Lecture 99/100 - path and clinical aspects of vasculitis

Question 1

what are the three classes of vasculities

Answer 1

large vessel – (aorta, arotic arch, branches)

Medium vessel – (muscular arteries) - vessels which feed the organs

Small vessel

Question 2

what are the 2 large vessel vasculitises

Answer 2

Temporal (Giant Cell) arteritis

Takayasu Disease

Question 3

what are the 3 medium vessel arteritis

Answer 3

Polyarteritis Nodosa

Kawasaki Disease

Buerger Disease

Question 4

what are the 6 small vessel arteritis

Answer 4

GPA (Wegner’s Disease – Granulomatosis with Polyangitis)

MPA (Microscopic Polyangitis)

EGPA ( Churg Struass Syndrome – Eosinophilic Granulomatosis with Polyangitis)

Henoch Schonlein Purpura – IgA Vasculitis

Hypersensitivity Vasculitis –

Cryoglobullonemic Vasculitis

Question 5

Temporal (Giant Cell) Arteritis –

who is affectd; 
what are the classic histo features
Clinical manifestations
may be associated with\_\_\_\_\_\_which presents as \_\_\_\_\_\_
Complications:
Treatment:

Answer 5

Older individuals (>50yo); women; northern European descent

Histo: loss of internal elastic lamina to inflammation; Giant cells + Intimal Fibrosis

Clinical manifestations:
Temporal Artery – HA + tenderness to temporal artery
Ophthalmic artiery –visual disturbance
Jaw Claudication

May be a/w polymyalgia Rheumatica – presents as Flu like; Girdle weakness

Complications: Irreversible blindness

Treat: ASAP with steroids

Question 6

you are suspicious for Giant cell arteritis, but the biopsy is negative, how should you proceed?

what must be taken into consideration for the biopsy?

Answer 6

treat with Corticosteroids as soon as possible

if high clinical suscpicion, don’t hold off on treatment

the biopsy may be false negative considering Lesions are segmental and requires a long strip of artery to detect

Question 7

Takayasu Disease –

who is affected?
what vessels are commonly affected?
what is the gross path described as?
Clinical presetnation (acute vs chronic) 
Complications -- 
Treatment:

Answer 7

Granulomatous Vasculitis that affects the aortic Arch at Branch Points

who: asian females

Question 8

polyarteritis Nodosa

what organs can be involved?
what organ is notable spared? 
Histopath findings?
Gross path/angiogram findings? 
Clinical manifestations -
treatment

what virus is this associated with 30% of the time

Answer 8

(nodosa = nodular) – string of peals/beads

organs: Kidney, Heart, Muscle, Skin, Mesnetery
Spared: NO LUNG INVOLVEMENT
Histo path: transmural fibrinoid necrosis
Gross/angiogram: String of peals/beading – nodules
Manifestations:
constituional symptoms, HTN (renal artery), Abd Pain, Melena, Foot drop, Skin nodules/purpura, Testicular Pain

treat: Corticoisteroids, cyclophosphamide

a/w HBV 30% of the time

Question 9

\Kawasaki Disease —

what is it?
who is affected?
Classical clinical presentation:

Complications:

Treatment:

Answer 9

Asian Children

Question 10

children with viral illness + given ASA =

Answer 10

Reye’s Sydrome;;

Question 11

What is buerger Disease?
(aka?)

who:
Cliincal manifestations:
Treatment:

Answer 11

Thromboangitis Obliterans
Necrotizing vasculitis of the digits –
In Male Smokers > 40 yo

gangrene, ulceration and autoamputation of the fingers and toes; Raynaud phenomena

treat: Stop smoking

Question 12

GPA (Granulomatosis with Polyangitis)

Formerly called?
what is it? 
who is affected?
Clinical manifestations (triad or organs) 
Pathology -- (vessels vs kidneys)
Labs: 
Treatment

Answer 12

Wegner’s Syndrome (weCners)

what: Necrotizing Granulomatous vasculitis involving the nasopharynx, lungs and kidneys
who: middle aged men
clinical manifeatsion:
Nose - -sinusitis/Nasal Ulcers
Lungs- nodular infiltrates, Pulm HTN
Renal – Hematuria, RPGN (crescentic)

Path -
Renal - crescenting
vessels - granulomas

Labs: cANCA

Treatment: Cyclophosphamide, Rituximab

Question 13

MPA (Microscopic Polyangitis)

how is this different from GPA
What organs can be affected?
Pathology --
Labs
Treatment:

Answer 13

Different from GPA: no Granulomas and No Nasopharynx

Organs; Lung, kidney, skin GI, brain

Path:
crescentic GN,

P Anca

Treatment: cyclophospohamide, Corticostreoid

Question 14

EGPA (Eosinophilic Granulomatosis with Polyangitis = Churg Struass Syndrome)

what?
who?
clinical manifestations?
Labs

Answer 14

What: Necrotizing Granulomatous inflammation with Eosinophils involving multiple organs, especially lungs and heart

who: history of asthma and allergic rhinitis
clinical: Mononeuropathy, Pulmonary opacitis, Crescentic GN, Cardiac arrythmias,
labs: P-ANCA

Question 15

what are the ANCA associated vasculitic disease?
which are p vs c anca
on IF what is the distribution of p vs c anca

Answer 15

cAnca = Wegners
IF: cycoplasmic staining pattern (everything but the nuc)

P Anca - EGPA, MPA
IF: Perinuclear staining pattern

Question 16

Young patient presents with palpable purpura do his lower extremities; he recently recovered from a URI last week. what is your leading dx?

Answer 16

Henoch Schonlein Purpura – IgA Vasculitis

Question 17

Henoch Schonlein Purpura – IgA Vasculitis

what is it?
who?
clinical manifestations:
treatment:

Answer 17

Vasculitis due to IgA immune complex deposition

Most common vasculitis in Children (

Question 18

a Patient presents with palpable purpur and gross hematuria— patient recently started taking XYZ drug …what is your leading diagnnosis?

what do you expect to find on histopath?

Answer 18

Hypersensitivity Vasculitis — secondary to Drug intake

histopath: Leukocytoclastic vasculitis

Question 19

what is ○ Cryoglobullonemic Vasculitis ?

what is the unique lab finding?

Answer 19

Vasculitis associated with Cryoglobulins – mixture of Ig + Complement that deposit in the walls of small vessels, capillaries, venules, arterioles

Cryoglobulins precipitate when cold –