Lupus + APS + Sjogren

Question 1

anti dsdna is assocaited with what ?

Answer 1

Associated with lupus nephritis

Question 1

ddx of ANA

Answer 1

• 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%)

Question 2

can anti dsdna used for monitoring ?

Answer 2

yes !

Question 3

anti histone in lupus is good for what ?

Answer 3

Drug induced lupus
SLE

Question 4

what labs is required to diagnose MCTD ?

Answer 4

anti RNP

Question 5

those with lupus and antiro/antila , are more at risk of what as a bb ?

Answer 5

risk of congenital heart block and neonatal cutaneous lupus

Question 6

managment of non renal SLE tx?

Answer 6

1. HCQ

Question 7

if have non renal; SLE but have UPCR > 500/htn, dshould give what ?

Answer 7

ACEi/ARB

Question 8

how often do you do cervical cancer screening in SLE patients ?

Answer 8

Annual basis regardless of immunosuppession

Question 9

benefit of hcq in non renal sle

Answer 9

• increase survival
• decrease renal flare risk

Question 10

mild non renal SLE ( const sx, mild arthritis, rash <9% BSA, plt 50-100) : tx option ?

Answer 10

HCQ ( +/- GC)

Question 11

Moderate non renal SLE ( ra like arhtritis, rash 8-18% , cutatenous vasculitis, plt 30-50 , serositis) -: tx options

Answer 11

-mtx
- azathioprine
-consider biologis : Belimumab

Question 12

severe systemic lupus erythematous ( organ threatening disease - nephritis/cerebritis/myelitis/pneumonitis/mesenteric vasculitis), plt <20, ttp like disease, AIHA< rash >18%
-tx ?

Answer 12

mmf
bel
Anifrolumab
cyclcospoprin
ritux

Question 13

when is belimumab and anifrolumab recommended first line in non renal SLE ?

Answer 13

• 1st line in severe disease refers to cases of extrarenal SLE with non-major organ involvement, but extensive disease from skin, joints

Question 14

which biologics in non renal SLE is great for skin involvement

Answer 14

anifrolumab

Question 15

which meds ot aovid in non renal SLE if pt has neuropsych disease

Answer 15

aniflrolumab
belimumab

Question 16

who should be biopsied in ALL SLE

Answer 16

– Glomerular hematuria and/or cellular casts
– Proteinuria >0.5g/24h or UPCR >500 mg/g
– Unexplained dec in GFR

Question 17

renoprotective meds soin lupus nephritis?

Answer 17

RAAS blockage
SGLT2

Question 18

if have APS nephropathy, what do you give as well ?

Answer 18

VKA
heparin

Question 19

when do you treat lupus nephritis class 1-2 with immunosup

Answer 19

if nephrotic syndrome –> immunosup

Question 20

what do you tx class 3-5 with in terms of lupus nephritis

Answer 20

• aggressive immunosuppression
• antiproteinuric/ antiHTN meds

Question 21

what do you teat class 6 lupus aka advanced sclerotic LN ( 90% of glomeruli globally sclerosed w/o residual activity)

Answer 21

• supportive therapy
• +/- tx extra renal manifestaiton

Question 22

lupus renal syndrome : SLE + TMA
- what to measure ?
- how to tx

Answer 22

• adamst 13, APLAs
• plex, steroids, eculizumab, anticoag

Question 23

class 3 , class 4 lupus nephritis
- HOW DO THEY PRESENT
- induction tx ?
- then what ?

Answer 23

• hematuria, proteinuria, hypertension, renal failure
• high dose GC + other agent ( MMF or BEL/CNI/CYC )
• maintenance : HCQ+ opther agent +/- low dose prendisone

Question 24

risk of cyclophosphomide

Answer 24

Risks infertility (significant) others : infection, malignancies esp GU (++hydration), cytopenias

Question 25

is mmf safe for pregnancy ?

Answer 25

not but can use it if considering future fertility consideration

Question 26

what other meds do you give for all lupus nephriits

Answer 26

HCQ , ACEi

Question 27

who can get rituximab in LN ?

Answer 27

persistent disease activity or inadequate response

Question 28

if you induced with cyclosporine, do you keep for maintenance ? if not what to do ?

Answer 28

MMF

Question 29

if have pregnancy plans and on MMF for LN, what can you give ?

Answer 29

AZA

Question 30

how long do you treat LN ?

Answer 30

>36 months

Question 31

antiphospholid syndrome - obstetrical crtierias , more at risk of what ?

Answer 31

- >3 prefetal losses - fetal death after 16 weeks - severe pre eclampsia

Question 32

entry criterion for antti phospholipid syndrome ?

Answer 32

1 documented clinical criteria + positive APL test within 3 years

Question 33

cardiac valve issues in APS ?

Answer 33

- thickening - vegetation

Question 34

hematology issues in APS

Answer 34

thrombocytopenia

Question 35

what's high risk APS profile wthout p[rior thromobsis ? tx ? what if SLE also prsent ?

Answer 35

LA + double/triple positivity ASA for life if SLE + APS --> HCQ

Question 36

why not use DOAC in thrombotic APS ?

Answer 36

associated with risk of arterial/venous thrombotic events

Question 37

eculizimab is good in what APS context ?

Answer 37

if TMA-Renal manifestation

Question 38

catastrophic APS , how can you define

Answer 38

concomitant/successive thrombosis in more than 3 organs

Question 39

how do you treat catastrophic APS ?

Answer 39

- full dose anticoagulation - high dose glucocorticoid + PLEX/IVIG

Question 40

should you continue HCQ in pregnancy ?

Answer 40

yes

Question 41

safe immunosuppressive treatment in pregnancy

Answer 41

HCQ steroids AZA tacroliumus Cyclosporine ( not cyclophosphomide)

Question 42

what should you start in lupus + pregnancy , benefit ?

Answer 42

ASA prior 16 weeks and reduce preeclampsia risk

Question 43

if history of neonatal lupus, what shouldd be done ?

Answer 43

HCQ + serial fetal echo weekly from week 16-26

Question 44

with lupus nephritis + pregnnacy, can you continue ACEi . how long LN should be inactive ?

Answer 44

no want to make sure that LN inactive for > 6M

Question 45

if positive APL but no APS, tx ?

Answer 45

ASA alone

Question 46

if obs APS , tx ?

Answer 46

ASA 81 + ppx hep until 6-12 PP

Question 47

if thrombotic PAS, tx ?

Answer 47

ASA 81 + therapeutic heparin during pregnancy and pp

Question 48

drug induced lupus - name common drugs - serology - tnfi serology particularity - tx :

Answer 48

- hydralazine, quinidine,procainamide, tnfi - ANA, anti histone - ANA, but not anti histone, dsdna + - NSAID, topical steroid, HCQ if derm msk, rarely po steroid

Question 49

Shrinking lung syndrome, what is it ?

Answer 49

related tod iaphragmatic muscle weakness in lupus lungs are clear decrease in MIP and MEPs Reduced volumes on PFTS

Question 50

Libman Sacks endocarditis - associated with what - whatt ? - results in what phenomena ? - tx ?

Answer 50

- APLA - immune complex accomulation with mononuclear cells, hematoxylin bodies + fibrin + platelet forming a thrombus - embolic phenomena - tx : steroids and anticoagulation

Question 51

DDX for bilateral parotid gland enlargement

Answer 51

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.

Question 52

if you have lacrimal glandd enlargement, even in the context of sjogren syndrome, raises concern for what ?

Answer 52

concern for lymphoma !

Question 53

dx proceddure for sjogern

Answer 53

Schirmer's test ( tears in eye assessment) Unstimulated salivary flow ENT : minor salivary gland bx with focal lymphocytic sialadenitis