Vasculitis Flashcards
Etiology of vasculitis
Mostly unknown
Can be associated to infectious organisms, drugs, tumors and allergic reactions.
Common denominator in vasculitis
Formation of immune complexes
Symptoms of vasculitis
A variety since its a systemic disorder
Treatment of vasculitis can have a different approach depending of
According to wether the trigger is immune or infectious
Clinical manifestations that are common in most vasculitis
Constitutional symptoms: fatigue, weight loss, fever and weakness
Anemia of chronic disease
Can affect a lot of organs
Chronic inflammatory disorder will show
Elevated ESR or CRP
The type of anemia associated to vasculitis
Anemia of chronic disease
Giant cells arteritis
Size of artery affected:
Typical symptoms:
Eye correlate:
Targets all size arteries, but specifically in the head
-Claudication of jaw & tongue
-Tenderness of skull in area and can palpate temporal arteries (reduced pulsation)
-Optic arteritis can cause permanent blindness
Giant cell lab findings and treatment
Elevated ESR, anemia/ thrombocytosis
Treat with corticosteroids bc its an inflammatory response
Takayasu arteritis
Common location affected:
Typically symptoms:
-Chronic inflammatory disorder
-granulomatous vasculitis of the aorta & narrowing branches from the aorta
- weakening of pulses in upper extremities (pulseless)
Clinical presentation of takayasu arteritis
Early phase shows non specific symptoms: night sweats, fever, weight loss , Myalgia + arthralgia (muscle and joint pain)
Later occlusive phase: once it keeps narrowing the arteries occlusion will occur -> Claudication pain in upper extremities.
Can cause TIA
Decreased flow to upper extremities, aortic regurgitation
How does Claudication of upper extremities occur in takayasu?
Narrowing of the aorta decreases blood flow to the upper extremities which causes the claudicating pain. The flow of the circulation is unable to keep up with the demand.
Takayasu diagnosis and treatment
Chest X-ray will show widened aortic shadow, irregularity of descending aorta, or cardiac enlargement
Arteriography is most helpful
Treat with corticosteroid but might need to add cytotoxic drugs in patients failing steroid treatment
Surgery/ angioplasty might be needed in advanced cases
Polyarteritis Nodosa (PAN)
Associated to:
Severity:
Treatment:
-Associated with Hepatitis B
-Necrotizing vasculitis in different organs, killing walls of the artery
-fatal if untreated, curative with steroids and cyclophosphamide
PAN characteristic symptoms:
Symptoms everywhere but kidneys are the most commonly affected (glomerulonephritis)
-coronary arteritis with angina/MI
-skin: purpura, gangrene
Myalgia+ arthraigas
Or hit is & epididymitis
If you have kidney damages in PAN, lab will show
Hematuria and proteinuria
Treatment of PAN
Corticosteroids + cyclophosphamide in severe cases
Hepatitis B related: antiviral, plasma exchange + corticosteroids
Kawasaki disease is AKA
Common in:
Consequence of”
Most severe complication:
“Mucocatenous lymph node syndrome”
Young children
Consequence of abnormal response to an infection, is usually self limited
Complication: involvement of coronary arteries can cause inflammation of heart
Kawasaki common symptoms
Painful cervical lymphadenopathy: enlargement of lymphatic syndrome is shown after infection and fever of 1-2 weeks
Bilateral conjunctival congestion
Strawberry tongue: dry + red, fissured lips
Athritis/ hand edema
Redness of palms and soles is seen in what disease?
Kawasaki disease
Rash that doesn’t respect palms and soles like other infections in children
Exanthema is seen in trunk, palms/soles as well as hand edema
How can Kawasaki can have effect on the heart
Carditis, heart murmurs, CAD
Kawasaki treatment includes
Supportive treatment since it is self limited
High does IV gamma globulin
What disease shows elevated c-ANCA
Wegener Granulomatosis
Wegener granulomatosis triad
Respiratory tract necrotizing granulomas
Renal disease (focal necrotizing GN)
Systemic vasculitis
c-ANCA positive and T cell mediated hypersensitivity response
Wegener granulomatosis
What diseases have necrotizing granulomas
PAN and wegener
Saddle nose deformity
Cartilage destruction affecting the nose is seen in wegener
Ocular manifestations of Wegner
Can affected any part of the eye
Conjunctivitis m Episcleritis, uveitis, retinal vasculitis
Anterior segment might be resistant to the anti inflammatory therapy
Decompressive surgery might be needed when ON is compromised
Lab data seen in wegener
C anca
3 antibodies (PR3)
Biopsy
Treatment of wegner
Cyclophosphamide + prednisone + methotrexate + azathioprine
Churn Strauss syndrome is similar to wegener but it has
Milder renal disease
And is associated with allergies
Vasculitis that is associated with allergies
Churg Strauss syndrome
Unique characteristic of this disease is Peripheral neuropathy
Charge Strauss syndrome
Shown in 70% of patients
p-ANCA positive
Charg Strauss
Diagnosis and treatment of charg Strauss
P-anca
Elevated ESR
Anemia, eosinophilia
Treatment of churg Strauss
Prednisone if mild
If severe a more potent immune suppressant
What is the difference between p-ANCA and c-ANCA
Different kinds of antibody deposition
P anca show a perinuclear stain
C anca show a diffusely granular, cytoplasmic stain
Most common hypersensitivity vasculitis in childhood and young adults
Henoch- Schonlein purpura
Vasculitis more commonly found in kids
Kawasaki and Henoch
Vasculitis that is preceded by an upper respiratory tract infection
Henoch-schonlein purpura
(Usually self limiting)
Henoch triad
Palpable purpura: Lower extremities + buttocks NOT DUE TO THROMBOCYTOPENIA
Abdominal pain (colicky/ GI bleeding)
Athritis + fever (knees and ankles)