Vasculitis Review: Intern Conference

1
Vasculitis ReviewIntern 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)
• Takayasus arteritis
• Distinguished only by age of pt gt or lt 50 yo
• Both involve the aorta and its primary branches

6
Medium Vessel Vasculitides (3)

• Polyarteritis nodosa (PAN)
• Buergers Thromboangiitis obliterans
• Kawasakis disease Mucocutaneous LN syndrome

7
Small Vessel Vasculitides (many)

• ANCA-associated (5)
• Wegeners 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, Bechets, Sjogrens)
• Drug-related (cutaneous leukocytoclastic
  vasculitis)
• Henoch-Schonlein Purpura (HSP) IgA deposits
• Goodpastures disease
• Cryoglobulinemia
• IBD-related
• Urticarial vasculitis
• Paraneoplastic

9
Large 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

10
Takayasus 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 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

13
Polyarteritis Nodosa (PAN)
14
Medium Vessel VasculitisBuergers Disease

• Young, 20-40 y.o
• Male
• Inflammation/thrombosis in hands/feet ? pain
• Recent or current tobacco use
• Tx with smoking cessation

15
Medium 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

16
Medium Vessel VasculitisKawasakis Disease
17
Small Vessel VasculitisChurg-Strauss Syndrome

• Middle-aged adults
• Asthma, eosinophilia (gt10), neuropathy (mono- or
  poly- in stocking/glove), pulmonary infiltrates
• ANCA vasculitis
• Tx Prednisone, cyclophosphamide

18
Small 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

19
Small Vessel Vasculitis Goodpastures Syndrome

• Hemoptysis/hematuria
• Linear IgG deposits
• across basement
• membrane
• Tx
• Stx, plasmapheresis

20
Small 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
21
Small 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

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