SS,raynaud, myositis

Question 1

serologies in SS
- which one specific to limited ( CREST)
- which one specifgic to diffuse SS ?

are they good for monitoring ?

Answer 1

• anticentromere
• Anti Scl 70 / Topo

no

Question 2

internal organ involvement in diffuse SS , consequences ?

Answer 2

ILD
hypertensive renal crisis

Question 3

Crest syndrome ? what is it

Answer 3

Calcinosis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasias

Question 4

Scleroderma renal crisis
- how does it present
- worsen with what
- tx ?

Answer 4

• progressive renal failure, htn, bland UA
• prednisone
• acei ( captopril)

Question 5

pulmonary hypertenson, more common is limited diffuse SS ?

Answer 5

limited

Question 6

ILD pattern in SS ?

Answer 6

NSIP
UIP

Question 7

GI complication of SS ?

Answer 7

Gave !

Question 8

5 SS manifestation

Answer 8

1. Skin
2. Raynaud’s +/- ulcers
3. Lung (ILD, PH)
4. GI
5. Kidney (SRC)

Question 9

is htn always present in SRC ?

Answer 9

no

Question 10

SRC autoantibody ?

Answer 10

RNAP3

Question 11

ANA + in raynaud ?

Answer 11

non

Question 12

in secondary renaud, what’s a particular abnormality that you can see ?
is it symetrical or assym
progressive or non progressive
male or female
age onset

Answer 12

abnormal nail folds
asymetrical
progressive
male
> 40 y

Question 13

2nd raynaud etiology

Answer 13

CTD
vasculitis
infection

precisely
ssc, mctd, sle, hypot4, carcinoid , pcc, hbv, hcv, pavob19, heme malignancy

Question 14

raynaud tx

Answer 14

CCB = 1st line
2nd line = topical nitrates, PDE5 inhibitor

Question 15

what screening should be uptodate for all DM/PM at presentation or any disease flare?

Answer 15

cancer screening

Question 16

wha’t anti synthethase syndrome

Answer 16

Anti-synthetase Syndrome = Anti-Jo1 Antibody * Raynaud’s phenomenon, mechanic’s hands,
arthritis

Question 17

what’s anti mda5

Answer 17

Rapidly progressive ILD, skin ulceration

Question 18

Anti NXP2 and anti TF1 gamma antibodies

Answer 18

Highly associated with malignancy

Question 19

myositis
- ?acute
- sym?
- progressive
- distal or proximal ?

Answer 19

• acute
• sym
• progressive
• proximal

Question 20

ddx of myositis/myopathy

Answer 20

• Drugs
• Statins, colchicine, alcohol, etc
• Infectious/ Viral
• HIV, influenza, EBV, CMV
• Pyomyositis
• Hypothyroid myopathy
• Electrolyte disorders
• Severe hypokalemia, hypophosphatemia
• Genetic myopathy
• (eg muscular dystrophy, or
  disorders of glycogen/lipid metabolism, mitochondrial disorders)

Question 21

Clinical features of DM/PolyM

Answer 21

• Muscle weakness: Insidious over weeks/months, symmetric and proximal > distal, neck flexor
• Can involve: Heart, diaphragm, oropharynx, and esophagus
• Cardiac: myocarditis, arrhythmias, CHF
• Pulmonary: ILD (NSIP, UIP) DLCO or CT abnormalities, Pulm HTN
• Skin: Gottron’s papules, shawl sign, heliotrope rash, generalized erythroderma, periungal
  erythema, mechanic’s hands, scalp psoriasiform changes, calcinosis cutis

Question 22

investigations for DM/PM

Answer 22

labs including atb
muscle mri : edema ( and will guide bx )
muscle biopsy
muscle emg
cardiac work up
slp assessment
spirometyr & MIP/MEP for diaphragmatic weakness
age appropriate screening

Question 23

why need spirometrry with MIP/MEPS in DM/PM?

Answer 23

to r/o diaphragmatic involvement

Question 24

HCQ vs DM/PM management - helpful when ?

Answer 24

only in skin manifestations

Question 25

if have rapidly progressive ILD in DM/PM - what to do ?

Answer 25

needs triple therapy (IV steroids + 2 immunosuppressive options) -Ritux, CYC, IVIG, CNI, MMF

Question 26

if have refractory or severe PM/DM

Answer 26

* IVIG * Rituximab * Cyclophosphamide * Abatacept

Question 27

Inclusion body myositis - age - gender - onset - CK level - distal vs prox - tx response

Answer 27

* Older, M > F, insidious onset * CK tends to be lower * Distal > proximal muscle weakness * Poor treatment response

Question 28

Immune mediated necrotizing myopathy - what's the antibody - what to rule out - level of CK

Answer 28

- anti HMGcoa reductase atb - paaneoplastic - very high

Question 29

which SARDs has highest ILD

Answer 29

RA, Systemic sclerosis, Idiopathic inflammatory myositis (incl PM, DM, MDA5, IIM), MCTD, Sjogren’s

Question 30

does SLE have lots of ILD

Answer 30

not really

Question 31

how to screen for ILD in Rheumatic diseasse

Answer 31

Screenwith: PFT that includes DLCO+TLC and/or HRCT(HRCT+PFT>PFTalone)