Reumotology finals Flashcards
(163 cards)
Which Vasculitis are associated with P-ANCA and which with C-ANCA
and each of those Ab, agianst which part?
**only the small vessel vasculitis:
**C-ANCA = Wegener (granulomatosis with polyangitis)
P-ANCA= Shug straus (EGPA) , microscopic polyangitis (MPA)
C- cytoplasmin»_space; PR 3
P- perinuclear»_space; MPO
If theres a vasculitis with both P and C anca positive.
it can be?
Drug induce vasculitis
mainly secondary to Cocaine use
What is the main Tx in vasculitis?
Steroids + cyclophophamide
what can be the use in Azathioprine (imuran) in vasculitis
what we have to check first?
maintanance- in GPA/MPA/GCA
must check levels of TPMT - related to severe cytopenias
RTX can be use in which type of vasculitis?
Microscopic polyangitis
granulomatosis with polyangitis - wegener
late onset neutropenia + HBV reactivation
small veseels.
which vascilitus can be treated with tocilizumab?
and what is the MOA
and C/I
GCA- giant cell arthritis (Temporal)
* also for RA as 2nd line
MOA- anti IL-6
C/I- Hx of diverticulitis
which anti suppresive medication can cuase hyperlipidemia + GI perforation?
Tocilizumab
toci li ze m-abdomnial
Mepolizumab can be use in which type of vasculitis?
EGPA
also for asthma
*remember- EGPA present with weird asthma
when we should give folate after tx with MTX?
a day after treatment
Granulomatosis With Polyangiitis (GPA)
What is the clinical presentation (triad)
Sinusitis, Otitis media, hemoptysis (think URI, LRI)
+
nephritic syndrome- hematuria + RBC casts
Upper resp. involv - 95%
lower- 85-90%
kidney - 77%
What we will see in CXR of Granulomatosis With Polyangiitis?
bi-lateral nodular infiltrate - to cavitations
in biopsy- necrotic vasculitis w/ granulamotosis
In which types of Vasculitis we will see pauci-immune?
all ANCA diseases:
GPA
EGPA
MPA
except- goodpasture syndrome (not a vasculitis)
Granulomatosis With Polyangiitis how we diagnose?
Biopsy- mainly from the lung
DDx for GPA- use of cocaine
C-ANCA
Which type of def. and substacne exposure will increase the risk for GPA
a1-AT and selica exposure
Tx for GPA + MPA
induction steroids + cyclophospamide (PO) (3-6month)
RTX- good as cyclo, given to pt with relapse
maintance- RTX (most effective )/ AZA/ MTX/ MMF (less effective) - at least for 2 years
50-70% יחוו הישנות
what is the different between
GPA to MPA
- MPA- w/o Upper respiratory tract involvment
- P-ANCA (and no C)
- No granulomas
could also be- mononeuritis multiplex (also in GPA)
almost the same disease
Clinical presentation of EGPA (Chaug strauss)
- late onset of weird asthma - rsistant to therapy,
- sinusitis
- neuropathy- Mononeuritis multiplex- second most common finding
- eosinophilis in labs, P-anca
Why should we perform echocardiography for every pt with EGPA?
will determine the prognosis and the aggresive to tx
What is the most significant cause of death in EGPA
cardiac disease
What is the prognosis of EGPA w/o Tx?
25% for 5 year survival
What is the Tx of EGPA?
mild disease- steroids, consider Mepolizumab
severe- like GPA and MPA
Which type of infection is associate with Polyarthritis nodusa (PAN)
HBV+
*check also for HBC
Which leukemia is a/w PAN?
hairy cell leukemia
Why kidney biopsy is not a good way to Dgx PAN?
beacuse this disease damage medium size blood vessels.
which mean »_space; the injury will be ischemic and will not see GN.