
Type 1: mono IgM -->**vasculopathy** --> cold induced Type2&3 (mixed): mono or poly IgM aginst poly IgG--> **vaculitis** --> not cold induced
45
What are the Asw of Cryo I and skin manifestations
As/w: Plasma cell dyscrasia Lymphoproliferative dis skin: Raynuad's Acrocyanosis Retiform purpura Gangrene.
46
What are the Asw of Cryo II and III (mixed) and skin manifestations?
Asw: **MC is Hep C** HepB, HIV AI-CTD Lymphoprilferative dis manifestations: **Plaplable purpura - MC** Glomerulonephritis Arthralgia peripheral Neuropathy
47
What is the most common infection and malignancy As/w Mixed cryo II&III
overall is Hep C malignancy -> B cell NHL
48
list 5 ddx for **Lymphocytic** vasculitis | 5Ps/PS5/4
**PS4**: Pernio PLEVA Panniculitis luPus relapsing Polychondritis Sneddon
49
What is the role of hep C in mixed cryo II&III
t(14;18) due to HCV → rearrange anti-apoptotic bcl-2→↑B-cell Chronic immune stimulation
50
Predictors of relapse in non-infectious mixed cryoglubinemia | PNA
Purpura, Necrosis Articular involvement
51
If biopsy done on lesions >72h what would be +ve in DIF
only C3 will be detected in lesions present for >72 hours (in 30% of cases).
52
DIF pattern in vasculitis
granular pattern
53
Microscopic polyangitis (MPA) epideiomology
Older patient F>M
54
MC Asw MPA
infectious endocarditis
55
MC skin manifestation of all ANCA and PAN vasculitis
**Palpable purpura** MC GPA and EGPA can also present with Papulonecrotic lesions: palisading neutrophilic dermatitis
56
MC systemic involvment in MPA
**Renal >90%** Pauci-immune, crescentic, necrotizing **glomerulonephritis**.
57
Systemic sx of MPA vs GPA (wegener)
**MPA**: Renal >90% Lungs ?capiliritis mononeuritis multiplex **GPA**: U/LRT involvment 90% renal 20% other organs
58
Which vasculitis has highest risk og renal invlovment
MPA
59
Which medium/small vessel vasculitis has no granuloma
MPA
60
Which ANCA and PAN vasculitis has highest risk of relapse
1. GPA (wegner) 1. MPA 1. PAN
61
What are the **predictors of relapse** in ANCA vasculitis and PAN
MPA: ANCA+ after remission GPA: ANCA+ after remission Nasal carriage of Satph A EGPA: ↓Eosin count at diagnosis PAN: Skin Sx non-HBV classic PAN
62
1st line tx Tx for MPA and GPA Tx for EGPA and PAN
MPA & GPA **Induction**: SCS+Cyc or + Ritux or + MTX **Maintainence**: Ritux EPGA and PAN: CS
63
Demographics of GPA (wegner)
↑Caucasian ♀at 45–65 Yrs. - Common systemic vasculitides of children
64
GPA pathophysiology
↓Activity of 𝜶-1 antitrypsin Th1- induced granuloma Satph A --> priming of neutrophilis
65
Which ANCA is associted with each ANCA vasculitis ?
MPA: P-ANCA > C-ANCA GPA: C-ANCA EGPA: C-ANCA
66
Which ANCA vsculitis present with strawberry gum
GPA (wegner)
67
What is the triad of Wgener systemic involvment ?
1. Granulomatous inflammation of U/LRT 1. systemic necrotizing small vessel vasculitis 1. pauci-immune glomerulonephritis
68
what is the European criteria for Wegener dx
European criteria (3 of 6): 1. URT inflammation 2. Typical radiologic features on CXR or chest CT 3. Airway stenosis esp in children 4. Abnormal urine analysis 5. Biopsy-proven granulomatous inflammation 6. Serology e.g. anti-PR3 ANCAs.
69
What are the path findings of Papulonecrotic lesion of GPA vs EGPA
GPA: **neutrophilic** dermatitis w/ granulomatous inflammation surrounding foci of **basophilic necrobiosis** EPGA: palisading dermatitis w/ **eosinophil** infiltration, granuloma & **eosinophilic necrobiosis**
70
Mention triggers for EPGA
1. vaccines 1. desensitization Rx 1. **leukotriene inhibitors** 2. **Omalizumab** 1. rapid D/C of steroids Th2&1-mediated granuloma formation
71
Mention phases of EGPA
**3 successive phases**: **1st prodromal phase**: Allergic rhinitis, nasal polyps & asthma Asthma; usually severe in almost all patients Persist for Yrs **2nd eosinophilic phase**: Peripheral eosinophilia, resp infections & GI Sx **3rd Vasculitic phase**: Necrotizing vasculitis w/ granulomatous inflammation after Yrs - decades of initial Sx
72
What is the leading cause of death in EGPA
**Cardiac Sx** 50%: Cardiomyopathy or pericarditis Leading cause of death
73
Mention Churg strauss +ve ANAC and -ve ANCA Asscociations
**+ve ANCA**: 1. ↑cardiac Sx 2. Pleural effusions 3. Fever 4. Livedo reticularis **-ve ANCA**: 1. purpura 2. renal 3. sinus 4. neuro Sx
74
what are the subtypes of PAN and MC infection asscociated
Skin limited PAN (10%) --> strep in kids others: parvo B19 & HIV Classic/Systemic PAN (90%) --> HepB spares lung
75
Associations of PAN
A/w: **Infections**: *Classic PAN*: HBV in ~7% ↑GI, neuro & renal Sx + ↓1-Yr survival HCV *Cutaneous PAN*: Strept esp kids, parvo B19 & HIV **Inflammatory** : IBD, SLE & familial Mediterranean fever **Malignancies** esp hairy cell leukemia **Medications**: Minocycline w/ cutaneous PAN **Gene**: ✗ CECR1 gene encodes for ADA2
76
What are APL associated vasculitis and vasculopathy
**Vasculopathy**: Sneddon **Vasculitis**: PAN Both can be associated with ADA2 deficiency/mutations
77
Which organ spared in PAN classic
Lungs
78
What sx Asw Skin-limited PAN
Skin-limited PAN a/w fever, myalgia, arthralgia & peripheral neuropathy
79
Renal invlovment of PAN vs ANCA vasculitis
PAN: interlobar renal arteries /Renovascular , No GN ANCA: Pauci-immune GN
80
Which systemic invlovment Asw low prognosis in PAN
GI Sx esp **mesenteric ischemia**
81
Histopath of PAN
Biopsy: **Segmental** necrotizing vasculitis of medium-sized arteries. Cutaneous PAN involves arteries of deeper dermis & SC fat
82
Ddx of nasal destruction
**Inflammatory disorders** 1. Wegener’s granulomatosis 1. Relapsing polychondritis 1. Sarcoidosis **Neoplastic disorders** 1. Nasal natural killer/T-cell lymphoma (lethal midline granuloma) 1. Squamous cell and basal cell carcinomas 1. Neuroblastoma, salivary gland tumors, sarcomas (e.g., rhabdomyosarcoma) **Infectious disorders** 1. Bacterial: rhinoscleroma, glanders, noma, syphilis (late congenital or tertiary), yaws 1. Mycobacterial: leprosy, tuberculosis (lupus vulgaris) 1. Fungal: paracoccidioidomycosis, zygomycosis, aspergillosis 1. Parasitic: mucocutaneous leishmaniasis, acanthamoebiasis, rhinosporidiosis **Other** 1. Cocaine use (cocaine-induced midline destructive lesion) 1. Nasal myiasis 1. Factitious or traumatic
83
Which initial labs ordered for vasculitis and to be repeated with flares
CBC+ diff ESR /CRP Hepatic panel Renal + UA Stool guaiac
84
When to do bx for vasculitis
Lesional bx H&E >24h DIF <24h
85
Why bx of CSVV DIF
r/o IgA
86
Check diagram for work up and approach at chapter/ notes
87
which vasculitis can present as inflammatory or non-inflammatory retiform purpura, and need to be ruled out in any patient presenting with retiform purpura
ANCA vasculitis and PAN
88
Abs for each
* GPA → c-ANCA, anti-PR3 * MPA/EGPA → p-ANCA, anti-MPO * Levamisole → p-ANCA "p = pan-ANCA" (aka positive in almost everything except
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