Lupus + APS + Sjogren Flashcards
anti dsdna is assocaited with what ?
Associated with lupus nephritis
ddx of ANA
- Rheum: SLE, scleroderma, MCTD, drug-induced lupus, polymyositis/ dermatomyositis, rheumatoid arthritis
- Thyroid disease, autoimmune hepatitis, PBC, IBD, IPF
- Infection: hepatitis C, parvovirus, TB
- Family history of any of above
- Healthy (healthy titres: 1/40 = 20%, 1/80 = 10%, >1/160 = 5%)
can anti dsdna used for monitoring ?
yes !
anti histone in lupus is good for what ?
Drug induced lupus
SLE
what labs is required to diagnose MCTD ?
anti RNP
those with lupus and antiro/antila , are more at risk of what as a bb ?
risk of congenital heart block and neonatal cutaneous lupus
managment of non renal SLE tx?
- HCQ
if have non renal; SLE but have UPCR > 500/htn, dshould give what ?
ACEi/ARB
how often do you do cervical cancer screening in SLE patients ?
Annual basis regardless of immunosuppession
benefit of hcq in non renal sle
- increase survival
- decrease renal flare risk
mild non renal SLE ( const sx, mild arthritis, rash <9% BSA, plt 50-100) : tx option ?
HCQ ( +/- GC)
Moderate non renal SLE ( ra like arhtritis, rash 8-18% , cutatenous vasculitis, plt 30-50 , serositis) -: tx options
-mtx
- azathioprine
-consider biologis : Belimumab
severe systemic lupus erythematous ( organ threatening disease - nephritis/cerebritis/myelitis/pneumonitis/mesenteric vasculitis), plt <20, ttp like disease, AIHA< rash >18%
-tx ?
mmf
bel
Anifrolumab
cyclcospoprin
ritux
when is belimumab and anifrolumab recommended first line in non renal SLE ?
- 1st line in severe disease refers to cases of extrarenal SLE with non-major organ involvement, but extensive disease from skin, joints
which biologics in non renal SLE is great for skin involvement
anifrolumab
which meds ot aovid in non renal SLE if pt has neuropsych disease
aniflrolumab
belimumab
who should be biopsied in ALL SLE
– Glomerular hematuria and/or cellular casts
– Proteinuria >0.5g/24h or UPCR >500 mg/g
– Unexplained dec in GFR
renoprotective meds soin lupus nephritis?
RAAS blockage
SGLT2
if have APS nephropathy, what do you give as well ?
VKA
heparin
when do you treat lupus nephritis class 1-2 with immunosup
if nephrotic syndrome –> immunosup
what do you tx class 3-5 with in terms of lupus nephritis
- aggressive immunosuppression
- antiproteinuric/ antiHTN meds
what do you teat class 6 lupus aka advanced sclerotic LN ( 90% of glomeruli globally sclerosed w/o residual activity)
- supportive therapy
- +/- tx extra renal manifestaiton
lupus renal syndrome : SLE + TMA
- what to measure ?
- how to tx
- adamst 13, APLAs
- plex, steroids, eculizumab, anticoag
class 3 , class 4 lupus nephritis
- HOW DO THEY PRESENT
- induction tx ?
- then what ?
- hematuria, proteinuria, hypertension, renal failure
- high dose GC + other agent ( MMF or BEL/CNI/CYC )
- maintenance : HCQ+ opther agent +/- low dose prendisone
risk of cyclophosphomide
Risks infertility (significant)
others : infection, malignancies esp GU (++hydration), cytopenias
is mmf safe for pregnancy ?
not but can use it if considering future fertility consideration
what other meds do you give for all lupus nephriits
HCQ , ACEi
who can get rituximab in LN ?
persistent disease
activity or inadequate response
if you induced with cyclosporine, do you keep for maintenance ? if not what to do ?
MMF
if have pregnancy plans and on MMF for LN, what can you give ?
AZA
how long do you treat LN ?
> 36 months
antiphospholid syndrome - obstetrical crtierias , more at risk of what ?
- > 3 prefetal losses
- fetal death after 16 weeks
- severe pre eclampsia
entry criterion for antti phospholipid syndrome ?
1 documented clinical criteria + positive APL test within 3 years
cardiac valve issues in APS ?
- thickening
- vegetation
hematology issues in APS
thrombocytopenia
what’s high risk APS profile wthout p[rior thromobsis ? tx ? what if SLE also prsent ?
LA + double/triple positivity
ASA for life
if SLE + APS –> HCQ
why not use DOAC in thrombotic APS ?
associated with risk of arterial/venous thrombotic events
eculizimab is good in what APS context ?
if TMA-Renal manifestation
catastrophic APS , how can you define
concomitant/successive thrombosis in more than 3 organs
how do you treat catastrophic APS ?
- full dose anticoagulation
- high dose glucocorticoid + PLEX/IVIG
should you continue HCQ in pregnancy ?
yes
safe immunosuppressive treatment in pregnancy
HCQ
steroids
AZA
tacroliumus
Cyclosporine ( not cyclophosphomide)
what should you start in lupus + pregnancy , benefit ?
ASA prior 16 weeks and reduce preeclampsia risk
if history of neonatal lupus, what shouldd be done ?
HCQ + serial fetal echo weekly from week 16-26
with lupus nephritis + pregnnacy, can you continue ACEi . how long LN should be inactive ?
no
want to make sure that LN inactive for > 6M
if positive APL but no APS, tx ?
ASA alone
if obs APS , tx ?
ASA 81 + ppx hep until 6-12 PP
if thrombotic PAS, tx ?
ASA 81 + therapeutic heparin during pregnancy and pp
drug induced lupus
- name common drugs
- serology
- tnfi serology particularity
- tx :
- hydralazine, quinidine,procainamide, tnfi
- ANA, anti histone
- ANA, but not anti histone, dsdna +
- NSAID, topical steroid, HCQ if derm msk, rarely po steroid
Shrinking lung syndrome, what is it ?
related tod iaphragmatic muscle weakness in lupus
lungs are clear
decrease in MIP and MEPs
Reduced volumes on PFTS
Libman Sacks endocarditis
- associated with what
- whatt ?
- results in what phenomena ?
- tx ?
- APLA
- immune complex accomulation with mononuclear cells, hematoxylin bodies + fibrin + platelet forming a thrombus
- embolic phenomena
- tx : steroids and anticoagulation
DDX for bilateral parotid gland enlargement
Ø Sjogren’s
Ø Infectious – Mumps, TB, bacterial ,
Hep C, HIV
Ø Sarcoidosis
Ø Lymphoma
Ø Alcoholism, Anorexia/bulimia
Ø IgG4 related disease*
*Sending IgG4 is now routine – esp if enlargement of submandibular glands / lacrimal glands without parotid enlargement.
if you have lacrimal glandd enlargement, even in the context of sjogren syndrome, raises concern for what ?
concern for lymphoma !
dx proceddure for sjogern
Schirmer’s test ( tears in eye assessment)
Unstimulated salivary flow
ENT : minor salivary gland bx with focal lymphocytic sialadenitis
