Trigger - Vasculitis Flashcards
HLA halpotype in pts >50 y/o
polymyalgia rheumatica and temporal arteritis
pain/stiffness in neck, shoulders, lower back hips and thighs leading to provlems with ADLs such as combing hair, putting on coats and rising from chairs
polymyalgia rheumatica
this increases risk of thoracic aortic aneurysm by 17-18x
temporal arteritis (GCA)
what is the cause of blindness in temporal arteritis (GCA)
anterior ischemic optic neuropathy
Elevated ESR
thrombocytosis
elevated alkaline phosphatase
normochromic normocytic anemia
temporal arteritis (GCA)
what are the 3 medications we learned about that can potentially be used in the tx of temporal arteritis (GCA) w no visual loss
- high dose prednisone x 1 month
- ASA to reduce stroke/vision loss
- tocilizumab (reduces prolonged use of prednisone)
10% of people with this disease are found to have Hep B infections
Polyarteritis Nodosa (PAN)
primarily affects the renal and visceral arteries causing nectrotizing arteritis
PAN
Does polyarteritis cause muscular weakness?
NO!!
This diagnosis spares the lungs but CAN affect the bronchial vessels
PAN
aneurysmal dilation up to 1cm in involved arteries is characteristic for…
PAN
Just look at this pathophys
Presents with levido reticularis, uclers on the malleoli, and gangrene of the digits
PAN
Will also present with flu like symptoms!!!
Flu like symptoms is the hallmark presenttion for this
PAN
Associated with secondary renin-mediated hypertension
PAN with renal artery involvement
Negative ANCA
PAN
GPA and MPA are positive!
what medication in PAN treatment has morality benefit
cyclophosphamide
tx with high dose prednisone, IV if severe.
Just tell me how to treat PAN
- high dose CS (60mg/day)
- IV methylprednisone if severe!
- add cyclophosphamide to reduce mortality
- If HBV+ then use anti-HBV and plasmapharesis
- Once in remission use azothioprine or MTX to maintain remission
what are the poor prognostic factors for PAN and how much do they reduce mortality
- CKD w Cr>1.6
- Proteinuria >1g/day
- GI ischemia
- CNS disease
- Cardio involvement
characterizied by vasculitis of the upper and lower respiratory tracts with glomerulonephritis
Granulomatosis w polyangitis (GPA)
classic triad!!
saddle nose deformity, serous otitis media, and chronic nasal staph aureus are all associated with what diagnosis
Granulomatosis w polyangitis (GPA)
clinical presenttion includes severe upper respiratory problems with skin lesions, conjunctivitis, and possible severe airway obstruction
Granulomatosis w polyangitis (GPA)
this is alot:(
pulmonary tissue biopsy has the highest diagnostic yield for this disease
Granulomatosis w polyangitis (GPA)
can also use renal biopsy to confirm pauci immune glomerulonephritis (idk seems important)
labs show elevated ESR, leukocytosis, mild hypergammaglobulinemia, and mild elevation of RF
also thrombocytosis
Granulomatosis w polyangitis (GPA)
will also see + ANCA but you KNOW THAT!!!
How do you treat severe Granulomatosis w polyangitis (GPA)
- cyclophosphamide + prednisone x1 month
- continue prednisone x 6-9 mo
- CBC Q 2 weeks
- Rituximab
- MTX or azathiprine to maintain remission (if cant take these then use mycophenolate mofetil)
I think you do all of these in this order but idk help plz
How do you treat non-severe Granulomatosis w polyangitis (GPA)
MTX + glucocorticoid
like if its not life threatening
if GPA is confined to the upper airway and is NOT found anywhere else, what can be used as treatment
bactrim. dont use alone if GPA is outside upper airway
What are the concerns with treating GPA w cyclophosphamide
- renally eliminated (monitor kidney fxn)
- infertility
- DVT and PE risk
- NEVER irradiate upper airway lesions!
what is the MCC of pulmonary-renal syndrome
Microscopic polyangiitis (MPA)
causes diffuse alveolar hemorrhaging + glomerulonephritis that often occurs simultaneously
how do you differentiate GPA and MPA
biopsy showing granulomatous inflammation = GPA
if Lack of granulomatous inflamm = MPA:)
What is the pattern of ANCA followed by MPA
p-ANCA!
I think GPA = c-ANCA
what medication must have normal renal function in order to use?
MTX
leucocytoclastic vasculitis with IgA depostition
henock Shonlein purpura (HSP)
what is the treatment of henock Shonlein purpura (HSP) in children with chronic persistent or intermittent type
what is the catch with this treatment
prednisone to decrease tissue edema, arthralgias and abdominal discomfort
DOES NOT decrease proteinuria or shorten/decrease chance of recurrence.
Tx of severe henock Shonlein purpura (HSP)
- mycophenolate mofetil
