Lec 19: Vasculitis

Question 1

First of all, manifestations of vasculitis vary so widely that the key to diagnosing vasculitis is what

Answer 1

clinical suspicion

Question 2

Large vessel Vasculitis

Which one commonly causes Renal HTN?

What clinical features do you see that scream GCA?

What other disease is usually concomitant with GCA?

What does GCA respond well too ASA far as medication management?

What does biopsy show in temporal arteritis?

How to treat GCA?

Answer 2

takayasu and temporal arthritis/giant cell arteritis

-takayasu
Young, Asian females
Affect aorta and its branches
Commonly causes renal HTN
”pulseless disease”

-temporal arteritis/ GCA
Infiltrative vasculitis w/ formation of giant cells in large/medium arteries
Usually older than 50 year old
Affects external carotid artery branches
Clinical features seen are new onset headache, jaw Claudia action, and vision symptoms!

polymyalgia rheumatica (aching and stiffness of proximal muscle groups)

*responds well to steroids and IL-6 inhibition —>tocilizumab

Biopsy shows disruption of the vessel wall layers

Treat GCA with high dose steroids high dose steroids
Check bone density at start and every 6 months for osteoporosis; supplement and taper as fast as tolerated

Question 3

Medium vessels w/ vasculitits

What is Polyangitis nodosa associated with?

PAN does not have ______, ________, or ___________

PAN does have ___________, _________, and ___________

Answer 3

polyangitis nodosa and Kawasaki disease

Polyarteritis Nodosa
non-ANCA systemic medium vessel vasculitis commonly a/w HEP B!!! causing tissue ischemia !!!
look for aneurysms on CT/MRA

DOES NOT HAVE
No ANCA
No pulm involvement
No kidney involvement

DOES have
Systemic sx, CNS involvement (aneurysms), peripheral neuropathy, and cutaneous involvement = it is necrotizing

Kawasaki.. a rare childhood dz

Question 4

Small Vessel Vasculitidies

ANCA associated small vessel vasculitis

Immune complex associated

Answer 4

Microscopic PAN, EGPA/churn-strauss, GPA/wegener’s, Enoch-schonlein

ANCA associated: Microscopic PAN, EGPA/churg-strauss, GPA/Wegeners

-microscopic PAN: p-ANCA
-EGPA: p-ANCA
-GPA: c-ANCA

Immune-complex associated: henoch-schonlein purpura
cryoglobulin related, hypersensitivity aka allergic vasculitis

Question 5

What is microscopic polyangitis nodosa?

Answer 5

Microscopic PAN
-necrotizing glomerulonephritis
-alveolitis/pneumonitis ( w ground glass appearance )
-renal HTN
-no Hep B association unlike…
-no Granulomas
-Tx with RITUXIMAB

Question 6

What is Churg-strauss?

What is Wegener’s?

Answer 6

EGPA aka Churg-Strauss: esosinophil rich granulomatous inflammation of resp tract: a/w asthma and esosinophilia
-interstitial nephritis = p-ANCA (anti-MPO)
-manifests in skin, neuro, pulm, joints and GI
-think atopy = eosinophils, IgE, asthma, AIN
Tx with Mepolizumab

GPA: granulomatous inflammation of respiratory tract with necrotizing inflammation of small and medium BV
-ELK or E for ENT, L for lungs, and K for kidney

Question 7

Henoch-Schonlein Purpura

Cryoglobulin related

leukocytoclastic vasculitis

Behcet disease

Answer 7

Tetrad of sx
Purpura + arthritis + abd pain + renal disease
mostly children with increased igA levels, preceded by Strep URI or GI infection

-PAPAH: Peds call their papah: *purpura, ab pain, arthralgia, heme in feces and urine

Hep C associated with Type II (mixed monoclonal igG + polyclonal igG)
-type I is monoclonal; type III is mixed polyclonal IgM and igG

Hypersensitivity. Allergic vasculitis.
Caused by infections, medicine, idiopathic

recurrent oral/genital ulcers, uveitis, arthritis, vasculitis, thrombosis
Zero negative, a/w HLA-B51
goodpasture’s = pulmonary and renal involvement with anti-GBM antibodies