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RH18 Rheumatology
Vasculitides
Toronto Notes 2019
Large artery
q
Medium artery
2
Tunica externa
Arteriole
3
Tunica media
Tunica intima
1 Giant cell arteritis (GCA) Takayasu’s arteritis
2 Polyarteritis nodosa (PAN) Kawasaki disease
Small artery
3
3 Granulomatosis with polyangiitis (GPA) Microangiopathic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA) IgA vasculitis (IgAV)
Non-ANCA-associated
Figure 9. Classification of vasculitides by vessel size
Capillary vessel
3
Venule
3
Small Vessel Non-ANCA Associated Vasculitis
CUTANEOUS VASCULITIS
• subdividedinto:
■ drug-induced vasculitis
■ serumsicknessreaction
■ vasculitis associated with other underlying primary diseases (CTD, infections, malignancies –
hematologic > solid tumours)
Etiology and Pathophysiology
• cutaneousvasculitisfollowing:
■ drug exposure (allopurinol, gold, sulfonamides, penicillin, phenytoin) ■ viral or bacterial infection
■ idiopathic causes
• smallvesselsinvolved(post-capillaryvenulesmostfrequently)
• usuallycausesaleukocytoclasticvasculitis:debrisfromneutrophilsaroundvessels • sometimesduetocryoglobulinswhichprecipitateincoldtemperatures
Signs and Symptoms
• palpablepurpura±vesiclesandulceration,urticaria,macules,papules,bullae,subcutaneousnodules ■ renal or joint involvement may occur, especially in children
Investigations
• vascularinvolvement(botharterioleandvenule)establishedbyskinbiopsy
Treatment
• stoppossibleoffendingdrug
• NSAID,lowdosecorticosteroids
■ immunosuppressive agents in resistant cases • usuallyself-limiting
Small Vessel ANCA-Associated Vasculitis
GRANULOMATOSIS WITH POLYANGIITIS
(GPA, formerly known as Wegener’s Granulomatosis)
Definition
• granulomatousinflammationofvesselsthatmayaffecttheupperairways(rhinitis,sinusitis),lungs (pulmonary nodules, infiltrates), and kidneys (glomerulonephritis, renal failure)
• highlyassociatedwithc-ANCAbyindirectimmunofluorescence(IIF)andpR3-ANCAbyELISA; however, changes in ANCA levels do not predict remission or relapse
• incidence2-3per100,000;morecommoninNorthernlatitudes
Table 22. Classification Criteria for GPA*
Classic Features of GPA
• Necrotizing granulomatous vasculitis of lower and upper respiratory tract
• Focal segmental glomerulonephritis
Criteria
1. Nasal or oral involvement 2. Abnormal findings on CXR 3. Urinary sediment
4. Biopsy of involved tissue
*Diagnosed if 2 or more of the above 4 criteria present
Description
Inflammation, ulcers, epistaxis
Nodules, cavitations, etc.
Microscopic hematuria ± RBC casts
Lungs show granulomas, kidneys show necrotizing segmental glomerulonephritis
American College of Rheumatology, 1990
© Andrew Q. Tran