HY from vasculitis lecture Flashcards
definition of vasculitis
Inflammation of blood vessels with necrosis of blood vessels walls, narrowing, and occlusion. In some types of vasculitis, aneurysms may be present
how is vasculitis classified?
Classification is by size of blood vessels involved, current knowledge of the pathophysiology and pattern of organ involvement
what are the large vessel vasculitis? (4)
takayasu arteritis
giant cell arteritis
cogan’s syndrome
Bechet’s disease
what are the predom medium vessel vasculitidies?
polyarteritis nodosa
cutaneous polyarteritis nodosa
Buerger’s disease
kawasaki disease
primary angiitis of the central nervous system
what are the predominantly small vessel vasculitidies that are immune complex mediated
medium vessel: PAN
small vessel
good pasture’s
cutaneous leukoctoclastic angiitiis(hypersensitivity angiitis) henoch-schonlein purpura
hypocomplementemeic urticarial vasculitis
essential cyroglobulnemia
erthema elevatum diutinum
what are anca associated small vessel vasculitis?
Wegener’s (granulomatosis with polyangiitis) microscopic polyangiitis churg-strauss syndrome renal-limited vasculitis
what are some connective tissue disorders associated with medium vessel vasculitides?
SLE, rheumatoid, inflammatory bowel disease, paraneoplasitc, infection, Drug-induced vasculitis
what are 8 vasculitis associated with granulomatous inflammation?
giant cell arteritis
takayasu areteritis
cogan’s syndrome
Wegener’s
churg-strauss syndrome
primary angiitis of the CNS
buerger disease
rheumatoid vasculitis
skin manifestations of PAN often include:
As noted above, skin manifestations of PAN may include livedo reticularis, skin ulcers, and a bullous or vesicular eruption.
which organ MC involved in PAN?
_ kidneys_ are the most commonly involved organ. Renal involvement frequently leads to variable degrees of renal insufficiency and hypertension. Renal ischemia, leading to activation of the renin-angiotensin system, is thought to be the primary mechanism for the development of hypertension.
is PAN anca associated?
PAN are typically ANCA-negative; a positive test suggests either microscopic polyarteritis or Wegener’s granulomatosis.
describe cutaneous PAN
The term cutaneous PAN usually implies a separate entity from PAN that is limited to the skin
which virus is involved w/cutaneous PAN?
Hepatits C viral infection has been associated with cutaneous PAN
how to do you treat polyarteritis nodosa?
If HBV present, need to treat this with antiviral agents and use plasma exchange Corticosteroids/cytotoxic agents Prognosis is variable, but often high morbidity, mortality
thromboangiits obliterans =
buergers disease
define buerger’s disease
is an inflammatory vaso-occlusive disease that predominantly affects the vascular supply to the lower limbs
buerger’s MC in ______
young adult male tobacco smokers, although women and older adults can also be affected
how does Buerger’s begin?
Buerger’s disease typically begins with bilateral pain and ischemia in the lower extremities although the upper extremities may be the site of initial symptoms with pain on exposure to cold.
how does Buerger’s progress?
Most cases evolve rapidly, however, with increasing ischemic (claudicant) limb pain, digital cyanosis, splinter hemorrhages, and skin vesicles and digital ulcers often occur, especially after minor trauma.
what does the tx of Buerger’s include?
Treatment includes stop smoking; and anecdotal use of calcium channel blockers and pentoxifylline has improved some cases.
what are MC types of immune complex mediated vasculitis?
The most common types of IC-mediated vasculitis are hypersensitivity vasculitis, Henoch-Schönlein purpura (HSP), and mixed cryoglobulinemia. Rarer forms of this condition include hypocomplementemic urticarial vasculitis and erythema elevatum diutinum.
which systemic diseases associated with immune complexes?
Connective tissue disorders such as systemic lupus erythematosus, Sjögren’s syndrome, and rheumatoid arthritis can be associated with IC-mediated vasculitis.
most prominent features of immune complex vasculitis
The classic cutaneous finding in small vessel vasculitis is palpable purpura, but a variety of other skin lesions may be found including pustules, vesicles, urticaria, and small ulcerations.
what do immunoflourescent studies find in immune complex vasculitis?
Direct immunofluorescence studies of involved blood vessels demonstrate characteristic types and patterns of immunoglobulin (Ig) and complement deposition.
what causes hypersensitivity vasculitis?
reaction to a medication or an infection.
the following describes associated with purpura, arthritis, glomerulonephritis, and colicky abdominal pain. IgA deposition is found within blood vessel walls.
HSP, remeber this has tetrad of A GAP (palpable purpura, arthritis, abdominal pain, and glomerulonephritis)
which virus is mixed cryoglobulinemia associated with?
Cryoglobulinemic vasculitis is most often associated with long-standing hepatitis C virus infection. The term mixed cryoglobulinemia is sometimes used for this disorder because the immunoreactants involved in the disease include both IgG and IgM.
The term ____________ refers to an immune complex (IC)-mediated small vessel vasculitis of the skin that spares internal organs and usually following _______ and ______
what characterizes this disease?
just know: A similar illness-serum sickness- is a systemic illness that includes rash and prominent arthralgia or arthritis occurring 1-2 wks. after exposure to a drug or foreign antigen
what is another name for this disease?
The term hypersensitivity vasculitis refers to an immune complex (IC)-mediated small vessel vasculitis of the skin that spares internal organs and usually following drug exposures or infections.
The disease is characterized by IC deposition in capillaries, postcapillary venules and arterioles.
Cutaneous Leukocytoclastic Angiitis
Henoch schonlein purpura:
what is mostly affected?
granulomas?
what does it follow?
common in whom?
which antibody present?
Necrotizing vasculitis of small vessels, primarily postcapillary
venules
1. NOT associated with granulomas
2. Often follows a specific exposure (drug, infection)
3. More common in children, and less severe in children, than
adults
IgA is characteristically present, both in biopsy of involved tissue
and is elevated in the serum (IgA1)
what is the classic tetrad of HSP?
Classic tetrad of purpura(buttocks/extremeities), arthritis, abdominal pain(and hemorrhage), Glomerulonephritis
labs for HSP
Anemia, thrombocytosis, increased ESR, CRP
Hematuria, elevated creatinine, and RBC casts if renal involvement
Diagnosis is by presentation and biopsy of skin or kidney
Pathology reveals a leukocytoclastic vasculits consisting of PMN’s with nuclear debris, RBC extravasation. IgA characteristically present
how do you tx HSP?
Self limited, usually with good prognosis
If renal disease, steroids may be used
When plasma is refrigerated at 4°C for up to 72 hours, proteins may precipitate (cryoprecipitate). If paired serum from the same patient also forms a precipitate, then the precipitated proteins are referred to as _____________. If, however, precipitation develops after refrigeration of plasma but does not occur in cold serum, the plasma precipitate is referred to as _____________________
When plasma is refrigerated at 4°C for up to 72 hours, proteins may precipitate (cryoprecipitate). If paired serum from the same patient also forms a precipitate, then the precipitated proteins are referred to as cryoglobulins (CGs). If, however, precipitation develops after refrigeration of plasma but does not occur in cold serum, the plasma precipitate is referred to as cryofibrinogen (CF).
components of Cryofibrinogen?
compoenet of cyroglobulin?
cyroglobulin = immonglobulin + complement components
CF = may contain immunoglobulins they characteristically are composed of fibrinogen, fibrin, fibronectin and/or fibrin split products. By definition, CF are undetectable in serum.
