Vasculitis, Noninfectious Vasculitis Flashcards
What is vasculitis? What are the clinical SX?
Inflammation of vessels
Clinical symptomology depends on vascular bed affected (CNS VS. heart etc.)
Constitutional SX = fever, malaise, arthralgias, myalgias
In vasculitis, are more vessels more commonly affected than others?
Any type may be affected - more often arterioles, capillaries, & venules
What are the 2 types of vasculitis? Describe them.
Non-infectious vs. infectious - can be immune mediated inflammation vs. direct invasion by infectious pathogens (can provide immunosuppressive TX for immune-mediated)
Infections can also INDIRECTLY induce a noninfectious vasculitis (generating immune complexes or triggering cross-reactive immune response)
Physical & Chemical injury can also cause vasculitis ( irritation, mechanical trauma, toxins)
What is the major cause of noninfectious vasculitis? What are some examples?
Immune response - local or systemic
Giant cell (Temporal) arteritis Polyarteritis Nodosa (PAN) Granulomatosis with Polyangiitis Thromboangiitis Obliterans (Buerger Dz)
What are the characteristics of Giant Cell (temporal) Arteritis? Include sites of involvement, inflammatory cells present, and clinical Hx.
Sites = AORTA, medium sized arteries
Inflamm. cells = Lymphocytes, Macrophages (neutrophils/eosinophils rare)
Features:
Granulomas, Thrombosis , Giant cells (often, not required)
May present with constitutional Sx = fever, fatigue, weight loss
Facial Pain, Headache
Clinical Hx = >40 yo +- polymyalgia rheumatica
What are the characteristics of Churg-Strauss syndrome (allergic granulomatosis and angiitis? Include sites of involvement, inflammatory cells present, and clinical Hx.
Sites = Medium and Small sized arteries (small vessel necrotizing vasculitis) assoc with:
Clinical Hx = Asthma, allergic rhinitis, hypereosiniphilia
Inflamm cells = EOSINOPHILS required
+ Lymphocytes, Macrophages, Neutrophils
May resemble PAN/microscopic polyangiitis w/ addition of eosinophils and granulomas
Features:
Granulomas, thrombosis, Serum ANCA positivity (<1/2, mostly MPO)
Many organ systems involved = palpable purpura, GI tract bleeding, renal disease (focal/segmental glomerulosclerosis)
Myocardial involvement –> cardiomyopathy (60%, half deaths)
What are the characteristics of Leukocytoclastic vasculitis? Include sites of involvement and inflammatory cells present.
Sites = Small sized arteries, capillaries, veins
Inflamm cells = Lymphocytes, macrophages, neutrophils, eosinophils (all +-)
Features:
Thrombosis
What are the characteristics of Buerger Dz (thromboangiitis obliterans)? Include sites of involvement, inflammatory cells present, and clinical Hx.
Sites = Medium/small sized arteries, veins
- Segmental, thrombosing
- Tibial and Radial arteries
- Occ secondary extension to veins and nerves of extremities (pain!)
May lead to vascular insufficiency of extremities
Chronic ulcerations –> may lead to gangrene
Inflamm cells = Lymphocytes, macrophages, neutrophils, eosinophils
Features:
THROMBOSIS Required
Clinical HX:
Young male smoker, <35 yo
Hypersensitivity to intradermally injected tobacco
Assoc. with HLA haplotypes of certain ethnic groups: Israeli, Indian, Japanese
What is immune complex vasculitis?
Autoantibody production and formation of immune complexes
Deposition of antigen-antibody complexes in vascular wall
-incites inflammatory reaction within the wall
-Ag is often unidentified
In what conditions may you see immune complex vasculitis?
Systemic immunologic diseases (ex: SLE)
Drug hypersensitivity
Secondary to exposure to infectious agent
Describe drug hypersensitivity in relation to immune complex vasculitis (ex: PCN). What may you see clinically? What happens if you stop the drug?
PCN acts as hapten binding to serum proteins of vessel walls
-Streptokinase acts as foreign protein
Clinically = mild to fatal; SKIN LESIONS most common
Always consider drug hypersensitivity. Stop drug –> resolution of vasculitis
How can you get immune complex vasculitis secondary to an infectious agent?
Ab to microbial constituents form immune complexes that deposit in vascular lesions
Polyarteritis nodosa = 30% associated with HBsAg and anti-HBsAg
What is Antineutrophil cytoplasmic antibodies (ANCA)?
A heterogenous group of antibodies reactant with cytoplasmic enzymes found in neutrophil granules, monocytes, and endothelial cells
What are the antibodies associated with ANCA?
Anti-proteinase-3 –> seen with PR3-ANCA
Anti-myeloperoxidase –> seen with MPO-ANCA
What dz is PR3-ANCA associated with?
Polyangiitis
What dz is MPO-ANCA associated with?
Microscopic polyangiitis and Churg-Strauss syndrome
Induced by RX: propylthiouracil
What do ANCA TITERS primary detect?
Disease SEVERITY
ANCA is pauci-immune. What does that mean?
ANCA Ab directed at cellular constituents and do not form circulating immune complexes.
You will not see a lot of immune cells
Vascular lesions do NOT typically contain Ab and complement