Flashcards in Rheumatology: Vasculitis Deck (52)
The vasculitides are a heterogeneous group of clinical disorders characterized by ______.
inflammation of blood vessels
Vasculitis classification can be based on ____, ____, and ____.
the size of the vessel involved
type of pathologic change in the vessel wall
the clinical presentation
Vasculitides can be _____, occurring separately from any known underlying disease, or _____ to infectious disorders such as hepatitis B or C and endocarditis, drug hypersensitivity, connective tissue diseases (RA, SLE, Sjögren’s), cryoglobulins, and malignancies.
The Chapel Hill Classification of vasculitis is based largely on _____ involved.
the size of the vessels
Large-cell vasculitis can either be ____ or ____.
giant cell arteritis, Takayasu's arteritis
What BVs are involved in giant cell arteritis?
temporal arteries, vessels originating from the aortic arch, other arteries (less common)
What are the s/s of giant cell arteritis?
What BVs are involved in Takayasu's arteritis?
aortic arch and its branches (any part of the aorta)
What are the s/s of Takayasu's arteritis?
claudication of upper>lower extremities
Medium-vessel vasculitis can be ____ or ____.
polyarteritis nodosa; Kawasaki's disease
What BVs are involved in polyarteritis nodosa?
small and medium sized arteries, especially at vessel bifurcations
What are the s/s of polyarteritis nodosa?
any organ can be involved, esp skin, joints, peripheral nerves, gut, and kidney
What BVs are involved in Kawasaki disease?
small and medium sized arteries
What are the s/s of Kawasaki disease?
prominent mucocutaneous changes
erythema and edema of hands and feet
The 2 broad categories of small vessel vasculitis are ____ and ____.
antineutrophil cytoplasmic antibody (ANCA) positive vasculides and antineutrophil cytoplasmic antibody (ANCA) negative vasculitides
ANCA positive vasculitides can be?
Granulomatosis with polyangiitis (Wegener’s) aka GPA
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Microscopic polyangiitis (MPA)
What arteries does GPA affect?
small and medium-sized arteries
Where does GPA manifest?
upper respiratory tract (sinuses)
may affect other organs
GPA is usually assoc. with serum _____.
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) affects ____.
small arteries and venules
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) manifests where?
Microscopic polyangiitis (MPA) affects which BVs?
arterioles, capillaries, and venules
What are the s/s of microscopic polyangiitis (MPA)?
joint and abdominal pain
Serum _____ is common in microscopic polyangiitis (MPA).
perinuclear- ANCA (p-ANCA)
Antineutrophil cytoplasmic antibody (ANCA) negative vasculitides can be either ____, ____, or ____.
Henoch-Schönlein Purpura (HSP), Essential cryoglobulinemic vasculitis, or Cutaneous leukocytoclastic angiitis
Henoch-Schönlein Purpura (HSP) affects which BVs?
arterioles and venules
Essential cryoglobulinemic vasculitis affects which BVs?
small vessels, including glomerulocapillaries
Henoch-Schönlein Purpura (HSP) presents as?
palpable purpuric skin lesions in lower extremities
Essential cryoglobulinemic vasculitis presents as?
Often pts are ____ and _____ positive in essential cryoglobulinemic vasculitis.
rheumatoid factor; hepatitis C antibody
Cutaneous leukocytoclastic angiitis manifests in which BVs?
arterioles and venules
What are the manifestations in cutaneous leukocytoclastic angiitis?
palpable purpuric skin lesions
systemic symptoms may be present, usually secondary
to an immune response
What are the common clinical s/s for all the vasculides?
constitutional symptoms (fever, anorexia, weight loss, weakness, fatigue)
musculoskeletal symptoms (arthralgias, arthritis, myalgias, peripheral neuropathy)
What are the common lab features for all the vasculides?
anemia of inflammatory disease
elevated sedimentation rate and C-reactive protein
possibly elevated liver enzyme tests
low complement levels
How are the vasculides diagnosed?
hx and phys exam
detection of serum Abs (RF, ANA, ANCA)
At what age do the vasculides typically present?
mean = 5th decade
The vasculides are as common in men as they are in women, except for in ____ and ____, in which women have a higher incidence.
Takayasu’s and giant cell arteritis
Although these disorders can occur in any population, there is a higher incidence of _____ in Japanese and Asian populations, and _____ in populations of Northern European background.
Takayasu’s arteritis; giant cell arteritis
What are the treatments for the vasculides?
treat or remove the inciting agent or antigen; glucocorticoids, cytotoxic drugs, plasmapheresis, Rituximab
What is the cause of giant cell arteritis?
disruption of the internal elastic lamina
What is the cause of Takayasu’s arteritis?
What is the cause of polyarteritis nodosa?
focal but panmural necrotizing arteritis with a predilection for involvement at the vessel bifurcation
What is the cause of Kawasaki’s disease?
probable infectious vector resulting in cytokine-mediated endothelial damage
What is the cause of Granulomatosis with polyangiitis (Wegener’s) (GPA)?
pauci-immune, necrotizing, granulomatous arteritis usually associated with serum cytoplasmic-ANCA (c-ANCA)
What is the cause of Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)?
necrotizing extravascular granulomas and vasculitis of small arteries and venules; eosinophils present in early stage
What is the cause of Microscopic polyangiitis (MPA)?
pauci-immune, necrotizing vasculitis, serum perinuclear-
ANCA (p-ANCA) common
What is the cause of Henoch-Schönlein Purpura (HSP)?
leukocytoclastic (neutrophilic perivascular/transmural infiltrate) or necrotizing vasculitis often with IgA deposition
What is the cause of essential cryoglobulinemic vasculitis?
cryoglobulins deposited on the vascular wall stimulate complement activation and a cellular inflammatory response
What is the cause of cutaneous leukocytoclastic angiitis?
What is the general pathology for the large and medium vessels?
- panarteritis with infiltration of lymphocytes, monocytes, histiocytes, eosinophils, and PMNs through the vessel wall
- granuloma or giant cell formation in some disorders
- disruption of the elastic lamina
- thickening results in narrowing or obliteration of the vessel lumen
What is the general pathology for the small vessels?
- fibrinoid necrosis of the vessel wall with a transmural
- “leukocytoclastic” vasculitis – a pathologic
term used to describe necrotizing vasculitis with PMN-derived nuclear debris that often accompanies the neutrophilic perivascular infiltrate of the vessel wall
- immunoglobulin (IgM, IgA in HSP) and complement (C3) vascular deposition