Title: Vasculitis Review: Intern Conference
1Vasculitis ReviewIntern Conference
2Overview
- Nomenclature of vasculitis
- Overview of most important diseases
- Quiz review
3Nomenclature
- 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)
4Jennette and Falk, Curr Op Rheumatol, 2007
5Large Vessel Vasculitides (2)
- Giant cell arteritis (formerly called temporal
arteritis) - Takayasus arteritis
- Distinguished only by age of pt gt or lt 50 yo
- Both involve the aorta and its primary branches
6Medium Vessel Vasculitides (3)
- Polyarteritis nodosa (PAN)
- Buergers Thromboangiitis obliterans
- Kawasakis disease Mucocutaneous LN syndrome
7Small Vessel Vasculitides (many)
- ANCA-associated (5)
- Wegeners granulomatosis (WG)
- Churg-Strauss syndrome (CSS)
- Microscopic polyangiitis (MPA) no granulomas
- Drug-induced ANCA vasculitis
- Idiopathic
8Small Vessel Vasculitides (many)
- Non-ANCA-associated (many)
- Infectious (Hep B, Hep C, HIV, Parvo, CMV, EBV)
- CVD-related (RA, SLE, Bechets, Sjogrens)
- Drug-related (cutaneous leukocytoclastic
vasculitis) - Henoch-Schonlein Purpura (HSP) IgA deposits
- Goodpastures disease
- Cryoglobulinemia
- IBD-related
- Urticarial vasculitis
- Paraneoplastic
9Large Vessel VasculitisTakayasus arteritis
- Affects women lt 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
10Takayasus arteritis
MRA
11Large 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
12Medium Vessel VasculitisPolyarteritis 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
13Polyarteritis Nodosa (PAN)
14Medium Vessel VasculitisBuergers Disease
- Young, 20-40 y.o
- Male
- Inflammation/thrombosis in hands/feet ? pain
- Recent or current tobacco use
- Tx with smoking cessation
15Medium Vessel VasculitisKawasakis 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
16Medium Vessel VasculitisKawasakis Disease
17Small Vessel VasculitisChurg-Strauss Syndrome
- Middle-aged adults
- Asthma, eosinophilia (gt10), neuropathy (mono- or
poly- in stocking/glove), pulmonary infiltrates - ANCA vasculitis
- Tx Prednisone, cyclophosphamide
18Small Vessel Vasculitis Wegeners Granulomatosis
- Granulomas in upper resp tract (sinusitis,
otitis, epistaxis), lower resp tract
(hemoptysis), and kidneys (GN), can involve eye
and nose - Tx Stx, cyclophosphamide
19Small Vessel Vasculitis Goodpastures Syndrome
- Hemoptysis/hematuria
- Linear IgG deposits
- across basement
- membrane
- Tx
- Stx, plasmapheresis
20Small Vessel Vasculitis Bechets Syndrome
- Highest in Iran, Turkey, and Japan
- Recurrent oral and genital ulcers
- Uveitis causes blindness in 10
- Skin pathergy
- gt 2mm nodule after 48 hours
- Multiple systems involve
- Venous thrombosis
- Tx Stx, TNF blocker
T 0
T 48h
T 24h
21Small Vessel VasculitisHenoch 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
22Small Vessel Vasculitis Cryoglobulenemia
- Nonspecific Sx
- Arthralgias, myalgias, fever, glomerularneprhitis
- Palpable pupura
- Associated with
- Hep C, RA, SLE
- Low complement levels
- Tx primary disease
23Quiz Review