1. Vasculitis Review: Intern Conference
Jason Haag

2. Overview Nomenclature of vasculitis
Overview of most important diseases
Quiz review

3. Nomenclature
Vasculitis: Inflammation on blood vessels causing vessel and tissue damage
Classification is according to the Chapel Hill Consensus Conference of 1994
Large-vessel vasculitides (2)
Medium-vessel vasculitides (3)
Small-vessel vasculitides (many)
ANCA-associated (5)
Non-ANCA-associated (many)

4. Jennette and Falk, Curr Op Rheumatol, 2007

5. Large Vessel Vasculitides (2)
Giant cell arteritis (formerly called temporal arteritis)
Takayasu’s arteritis
Distinguished only by age of pt > or < 50 yo
Both involve the aorta and its primary branches

6. Medium Vessel Vasculitides (3)
Polyarteritis nodosa (PAN)
Buerger’s = Thromboangiitis obliterans
Kawasaki’s disease = Mucocutaneous LN syndrome

7. Small Vessel Vasculitides (many)
ANCA-associated (5)
Wegener’s granulomatosis (WG)
Churg-Strauss syndrome (CSS)
Microscopic polyangiitis (MPA) – no granulomas
Drug-induced ANCA vasculitis
Idiopathic

8. Small Vessel Vasculitides (many)
Non-ANCA-associated (many)
Infectious (Hep B, Hep C, HIV, Parvo, CMV, EBV)
CVD-related (RA, SLE, Bechet’s, Sjogren’s)
Drug-related (cutaneous leukocytoclastic vasculitis)
Henoch-Schonlein Purpura (HSP) – IgA deposits
Goodpasture’s disease
Cryoglobulinemia
IBD-related
Urticarial vasculitis
Paraneoplastic

9. Large Vessel Vasculitis: Takayasu’s arteritis
Affects women < 40 yo
Prepulseless phase: systemic sx
Pulseless phase: arm claudication, HA, dizziness, visual changes, decreased arm pulses or pressure differential, subclavian/abdominal bruits
Tx with Prednisone
Fever, weight loss, fatigue

10. Takayasu’s arteritis
MRA

11. Large Vessel Vasculitis: Giant Cell Arteritis
Sx: fatigue, HA, visual changes, scalp tenderness, jaw claudication
Often associated with PMR (pain and stiffness in neck, shoulder, pelvic girdles)
Prompt corticosteroids prevent blindness

12. Medium Vessel Vasculitis: Polyarteritis Nodosa (PAN)
Peaks in 5th decade, slight male preponderance
Sx: constitutional sx, skin lesions (purpura, livedo reticularis, ulcers, nodules), mononeuritis multiplex, hematuria, abdominal pain, testicular pain
Aneurysms and narrowing on MRA
Associated with Hep B
Tx with Prednisone, cyclophosphamide

13. Polyarteritis Nodosa (PAN)

14. Medium Vessel Vasculitis: Buerger’s Disease
Young, y.o
Male
Inflammation/thrombosis in hands/feet  pain
Recent or current tobacco use
Tx with smoking cessation

15. Medium Vessel Vasculitis: Kawasaki’s Disease
Classic Dx
5 days of fever + 4 of 5 criteria
Erythema/cracking of lips
strawberry tongue
Trunkal rash
Swelling of hands/feet
Conjuctivitis
Cervical LAD
Also can have joint pain, coronary aneurysms, myocarditis
Tx: IVIG

16. Medium Vessel Vasculitis: Kawasaki’s Disease

17. Small Vessel Vasculitis: Churg-Strauss Syndrome
Middle-aged adults
Asthma, eosinophilia (>10%), neuropathy (mono- or poly- in stocking/glove), pulmonary infiltrates
ANCA+ vasculitis
Tx: Prednisone, cyclophosphamide

18. Small Vessel Vasculitis: Wegener’s Granulomatosis
Granulomas in upper resp tract (sinusitis, otitis, epistaxis), lower resp tract (hemoptysis), and kidneys (GN), can involve eye and nose
Tx: Stx, cyclophosphamide

19. Small Vessel Vasculitis: Goodpasture’s Syndrome
Hemoptysis/hematuria
Linear IgG deposits
across basement
membrane
Tx:
Stx, plasmapheresis

20. Small Vessel Vasculitis: Bechet’s Syndrome
Highest in Iran, Turkey, and Japan
Recurrent oral and genital ulcers
Uveitis causes blindness in 10%
Skin pathergy
> 2mm nodule after 48 hours
Multiple systems involve
Venous thrombosis
Tx: Stx, TNF blocker
T= 0
T = 24h
T= 48h

21. Small Vessel Vasculitis Henoch Schonlein Purpura
Most common in children aged 3-15, but also occurs in adults
Presents with classic tetrad of purpuric rash, abdominal pain, renal disease, and arthralgias
Can progress to renal failure (adults)
Tx: Pain control, Stx if neccessary

22. Small Vessel Vasculitis: Cryoglobulenemia
Nonspecific Sx
Arthralgias, myalgias, fever, glomerularneprhitis
Palpable pupura
Associated with
Hep C, RA, SLE
Low complement levels
Tx primary disease

23. Quiz Review