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Rheumatology > CT disorder > Flashcards

CT disorder Flashcards

1
Q

SLE epidermiology

A

F>M

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2
Q

SLE pathogenesis

A

Genetics contributes 30%

  • Provides susceptibility
  • E.g. C1q deficiency (high penetrance for SLE); MHC class (HLADR2, DR3, DRB1), IFN related pathway genes

Environmental

  • UV light, DNA damage, skin damage
  • Infection ?EBV via molecular mimicry
  • Drugs e.g. TNFi, procainamide, hydralazine
  • Heavy metals
  • Smoking

B cell activation and autoantibody production + IFN production + effector T cells (TH17 cells) + problems with phagocytosis (increased apoptotic material serves as autoantigen)

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3
Q

ACR/EULAR classification criteria 2019

A

Does not equal diagnostic criteria. Used in research. But useful to look at it to help reach diagnosis.

Must have positive ANA
Different organs affected
If renal biopsy positive for lupus nephritis, and ANA positive, don’t need other organ manifestations.

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4
Q

Cutaneous manifestations for SLE

A 
Butterfly/malor rash - acute cutaneous lupus 
Subacute cutaneous lupus
Chronic discoid lupus (scarring)
Photosensitive
Bullous
Urticaria
Chill blains
Panniculitis
Alopecia (Rx JAKi)
Painless nasal ulcers

Remember cutaneous lupus does not equal systemic lupus!

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5
Q

MSK manifestations for SLE

A

Arthralgia - symmetrical, migratory, polyarticular

Arthritis (like RA< usually non-erosive but can be deforming = Jaccoud’s arthritis)

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6
Q

Serositis in SLE

A

Pericarditis (big globular heart shadow on CXR)

Also pleuritis

High CRP

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7
Q

Do you get CRP rise in SLE flare?

A

Not usually - must exclude infection!

Unless there is serositis

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8
Q

Pulmonary manifestations in SLE

A

Pleurisy/pleural effusion

Acute pneumonitis

Pulmonary haemorrhage (rare)

ILD: NSIP most common

PE ?APLS

Pulmonary HTN

“Shrinking” lung syndrome

  • Progressive SOB
  • Raised hemidiaphragm, reduced lung volumes
  • RFTs restrictive pattern
  • Unsure mechanism but can respond to immunosuppression
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9
Q

Cardiovascular manifestations in SLE

A 
Pericarditis (most common)
Premature CAD (most common)
Vasculitis
Libman-sacks endocarditis (valvular lesions as a result of microthrombi on heart valves)
Lupus myocarditis
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10
Q

Haematologic manifestations in SLE

A

Anaemia

  • Multifactorial
  • Chronic inflammation (most common), IDA, autoimmune haemolytic anaemia (DAT+), aplastic anaemia, anti-EPO ab

Leucopenia

  • Low lymphocyte count is common but not severe
  • Due to peripheral destruction but the cells are quite normal

Thrombocytopenia

  • Overlap with ITP (10% SLE have ITP; 50% ITP meet SLE criteria)
  • Mainly peripheral destruction
  • Different ab to pure ITP
  • MAHA (mainly TTP)
  • Myelofibrosis

Lymphadenopathy
Splenomegaly

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11
Q

How to differentiate TTP vs DIC?

A

DIC - abnormal coags, abnormal fibrinogen

TTP - normal coags. Platelets and Hb abnormal.

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12
Q

Neurological manifestations in SLE

A

CNS: headache, mood disorder, seizure, cognitive dysfunction, CVA, myelitis etc

PNS: sensory-motor axonal polyneuropathy

Consider catastrophic APLS (seizure, encephalopathy)
Need urgent ICU and immunotherapy and anticoagulation

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13
Q

Ribosomal P abs =

A

Neuro lupus

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14
Q

Immunologic manifestations in SLE

Antibody findings

A

Less specific findings

  • ANA positive >99% (homogenous pattern)
  • Low C3, C4
  • Raised ESR:CRP
  • Positive RF 15-35%

More specific

  • dsDNA 60% (disease activity, renal disease)
  • Anti-smith (most-specific)
  • SSA (Anti-Ro), SSB (anti-La)
  • U1RNP (also in mixed CT disease)
  • Ribosomal P (neuro lupus)
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15
Q

SLE and pregnancy

What are 2 important things to know?

A

Most have successful pregnancies

1) Anti Ro/La positive associated with
- Congenital heart block 1% (increases with subsequent pregnancies if previous babies with CHB)
- Weekly USS
- Consider fluorinated steroids (crosses placenta e.g. dex) +/- IVIG in 2nd degree HB to prevent progression

2) Antiphospholipid status. If positive:
- Asymptomatic: consider low dose aspirin
- Prior obstetric morbidity: low dose aspirin, prophylactic clexane
- Prior thrombosis: therapeutic clexane; continue 6/12 post partum

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16
Q

SLE treatment

A

Non-pharmacologic

  • UV protection
  • Smoking cessation
  • Immunisation
  • Avoid estrogen therapies, sulfonamides (A/W flares)
  • Replete vitamin D
  • CV risk management

Hydroxychloroquine
- Everyone should be on it

Others
Corticosteroids - useful in controlling disease; some stay on small dose lifelong
MTX - useful for polyarthritis, stay away in ILD, renal impairment
Azathioprine 
Leflunomide - stay away in ILD
Mycophenolate - esp renal/pulmonary
Cyclosporin
Cyclophosphamide

Rituximab in refractory disease

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17
Q

Risk of HCQ

A

Retinal toxicity
Accumulative toxicity, dose and duration related
Detected with retinal exam/OCT - “pre maculopathy” may be reversible. Later macular disease “bull’s eye”, visual field loss, often irreversible.

Baseline eye check before starting HCQ
After 5 years, need annual eye checks

Increased risk after 5 years, in older age, renal impairment
Keep dose <5mg/kg Ideal body weight

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18
Q

Biologics in SLE

A

1) Rituximab and veltuzumab (CD20 ab)
- Modest evidence in trials but does work well in the right patients
- Off label use
- Refractory disease, lupus nephritis

2) Belimumab
- Targets B cell activating factor (BAFF) - promotes B cell survival and differential
- FDA approved in lupus nephritis. SAS access only.
- Moderate SLE that has not responded to HCQ/MYC. Not in severe disease or lupus nephritis.

3) Tibulizumab
- Targets BAFF and IL17
- Ongoing trials

4) Anifrolumab
- Anti-IFN therapy
- Trials look promising

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19
Q

APLS presentation

A

Primary or secondary (SLE)

Thrombosis +/- obstetric complications
Also thrombocytopenia, cardiac valve abnormalities livedo reticularis, renal microangiopathy, chorea, myelitis

Associated conditions: HELLP, pre-eclampsia, MAHA

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20
Q

When you see livedo reticularis.. what should you check?

A

APLS ab

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21
Q

APLS ab

A

Anti-cardiolipin ab

Anti-Beta2-glycoprotein1

Lupus anticoagulant - highest risk for thrombosis

“Triple positive” at highest risk of thrombosis
Look out for the prolonged APTT!!

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22
Q

APLS treatment

A

Asymptomatic/primary prevention: no treatment

Secondary prevention:

  • Lifelong anticoagulation with warfarin (INR2-3)
  • DOACS not recommended

Management in pregnancy
Prior thrombosis: therapeutic clexane (continue at least 6/12 post partum) + aspirin
Prior pregnancy loss (meets criteria): low dose aspirin + prophylactic clexane
Asymptomatic: consider low dose aspirin

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23
Q

Pathophysiology Systemic sclerosis

A

Initially, have vascular damage

Autoimmunity - innate, cell mediated and humoral

Tissue fibrosis is the characteristic end result (by the time we see this its too late)

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24
Q

Systemic sclerosis specific ab

A

Anti-centromere = Limited SSc or CREST; PHTN

Anti-topoisomerase I = Anti-Scl-70 = ILD

Anti-RNA polymerase III = severe renal and skin disease

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25
Q

Disease subtypes of Systemic sclerosis

A

Limited cutaneous Systemic sclerosis (CREST)

  • Long history of Raynaud’s
  • Distal skin sclerosis, digital ulcers
  • Major mortality from pulmonary arterial hypertension
  • Anti-centromere ab

Diffuse cutaneous Systemic sclerosis

  • Short history of Raynaud’s
  • Proximal limb or trunk involvement, with skin sclerosis
  • Extensive skin disease (contractures) + distal ulcers + more internal organ involvement (lung and kidney)
  • Tendon friction rubs
  • Awful morbidity and mortality
  • Lack of therapy
  • Anti-Scl-70 (ILD) and RNA polymerase III (scleroderma renal crisis)
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26
Q

Pulmonary involvement in Systemic sclerosis

A

1) Lung fibrosis
- Occurs in the first 5 years in dcSSc
- Most have lung involvement. Clinically significant in 30%.
- Now the major cause of mortality
- If >20% lung involvement, they will likely progress
- Most prevalent pattern is NSIP - subpleural sparing, ground glass. No honeycombing.
- May lead to PAH

2) Pulmonary arterial hypertension
- Mostly group 1
- Can occur anytime
- Need to keep screening - 1-2% develop per year
- Very serious
- Present in limited > diffuse
- TTE alone may miss up to 30%. BNP is useful.

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27
Q

Rx for pulmonary hypertension in systemic sclerosis

A

Endothelin receptor antagonist

  • Bosentan, macicentan, ambrisentan
  • AE: LFT abnormality, tetatrogenic, fluid retention, anaemia

NO stimulation

  • Sildenafil (PDE5), tadalafil (PDE5), Riociguat (GC stimulator)
  • AE: headache, bleeding, visual disturbance

Prostacyclin analogues

  • IV Epoprostenol (severe SSc-PAH), iloprost, beraprost, trepostanol, selexipag
  • AE: headache, muscle cramps, diarrhoea

Lung transplant

  • CCBs monotherapy don’t work!
  • Benefits of PAH specific therapies were not as good as for iPAH
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28
Q

Cardiac manifestation Systemic sclerosis

A

Arrhythmias/conduction defects e.g. VT
Myocarditis (often with skeletal muscle disease)
Cardiac fibrosis (80% in autopsy studies)
Valvular heart disease

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29
Q

MSK/skin involvement Systemic sclerosis

A

Puffy fingers

Proximal progression of skin thickening

Joint contractures +/- arthritis

Unable to open mouth due to skin tightening

Tendon friction rubs (associated with kidney involvement)
- Especially common in diffuse SSc with RNA polymerase III

Calcinosis

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30
Q

GI involvement Systemic sclerosis

A

Most frequent internal organ manifestation ~90%

Dental disease
Oesophageal dysmotility, strictures, candidiasis
Reflux
Watermelon stomach (GAVE) - poor motility, telangiectasia that bleed
Small bowel overgrowth (due to gut wall scarring and motility disturbance) - bloating, diarrhoea, weight loss, high folate
Large bowel, diverticular disease
Faecal incontinence due to reduced sensation, altered bowel habits, constipation overflow diarrhoea

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31
Q

What’s an important renal manifestation in Systemic sclerosis?

A

Scleroderma renal crisis!

  • Occurs in the first 5 years in dcSSc
  • Accelerated hypertension (retinal changes, hypertensive encephalopathy, PRES). Normally these people have low BP ~90, so when they raise 20mmHg this is significant. OR new onset BP >150/85.
  • Renal impairment
  • Associated with RNA polymerase III, tendon friction rubs
  • Diffuse subtype
  • Association with steroids (be careful in using steroids in diffuse subtype)
  • 40% will need dialysis but recovery can happen up to 2 years after
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32
Q

Vasculopathy in Systemic sclerosis

Treatment

A

1) Raynaud’s (universal)
- Primary Raynaud occurs in those without CT disease. Negative ab. No pain. Short lasting ~15 min.
- Secondary (CT disease) - get tissue damage.

Rx: CCB, PDE5 inhibitor, IV prostacyclin (iloprost, epoprostenol) in severe disease after oral therapy

2) >50% have digital ulcers
- IV Prostacyclin (iloprost) or PDE5 inhibitor or endothelin receptor antagonist (Bosentan)
- IV prostacyclin can be protective for a few months after. Very effective
- May need surgical debridement in necrosis

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33
Q

Pregnancy in Systemic sclerosis

A

Limited: generally do well

Diffuse: high maternal mortality

Increased risk of hypertension, preeclampsia and CS, preterm birth, IUGR, organ complication

Dangerous in pulmonary HTN
Alot of the drugs can’t be used

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34
Q

Screening in systemic sclerosis

A

Screen for ILD and pulmonary HTN

Annual ECHO, PFTs

Baseline CT. If there are change in PFTs, then repeat CT.

35
Q

Stem cell transplant in systemic sclerosis

A

Great treatment if patients survive (5-10% mortality)

Good for skin and lung involvement, if done early.

36
Q

Therapy for systemic sclerosis-associated ILD?

A

Nintedanib + mycophenolate (combination therapy)
Less drop in FVC over time

Others
O2 to maintain SpO2 88% and above 
GORD therapy 
Quit smoking
Vaccinations
37
Q

What is Mixed CT disease?

Clinical features

A

It is an overlap syndrome (doesn’t mean we don’t know what it is)
Features of SLE, scleroderma, rheumatoid, myositis

Raynaud's very common
Hand oedema (puffy hands)
Arthralgia/arthritis
Myositis, trigeminal neuralgia
ILD
Pulmonary HTN (commonest cause of death)
38
Q

Mixed CT disease labs

A

ANA + speckled
Characteristic ENA: U1RNP (high titre)

Leucopenia, thrombocytopenia
Raised ESR
70% RF (more likely to have arthritis). CCP negative.

Important negatives
- dsDNA, anti-SM

39
Q

“Puffy hands”, RNP without dsDNA

Dx?

A

Mixed CT disease

40
Q

Sjogren’s syndrome presentation

A

Gland involvement

  • inflammation, fibrosis, lymphocyte infiltration
  • Dry mouth (dental caries, oral candidiasis), dry eyes
  • Glandular enlargement occurs in 30% (high rate of lymphoma)

Extraglandular

  • Fatigue, arthralgia/arthritis (universal)
  • Palpable purpura often on legs
  • Cystic ILD
  • Distal renal tubular acidosis/glomerulonephritis
  • Cryoglobinaemia (renal involvement, rash, mononeuritis multiplex)
  • Dorsal root ganglionopathy
  • Peripheral neuropathy
  • NHL
41
Q

Sjogren’s syndrome can be primary or secondary. Which conditions can it be secondary to?

A

RA >SLE > SSc

HIV

42
Q

Sjogren’s syndrome management

A

Symptomatic

Dry mouth
Citrus fruit or gum to stimulate saliva 
Saliva replacement/lubricants
Secretagogues (pilocarpine) for both dry mouth and eyes. But side effects. 
Avoid meds that cause dryness 
Dry eyes
Lubricating eye drops (preservative free)
Topical cyclosporin
Punctal plugs
Secretagogues

Extraglandular
Exercise
NSAIDs
HCQ for systemic manifestations

Life-threatening: pulsed methylpred +/- plasma X +/- RTX if cryoglobulinaemia

43
Q

DDx of parotid mass

A

Infections - viral (mumps, EBV, HCV, HIV); bacterial (acute parotitis, TB), fungal

Autoimmune - Sjogren’s, GPA

Inflammatory - IgG4 disease, allergic parotitis, kilmura disease

Metabolic - diabetes, bulimia, alcholism, hyperlipoproteinaemia

Neoplastic - lymphoma, leukaemia, Warthin tumour

Granulomatous - sarcoidosis

44
Q

What’s Schirmer’s test?

A

Leave blotting paper under lower lid
Then measure distance of moisture
Correlate with tear production

Useful in Sjogren’s

45
Q

Investigations in Sjogren’s

A

Anti-SSA (Anti-Ro60) and/or Anti-SSB - diagnostic
Positive RF & ANA titre >1:320 - diagnostic

Schirmer’s test (tear production)

Salivary gland biopsy

dsDNA absent
Elevated ESR
Polyclonal hypergammaglobulinaemia 30%
Anaemia, leucopenia (usually neuts) and thromboctopenia
Cryoglobulinaemia in 15%
46
Q

What are the subtypes of idiopathic inflammatory myopathies?

A

1) Dermatomyositis
2) Anti-synthetase syndrome
3) Immune-mediated necrotising myopathy (IMNM or NAM)
4) Inclusion body myositis
5) Polymyositis ?does this exist as a distinct entity

47
Q

Presentation idiopathic inflammatory myopathies

A

3 “Ps”

Progressive, painless, proximal weakness

BUT all will have extra-muscular manifestations - cutaneous, diaphragmatic and intercostal muscle weakness = T2RF, oesophageal muscle weakness = aspiration, dysphagia, ILD, myocarditis, MI

48
Q

Classic dermatomyositis cutaneous manifestations

A

Gottren’s sign/papules (over extensors of large joints)
Shawl sign
V sign
Heliotrope rash (peri-orbital)
Holster sign (lateral thigh where your gun holster would be )
Periungual (around finger nail) erythema

Skin findings may precede muscle weakness

49
Q

Myositis specific ab

Antisynthetase syndrome
Skin disease (dermatomyositis)
INMN
IBM

A

Antisynthetase syndrome
- 9 ab involved - Jo-1 (most common), PL7, PL12; all 3 are associated with lung disease

Skin disease (dermatomyositis)
- SAE, Mi-2, MDA5 (ILD), NXP2, TIF1

INMN
- SRP, Anti-HMGCR (very specific to stain associated IMNM)

IBM
- Anti-CN1A

50
Q

Anti-synthetase syndrome

Clinical Presentation

A 
Myositis
Jo1, PL7, PL12; total 9 ab ( all 3 are associated with lung disease)
ILD - most important cause of mortality
Mechanic's hands
Raynaud phenomenon
Inflammatory polyarthritis
Oesophageal dysmotility (distal)
Fever
51
Q

Inclusion body myositis
Clinical presentation
Treatment

A

M>F, older demographic 50-60yo

Muscle pattern is different to the other myositis
***Asymmetric
Distal upper limb (finger flexors, wasting of forearm muscles, watch falling off)
Proximal leg weakness (quadricep weakness, frequent falls going downstairs)

Mildly elevated CK (but not >10x ULN)

Anti-CN1A

Less responsive to IS unlike other inflammatory myopathies. Can have limited trial of glucocorticoids.

52
Q

Statin-associated IMNM
Clinical Presentation
Treatment

A

1:100,000 statin users

Associated with HLADRB1*11:01

Usually after months on treatment, persist after cessation (ongoing immune reaction), CK usually >10x normal

Anti-HMGCR ab very specific

Treatment
Statin cessation won’t work
Often will need steroids +/- IVIG

53
Q

MDA5+ dermatomyositis
Clinical presentation
Treatment

A

May not have muscle disease, or if they have, its subtle

Associated with rapidly progressive ILD

Very high mortality 50-60% in 6 months

Some distinctive features from other DM - ulcerating lesions, inverse gottren’s or palm papules (instead of MCP, PIP distribution, gottren’s papules are located on the palm), pneumomediastinum

Rx: EARLY AGGRESSIVE IMMUNOSUPPRESSION (may survive 2 years if done early)

54
Q

Do we need to avoid statins in idiopathic inflammatory myopathy?

A

NO

Unless they’re anti-HMGCR positive (statin-associated IMNM)

55
Q

Behcet disease

Clinical features

A

Cardinal features
- Relapsing oral and genital ulcers with bilateral posterior or panuveitis

Other features

  • Recurrent papulopustular lesions, erythema nodosa like lesions, non-erosive mono/oligo arthritis
  • Pathergy (exaggerated skin lesion after minimal trauma)
  • Vasculitis involving all vessels (thrombotic, pseudoaneurysms)
56
Q

Which genetic factor is associated with Behcet disease?

A

HLA-B*151

57
Q

What is sarcoidosis characterised by?

A

Systemic inflammatory disorder characterised by non-caseating granulomas

58
Q

Sarcoidosis presentation

A

Acute and chronic arthritis (knees and ankles)
Muscle involvement common although often asymptomatic
Bilateral hilar lymphadenopathy
Pulmonary infiltrates
Uveitis

Also cardiac, neurologic, cutaneous

59
Q

What’s Lofgren syndrome?

A

Acute sarcoidosis

Erythema nodosa
Acute polyarthritis (typically ankles)
Hilar lymphadenopathy
Fever

60
Q

What’s CREST syndrome?

A

Type of limited cutaneous sclerosis

C - calcinosis
R - Raymaud's (years)
E - esophageal dysmotility 
S - skin changes limited to UL, face; gradual onset 
T - telangectasia
61
Q

Does pulmonary hypertension occur in limited or systemic sclerosis?

A

Both

~10% in both subtypes (maybe slightly more in limited)

62
Q

ANA with centromere pattern is associated with which disease?

A

Limited sclerosis

63
Q

ANA with homogenous pattern is associated with which disease?

A

Lupus (dsDNA, histone +)

64
Q

Main causes of mortality in scleroderma

A

ILD and pulmonary HTN

Previously was scleroderma renal crisis but ever since the introduction of ACEI, mortality has fallen

65
Q

Who with systemic sclerosis-ILD is likely to progress in their ILD?

A

Early dcSSc with anti-Scl70
Early dcSSc with high CRP

= High risk phenotypes = need treatment

66
Q

ANA with nucleolar pattern is associated with which disease?

A

Progressive overt systemic sclerosis

67
Q

How do we monitor patients with systemic sclerosis-ILD?

A

Rapidly progression is likely to occur in the first 5 years
Monitor with spirometry and DLCO every 3-4 months for 5 years after disease onset then yearly

No advantage in serial HRCTs if PFTs stable

68
Q

Who with systemic sclerosis-ILD should receive immunosuppressive treatment?

A

Clinical ILD

- FVC% predicted or DLCO% predicted

69
Q

What investigations to do in someone with SSc-ILD and worsening respiratory symptoms?

A

Repeat

  • PFTs + DLCO
  • HRCT ?progressive ILD
  • ECHO
  • NT-proBNP
  • DETECT algorithm (online calc for pHTN)

Things to suggest pHTN

  • FVC/DLCO ratio >0.6
  • TR velocity >3.2msec (should = systemic pulmonary pressures)
  • NT-proBNP >2 fold ULN
70
Q

How to screen for pulmonary HTN in systemic sclerosis?

A

METHOD ONE
Screen every 12 months
PFTs - DLCO ≤50%
ECHO - sPAP ≥40mmHg

= If positive, do RHC
= ECHO uses TR jet which is absent in up to 39% of patients
= $$$, expertise required, poor image quality
= Sensitivity 88%, specificity 83%

METHOD TWO
Screen every 12 months
PFTs - DLCO pred <70% with FVC/DLCO ≥1.8
NT-proBNP ≥210

= If either positive, do RHC
= 98% NPV
= Cheaper than annual ECHOs

*If recent decline in ET, syncope, RHF or ECHO features of Rt heart strain, should do RHC regardless of ECHO/DLCO findings

71
Q

How is iron related to systemic sclerosis?

A

Iron deficiency in SSc-PAH is associated with worse survival

Replace iron!

72
Q

Signs of severe scleroderma renal crisis

A 
Microangiopathic haemolytic anaemia and thrombocytopenia
HF and flash APO 
Blurred vision due to retinopathy
Headache, fever, malaise
Encephalopathy, generalised seizures
Pericardial effusion
73
Q

Treatment of scleroderma renal crisis

A

Control BP with ACEI within 72h

  • Captopril preferred due to short t1/2
  • Avoid beta-blockers
  • Aim 10% reduction in SBP/DBP per day
  • Can stabilise or improve renal function and survival

If CNS involvement: captopril + IV nitroprusside

MAHA: consider PLEX

74
Q

When might you consider haematopoietic stem cell transplant in systemic sclerosis?

A

In those with early progressive SSc at risk of organ failure

Idea is to wipe out the existing immune system and replace with non-autoreactive immune system with stem cells

High early treatment-related mortality!! Also 1/3 get disease relapse

75
Q

How does drug induced lupus present?

A

Usually mild
Common to get skin and joint manifestations
ANA +, dsDNA +, histone ab +

Generally resolves with stopping the drug

76
Q

GI manifestations of SLE

A

Abnormal LFTs (significant liver disease rare)

Lupus hepatitis (ribosomal P ab)

Ileal/colonic perforation (associated with vasculitis)

Acute pancreatitis

77
Q

Renal manifestations of SLE

A

Glomerulonephritis

6 classes (based on histology)
Class 3-4 +/- 5 reflect activate inflammation and warrants prompt immunosuppression
78
Q

When does someone with SLE need a renal biopsy?

A

1) Increased serum Cr without alternate cause
2) Proteinuria ≥1g/24h
3) Proteinuria ≥0.5g/24h AND haematuria
4) Proteinuria ≥0.5g/24hr AND cellular casts

79
Q

Treatment of lupus nephritis

A

Treat class 3, 4, +/- 5

INDUCTION PHASE
IV pulse steroids AND
MMF or CYC
Refractory disease: rituximab or calcineurin inhibitor (cyclosporin)

MAINTENANCE PHASE
MMF or AZA

80
Q

Should you continue hydroxychloroquine in lupus during pregnancy?

A

YES

Reduce risk of cardiac neonatal lupus (CHB)

81
Q

What’s overlap myositis?

A

Combination of myositis and another CT disorder e.g. SLE, scleroderma, Sjogren

OR

Myositis with overlap features without fulfilling criteria for another CT disorder (clinical feature or antibody)

82
Q

Which subtypes of idiopathic inflammatory myopathy do you get increased risk of malignancy?

A

Dermatomyositis (x5-7 times)
- Colon, lung, breast, ovarian

IMNM

83
Q

Empirical therapy for DM, PM, IMNM (idiopathic inflammatory myopathy)

A

1) high dose glucocorticoids tapering over 1 year
2) steroid sparing agents - AZA, MTX, IVIG, rituximab, PLEX
3) monitor CK, muscle power, PFTs

Rheumatology (5 decks)

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