Vasculitis Flashcards
Medium vessels
Immune complexes : polyartheritis nodusa
Anti endothial cell antibody : Kawasaki
Large vessel vasculitis
Granulomatous disease : giant cell , takayasu
Small vessel
Microscopic polyangitis
Wegners granulomotosis
Chrug Strauss
Sle, IgA henoch schonlein, cryoglobulin, good pastures
Pr3 - anca
C-anca
Wegners, microscopic poly, pan, Chrug, good pastures
MPO- anca
P-anca
Primary vasculitis, microscopic polyangitis , Chrug, pan
Felty, sle, sjorgen, uc, crohns, primary sclerosing cholangitis
Microscopic polyarteritis
Necrotising vasculitis affecting arteries, capillaries, venules
Glomerulonephritis and pulmonary capillaritis common ( not in PAN)
Presents as palpable purpura
Microscopic polyarteritis
Skin biopsy : lesions are all the same stage
Segmental fibrinoid necrosis of media
Microscopic polyarteritis
Have fragmented pmns - leucocytoclasia
Microscopic polyarteritis
Cause of microscopic polyarteritis
Reaction to antigens once antigen is removed it goes away
Triad :acute necrotizing Granulomatous inflammation of medium to small vessels in upper and lower respiratory tract
Affects other body sites- eye and skin
Focal necrotizing glomerulonephritis
Wegners granuolmatosis
Age of onset and test for Wegners
5th decade m>f and c-anca detectable in 95%
Begins as severe type of asthma
Sinusitis ~>asthma ~> organ involvement
Churg Strauss
Diagnostic markers for churg Strauss
Blood eosinophilia, eosinophils and granulomas in affected tissue and p- anca . Mainly affects lungs
Segmental transmutation necrotizing vasculitis in medium and small arteries . Doesn’t affect arterioles, capillaries, venules
Aneruysms and thrombosis common
Polyarteritis nodosa
Spares the lungs and if a disease of young adults ….m>f
PAN
Clinical signs of PAN
Ischemia and infarction often episodic. And general symptoms of malaise, fever weightless, etc
30% have HBsAg in serum
Both c-ANCA and p-ANCA but p is more common
Treatment pan
Untreated - fatal unless cutaneous
Corticosteroid and cyclophosphamide
Vasculitis of children and infants parents as acute fever rash,viral erythema, red lips and strawberry tongue
Kawasaki
Kawasaki diseases symptoms and problems
Asymptomatic to mi coronary artery aneurysms with rupture and death
Leading cause of acquired heart disease in the us and japan. Similar lesions as pan .
Cause is thought to be viral induced in genetically susceptible patients
Tx with anti- pyretics and IVIG
Affects medium to to small vessels - mainly radial and tibial arteries ususally young male and women smokers
Buergers disease
Buergers disease age and hla type
Before the age of 35
Hla -a9 and b5
Common in Japan, Israel, India
Hypersensitive to injected tobacco extract
Inflammation of. Large medium and small arteries.
Usually affects head arteries ( temporal, ophthalmic, vertebral) or aorta
Giant cell (temporal) arteritis
Most common vasculitis in adults. You see segmental modular thickenings, lumen is reduced.
Giant cell arteritis
Giant cell arteritis clinical symptoms
Usually elderly 70-80 - half pts have visual symptoms - vague constitutional symptoms( fever, fatigue, weightloss, HA) Facial pain over the temporal artery May cause thoracic aortic aneurysm
What do you see on biopsy giant cell
Fragmentation of internal elastic membrane
Granulomatous inflammation of emerita near the internal elastic membrane
Multinucleated giant cells
Granulomatous inflammation of large to medium sized arteries. Fibrous thickening of aortic arch and it’s branches and in half of cases the pulmonary arteries are affected
Takayasu arteritis
Takayasu clincial symptoms and biopsy results
Females > 40
Wake pulses and low BP in upper extremities.
Ocular probs , neuro deficits, pulm HTN, HTN, claudication.
Narrowing and obliteration of lumens On angiogram
Biopsy: intimate thickening, mononuclear inflammation, giant cells, lumens occluded by partially organized thrombus
Vasculitis that look like micros poco polyarteritis and pan
RA, sle , henoch- schonlein purpura , mixed cryoglobulinemia, malignancy
Henoch- schonlein purpura
Generalized vasculitis of small vessels of the skin (purpura), GI tract,
joints (arthritis), kidneys (rarely - lungs & CNS)
Most common vasculitis in children.
75% of cases occur in children 2-11 years old.
Rare in adults.
IgA antibody– antigen complexes ĺ deposit locally - throughout bodyand activate pathways leading to necrotizing vasculitis.
Bacterial, viral, vaccine, drug, idiopathic causes. No single etiology has been clearly identified.
Why is it important to differentiate sle from what
APLA syndrome because treatment is different steroids vs anti coagulation
Anti phospholipid antibody (APLA) Hughes syndrome
3 different antibodies cause both arterial & venous thrombosis (blood clots).
- anticardiolipin antibodies
- antibodies directed against specific molecules (e.g. beta-2-glycoprotein 1).
- lupus anticoagulant (LA)
> 50% patients have primary APLAS. Prevalence in general population ~2-4%. Any age (esp. females with SLE)
Secondary APLAS - ~ 30% of patients with SLE develop.
Anticardiolipin antibodies are more common than lupus anticoagulant (~ 5X)
In patients with 1º APLAS, ~ 10% will eventually be diagnosed with an autoimmune disorder
(e.g. SLE or a mixed connective tissue disorder).
Antibodies induce a thrombophilic state. Exact mechanism is unknown.
APLA react will endothelial cell membrane phospholipids ĺ cell damage ĺ thrombosis
APLAS is associated with miscarriages, preterm labor, preeclampsia, and eclampsia.
Thrombocytopenia (low platelets) observed in 20-40% of patients.
Stroke, MI, kidney damage, PE If multiple clots – catastrophic APS (CAPS)
Red papule forms a vesicle
in the center.
Dries, forms a black scab.
No scarring.
Papule is usually on face, trunk, extremities. Palms, soles, and oral mucosa may be involved.
Papule precedes fever.Serology.
Rickettsialpox - R. akari
Papule becomes a vesicle
on an erythematous base.
Resembles a “dew drop”
on a rose petal.
Papule begins on head
ĺ progresses to trunk,
arms, then legs.
Vesicles present in all
stages.
Common in children.
Positive Tzanck test or DFA.
Chicken pox (VZV)
Red macule or papule with central vesiculation surrounded by a red areola. Papule crusts in a few days. No scarring.
Palmar surfaces and
sides of fingers, hands,
and plantar toe skin.
After fever, in 1-2 days
oral ulcers on the hard palate, tongue, buccal mucosa.
Cell culture & PCR.
RX: symptomatic relief
Hand-foot-and-mouth Disease
(enteroviruses, e.g.
coxsackievirus A16)
At tick bite site, an ulcer with a red halo forms. Later becomes generalized maculopapular rash. Rash is generalized, involves palms and soles, and is rarely hemorrhagic.
Fever, headache, and
generalized lymphadenopathy
Symptoms similar to a milder
form of RMSF.
Serology – R. conorii Ab
Boutonneuse fever
At mite bite site, vesicle or
black scab appears on an
erythematous base.
Vesicles are usually on
trunk or extremities.
Rash fades within a few days.Pneumonitis is frequent.
Serology.
Scrub typhus - o. Tsutsugamishi
Louse-borne typhus
epidemic
R. prowazekii
Human body louse (Pediculus humanus)
Murine typus
R. typhi
Rat flea
(also endemic CA, HI, TX)
African tick bite fever
R. africae
Buffalo ticks - usually live on cattle
Scrub typhus
O. tsutsugamushi
Chigger mite
Hands and feet then to trunk
Rmsf
Extremities to trunk
Small pox and kawasakis
Lower extremities
N. Meningitidis
Hair line and downward
Measles
Head to trunk to extremities
Varicella
Neck to trunk
Scarlet fever group a strep s. Pyogenes
Localized or generalized skin desquamation
Staph aureus, scalded skin syndrome. Exfoliatins toxin
Scarlatina like rash that desquamates
Staph aureus TSS . Toxic shock toxin (tsst 1 )
Cowdry type a inclusions
Intranuclear - VZV - herpes zoster
Molluscum contagiosum
Benign papular eruption of multiple umbilical end cutaneous lesions
H and e stain of Molluscum contagiosum
Epidermal acanthosis (hyperplasia) - MC grows into dermis to form multiple lobules.
Eosinophilic intra-cytoplasmic inclusion bodies - at the lobule base,
more basophilic at superficial layer.
Central crater - into which the inclusion bodies discharge their contents.
Intracellular growth
Rickettsia (RMSF, typhus), VZV, HSV, MCV, rubella
Endotoxin
Neisseria meningitidis
LPS causes vasculitis & DIC
Exotoxin
Streptococcus pyogenes (Group A Strep.)Pyrogenic (erythrogenic) toxins A-C
Bacteria in vessel wall
Treponema pallidum
endothelial proliferation
lymphoplasmocytic vasculitis
endarteritis obliterans (fibrosis/healing)
Immune Attack
of infected cells
Measles (Rubeola)
T cells attack viral antigen in infected
endothelium of small vessels ĺ Koplick Spots:(oral lesions on mucous membrane enanthem)
Complement Activation
[Immune / Autoimmune]
Cutaneous Necrotizing Vasculitis:
Microscopic polyarteritis (small vessels)
Giant Cell Arteritis (large temporal art. & aorta)
Polyarteritis Nodosa (PAN) (medium & small)
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Petechia rash
Fulminant disease is Waterhouse friderichsen syndrome what is that
N. Meningitidis
non-palpable purpura ecchymosis shock DIC adrenal gland destruction Grey/black necrosis with DIC.
Septic arthritis ĺ most common initial manifestation (migratory polyarthralgia).
Rash ĺ occurs below the neck (spares scalp, face, mouth).
Small papules or petechiae located on trunk or extremities, palms and soles.- turn into pustules on broad erythematous bases and necrotic centers.
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Neisseria Gonorrhoeae
Impetigo
Vesicles that crusts
Strep pyogenes, staph aureus
Erysipelas
Superficial cellulitis variant
Margin sharply demarcated, painful
Step. Pyogenes ans staph aureus
Spreads via lymphatics
Gumma
Syphilis
1° Tiny red papule on legs and other exposed areas,
becomes an elevated erythematous plaque.
Satellite papules form after few months; may fuse together.
No ulceration (unlike syphilis).
2° 5 -12 months after 1° stage, erythematous circular patcheson extremities and face; may go unnoticed.
3° Lesions with blue pigmentation on face, head, neck.
Replaced by depigmented spots resembling vitiligo.
T. Careteum -‘blue stain disease
1°Skin papules develop on extremities ~ 1 month after infection, Enlarge to form papillomatous “frambesiform” nodules.
Usually children are affected.
2 Yellow crusted granulomatous nodules appear on extremities
and face and usually heal; may reoccur.
3 Long chronic course of remissions and relapses.
Systemic symptoms ~>fever, malaise, LN
May have skin destruction ~>large ulcerated nodules
Disfiguration esp. around nose ~>gangosa
Painful open sores on feet soles~> crab yaws
Later, bone destruction ~>tibias
Tp subsp. Pertenue
When does the rash appear with measles
4th febrile day . Hair line to toes in 2-3 days . Maculopapular
