SS,raynaud, myositis Flashcards
serologies in SS
- which one specific to limited ( CREST)
- which one specifgic to diffuse SS ?
are they good for monitoring ?
- anticentromere
- Anti Scl 70 / Topo
no
internal organ involvement in diffuse SS , consequences ?
ILD
hypertensive renal crisis
Crest syndrome ? what is it
Calcinosis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasias
Scleroderma renal crisis
- how does it present
- worsen with what
- tx ?
- progressive renal failure, htn, bland UA
- prednisone
- acei ( captopril)
pulmonary hypertenson, more common is limited diffuse SS ?
limited
ILD pattern in SS ?
NSIP
UIP
GI complication of SS ?
Gave !
5 SS manifestation
- Skin
- Raynaud’s +/- ulcers
- Lung (ILD, PH)
- GI
- Kidney (SRC)
is htn always present in SRC ?
no
SRC autoantibody ?
RNAP3
ANA + in raynaud ?
non
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
abnormal nail folds
asymetrical
progressive
male
> 40 y
2nd raynaud etiology
CTD
vasculitis
infection
precisely
ssc, mctd, sle, hypot4, carcinoid , pcc, hbv, hcv, pavob19, heme malignancy
raynaud tx
CCB = 1st line
2nd line = topical nitrates, PDE5 inhibitor
what screening should be uptodate for all DM/PM at presentation or any disease flare?
cancer screening
wha’t anti synthethase syndrome
Anti-synthetase Syndrome = Anti-Jo1 Antibody * Raynaud’s phenomenon, mechanic’s hands,
arthritis
what’s anti mda5
Rapidly progressive ILD, skin ulceration
Anti NXP2 and anti TF1 gamma antibodies
Highly associated with malignancy
myositis
- ?acute
- sym?
- progressive
- distal or proximal ?
- acute
- sym
- progressive
- proximal
ddx of myositis/myopathy
- 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)
Clinical features of DM/PolyM
- 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
investigations for DM/PM
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
why need spirometrry with MIP/MEPS in DM/PM?
to r/o diaphragmatic involvement
HCQ vs DM/PM management - helpful when ?
only in skin manifestations
if have rapidly progressive ILD in DM/PM - what to do ?
needs triple therapy (IV steroids + 2 immunosuppressive options) -Ritux, CYC, IVIG, CNI, MMF
if have refractory or severe PM/DM
- IVIG
- Rituximab
- Cyclophosphamide
- Abatacept
Inclusion body myositis
- age
- gender
- onset
- CK level
- distal vs prox
- tx response
- Older, M > F, insidious onset
- CK tends to be lower
- Distal > proximal muscle weakness
- Poor treatment response
Immune mediated necrotizing myopathy
- what’s the antibody
- what to rule out
- level of CK
- anti HMGcoa reductase atb
- paaneoplastic
- very high
which SARDs has highest ILD
RA, Systemic sclerosis, Idiopathic inflammatory myositis (incl PM, DM, MDA5, IIM), MCTD, Sjogren’s
does SLE have lots of ILD
not really
how to screen for ILD in Rheumatic diseasse
Screenwith:
PFT that includes DLCO+TLC and/or HRCT(HRCT+PFT>PFTalone)
