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1. Rheum RC > Vasculitis (LVV, MVV, SVV, MVV), PMR > Flashcards

Vasculitis (LVV, MVV, SVV, MVV), PMR Flashcards

1
Q

** Rheumatic diseases assoc’d w aortitis**

A

-Seronegative SpA
-Relapsing polychondritis
-Behcet’s
-Cogan’s
-Sarcoid
-LVV: GCA, TAK
-AAV

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2
Q

DDx ascending aorta vasculitis/aneurysm

A

Inflamm: GCA, TA, Behcet

-Noninflam: Marfan, Ehlers-Danlos type 4, familial thoracic aneurysm/dissection, loey-diatz

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3
Q

DDx isolated descending aorta vasculitis/aneurysm

A

TA (india/pakistan)
-Inflammatory abdominal artery aneurysm

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4
Q

DDx isolated pulm artery vasculitis/aneurysm

A

Behcet’s (huge’s stovin syndrome)

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5
Q

DDx periaortic vasculitis/aneurysm

A

-IgG4 related disease
-Lymphoma
-Erdheim Chester

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6
Q

GCA Presentation

A

Cranial: H/A, scalp tenderness, jaw/tongue claudication, trismus, diploplia, visual sx

-Extracranial: abnormal pulse, stroke, dizziness, claudication, abdo pain, HTN, angina

-Systemic: fever, wt loss, UE claudication, arthritis, cough

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7
Q

Non-productive Cough in GCA Mechanisms

A

-Pharyngeal artery vasculitis (branch of external carotid)
-Stimulation of cough receptors in the bronchus due to aortic inflammation

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8
Q

Physical findings GCA

A

-Scalp tenderness

-Temporal artery tenderness, reduced pulse

-Visual sx: diploplia, amaurosis fugax, vision loss, optic neuritis, optic atrophy

-BP >10mmHg diff between arms
-Carotid/Subclavian bruits

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9
Q

What single physical exam finding has the highest likelihood of having a positive temporal artery biopsy?

A

Temporal artery beading

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10
Q

Ocular Symptoms GCA

A

Diplopia (Blurry vision)
-Amaurosis fugax, aura
-Painless vision loss
-AION, PION
-Iritis, Conjunctivitis
-Ophthalmoplegia (ischemia EoM)

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11
Q

Mech of vision loss in GCA

A

-1. AION caused by vasculitis of posterior ciliary artery
-2. central retinal artery occlusion
-3. posterior ischemic optic neuropathy (PION)
-4. occipital cortex ischemia

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12
Q

Nonvasculitis ocular symptoms in GCA patients on prednisone

A

Glaucoma
-Cataracts
-Central serous retinopathy

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13
Q

GCA vessels

A

Aorta
-Subclavian
-Vertebral
-Carotid
-Iliac
-Mesenteric
-Renal
-Coronary
-Rarely pulm artery

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14
Q

Aneurysm monitoring/management

A

MRA/CTA q6-12mo
-Periodic CXR, TTE, Abdo US
-
-Surgery/Endovascular repair >5cm or dissection
-

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15
Q

GCA Imaging - US (vessels, signs, adv, disadv)

A

Vessels: Temporal, axillary, subclavian
-Signs: halo sign, compression sign
-Adv: cheap, no radiation, noninvasive
-Disadv: operator dependent

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16
Q

GCA Imaging - MRI (vessels, signs, adv, disadv)

A

Vessels: Temporal, Cranial, aorta, intrathoracic/intraabdo large/medium branches
-Signs: active vessels uptake contrast, structural changes (stenosis, aneurysm)
-Adv: no radiation, noninvasive, good sensitivity
-Disadv: cost, access, procedural time

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17
Q

GCA Imaging - CT (vessels, signs, adv, disadv)

A

Vessels: Aorta, intrathoracic/intraabdo large/medium branches
-Signs: active vessel uptake contrast, structural changes (stenosis/aneurysm)
-Adv: fast, noninvasive
-Disadv: radiation, cost higher than US (lower than MR)

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18
Q

GCA Imaging - FDG PET (vessels, signs, adv, disadv)

A

Vessels: Temporal, axillary, subclavian
-Signs: FDG uptake
-Adv: Can look for GCA and mimickers (ca, infxn)
-Disadv: radiation, high cost, access, NOT GOOD for cranial vessels

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19
Q

** GCA Bx Histology**

A

** Media thickening with TRANSMURAL GRANULOMATOUS inflammation (panarteritis)

  • GIANT CELLS, lymph/macrophages, eo

-Intimal hyperplasia/fibrosis

-Fragmentation/destruction internal elastic lamina’

-SHOULD NOT SEE FIBRINOID NECROSIS (look for GPA)

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20
Q

4 reasons for negative TAB in someone you suspect GCA in

A
  • Previous corticosteroid use
    – GCA with phenotype not associated with cranial arteritis
    – Sampling error& missed lesion due to skip lesions
    – Inadequate sample
    – Delayed biopsy after treatment
    – Biopsied temporal vein
    – Patient does not have GCA, i.e alternative diagnosis
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21
Q

**4 clinical or lab findings that are associated with positive TAB **

A

Limb claudication (positive LR, 6.01; 95% CI, 1.38-26.16),
-Jaw claudication (positive LR, 4.90; 95% CI, 3.74-6.41),
-Temporal artery thickening (positive LR, 4.70; 95% CI,2.65-8.33),
-Temporal artery loss of pulse (positive LR, 3.25; 95% CI, 2.49-4.23),
-Platelet count of greater than 400 (positive LR, 3.75; 95% CI, 2.12-6.64),
-Temporal tenderness (positive LR, 3.14; 95% CI, 1.14-8.65),
-Erythrocyte sedimentation rate greater than 100 mm/h (positive LR, 3.11; 95% CI, 1.43-6.78).

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22
Q

Layers of vessel from lumen outwards

A

Tunica Intima
-Internal elastic lamina
-Tunica media
-External elastic lamina
-Tunica adventitia
-Vasa vasorum

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23
Q

Where is vasovorum and what does it do

A

Located in adventitia
-Microvascular network supplies O2 and nutrients to vessel

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24
Q

GCA labs

A

Elevated ESR, CRP
-Anemia, Thrombocytosis, Transaminitis (ALP)
-

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25
Q

GCA Pathogenesis

A

Ag trigger activates vascular DCs at adventitia → T cell activation, attraction of lymphocytes/macrophages → granuloma formation, proinflammatory cytokines (IL 1,6, 21, 17), injury of media and external lamina, neoangiogenesis, hyperplasia of intima, and vessel stenosis/occlusion

-DCs at adventitia
→ recruit macrophage, lymphocytes
→ produce IL12/18 for Th1 differentiation → IFNg (macrophage activation and granuloma formation)
-→ IL1/6/21 → Th17 differentiation → IL17 (vascular inflamm, MMP production to destroy internal elastic lamina)
-VEGF brings more T cells and macrophages
-Plt derived growth factor IL1 → intimal proliferation and ischemia

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26
Q

GCA HLA link

A

HLA DR4
HLA DRB1*04

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27
Q

GCA Tx

A

Pred 1mg/kg (pulse if vision threatening/loss) x1 mo or until ESR/CRP normal, then long taper

-ASA 81 daily for critical/flow limiting involvement of vertebral/carotid arteries

-MTX if diabetes, relapsing, or steroid side fx

-Toci 162mg SC weekly(if visual sx or critical cranial ischemia)

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28
Q

TA presentation phases

A

1: prepulseless inflammatory period: fevers, arthralgias, wt loss
-2: vessel inflammation: pain/tenderness
-3: fibrotic: bruits and ischemia

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29
Q

TA clinical manifestations

A

Bruits, decreased pulse, asymmetric BP
-HTN, arthralgia, constitutional sx
-
-Subclavian/Iliac → limb claudication
-Carotid/Vertebral → H/A and presyncope
-Pulm artery → pHTN
-Cardiac → angina, MI, HF, death, regurg

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30
Q

TA Dx

A

MRA > CTA, FEG-PET, US
-Biopsy NOT necessary to diagnose

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31
Q

TA histopath

A

-Different from GCA : cellular infiltrate in adventitia and outer parts of media (including vasa vasorum) with gamma-delta T lymphocytes, NK cells, and CD8 T cells (vs Th1/Th17 lymphocytes in GCA)

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32
Q

TA Tx
-Initial
-Adjunct
-Refractory
-Salvage
-Other
-Refractory to meds

A

GC + MTX/AZA/TNFi
-Adjunct: ASA (if cranial/vertebrobasilar involvement)
-Refractory: LFN, MMF, AZA, ustekinumab, infliximab, toci
-Salvage: CYC
-Other: BP control, antiplatelet, smoking cessation, lipid mx
-Surgery only if ischemic once dz quiescent (angioplasty, stenting, bypass graft surgery)

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33
Q

TA morbidity

A

Cardiac:
-Aneurysm, rupture, dissection
-LVH
-Aortic root dilatation, regurg
-pHTN
-MI
-Stroke

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34
Q

Infectious aortitis causes

A

-TB
-Syphilis (tertiary)
-Bacterial (Staph, Strep, Salmonella
-Viral

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35
Q

Idiopathic isolated aortitis

A

-Isolated idiopathic thoracic or abdo aortitis
-Giant cell aortitis: indistinguishable from GCA
-IgG4RD
-Chronic periaortitis (abdo)
-Inflamm abdo aortic aneurysm

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36
Q

LVV mimickers

A

-FMD
-Atherosclerosis
-Radiation fibrosis
-IgG4
-Sarcoid
-Inflamm aortic aneurysm
-Syphilitic aortitis
-Genetic disease (marfan, loeys dietz)
-Congenital coarctation of aorta
-Neurofibromatosis type 1

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37
Q

** PAN organ involvement **

A

Peripheral nerves > skin > abdo > muscle > kidney
-Unlikely: ENT, lungs

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38
Q

**Clinical features PAN **
- Classification criteria
- Other features

A

3+ of the following
-Weight loss
-Livedo
-Testis: pain (more w/ hep B)
-Myalgias (excluding shoulder/hip girdle) or weakness
-Nerves: Mononeuritis multiplex (motor/sensory), foot drop, polyneuropathy
-dBP>90mmHg
-Elevated BUN/Cr
-HBV
-Arteriographic abN
-Biopsy small/medium artery showing PMN

Others:
-CNS: stroke, seizure, jaw claudication
-Eye: retinal hemorrhage, optic ischemia
-Skin (LE): Purpura, ulcers, livedo, nodules, fingertip infarct, necrotic lesions
-Cardiac: MI, HF
-Abdo: pain, liver fcn, mesenteric arteritis
-Renal: infarcts, HTN, Proteinuria

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39
Q

Clinical features more found in HBV assoc’d PAN vs PAN

A

HBV assoc’d: more orchitis, HTN, renal infarct

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40
Q

PAN cutaneous findings

A

Sam as GPA/eGPA/IC SVV

-Purpura
-Ulcers,
-Livedo,
-Nodules,
-Fingertip infarct,
-Necrotic lesions

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41
Q

Labs PAN

A

-AoCD, Thrombocytosis, ESR/CRP,
-Low albumin
-Can hv elevated Cr (from renal vessel occlusion) with BLAND urine
-Classically: negative ANA, cryo, RF, ANCA
- HBV

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42
Q

Dx PAN

A

Biopsy symptomatic (skin, sural nerve, muscle, rectum, testicle)showing granulocytes +/- mononuclear leukocytes in artery wall

-Angiogram if cannot biopsy (usually abdo: renal, liver, spleen, stomach, large/small bowel; rarely hand/foot) showing aneruysm/occlusion of visceral arteries (not due to atherosclerosis, FMD, or other cause)

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43
Q

PAN histology

A

Focal & segmental transmural **necrotizing vasculitis ** of medium/small arteries (>arterioles; no vein or large vessel involvement; uncommon splenic, rarely pulmonary)

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44
Q

PAN purpura Biopsy histology

A

LCV

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45
Q

PAN angiogram findings

A

Microaneurysm (saccular aneurysm)
-Occlusion/cutoff
-Luminal irregularities
-Stenoses
-Rosary bead sign

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46
Q

Ddx aneurysms on abdo visceral angio

A

Segmental arterial mediolysis (SAM)
-FMD

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47
Q

Segmental arterial mediolysis (SAM)
-Pathogenesis
-What vessels
-Dx
-Tx

A

** Vasospasm/FMD variant**
-Nonatherosclerotic & ** noninflamm** lytic loss of medial muscle → dilation/aneurysm (muscular, splanchnic, basilar cerebral, coronary arteries)
-Dx: Angiogram, Biopsy
-Tx: Surgery, BP control

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48
Q

2 imaging findings to differentiate segmental arterial mediolysis (SAM) from PAN

A

-SAM: NO significant vessel wall thickening. String of beads (stenosis, dissection, aneurysm, thrombosis) at multiple renal/ mesenteric arteries

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49
Q

1 lab finding to differentiate SAM from PAN

A

PAN has elevated ESR, CRP, abnormal UA
-SAM DOES NOT

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50
Q

PAN pathogenesis theories

A

Immune complex (complements / complexes seldom found)
-Endothelial injury → cytokines/inflamm (no Ag or trigger found)

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51
Q

Conditions assoc’d w/ PAN or PAN vasculitis

A

Viral infxn: HBV > CMV, HTLV1, HIV, parvo, EBV, Hep C,
-Hairy cell leukemia
-Genetic: loss of adenosine deaminase 2 (DADA2)
-Autoimmune: SLE, RA, childhood DM, Cogan
-Med rxn: allopurinol, sulfa

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52
Q

Tx systemic PAN

A

Mild (No cardiac, GI, CNS): pred 1mg/kg x4 wks, taper 6-12mo + AZA (2mg/kg) or MTX (20-25/wk)

-Mod/severe: pred 1-2mg/kg + CYC

-Organ/life threatening: IV pulse GC + CYC (2mg/kg PO or 0.5-1g/m2 /mo IV) x4-12mo. (PLEX ONLY if catastrophic)
CYC → AZA/MTX once in remission for total 18mo (MMF if can’t AZA/MTX, Ritux if can’t CYC) + pred taper

-Adjunct: HTN control, PJP ppx, OP prevention

  • PT if nerve/muscle involvement
  • DADA2: TNF > GC alone
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53
Q

Tx HBV assoc’d PAN (HBsAg+ and HBeAg+)

A

HBV assoc’d PAN:
-Pred 60 mg/d x1 wk then taper by 50% dose daily until off by end of 2nd week
-PLEX to remove immune complex 3x/wk for 3 wks, 2x/wk for 2 wks, then once/wk until HBeAg-neg
-Antiviral agents on day of PLEX after procedure

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54
Q

5 factor score - Indications and Components w/ 5 year survival

A

At diagnosis to predict prognosis of PAN, MPA, eGPA

-Cardiac sx
-GI involvement
-CNS involvement
-Creatinine >140umol/L
-Proteinuria >1g/24h

0 = 88% 5 year survival
1 = 75%
2+ = 54%

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55
Q

PAN Cause of death
-1 year
->1 year

A

1 year: uncontrolled vasculitis
>1 year: complication tx, infxn, vascular event (stroke, MI)

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56
Q

Tx Isolated PAN
-Which organs
-How to treat

A

Cutaneous (nodular lesions, livedo, polyneuropathy):
-Pred 20-40mg/d w/ taper
-Steroid sparing: MTX, AZA, colchicine, dapsone
-
-Localized gallbladder, uterus, breast, appendix:
-Sugical excision
-No immunosuppression

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57
Q

Thromboangiitis obliterans aka Buergers
-What is it

A

Inflamatory , obliterative, nonatheromatous vascular dz of small/medium arteries AND veins.

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58
Q

TO Pathology

A

Acute: hypercellular inflammatory thrombus with microabscess within thrombus

-Subacute: microabscess surrounded by granulomatous inflamm –> organization/recanalization of thrombus

-Late: mature thrombus w/ vascular fibrosis

-Internal elastic lamina INTACT

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59
Q

TO etiology

A

Unknown
-Possible:
-Smoking (pipe, cigar, tobacco, weed)
-Autoimmune: antiendothelial cell antibodies in active dz (not remission)
-DNA fragments of anaerobic bacterial in arterial lesions and oral cavities

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60
Q

TO features

A

Ischemia/claudication feet > hands (distally progressing up)
-Instep/pedal claudication
-Digital ulcerations, gangrene, rest pain
-Dysesthesias
-Cold sensitivity
-Superficial migratory thrombophlebitis
-Raynaud’s, rubor, cyanosis
-Arthralgias

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61
Q

TO DDx

A

Collagen Vascular dz: SLE, SS, MCTD
-Vasculitis: PAN, GCA, TA, cocaine, SVV
-APLA
-Blood dyscrasias: PV, ET, hyperviscosity Occupational: vibratory tools, hypothenar hammer syndrome
-Embolic: thoracic outlet syndrome, myxoma, cholesterol emboli
-Ergot/emphetamine tox
-Atherosclerosis
-Pernio
-Frostbite

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62
Q

TO Ix

A

Normal: CBC, LFT, UA, BG, ESR/CRP, ANA, RF
-TTE for cardiac thrombi
-Duplex US / ABI
-Arteriogram

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63
Q

TO arteriographic findings

A

B/L focal segments of stenosis or occlusion with NORMAL proximal or intervening vessels (no emboli or atherosclerosis). Collaterals around occluded areas (treet root, spider web, corkscrew appearance)

-Small/medium artery AND veins involved
-Common: digital arteries fingers/toes, palmar, plantar, tibial, peroneal, radial, ulnar arteries

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64
Q

TO Tx

A

-Treat ischemic ulcer (wound care, CCB, pentoxifylline, iloprost, sympathectomy, bosenta, GCSF, fibroblast/hepatocyte growth factor, BM mesenchymal stem cells)
-
-Treat cellulitis (abx)
-
-Treat superficial phlebitis (NSAIDs)
-
-Angioplasty, Revascularization, Bypass graft
-
-Amputate (last resort)

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65
Q

PACNS / PCNSV manifestations

A

H/A
-Neuro sx: cog dysfcn, stroke, FND, sz, hemorrhage, encephalopathy, myelopathy, radiculopathy, neuropathy, ataxia, coma
-
-Systemic vasculitis sx ABSENT (fever, rash, mononeuritis multiplex)

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66
Q

PACNS/PCNSV DDx

A

-Malignancy: CNS lymphoma, lyphomatoid granulomatosis
-
-Drugs: Cocaine, heroine, amphetamines (all cause vasospasm vs vasculitis)
-
-CTD: SLE, Sjogren, Behcet, PAN, AAV, APLA
-Susac: endotheliopathy w/ sensorineural hearing loss, encephalopathy, retinal artery occlusion
-
-Others: Sarcoid, CADASIL, FMD, Moyamoya, cardiac emboli

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67
Q

PACNS/PCNSV Dx

A

Brain biopsy (higher yield from granulomatous vasculitis vs lymphocytic vasculitis)

-Imaging:
-Angiogram (not specific: stenosis/ectasis in 1+ vascular bed) > CTA/MRA
-MRI (cortical/subcortical infarcts, white matter hyperintesity, gadolinium enhanced intracranial lesions)

-CSF: lymphocytic pleocytosis, elevated protein, occasionally high IgG and oligoclonal bands. (consider RCVS if normal CSF)

-exclude intravascular lymphoma if LDH elevated

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68
Q

PACNS/PCNSV Tx

A

High dose pred + CYC (or ritux) x3-6mo (while taper pred)
-Remission (no new sx or MRI lesion): AZA or MMF x1y +

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69
Q

RCVS manifestations

A

-Acute thunderclap headache
-Seizures
-Brain edema
-SAH (lobar/convexity)
-PRES

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70
Q

RCVS Investigations

A

CSF and labs NORMAL

-Angiogram: reversible arterial narrowing of intracerebral arteries in bilateral hemispheres

Vasogenic edema
SAH

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71
Q

RCVS Tx

A

-CCB (nipodipine 60mg q6h, verapamil 80mg BID< verapamil SR 180-20 daily)

-Sometimes short course high dose prednisone

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72
Q

MVV mimickers

A

-Cholesterol emboli
-Atrial myxoma
-FMD
-Lymphomatoid granulomatosis
-Angioblastc Tcell lymphoma
-Thomboembolic disease
-Ergotism
-Type 4 EDS
-Segmental arterial mediolysis
-Grange syndrome
-Pseudoxanthoma elasticum

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73
Q

PMR clinical features

A

SECRET
-Stiffness/pain (prox muscles, NO weakness, >2wks)
-Elderly (>50, mostly >60, mean age onset 70)
-Constitutional, Caucasian
-Rheumatism (arthralgias/arthritis - NONEROSIVE, asymmetric distal joint, Knee/wrist/SC joint; MTP/ankle RARE)
-ESR>40 +/- high CRP
-Temporal arteritis

-Absence of RF/CCP
-Rapid GC response
-AM stiffness >45min
-Nocturnal
-Constitutional sx (fever, wt loss, sweats, fatigue)

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74
Q

PMR Pathogenesis

A
  • HLA DR4 association
    -Dendritic cells activated at adventitia-media border of large vessel →IL1/6 production and suppression of Treg/Th17 response
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75
Q

PMR ACR/EULAR classification criteria

A

Required:
– Age >50,
– Bilateral shoulder discomfort,
– Abnormal inflamm markers

-Points (at least 4 required or 5 if optional US criteria used)
-AM stiffness >45min (2)
-Hip pain/ROM limitation (1)
-No RF/CCP (2)
-Absence of other joins (1)

  • Shoulder bursitis, biceps tenosynovitis, or glenohumeral synovitis AND at least one hip w/ synovitis and/or trochanteric bursitis (1)
  • Bilateral shoulder w/ subdeltoid bursitis, biceps tenosynovitis, or GH synovitis (1)
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76
Q

Source of PMR symptoms

A

Tenosynovitis (biceps)
-Bursitis (subdeltoid, subacromial, trochanteric, interspinous)
-Synovitis (hip/shoulders)

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77
Q

PMR labs

A

Inflammatory markers
-Normocytic anemia
-Thrombocytosis
-INCREASED GAMMA GLOBULINS
-Elevated ALP

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78
Q

Indications for TAB in PMR

A

Sx of GCA:
– Fever,HA, Claudication (jaw, tongue) scalp tenderness
– Visual changes,
– Tender/enlarged arteries, bruits,
– Decreased pulsation or discrepant BP

– Failure of prednisone 20mg/d to improved symptoms or normalize ESR/CRP within 1mo

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79
Q

PMR DDx

A

-Chronic pain, depression
-Hypothyroid

-Polymyositis (weakness w/o pain)
-Malignancy (esp lymphoma, myeloma)]
-Occult infxn (TB, HIV, SBE)

-Late onset spondyloarthropathy
-RA
-Shoulder OA, Rotator cuff, Frozen shoulder

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80
Q

PMR Tx

A

12.5-25mg pred daily x1-2yrs
OR
-IM methylpred 120mg q3wks
OR
-MTX 7.5-10mg/week + GC (if DM, OP, or can’t taper GC)
-NEWLY FDA APPROVED SARILUMAB (IL6Ra blocker)

-Exercise

-NO TNFi or CHINESE HERBAL SUPPLEMENTS

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81
Q

PMR poor prognostic factors

A

Older age
-Female
-Very high ESR/CRP
-Failure of CRP to normalize within 1 wk of pred

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82
Q

Pred taper in PMR

A

-Or tapering 1mg q2mo if flare occurs during taper

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83
Q

Steroid sparing agents for PMR

A

MTX 10-20mg/wk

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84
Q

Nonmedication treatment plan for PMR

A

ROM exercises
-Vitamin D
-Calcium supplements
-Regular DEXA scan
-Flu vaccines

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85
Q

** How does cANCA stain and what does it bind? And what disease **

A

C Anca = diffuse staining of neutrophil cytoplasm
-Binds PR3 (serine protease in neutrophilic granules)
-GPA (cANCA 90% Sens/spec)

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86
Q

** How does pANCA stain and what does it bind? **

A

pANCA = perinuclear cytoplasmic staining
-Binds MPO, but also elastase, lactoferrin, catalase, lysozymes

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87
Q

** False positive ANCA**

A

-CTD: RA, SLE, SS, SSc, PM, RP
-Goodpasture
-Autoimmune GI: IBD, Autoimmune liver, PSC, PBC
-Cystic fibrosis
-Infxn: HIV, HBV/HCV, TB, IE, leprosy, malaria, Parvo B19, Mono
-Meds: PTU, methimazole, carbimazole, hydralazine, minocycline, cocaine with levamisole

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88
Q

GPA / MPA pathogenesis

A

1) Infxn → cytokine release (IL1, TNF) →
neutrophil/monocytes to transport PR3/MPO to cell surface.

2) ANCA bind to surface PR3/MPO activating neutrophils/ monocytes →
- degranulate ROS + lysosomal enzymes
- upregulate adhesion molecules on endothelial cells so activated neutrophils bind/transmigrate into vessel wall → vasculitis
- NETs damaging endothelium and causing more AB production

Activation of alternative complement pathway (by infxn/injury) → C5a release to further prime neutrophils for activation and promote NET formation and more PR3/MPO

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89
Q

** 5 causes of DAH with capillaritis**

A

Primary SVV: GPA, MPA, eGPA, anti-GBM, HSP/IgA
-2ndary vasculitis: SLE, RA, APLA, MCTD, DM, behcet, UC, APLA, SSc
-Cryo vasculitis
-Lung or autologous BM transplant rejection
-Drug induced (chemo, PTU, hydralazine, TNF)
-Infxn: IE, leptospirosis

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90
Q

** 5 causes of DAH without capillaritis**

A

-Idiopathic pulmonary hemosiderosis
-Coagulopathy (DIC, anticoag, antiplatelets, thrombolytics)
-Mitral stenosis
-Pulmonary veno occlusive disease

Diffuse alveolar damage
- Anything causing ARDS
- PNA
- Drug associated: amio, penicillamine, nitrofurantoin
- Toxin or inhalation injury (crack cocaine)
- PM, SLE
- Radiation therapy
- PE

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91
Q

** DAH workup**

A

Serologies: ANCA, AntiGBM, ANA
-CT chest
-Bronchoscopy: fungal, viral, bacterial, mycobacterial cultures, PJP
-Biopsy shows hemosiderin laden macrophages
-PFT shows increased DLCO

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92
Q

** DAH tx**

A

O2 supplementation +/- mech vent
-+/- ECMO
-GC (methylpred 1g IV daily x 3d → 1mg/kg PO)
-CYC IV 15mg/kg q2wk x 3, then q3wk x 3 mo or
-Rituximab 375mg/m2 x 4;
-PLEX.

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93
Q

** Non Rheum DDX for saddle**

A

Trauma
-Cocaine
-Surgery

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94
Q

** Rheum DDX for saddle**

A

GPA
-Relapsing polychondritis
-Sarcoid
-IgG4 related disease
-RA

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95
Q

Resp Manifestations of GPA

A

Upper
- Sinusitis, discharge, ulcers, septal perforation, epistaxis
- Saddle nose
- Laryngeal granuloma –> subglottic stenosis, stridor & hoarseness

Lower
- Granuloma (vessel, airway walls, interstitium →
- Nodules +/- cavitation → post-obstructive PNA
- Capillaritis → DAH
- Resp insuff from fibrosis

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96
Q

Renal Manifestations of GPA

A

Active urine: blood, WBC, protein, casts
-Acute/ chronic renal failure

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97
Q

Eye Manifestations of GPA

A
  • Proptosis from orbital inflamm disease
    -Episcleritis/scleritis
    -Peripheral ulcerative keratitis (risk of corneal melt)
    -Uveitis
    -Conjunctivitis
    -Optic neuritis
    -Lacrimal duct obstruction
    -Retinal artery thrombosis
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98
Q

Ear Manifestations of GPA

A

-Vertigo
-Hearing loss (conductive and sensorineural)
-Otitis media
-Mastoiditis

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99
Q

Skin Manifestations of GPA/ eGPA / IC SVV

A

-Purpura, petechiae
-Ulcers , infarctions
-Subcutaneous nodules
-Vesicles
-Livedo reticularis
-Urticarial lesions
-Erythema multiforme(more eGPA and IC SVV)

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100
Q

MSK Manifestations of GPA

A

Arthralgias
-Myalgia
-Synovitis

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101
Q

CNS Manifestations of GPA

A

CNS:
- Pachymeningitis,
- Cranial neuropathy,
- Ocular palsy,
- CVA, SAH/SDH
- Seizure,
- Pituitary involvement,

-PNS: mononeuritis multiplex

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102
Q

Cardiac Manifestations of GPA

A

RARE Pericarditis, or involvement of myocardium/coronary vasculature

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103
Q

GI Manifestations of GPA

A

Intestinal perforation

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104
Q

GU Manifestations of GPA

A

Bladder/urethral vasculitis
-Orchitis
-Epididymitis
-Prostatitis

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105
Q

GN histology GPA

A

-ABSENCE of immune complex deposition
-Focal &segmental
-Necrotizing and crescentic

106
Q

GPA histology

A

-Necrotizing vasculitis of small arteries/veins
-Extravascular & vascular wall granulomas
-Fibrinoid necrosis in vessel walls
- Infiltration of soft tissue by neuts, lymphs, macrophages, giant cells

107
Q

Generalized vs limited GPA

A

-Limited: NO Renal, pulm hemorrhage, orbital pseudotumor, progressive neuropathy, and CNS dz

108
Q

What to consider if hard palate lesions seen in ?GPA

A

Cocaine induced
-Infiltrative Ca (NK cell or T cell lymphoma)

109
Q

Bloodwork abnormalities in GPA

A

Anemia of chronic inflamm
-Leukocytosis
-Thrombocytosis
-ESR, CRP
-Low albumin
-Elevated globulin levels
-UA: hematuria, pyuria, casts, proteinuria

110
Q

How to differentiate GPA/MPA, Goodpastures, and SLE on imunofluorescence

A

Immunofluorescence:
-Goodpasture: linear deposition of anti-basement membrane AB binding epitopes in glomeruli/alveoli in basement membrane
-
-SLE: granular/lumpy deposition of Ig in glomerulus (immune complex deposition)
-
-GPA/MPA: NEGATIVE, scant Ig deposition in area of necrosis

111
Q

Mimickers of GPA (and how to tell)

A

eGPA (atopy, eosinophilia)
-MPA (NO granuloma, cavitary pulm nodules, or destructive upper airway dz)
-Lymphomatoid granulomatosis (NO GN)
-Goodpasture (antiGBM positive, linear deposition)
-SLE (ANA, dsDNA, Sm, granular deposition)
-Granulomatous infxn: mycobacterium fungi syphilis, actinomycosis
-Cocaine: anti neutrophil elastase antibody, nasal septum pathology
-Atrial myxoma: TTE
-IE: blood cultures
-CHolesterol emboli: TEE, angio, skin bx
-Lethal midline granuloma: NK T cell lymphoma, nasal/palate destruction

112
Q

**GPA induction therapy **

A

Pulse steroids
-CYC - IV (more relapse) = PO
-Ritux > CYC for induction/ relapsing dz
-CYC if severe (ARF Cr >4.0mg/dL)
-
-PLEX not recommended (consider if high risk ESRD PEXIVAS showed nonstatistically signif decrease in ESRD; not for DAH)
-
-Limited: MTX can be considered as steroid sparing agent
-
-** PPX SEPTRA 1DS MWF if >15-20mg/d pred (or dapsone, atovaquone, pentamidine if allergic)

113
Q

Why ritux over CYC based on evidence

A

-RITUXVAS: no diff in sustained remission at 12 and 24 mo (vs CYC-AZA)

114
Q

CYC IV vs PO

A

Relapse: IV>PO
-Leukopenia: PO>IV

115
Q

**PEXIVAS summary **

A

PLEX → no signif reduction of mortality and ESKD in pt w/ organ/life threatening AAV

116
Q

**RTX + GC tx 0.5mg/kg vs Ritux + GC 1mgkg **

A

Noninferior but excluded severe renal/pulm involvement
-Most in study were MPO (lower relapse risk)

117
Q

Induction MMF vs CYC

A

MMF noninferior but higher relapse in PR3

118
Q

Avacopan when and how to use

A

Avacopan 30mg BID for induction of new or relapsing GPA/MPA treated w/ CYC or RTX

-For induction: continue for one year

-DC GC by end of week 4

-**caution if GFR<15 and DAH req mech vent

119
Q

Maintenance Tx trials: RTX vs AZA

A

RITAZAREM & MAINRITSAN showed:
-RTX = less relapse than AZA
-RTX for 36mo > 18 mo

120
Q

AAV Maintenance Tx trials: MTX vs AZA vs MMF

A

Similar efficacy for maintenance but relapse higher in MMF vs AZA

121
Q

Tx subglottic stenosis

A

Radial CO2 laser incision, dilation
-Intralesional steroid injxn +/- topical mitomycin C

122
Q

CYC dosing

A

NIH: 0.5-1.0g/m2 monthly x3-6mo

123
Q

GPA maintenance therapy

A

AZA, MTX, Ritux within 3-6 mo after CYC start, 1-3years dep resp Mx, PR3 etc
-Ritux > AZA (MAINRITSAN)

124
Q

Options for treatment resistant GPA

A

PLEX

125
Q

** Preventing complication of GPA Tx **

A

CYC:
- Malignancy screening (esp >36g),
- Ovarian/sperm protection
- Decrease dose based on WBC
- Mesna for hemorrhagic cystitis

Infection:
- PJP ppx : septra 1ds mwf (or dapsone, atovaquone, pentamidine if allergic)
- Vaccinations
-IVIG if hypogammaglobulinemia

-OP prevention
-Nasal irrigation w/ mupirocin, budesonide, shampoo (as surfactant)

126
Q

MPA vessels affected

A

Small vessels: capillaries, venules, arterioles

127
Q

MPA presentation & histology

A

RPGN: Focal segmental necrotizing GN w/ crescents w/ NO immune deposits
-DAH/hemoptysis: Pulmonary capillaritis w/ NO immunofluorescence
-
-Fever
-Arthralgias
-Purpura
-PNS/CNS disease

128
Q

MPA vs PAN
Vessels involved
Pulm/Renal
Lab data
Angio results
Relapse

A

PAN:
- Small/medium sized muscular arteries
-NO RPGN or DAH
- HBV and RARE ANCA
- Necrotizing vasculitis
- Microaneurysm with vessel occlusion/stenosis of kidney, liver, spleen, stomach, small bowel
- Rare relapse

MPA:
- Capillaries, arterioles, or venules
-RPGN and DAH
- NO HBV
- MPO, pANCA
- Necrotizing vascultiitis (NO Granulomas)
- Angiography normal
- Rare relapse

129
Q

MPA vs GPA

A

NO granuloma in MPA

130
Q

MPA antibody

A

p-ANCA against MPO
-c-ANCA against PR3 in 15-30%

131
Q

eGPA vessels involved

A

Small and medium vessels

132
Q

eGPA patient phenotype

A

-Previous atopy
-Nasal polyps
-Asthma
-Eosinophilia

133
Q

Cytokines involved in eGPA

A

IL5 and eosinophil granule protein

134
Q

eGPA 3 phases

A

Prodromal: allergic sx of rhinitis, asthma, polyposis, recurrent fevers

-Peripheral blood/tissue eosinophilia: shifting pulm infiltrates and eosinophilia, chronic eosinophilic PNA, eosinophilic gastroenteritis, myocarditis, fevers

-Life threatening systemic vasculitis: asthma STOPS, myocarditis, valvular insuff, neuro sx (vasculitis peripheral neuropathy), eosinophilic gastroenteritis, pupura, testicular pain

135
Q

eGPA clinical features - paranasal sinus

A

Sinus pain/tenderness
-Rhinitis
-Polyposis

136
Q

eGPA clinical features - lungs

A

Asthma (adult onset)
-Pulm infiltrates- patchy, shifting, nodular, NO cavitations
-Effusions
-ILD
-Pulm hemorrhage

137
Q

eGPA clinical features - NS

A

Mononeuritis multiplex
-Asymmetric sensorimotor polyneuropathy
-Rarely CNS involvement

138
Q

eGPA clinical features - joints

A

Arthralgias, arthritis (RARE)

139
Q

eGPA clinical features - GI

A

Eosinophilic gastroenteritis (abdo pain, bloody diarrhea), abdo masses

140
Q

eGPA clinical features - misc

A

Renal failure (uncommon), CHF, corneal ulcerations, panuveitis, prostatitis

141
Q

eGPA lab findings

A

Eosinophilia with high IgE
-Anemia
-Inflamm markers
-RF in 70%
-pANCA against MPO

142
Q

** 6 rheum causes of peripheral eosinophilia**

A

-Eosinophilic Fasciitis/ Myositis / Myalgia syndrome
-IgG4 / Sjogren
-SLE
-RA
-eGPA
-Sarcoid

143
Q

** 6 nonrheum causes of peripheral eosinophilia**

A

Leukemia, lymphoma
-Infection: parasitic, helminth (eg strongyloides), fungi (histo, coccidiomyocosis, aspergillosis), viral (HIV)
-Allergic bronchopulmonary aspergillosis
-Asthma/atopy
-Eosinophilic PNA
-Drugs (NSAID, nitrofurantoin)
-IBD
-Idiopathic hypereosinophilic syndrome
-Cholesterol emboli
-Radiation exposure

144
Q

eGPA histopath

A

-Necrotizing granulomas (extravascular) with central EOSINOPHILIC core (not basophilic) surrounded by macrophages and giant cells
-Eosinophils > PMN and lymphocytes

-Necrotizing vasculitis of small arteries/veins

145
Q

Drugs to avoid in eGPA

A

Leukotriene inhibitors

146
Q

FFS what is it for and what are they

A

5 features w/ poor prognosis in eGPA:
-Cr > 1.58
-Proteinuria >1g/d
-CNS
-GI
-Myocardial involvement

147
Q

eGPA Tx

A

FFS 0: GC alone
-FFS≥1: GC + CYC
-
-Resistant: Ritux or mepolizumab (IL5 AB)

148
Q

Meds causing drug induced AAV

A

Thyroid: PTU, methimazole, carbimazole
-Hydralazine, minocycline
-Cocaine with levamisole

149
Q

Drug induced AAV manifestations

A

Constitutional sx
-Arthralgias w/ occasionals synovitis
-Cutaneous vasculitis
-Necrotizing GN (PAUCI IMMUNE)
-DAH

150
Q

Cocaine with levamisole manifestations (clinical)

A

Constitutional: fevers, sweats, weight loss
-ENT: sinusitis, septal cartilage necrosis and perforation
-Derm: Purpura, digital abscess, necrotic lesions, ecchymotic bullous skin lesions
-MSK: arthralgia
-Pulm: DAH
-Renal: GN
-Heme: leukopenia, neutropenia

151
Q

Levamisole cocaine vasculitis etiology

A

-Cocaine induces B cell activating factor secretion from neutrophils that produce more ANCA and cause more NETosis → mitochondrial DNA (immunogenic)

-Cocaine acts as haptan to trigger immune response an increase Ig formation and IC deposition → 2ndary hypercoag, tissue/skin thrombosis

152
Q

**Cocaine with levamisole manifestations (labs) **

A

-Bone marrow suppression → leukopenia, AGRANULOCYTOSIS, Thrombocytopenia
-AB against PR3, MPO,APLA
-Human neutrophil elastase

153
Q

Differential for purpura and LCV on biopsy

A

Infection: meningococcemia, gonococcemia, IE
-Cancer: paraneoplastic, lymphoproliferative dz
-Drugs: glucocorticoid induced,
-CTD, RA, EDS,
-Cholesterol emboli
-Idiopathic
-Thrombocytopenia: ITP, TTP, DIC, HITT
-Vasculitides: MPA, GPA, eGPA, IgAV, PAN, urticarial vasculitis, cryo vasculitis

154
Q

**Purpura Tx **

A

Colchicine
-Dapsone
-AZA
-MMF
-Prednisone
-Treat underlying problem
-DC drug

155
Q

**Erythema nodosum causes - 4 families w/ 3 examples each **

A

Idiopathic
-Pregnancy

-Systemic: Sarcoid, IBD, Celiac, SLE, APLA, RA, Ank Spond, TA, PAN, GPA, Sweet, SS,

-Infectious: TB, fungal (coccidiodo/ blasto/ histo), IE, viral (herpes), strep, leprosy, list any bacteria (E coli, staph, strep, campylobacter)

-Drugs: OCP, antibiotics (amox, penicillin, cipro, cephalosporin)

-Cancer: HL, NHL, leukemia, solid (GI/GU)

156
Q

IC-mediated SVV histopath / LCV features

A
  • Infiltration w/ PMN and mononuclear cells
  • FIBRINOID NECROSIS of vessel wall
  • Leukocyte fragments (leukocytoclasis)
  • Vessel wall destruction
  • Endothelial cell damage

-*eosinophils on biopsy suggest drug induced

157
Q

IC mediated V causes

A

New meds
-Recent infxns
-Hep C

158
Q

IC mediated V pathophys

A

Immune complex deposition in vessel wall → complement activation → PMN migration to area → lysosomal enzyme release and vessel wall damage

159
Q

IC mediated V other manifestations

A

Constitutional (fever, malaise)
-Arthralgia (RARE arthritis)
-GI: pain, GIB
-GU: proteinuria, hematuria, renal insuff

160
Q

IC mediated V causes

A

Hypersensitivity vasculitis (idiopathic or drug rxn)
-Urticarial vasculitis
-IgA vasculitis
-Cryo vasculitis (hep B, C, Ca, rheum dz)
-Rheum d/o (RA, SLE, SS, crohns)
-Infxn (SBE, Flu, Mono, HIV, Hep B/C)
-Ca (Leuko, lymphoma, myeloma, solid tumor, MDS, hairy cell)
-Anti GBM (NO SKIN)
-Erythema elevatum dilutinum (LCV limited to skin of extensor surfaces - knuckles, knees, butt)

161
Q

IC mediated V MIMICKERS

A

-APLA
-Livedoid vasculopathy
-Cholesterol emboli, Atrial myxoma, IE
-Low Plt: ITP, TTP, DIC
-Meningococcemia
-Calciphylaxis
-Pigmented purpuric dermatoses
-Scurvy
-Amyloid

162
Q

SVV Ix to send

A

CBC, Cr, UA, Hep B/C, RF, ANA, dsDNA, complements, ANCA, CRYO, HIV, SPEP, CXR, ESR/CRP

163
Q

IgA V histopath

A

LCV or necrotizing SVV
-Immunofluorescence shows IgA deposition in blood vessels and in glomerular mesangium

164
Q

IgA V pathogenesis

A

-IgA binds mesangial cells in kidney → prolif & proinflamm cytokines

165
Q

IgAV manifestations

A

TETRAD:
-GI: abdo pain, intussusception, hemorrhage, ileal perforation
-Renal dz (proteinuria, hematuria, ARF)
-Rash: macular erythema, urticarial lesions → purpura
-Arthritis: transient oligo, ankle/knee
-

166
Q

IgAV/HSP Tx

A

Mild: supportive,
-Arthritis: NSAID
-GI: pred 1mg/kg
-Renal: ACE+ARB + pulse steroids w/ high dose PO after if proteinuria >1g/d, nephrotic syndrome, >50% crescentic GN

-Resistant: AZA, CYC, Ritux

167
Q

AntiGBM Tx

A

Concurrent PLEX, high dose GC, and CYC

168
Q

Urticarial vasculitis subtypes

A
  • Normocomplementemic - idiopathic hypersensitivity
    -Hypocomplementemic (HUV) - low complement eg C1q
  • HUVS (HUV syndrome)
169
Q

Hypocomplementemic vasculitis syndrome criteria

A

HUV + 2 minor criteria
– LCV
– Arthralgia/arthritis
– Ocular inflamm
– GN
– Abdo pain
– Anti-C1q AB

170
Q

HUV manifestations

A

Urticaria
-Arthralgia
-Scleritis/uveitis/episcleritis
-Angioedema
-Obstructive pulm dz
-GN
-GI
-Cardiovascular disease

171
Q

HUV associations

A

75% in isolation
-25% assoc’d w/ SLE or SS

172
Q

Urticarial vasculitis vs urticaria differences

A

Duration: >24-48h w/ residual hyperpigmentation vs <24h w/o trace
-Pain vs Pruritus
-0.5-5cm vs >10cm
-Systemic sx (fever, MSK, LN, active urine) vs rare in allergic urticaria
-Histology: LCV vs edema of upper dermis
-Distribution: Trunk/prox extremities > distal in other LCV

173
Q

UV assoc’d conditions

A

Autoimmune: SLE, SS
-Ca: Lymphoma, IgM Paraproteinemia
-Infxn: Hep B, C
-Drug rxn

174
Q

UV pathophys

A

IgG2 antiC1q AB (C1q precipitins) bind collagen like regions of C1q → IC → deposit in blood vessel to activate complement → inflamm
-Lung involvement if antiC1Q AB binds collagen like regions of surfactant in alveoli

175
Q

UV Tx

A

-Dapsone, HCQ, colchicine
-
-MSK: NSAIDs, or pred from 10-60mg
-
-Severe: CYC, Ritux, AZA, MMF
-
-Refractory: PLEX, IVIG

176
Q

Erythema elevatum diutinum biopsy

A

LCV w/ fibrinoid necrosis
-Nonspecific immunoflueocescence

177
Q

Erythema elevatum diutinum assoc’d conditions

A

Same as UV: CTD, Infxn, paraproteinemias, esp IgA

178
Q

Erythema elevatum diutinum Tx

A

Dapsone, sulfapyridine

179
Q

SVV mimickers

A

-Cholesterol emboli, IE, Mycotic aneurysm w/ emboli
-APLA
-Sepsis (gonoccoccal , meningococcal)
-Ca (lymphoma, solid tumor, myeloprolif)
-Ecthyma gangrenosum (Pseudomonas)
-TTP
-Drugs: Cocaine, Amphetamines, Minocycline, Hydralazine,
-HIV, Hep C
-Amyloid, sarcoid
-SLE, RA, SS, IBD
-Immunodef
-Calciphylaxis
-Livedoid vasculopathy

180
Q

What are cryo

A

Igs/complexes that precipitated out of serum/plasma at low temp

181
Q

Cryo classification

A

Type
-1: SINGLE MONOclonal Ig
-2 (MC): MIXED MONOclonal Ig w/ RF activity against POLYclonal Ig
-3: MIXED POLYclonal Ig w/ RF activity against POLYclonal Ig

182
Q

How to collect cryo

A

At 37C: Collect blood, let clot x1 hr, centrifuge
-At 4C: incubate serum and centrifuge. Qualitative screen visually for cryoprecipitate vs quantitative using Wintrobe tubes
-
-If present: electrophoresis and immunofixation for isotypes and clonality

183
Q

Type 1 Cryo assoc’d disorders

A

Essential

Lymphoproliferative disorders:
- MM
- Waldenstrom’s macroglobulinemia
- Chronic lymphocytic leukemia
- B cell lymphoma

184
Q

Mixed cryo assoc’d conditions: ie Type 2/3

A

Essential

-CTD: ** SS **, SLE, RA, APLA, Behcet’s, AAV, IBD

-Infxn: ** HCV **, HIV, TB, IE, HBV, EBV, CMV, Parvo, brucellosis

-Heme Ca (waldenstrom, NHL, CLL)

185
Q

** Mech of tissue injury in cryo vasculitis and manifestations: Type 1 and 2/3

A

Type 1: Cryo aggregation and precipitation → vascular occlusion → Raynaud, arterial thrombosis, distal ulceration/necrosis, hyperviscosity

-Type 2/3: HCV protein binds CD81 on B cells → stim and B lymphocyte expansion → Igs w/ RF activity bind HCV components form cryoglobulin immune complex → vasculitis, Raynaud’s, pupura, arthritis, renal dz, neuropathy

186
Q

Cryo clinical manifestations: Type 1, 2/3, 6 MC

A

Type 1: Raynaud, arterial thrombosis, distal ulceration/necrosis, HYPERVISCOSITY (bleeding vision changes, neuro symptoms

-Type 2/3: arthritis (poly, nonerosive), raynaud’s, renal dz, neuropathy, skin manifestations

6 MC: cutaneous, liver dz, arthralgia, renal dz, raynauds, neurologic,

187
Q

Mixed Cryo skin manifestations

A

Purpura,
Urticarial vasculitis,
Digital necrosis,
Bullae,
Livedo racemosa

188
Q

Mixed cryo renal manifesations

A

Type 1 membranoproliferative GN
-Nephrotic syndrome
-Nephritic syndrome
-HTN
-AKI/ARF

189
Q

Mixed cryo neuro manifestations

A

Sensory polyneuropathy (symm/asymm, insidious/abrupt)
-Mononeuritis multiplex

190
Q

Lab findings mixed cryo vasculitis

A

ELEVATED RF AND HYPOCOMPLEMENTEMIA
-C4 reduced MORE than C3
-Polyclonal hypergammaglobulinemia or monoclonal gammopathy

Other tests to order:
- Ana, ANCA, ENA, CH50, HCV RNA, SPEP

191
Q

Mixed cryo vasculitis Dx

A

High RF, low complements, and presence of monoclonal gammopathy
-LCV on biopsy and intravascular hyaline thrombi
-Immunofluorescece: Ig and C3 deposition
-Liver/BM biopsy may show clonal expansion fo B cells

192
Q

Poor prognostic factors for mixed cryo vasculitis

A

Male
-Age>60
-Type 2
-GI involvement
-Chronic HCV
-Diffuse vasculitis

193
Q

** Mixed cryo vasculitis Tx options **

A

GC (high dose if neuro/renal/diffuse vasculitis)
-PLEX if hyperviscosity, high cryo [ ], or signif renal insuff
-CYC for severe WITH PLEX
-Colchicine - for pain, weakness, pupura, leg ulcers
-Low antigen diet
-Antiviral (interferon based or direct acting) + RTX > either alone

194
Q

** Severe mixed cryo vasculitis + Tx (induction and maintenance) **

A

Ulcers/ischemia
-DAH
-Severe neuropathy
-GN w/ renal failure or nephrotic syndrome
-GI involved
-
-Ritux + Steroids → antiviral for maintenance

195
Q

Mild/mod mixed cryo vasculitis manifestations
-+ Tx (induction and maintenance)

A

-Purpura
-Mild neuropathy
-GN w/o renal failure

-Antiviral therapy +/- steroids → antiviral for maintenance

196
Q

Life threatening mixed cryo vasculitis manifestations
+ Tx (induction and maintenance)

A

-RPGN
-CNS involved
-Intestinal ischemia
-DAH

-PLEX + GC pulse + Ritux or CYC → antiviral for maintenance

197
Q

Cryofibrinogenemia - what is it

A

Insoluble complex of fibrin, fibrinogen, fibrin split products, plasma prot and Igs that precipitate from PLASMA (NOT SERUM) w/ cold

198
Q

Cryofibrinogenemia manifestations

A

Derm: vascular occlusion/ischemia → pupura, livedo, ulcers, gangrene, strokes, MI
-** Paradoxical bleeding ** bc clotting factors depleted

199
Q

Cryofibrinogenemia assoc’d conditions

A

CTD, infxn, Ca, drugs (OCP), DM, advanced DLPD

200
Q

Cryofibrinogenemia Tx

A

Avoid cold
-Smoking cessation
-Treat underlying
-Fibrinolytics (eg streptokinase)
-Low dose ASA
-GC for mild/mod
-DMARD for underlying CTD

201
Q

Cold agglutinin disease - pathophys

A

IgM AB against erythrocytes → hemolytic anemia and complement mediated RBC destruction in reticuloendothelial system (liver) → occlusion by agglutinated RBC

202
Q

Cold agglutinin disease - manifestations

A

Acrocyanosis
-Raynaud’s
-Ulcers over ears, nose, digits

203
Q

Cold agglutinin disease - Tx

A

Warmth
-GC
-RTX
-Rarely PLEX
-Treat underlying

204
Q

Examples of variable vessel vasculitis

A

Bechcet
-Cogan

205
Q

Behcet’s criteria (revised international criteria for adamantiades - behcent’s disease)

A

Recurrent oral ulcers at least 3x in one 12mo period
+ 2 of following:

  • Recurrent genital ulceration
  • Eye lesions (anterior/posterior uveitis, hypopyon (cells in vitreous)**, retinal vasculitis
  • skin lesion: pseudofolliculitis, papulopustular, erythema nodosum like, acneiform, pyoderma gangrenosum like
  • Positive pathergy (2mm erythema 1-2d after needle prick to 5mm)
206
Q

Behcet’s mimickers

A

IBD: Crohn’s
-SLE
-ReA
-Herpes
-Systemic vasculitis
-Sweet’s
-Periodic fever syndrome (PFAP, HIDS)

207
Q

Behcet’s histopath

A

Variable: from neutrophilic vascular rxn to LCV

208
Q

HLA assoc’d w/ Behcets

A

HLA B51

209
Q

Aphthous stomatitis differential

A

Idiopathic
-Metabolic deficiency: B12, folate, Fe
-Autoimmune: IBD, Behcets, SLE, ReA, Celiac, Autoinflamm (PFAPA, HIDS)
-Drug: MTX, SJS
-Infxn: HIV, HSV
-Recurrent (complex) apthosis
-Menstrual related
-Cyclic neutropenia
-Derm: pemphigoid, pemphigus, lichen planus

210
Q

Genital ulcers differential

A

Infxn:
– Venereal: HSV, syphilis, chancroid, LGV, granuloma inguinale
– Nonvenereal: pyogenic, yeast,

Inflamm:ReA, Crohn’s, Behcet
Trauma (mech, chem),
Cancer: BCC, SCC
Drug reaction

211
Q

Behcet’s eye manifestations

A

Ant/Post/Pan uveitis (bilateral, episodic)
-Conjunctivitis
-Corneal ulceration
-Papillitis
-Retinal vasculitis
-Optic neuritis
-Hypopyon (inflamm cells in anterior chamber of eye)

212
Q

Behcet’s MSK manifestations

A

Arthralgia > arthritis - mono, oligo, poly
-RARE erosions
-Enthesopathy

213
Q

Behcet’s Derm manifestations

A

Erythema nodosum
-Superficial thrombophlebitis
-Acneiform skin eruption or pseudofolliculitis
-Pyoderma gangrenosum like lesions
-Sweet’s
-Cutaneous SVV and pustular vasculitic lesion
-Pathergy

214
Q

Behcet’s Vascular manifestations

A

-Thrombosis: SVC, IVC, portal or hepatic veins, pulm arteries, DVT
-Arterial aneurysm of pulm arterial tree MORTALITY RISK
-Superficial thrombophlebitis
-Small artery vasculitis

-*Hughes Stovin = DVT + pulm artery aneurysm w/ hemoptysis

215
Q

Behcet’s Neuro manifestations

A

B/A
-Meningoencephalitis
-CN palsy
-Seizure
-Cerebellar ataxia
-Hemiplegia/Paraparesis
-Pseudobulbar palsy
-Extrapyramidal signs
-Intracranial HTN from dural sinus thrombosis

CNS MANIFESTATIONS MORTALITY RISK

216
Q

Behcet’s GI manifestations

A

Mucosal ulceration → perforation
MORTALITY RISK

217
Q

Behcet’s Cardio manifestations

A

RARE
-Valvular lesions
-Myopercarditis
-Coronary arteritis/aneurysm

218
Q

Behcet’s GU manifestations

A

GN is rare
-Epididymitis
-Salpingitis

219
Q

Behcet’s lab findings

A

Inflamm markers
-Leukocytosis
-Elevated IgG/A/M
-Elevated CSF prot/cell count

220
Q

** Behcet’s cause for mortality**

A

CNS involvement
-Valvular dz (ruptured pulm/periph aneurysm)
-Bowel dz (perf)

221
Q

** Behcet’s Tx for skin lesions**

A

Topical triamcinolone / sulcrafalate
-Dexamethasone swish and spit
-Oral Colchicine (for eyrthema nodosum and genital ulcers)
-GC - topical, intralesional, PO
-Apremilast if fail topical, colchicine, and can’t taper pred **
-AZA > MMF
-TNFi (WITH AZA)**
-Interferon alpha
-Dapsone, cyclosporine, IL1i

222
Q

** Behcet’s Tx for eye dz**

A

Topical (anterior uveitis), intraocular, and systemic GC (posterior)
-AZA + GC for posterior (uveitis, retina, optic nerve)
-TNFi (INFLIXIMAB best) WITH AZA or other DMARD +/- Cyclosporine (can worsen CNS behcet) or tacro
-Interferon alpha (NOT with AZA bc myelotox)
-Salvage: CYC, chlorambucil
-Others: MTX, MMF, Anakinra, Toci, Ritux

223
Q

** Behcet’s Tx for MSK **

A

Colchicine
-Refractory: GC, AZA, MTX, SFZ TNFi, Interferon-a

224
Q

** Behcet’s Tx for CNS **

A

GC
-AZA
-Interferon a
-CYC
-TNFi
-*Do not use cyclosporine unless coexisting ocular disease

225
Q

** Behcet’s Tx for GI**

A

GC
-SFZ
-AZA
-TNFi

226
Q

Behcet’s Tx for vascular thrombosis

A

GC and immunsuppressives
-**do not anticoag until inflamm controlled bc silent aneurysm can cause hemorrhage → death

227
Q

Behcet’s Tx for aneurysm/vasculitis

A

GC, CYC
-Endovascular embolizatin/surgery for hemorrahge

228
Q

**Biologics for Behcets*

A

TNFi - Infliximab, Humira, Etanercept

229
Q

**Nonbiologics for Behcets*

A

AZA
-Cyclosporine
-Apremilast
-Colchicine

230
Q

MAGIC

A

Mouth and genital ulcerations with inflamed cartilage
-*ie behcets and relapsing polychondritis

231
Q

Behcet’s pathogenesis

A

Infectious trigger in genetically predisposed host
-Heat shock protein release → interact w/ TLR → Cytokine release (IL1/8/12/17) → neutrophil and T helper 1 cell hyperactivity

232
Q

Cogan manifestations

A

-Ocular

-Audiovestibular

-Constitutional

-Vasculitic(large medium),

233
Q

Cogan Audiovestibular manifestations

A

Rapid onset sensorineural hearing loss (often bilateral)
-Vestibular dysfunction (vertigo and ataxia)
-Cochlear hydrops
-Tinnitus

234
Q

Cogan constitutional manifestations

A

Fever
-Weight loss
-LN
-HSM
-Arthritis

235
Q

Cogan vasculitic manifestations

A

-Aortitis w/ aortic insuff/aneurysm
-Coronary vasculitis
-Aortic/mitral valvulitis
-Purpura
-Gangrene

236
Q

** Cogan Ocular manifestations**

A

Nonsyphilitic interstitial keratitis (Red, painful, photophobic eyes)
-Scleritis/episcleritis
-Uveitis
-Chorioretinitis
-Optic neuritis
-Retinal vasculitis

237
Q

Cogan lab manifestations

A

Low Hgb, high WBC/Plt/inflamm
-RARE: ANCA, ANA, APS, RF,

-Anti HSP70 AB

238
Q

Cogan Tx

A

-Anterior ocular eg keratitis: topical steroids, topical CNI
-Posterior ocular: high dose GC
-Audiovestibular or Systemic: High dose GC

-Resistant or failure to taper GC: MTX, AZA, MMF

-Others: CYC, cyclosporine, TNFi, Toci

-Cochlear implants
-Vascular surgery (AoV replacement or aneurysm repair)

239
Q

Vasculitis mimic for Old guy with jaw claudication but normal CRP and test to rule it in

A

Atherosclerosis → carotid US
-TMJ disease → MRI

240
Q

Vasculitis mimic for Polyneuropathy, cardiac involvement, pulmonary infiltrates, and eosinophilia but no asthma

A

Sarcoid → ACE, CXR, biopsy
-IgG4rD → biopsy
-Lymphoma/heme malignancy → biopsy/flow cytometry

241
Q

**Vasculitis mimic for Mechanical heart valve with active urinary sediment **

A

IE → TTE, cultures

242
Q

Vasculitis mimic for 30 yr M with severe emphysema and ischemic digit

A

Buerger’s → Angio

243
Q

arthritis in Lyme vs Behcet vs Sarcoid vs HSP, and additional features

A

L: Migratory arthralgia → late episodic knee mono or asymmetric oligoarthritis
CN palsy, meningitis, AV block, myopericarditis

-B: mono/oligo/poly nonerosive distal medium joints (ankle, knee, wrist, elbow), with ulcers and enthesopathy

-S: symmetric (can be erosive), distal medium + PIP;
LN, granuloma, dactylitis.
Loftren’s (nodosum, LN, MSK),
Heerfordt (parotid, uveitis, facial nerve palsy)

-H: lower extremity large oligo (nondeforming). periarticular swelling

244
Q

Single organ vasculitidities

A

Cutaneous LCV
-Cutaneous arteritis
-Primary CNS vasculitis
-Isolated aortitis

245
Q

Vasculitis assoc’d w/ systemic dz

A

SLE, RA, Sarcoid vasculitis

246
Q

** SVV causes**

A
  • AAV: GPA, eGPA, MPA
  • IC: Cryo, IgA, Hypocomplementemic Urticarial (Anti C1q)

2ndary:
- Infection: Hep B/C, HIV, IE
- Drugs: Levamisole, penicillin, cephalosporin, diuretics, phenytoin, allopurinol
- CTD: SLE, RA, Sjogren
- Malignancy: lymphoma, leukemia, myeloma

247
Q

** Best time to biopsy LCV**

A

<24h = PMN infiltration w/o fibrinoid necrosis
24-48 = most likely to demonstrate LCV
>48h = inflamm cells –> lymphocytic and macrophages (vs PMN) and clear quickly leaving fibrinoid necrosis and nothign else

248
Q

** eGPA Cause of Death**

A

MI/CHF

249
Q

** GPA of larynx Tx**

A

Steroids +/- CYC

Endoscopic tx:
- Bronchoplasty (airway dilation)
- Airway stents
- Intralesional GC injxn into granuloma
- Dilation of subglottic lesion
- Carbon dioxide laser
- Tracheostomy

250
Q

eGPA classification criteria

A

Apply only when SVV or MVV diagnosis made

Clinical:
-Obstructive airway disease (+3),
-Nasal polyps (+3),
-Mononeuritis multiplex (+1)

Lab/Biopsy criteria
- Eosiniophilia >1E9 (+5)
- Extravascular eosinophil predom inflamm on bx (+2)
- cANCA or PR3+ (-3)
- Hematuria (-1)

251
Q

** Conditions assoc’d w/ mononeuritis multiplex**

A

Inflamm: RA, SS, SLE, GPA, PAN, Cryo, HUVS
Infxn: leprosy, sarcoid, lyme, HIV, EVC, CMV, Hep B/C
Metabolic: DM, Lead neuropathy
Infiltrative: sarcoid, amyloid, malignancy
Trauma
Ischemic: sickle cell, cholesterol emboli, livedoid vasculopathy
Wartenburg’s relapsing sensory neuritis

252
Q

**Neuropathic patterns seen in vasculitis **

A

Mononeuritis multiplex
Stocking glove distribution

253
Q

GCA Classification Criteria

A

Apply only when SVV or MVV diagnosis made
REQUIRES Age 50 or more
Positive if 6 or more points

Clinical:
-AMS neck/shoulder (+2),
-Sudden vision loss (+3),
-Jaw claudication (+2)
-NEW temporal headache (+2)
-Scalp tenderness (+2)
-Abnormal exam of temporal artery(+2)

Lab/Imaging/Biopsy criteria
- Maximum ESR >50 or CRP >10 (+3)
- Positive TAB or halo sign (+5)
- Bilateral axillary involvement (aneurysm, stenosis, occlusion on angio, CT, MR, US, or PET) (+2)
- FDG PET activity throughout aorta (+2)

254
Q

** Techniques to look for ANCA**

A

Indirect immunofluorescence
- Serum mixed w/ neutrophils fixed on slide
- ANCA from serum attaches to neutrophils and is detected w/ AB
- Titre reported and staining pattern (perinuclear or cytoplasmic)

ELISA with plate coated w/ PR3, MPO
- Incubate patient serum w/ ANCA
- Enzyme added with radiolabel that targets ANCAs

255
Q

** Stroke in Young DDX **

A

Inflamm/Infection
- SLE, APS
- Vasculitis (PACNS, GPA, TAK, Behcet, Cogan)
- Relapsing polychondritis
- RA, Sjogrens
- Neurosarcoid
- HIV, VZV, Neurosyphilis, TB

Nonatherosclerotic angiopathy:
- Amyloid
- Moyamoya
- FMD
- Susac
- Sneddon
- RCVS

Heme:
- Hypercoag from deficiency of Prt S/C/AT, Factor V mutation
- Acquired hypercoag: preg, OCP, steroids, nephrotic syndrome
- Sickle cell

256
Q

** ANCA disease association and % **

A

pANCA:
- MPA 50-60%
- eGPA 25-30%

cANCA
- GPA 75%

257
Q

** Erythema nodosum biopsy**

A

Panniculitis involving septa in subcutaneous fat

258
Q

Cryo path

A

Precipitated cryo on light microscopy
Diffuse IgM deposition in capillary loops in immunofluorescence

259
Q

**Components found in cryoprecipitate **

A

Fibrinogen.
Factor VIII.
Factor XIII.
Von Willebrand factor.

260
Q

** GCA how to biopsy**

A

Unilateral > Bilateral Within 2 weeks
Long segment >1cm preferred

261
Q

** Young patient with arthritis, oral ulcer and uveitis: List 5 differential diagnosis?**

A

Seroneg: PEAR
Behcet
Vasculitis: GPA
SLE
Relapsing polychondritis

262
Q
A

1. Rheum RC (23 decks)

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