Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

RACP 2023 > RHEUM > Flashcards

RHEUM Flashcards

1
Q

Which diseases are associated with scleritis vs uveitis

A

Scleritis: RA

Uveitis: ASpond, IBD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What would you see on joint aspiration for gout vs CPPD?

A

Gout: needle shaped, negative bifringence
CPPD: rhomboid/rod shaped, weak positive bifringence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

RA risk factors?

A

Smoking
DRB1-01 DRB1-04 DRB1-15 DRB1-13
PTPN11
Perionditis
Family history
Dysbiosis
CTLA4
STAT4
IRF5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Associations with dysbiosis

A

Parkinsons
RA
IBS
Metabolic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

VEXAS syndrome features? Treatment?

A

Vacuoles
E1 ubiquitin activating enzyme UBA1

X chromosome
Autoinflammatory
Somatic mutation

Features: MM, MDS, polychondritis, polarteritis, Sweets, GCA, recurrent fevers

Tx: steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Autoinflammatory treatment?

A

IL-1, NSAIDs, Steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Causes of spinal canal stenosis

A

Ligamentum flavum hypertrophy
Short pedicles
Spondylolisthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Management of fibromyalgia?

A

SSRIs
TCAs
SNRIs
Gabapentinoids
Propanolol
Tramadol
Naltrexone low dose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Effect of hydroxychloroquine on SLE progression?

A

Improved: renal/CNS/organ
Improved survival
Decr thrombosis, CHB if SSA+
Reduced lipids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Options for SLE treatment in pregnancy?

A

Steroids
Cyclo
Hydroxychloroquine
Tacrolimus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

APLS prophylaxis?

A

Aspirin: asymptomatic
Prev morbidity: aspirin + DVTp
Prior thrombosis: therapeutic anticoagulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the advantage of HCQ in those with SS antibodies?

A

Reduces risk of CHB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Treatment for SS + target

A

Skin/MSK: MTX, MMF
ILD: MMF, Tocilizumab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Features of Allopurinol HS Syndrome?

A

Fever, eosinophilia, end organ damage, rash

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Management of gout flare

A

NSAIDs
Steroids
Colchicine
IL-1 blocker: Anakinra, Canakinumab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Urate lowering therapy: describe MoA and names

A

Xanthine oxidase inhibitors: prevents purine catabolism and urate production –> allopurinol/ febuxostat

Uricosuric agents: increased urinary excretion
- InhibIts URAT1 and GLUT 9 in proximal tubule - reduced reabsorption and promotes elimination –> probenecid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the second hit hypothesis in RA?

A

1st hit = genetic –> antibodies
2nd hit = environmental/stress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Cytokines involved in RA?

A

IL1
IL6
IL17
TNF-alpha
JAK
RANKL
Blyss/BAF –> B cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Name 4 antibodies involved in RA

A

CCP
RF
CarP
PAD4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Features of RA on radiology?

A

Joint space narrowing
Erosions
Subchondral sclerosis
Subluxation
Ankylosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

RA csMARDs treatment?

A

MTX
HCQ
SLZ
MMF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

RA bDMARDs treatment?

A

TNF alpha
JAK
IL-6
CD 80/86
CD-20

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Most effective csDMARD in RA?

A

MTX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Biggest cause of mortality in RA?

A

CVD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Which part of the joint has no pain receptors?

A

Cartilage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Pathophys of OA?

A

Increased destruction
- synovitis –> cytokines (IL1, TNF, IL6) down regulates aggrecan and collagen type 2

Increased deposition
- BMP-2 and low TGF-beta –> osteophytes

Cytokines trigger osteoclasts + osteoblasts

Have CPPD at end stage

26
Q

COS 2 specific NSAIDs?

A

Meloxicam
Celecoxib

27
Q

Arthritis assoc with haemachromatosis?

A

Erosive 2nd and 3rd MCPs; does not improve with treatment

28
Q

Autoinflamamtory syndrome pathophysiology?

A

NF-kappa B
Inflammasomes
IL1
Interferons

29
Q

Common symptoms of autoinflammatory syndromes?

A

itis
Fevers
Rash
Lymphadenopathy
esp arthralgias

30
Q

Gout causes?

A

Overproduction
- Primary: PRPP hyperactivity, HGPRT1 def
- G6PD, Fructose 1 ph aldolase def
- Secondary: PV, hemolysis, TLSm hemoglobinopathies,

Incr intake
- fructose, alcohol, meat, seafood

Under excretion
- URATE1 GOF/ABCG2 LOF
- Thiazides, loops
- aspirin
- pyrazinimide, cyclosporine

31
Q

Gout Pathophysiology: which chemicals involved?

A

IL1
Inflammasomes
TLR 2+4

32
Q

What can colchicine interact with?

A

CYP450 inhibitors
- Cyclo
- Clarithro

33
Q

What can allopurinol interact with?

A

Aza
Mercapto
Thiazides: increases risk of AHS

34
Q

Allopurinol hypersensitivity HLA gene? who’s at risk?

A

HLA B5801
Han chinese, thai

35
Q

SE’s of probenicid?

A

Reduced secretion of cephs/ penicillins/ MTX
- higher serum concentration

36
Q

Features of scleroderma renal crises?

A

○ Hyper-rininemic, rapidly progressive renal impairment
○ Abrupt onset HTN
○ Mild proteinuria
○ MAHA
Progressive renal failure

37
Q

Differences between diffuse and limited scleroderma?

A

LIMITED
- Skin involvement limited to upper limbs; gradual onset
- A: perioral soft tissue loss
- B: sclerodactyly
○ Tapered, cyanotic
- C: facial teleangiectasia
- D: dilated nailfold capillaries
- E: calcinosis cutis
- Clinically significant ILD more rare
- pHTN + GERD and faecal incontinence similar

DIFFUSE
- Short history Raynaud’s
- Severe skin involvement –> contractures
- MSK and internal organ comps –> ILD, renal crisis
- Tendon friction rubs
- Constitutional symptoms
- Assoc: anti-Scl70, RNA polymerase III and I, ANA with nucleolar pattern
- pHTN + GERD and faecal incontinence similar

38
Q

What is GAVE?

A

Assoc with systemic sclerosis

Gastric enteral vascular ectasia (GAVE)
* Watermelon stomach
* Stomach liniing thickened and fibrosed
* Fe deficiency

39
Q

Cardiac treatment for scleroderma?

A
  • Systolic dysfunction: ACEI/ARB
    • Diastolic dysfunction: frusemide
    • Immunosupression for myocarditis
40
Q

Skin/MSK treatment for scleroderma?

A
  • MTX/ MMF
    • Low dose pred for friction rubs
    • Biologics: resistant arthritis
    • Hand, foot, nail care
    • Pruritis
      ○ Regular application of emollients + avoidance of soaps
      ○ Topical corticosteroids
      ○ Antihistamines
    • Hand/facial exercises
41
Q

Renal crises treatment for scleroderma?

A
  • BP control
    ○ ACEI/ARBs
    ○ Use additional if needed –> avoid beta blockers
    ○ PLEX if MAHA
    Dialysis
42
Q

Vasculopathy tx for scleroderma?

A
  • Ulcers
    ○ Iloprost
    ○ PDE-5 inhibitors
    ○ Bosentan
    • Digital ischemia
      ○ IV prostaglandin/prostacyclin
      ○ Vasodilator therapy/antiplatelet/anticoagulation
      ○ Statin
      ○ Abx
      ○ Botox/ digital sympathectomy
      ○ Debridement for necrotic tissue
    • Raynauds
      ○ Avoid cold
      Botox
43
Q

Pulmonary tx of scleroderma?

A
  • ILD
    ○ Mycophenolate / mycophenolic acid
    ○ PO/IV cyclophosphamide
    ○ Other
    § Nintedanib
    § Pirfenidone
    § Ritux
    ○ Tocilizumab preserves FVC
    • PAH
      Continuous IV epoprostenol –> severe PAH
44
Q

Indications for HSCT in scleroderma?

A

Non smokers non responsive for standard treatment
- diffuse for first 4-5 years mild-moderate organ involvement
- local with progressive organ involvement

45
Q

Prognostic factors for scleroderma

A
  • Reduced life expectancy
    • Mostly due to cardiopulmonary manifestations / pulmonary fibrosis/ paHTN
  • Renal crises
    • Higher BP = better outcome
      ACEI prior to crises = worse outcome
46
Q

Causes of vision loss: describe which arteries can be thrombosed

A

□ anterior ischaemic optic neuropathy (occlusion of posterior ciliary arteries) most common cause
□ Posterior ischemic optic neuropathy
□ Central retinal artery occlusion
□ Choroidal ischemia
Cerebral ischemia

47
Q

Most common symptoms of GCA?

A

Headache
jaw claudication
scalp tenderness

48
Q

GCA treatment

A

Visual symptoms
- IV methylpred
- Progression rare after initiating steroids
- SE’s
○ High risk infection in first year

Tocilizumab
- Refractory to steroids
- IL-6 receptor inhibitor
- Risk of relapse after being discontinued

Aspirin
- If other CVD risk factors

MTX/ lefluonamide

49
Q

Sjogrens: which cancers are assoc?

A

o Lymphoma
○ Highest risk among all AI diseases
○ Predicting factors
§ Tongue atrophy
§ Persistent parotid gland enlargment
§ Purpura
§ Mixed II cryoglobulinemia
§ Low C4
§ Autoantibodies
§ Extensive lymphocytic infilftration
○ Higher risk of NHL
○ DLBCL
MALT

50
Q

Extraglandular manifestations of Sjogrens

A

o Renal: RTA, cryos
o Neuro
o Fatiguability
o Low grade fever
o Raynauds
o Myalgias/ arthralgias
o Arthritis
o AI epithelitis
o Interstitial pneumonitis
o Interstitial nephritis
o RTA
o Vasculitis

51
Q

APLS treatment?

A

In individuals with suspected APS, warfarin is typically preferred over a direct oral anticoagulant (DOAC), although a DOAC may reasonably be used in selected individuals.

Asymptomatic
○ Primary prevention contentious
§ Aspirin
§ HCQ

Secondary prevention
○ Warfarin NOT DOACS LIFELONG aiming INR 2.5-3.5

Pregnancy
○ Prior thrombosis: therapeutic LMWH + aspirin
○ Prior pregnancy loss: low dose LMWH and aspirin daily

52
Q

Causes and treatment of catastrophic APLS?

A
  • Rare complication; high mortality rate

Causes: sepsis, warfarin discontinuation, inflamm states
Treatment: anticoagulation, PLEX, steroids

53
Q

APLS diagnostic criteria + antibodies?

A

Lupus anticoag affected by warfarin; carries highest risk of thrombosis
Beta 2 glycoprotein rare
Anticardiolipin often positive

DIAGNOSIS
- Thrombosis/ pregnancy morbidity
AND
Antibody positive on 2/more occasions

54
Q

Which meds are associated with drug induced lupus?

A

§ Hydralazine
§ Isoniazid
§ TNF-alpha inhibitors: clinical lupus rare but can induce AA (ANA, antidsDNA, APL Abs)
§ Procainamide
§ Carbamazepine
§ Minocycline
§ Methyldopa
§ Chlorpromazine
§ Statins
§ Sulfasalazine
§ Cardiac: some ACEI/BB, statins, HCT
§ PTU
§ Antipsychotics: lithium, chlorpromazine
§ Anticonvulsants: carbamazepine, phenytoin

55
Q

Pathogenesis of SLE?

A
  • Loss of self tolerance to auto antigens
    ○ Incr apoptotis material
    ○ Autoantigens form immune complexes with autoantibodies –> triggers inflammatory cascade
    ○ Self nucleic acids in complexes activate TLRs
    § Dendritic cells –> IFN
    § NETS –> TLRs
    § Elevated B cell survival factors BAFF/BLyS + APRIL
    • Major cells involved
      ○ Type I IFNs
      ○ B cells
      § End product: abnormal expansion of autoreactive B cells –> increased autoantibodies
      ○ Autoantibodies form immune complexes –> deposition + inflammation
      ○ T cells
      § Produce IL and proinflammatory cytokines
    • Lupus autoantigens accumulate inside cells: susceptible to modifications
      ○ Apoptotic debris accumulates –> AI response
56
Q

SLE antibodies + associations

A

ANA positive
- Presnt in almost all patients
- Some can be negative
Sensitive not specific

Anti-dsDNA >reference, if by ELISA 2x reference
Specific for SLE –> can be negative if disease in remission
Assoc: nephritis, TNFi induced SLE
Homogenous pattern

Anti-Smith
MOST specific for SLE –> assoc with renal and CNS disease

SSA + SSB
20-30%
RO-52 associated

U1RNP
25%

Drug induced
ANA more common
antinuclear and antihistone antibodies and, in some cases, anti-double-stranded DNA (D-penicillamine, anti-TNF-α) or perinuclear antineutrophil cytoplasmic antibodies (p-ANCA)

Ribosomal P Abs/ antineuronal/ smith
Neurolupus

57
Q

SLE treatment options

A

Minor disease
Hydroxychloroquine
NSAIDs
Steroids

Major disease
IV steroids
MTX
Azathioprine
Lefluonomide
If bad arthritis
Mycophenolate
- Renal lupus
- ILD

BIOLOGICS
Belimumab
B cell inhibition CD20
Tibulizumab
Rituximab
Anti CD20 depleting MAB
Veltuzimab
Leucocyte movements
Anifrolumab
IFN type 1 receptor antibody

JAK INHIBITORS

Baricitinib

CAR-T CELLS, CD19 TARGETED

58
Q

SLE treatment options in pregnancy?

A

NSAIDs: avoid in T3
Steroids: low dose as possible
AZA < 2mg/kg/day
Hydroxychloroquine
§ May reduce congenital heart block
CSA/tac

59
Q

Sarcoid pathophys

A
  • Infectious/non infectious environmental agent –> inflammatory response involving accumulation of inflammatory cells
    • HLACD4 complex –> APCs present antigen to helper T cell –> IFN, TNF, IL12/18/8
    • T cell mediated
60
Q

Cardiac manifestations of sarcoid?

A

pHTN
Arrhythmias
Heart block if AV node affected = MOST COMMON CARDIAC PRESENTATION
BASAL SEPTAL LV ANEURYSM

61
Q

Skin assoc changes in sarcoid?

A
  • Maculopapular lesions
    • Hyper/hypopigmentation
    • Keloid
    • Subcutaneous nodules
    • erythema nodosum
    • skin: lupus pernio
      ○ Rash on face
      Papules on nose bridge/ area beneath eyes/ cheeks
62
Q

Mechanisms of hypercalcemia in sarcoid?

A

Hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
- Renal calculi

63
Q

Sarcoid treatment

A

ACUTE
- Steroids

CHRONIC
Indications: avoid end orgen/life threatening disease, improve QoL
- Eye/heart/CNS

Steroids first
- Try and taper
- If not tolerated/ ineffective consider other therapy

Systemic therapy
- Hydrozychloroquine
○ SE: ocular toxicity
- Minocycline: cutaneous
- Pulmonary/extrapulmonary: MTX, azathioprime, leflunomide, mycophenolate, cyclophosphamide

Biologics
- - etanercept
- Golimumab
○ Not sig different to placebo
- Infliximab
○ Improves lung function
○ Higher risk of of TB reactivation
○ SE’s infection, carcinogen, allergic reaction

RACP 2023 (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions