Rheumatology: Vasculitis Flashcards
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.
primary; secondary
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?
temporal headache
jaw claudication
scalp tenderness
visual loss
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
CNS events
granulomatous panarteritis
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?
fever prominent mucocutaneous changes cervical lymphadenopathy polymorphous rash erythema and edema of hands and feet desquamation myocarditis coronary vasculitis
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)
lungs
kidneys
may affect other organs
GPA is usually assoc. with serum _____.
cytoplasmic-ANCA (c-ANCA)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) affects ____.
small arteries and venules
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) manifests where?
multiorgan involvement: lungs skin peripheral nerves gut heart renal (rare)
Microscopic polyangiitis (MPA) affects which BVs?
arterioles, capillaries, and venules
What are the s/s of microscopic polyangiitis (MPA)?
pulmonary hemorrhage glomerulonephritis palpable purpura peripheral neuropathy 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
arthritis
abdominal pain
hematuria
Essential cryoglobulinemic vasculitis presents as?
purpura arthralgias weakness peripheral neuropathy Raynaud’s phenomenon glomerulonephritis pulmonary hemorrhage
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
arthralgias
systemic symptoms may be present, usually secondary
to an immune response
What are the common clinical s/s for all the vasculides?
skin lesions 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 thrombocytosis low albumin elevated sedimentation rate and C-reactive protein polyclonal gammopathy possibly elevated liver enzyme tests low complement levels low cryoglobulin
How are the vasculides diagnosed?
hx and phys exam
detection of serum Abs (RF, ANA, ANCA)
biopsy
angiogram
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?
granulomatous panarteritis
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?
leukocytoclastic vasculitis
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
inflammatory reaction - “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
What are the 5 suspected mechanisms of vascular damage?
- immune complexes
- Antineutrophil Cytoplasmic Antibodies (ANCA)
- Antiendothelial antibodies
- T Cell Dependent Mediated Endothelial Cell Injury
- Infection of Endothelial Cells
