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Exam > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

ANA

A

SLE, JIA, psoriatic arthritis, JDM, scleroderma, MCTD
In JIA and psoriatic arthritis, increased risk of uveitis if positive
30% of children with pos ANA have no underlying rheumatological disease
Can also be positive in infection eg. EBV, endocarditis, parvovirus B19

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2
Q

dsDNA

A

SLE- 65%
High specificity for SLE
Associated with lupus nephritis

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3
Q

Anti- Smith

A

SLE- 25%
High specificity for SLE
Associated with lupus nephritis

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4
Q

Anti Smooth Muscle

A

Autoimmune hepatitis

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5
Q

Anti-Pm-Scl (polymyositis-schleroderma)

A

Assoc with sclerodermatomyositis (overlap syndrome)

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6
Q

Anti-SSA (Ro)

A

SLE, Sjogrens
Positive in 25%
Associated with neonatal lupus syndrome, subacute cutaneous lupus, thrombocytopenia

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7
Q

Anti-SSB (La)

A

SLE, Sjogrens
Positive in 25%
Usually coexists with SSA

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8
Q

Anti-RNP (ribonuclease protein)

A

MCTD, SLE
35%
Suggestive of MCTD unless meets criteria for SLE

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9
Q

Anti-histone

A

Positive in durg-induced lupus, SLE

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10
Q

Anti-centromere

A

Limited cutaneous systemic sclerosis (70%)

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11
Q

cANCA (PR3-ANCA)

A

Granulomatosis with polyangiitis (Wegner), CF

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12
Q

pANCA (MPO-ANCA)

A

Microscopic polyangiitis, eosinophillic granulomatosis with polyangiitis (Churg Strauss)
Polyarteritis nodosa, SLE, IBD, CD, PSC, HSP, Kawasaki

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13
Q

ACPA (Anti-CCP)

A

RF positive JIA 50-90%

Quite specific, may be positive before RF

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14
Q

Systemic JIA classification (ILAR)

A

Minimum duration 6 weeks, age of onset <16 years

Arthritis in >= 1 joint with, or preceded by, fever of at least 2 weeks that is daily for at least 3 days

> =1 of the following:

  • Evanescent rash
  • Hepato/splenomegaly
  • Generalised lymphadenopathy
  • Serositis

Exclusions:

  • Psoriasis (or 1st degree rel)
  • HLA pos boy >6 years
  • AS, enthesitis related arthritis, IBD with sacroiliitis, reactive arthritis, acute anterior uveitis (of 1st degree rel)
  • IgM RF positive on at least 2 occasions 3 months apart
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15
Q

Systemic JIA clinical features

A 
Onset 1-5 years
Male = female
5-15% all cases JIA
Usually polyarticular arthritis 
Positive ANA uncommon
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16
Q

Systemic JIA Rx

A

Less responsive to standard treatment

If systemic features present:

  1. NSAID
  2. Steroids
  3. IL-1 inhibitor or IL-6 inhibitor eg. Anakinra, tocilizumab

If active arthritis:

  1. MTX +/- NSAID, joint injection
  2. TNFa or anakinra
  3. Abatacept
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17
Q

Macrophage activation syndrome

A

Rare but potentially fatal complication of sJIA
Can occur at any time including while in remission
High spiking fevers, lymphadenopathy, hepatosplenomegaly, encephalopathy, thrombocytopenia, leukopenia, elevated LFTsLDH/ferritin/triglycerides
Low ESR due to hypofibronogenaemia- useful to distinguish MAS from flare of sJIA

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18
Q

Oligoarthritis JIA classification

A

Min duration 6 weeks, onset <16 years
Arthritis affecting 1-4 joints during the first 6 months of the disease
- Persistent = always affecting only 4 joints
- Extended = affects >4 joints after the first 6 months (worse prognosis)

Exclusions: not another form of JIA

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19
Q

Oligoarthritis JIA clinical features

A 
Age of onset 2-4 years
3:1 female:male
40-50% all cases JIA
Arthritis in the knees ++, fingers +, ankles
Almost never isolated hip involvement
Uveitis in 30%
ANA positive in 60%
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20
Q

Oligoarthritis JIA Rx

A
  1. NSAIDs
  2. Intra-articular steroids
  3. MTX if required +/- NSAID, joint injection
  4. Anti-TNFa +/- NSAID, joint injection
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21
Q

Polyarthritis JIA (RF negative) classification

A

Min duration 6 weeks, onset <16 years
Arthritis affecting >= 5 joints during the first 6 months of the disease, RF negative
Not fitting another JIA diagnosis

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22
Q

Polyarthritis JIA (RF negative) clinical features

A

Onset 2-4 years and 10-14 years
3:1 females (little kids), 10:1 females (older kids)
20-35% all JIA
Arthritis can be symmetric or asymmetric, small or large joints, C-spine (increased wirk atlantoaxial subluxation), TMJ (can cause micrognathia)
Uveitis in 10%
ANA pos in 40%

23
Q

Polyarthritis JIA (RF negative) Rx

A
  1. MTX +/- NSAIDs, joint injection
  2. Anti-TNF +/- NSAIDs, joint injection
  3. 2nd Anti-TNFa or abatacept
24
Q

Polyarthritis JIA (RF positive) classification

A

Min duration 6 weeks, onset <16 years
Arthritis affecting >= 5 joints during the first 6 months of the disease
RF positive 2x at least 3 months apart during the first 6 months
Not fitting another JIA diagnosis

25
Q

Polyarthritis JIA (RF positive) clinical features

A 
Onset 9-12 years
9:1 females
<10% all JIA
Aggressive symmetric polyarthritis 
Rheumatoid nodules 10% (more severe disease course)
Low grade fever
26
Q

Polyarthritis JIA (RF positive) Rx

A

Long-term remission unlikely- early aggressive therapy

  1. MTX +/- NSAIDs, joint injection
  2. Anti-TNF +/- NSAIDs, joint injection
  3. 2nd Anti-TNFa or abatacept
27
Q

Intra-articular steroids

A

Trimenoclone

Should result in improvement for 4 months if effective- can then be repeated as needed

28
Q

TNFa inhibitors

A

Etanercept, adalimumab
Monitoring: FBC, creatinine, LFTs
Before starting, Q3-6 months
?TB screening

29
Q

Psoriatic arthritis classification

A 
6 weeks duriation, <16 years
Arthritis + psoriasis
OR
Arthritis and 2 of:
- Dactylitis
- Nail pitting and onycholysis
- Psoriasis in a 1st degree relative
30
Q

Psoriatic arthritis clinical features

A 
Peak age 2-4 and 9-11
2:1 females
Asymmetric arthritis of small/medium joints
Uveitis 10%
Psoriasis 50%
ANA positive 50%
31
Q

Psoriatic arthritis Mx

A

NSAIDs
IA steroids
MTX
Anti-TNFa

32
Q

Enthesitis-related arthritis classification

A

6 weeks, <16 years
Arthritis and enthesitis
OR
Arthritis OR enthesitis + 2 of the following:
- Hx of SI joint tenderness or inflammatory lumbosacral pain
- HLA-B27 positive
- Male > 6 years at onset
- Acute (symptomativ) uveitis
- 1st degree relative with AS, enthesitis-related arthritis, sacroiliitis with IBD, reactive arthritis, acute anterior uveitis

33
Q

Enthesitis-related arthritis clinical features

A 
Peak age 9-12 years
7:1 boys
Lower limb joints and axial skeleton
Acute anterior uveitis
80% pos HLA-B27
34
Q

Enthesitis-related arthritis Rx

A

NSAIDs
IA steroids
Sulfasalazine

35
Q

Undifferentited arthritis classification

A

6 weeks, < 16 years

Fulfils no other JIA category or 2 or more categories

36
Q

Pathogenesis JIA

A

T lymphocytes - release proinflammatory cytokines favoring TH1 response

37
Q

Uveitis in JIA

A

High risk: shorter duration disease, ANA positive, younger age at diagnosis
Low risk: long duration illness, ANA negative, older age at diagnosis, systemic JIA
Min screening Q12 monthly

38
Q

Short stature in JIA

A

Initially arthritis in large joints esp knees will accelerate linear growth, but continued inflammation then closes growth plate
Can have limb length discrepancy

39
Q

Seronegative spondylarthropathies

A 
Ankylosing spondylathritis
Reactive arthritis
Psoriatic arthritis
Arthritis assoc with IBD
Isolated anterior uveitis

= RF negative, often ANA negative, often HLA-B27 positive

40
Q

Reactive arthritis

A

MC organisms: salmonella, shigella, Yersinia, campylobacter, chlamydia, E. coli, C. diff
Onset 2-4 weeks after infection, lasts weeks to months
75% HLA-B27 positive
Increased frequency of acute and symptomatic uveitis
Triad of arthritis, urethritis and conjunctivitis (classic triad uncommon in children)

41
Q

SLE genetics

A

Can be due to congenital deficiencies of C1q, C2, C4
Assoc with certain HLA typrs
2-5% concordance in dizygotic twins, 25-60% concordance in monozygotic twins

42
Q

Typical SLE patient

A

90% female
Reproductive age
Higher risk in Black, Asian, Hispanic, Native American, Pacific Islander patients

43
Q

SLICC classification - diagnostic criteria for SLE

A

Clinical and immunological criteria
A least 4 criteria present, with at least one clinical and one imunological
OR lupus nephritis (confirmed on biopsy) in the present of positive ANA or dsDNA

44
Q

SLICC classification- clinical criteria for SLE (11)

A 
Acute cutaneous lupus
Chronic cutaneous lupus
Oral or nasal ulcers
Non-scarring alopecia
Synovitis >= 2 joints
Serositis 
Renal
Neurologic
Haemolytic anaemia
Leukopenia or lymphopenia
Thrombocytopenia
45
Q

SLICC classification- immunological criteria for SLE (6)

A

Positive ANA (90-95% of patients)
Positive dsDNA
Positive anti-Smith Abs
Positive antiphospholipid antibody (66% children with SLE)
Low complement
Postitive DAT in absence of hameolytic anaemia

46
Q

Cutaneous lupus

A

Malar rash
Bullous lupus
TEN variant SLE
Photosensitive lupus rash

Chronic:
Discoid rash
Lupus panniculitis
Mucosal lupus
Chillblains
47
Q

Serositis in lupus

A

Pleurisy or pericardial pain >= 1 day
Pleural effusion or rub
Pericardial effusion or rub
ECG evidence of pericarditis

48
Q

Renal involvement in lupus

A

RBC casts
>500mg/24hrs urine protein
Most common- nephrotic syndrome

49
Q

Neurological involvement in lupus

A 
Seizures
Psychosis
Mononeuritis multiplex
Myelitis
Peripheral or cranial neuropathy
Acute confusional state
50
Q

Drug induced lupus

A

MC drugs: minocycline, AEDs, sulfonamides, antiarrhythmics
Usually anti-histone Ab positive
Recover over months-years once drug ceased

51
Q

Antiphospholipid antibody syndrome

A

Positive antibody + arterial or venous clotting event

Can occur in SLE or independent of SLE

52
Q

SLE management

A 
Sunscreen, avoidance off direct sunlight
Hydroxychloroquine for all pts if tolerated
- Treats rash and mild arthritis 
- Prevents flares
- Improves lipid profile
- Improves mortality and renal outcomes
- Toxicity: retinal pigmentation, colour vision impairment (ophthal exam every 6-12 months)
Corticosteroids for flares
Steroid sparing agents if frequent 
Lupus nephritis- cyclophosphamide or mycophenolate for 6/12 
Mx RF for atherosclerosis
Mx RF for OP
53
Q

Neonatal lupus syndrome

A

Annular or maculopapular rash affecting face, trunk and scalp- appears within the 1st 6 weeks after ligh exposure, lasts 3-4 months
Can be present at birth
Cytopenia
Hepatitis
Congenital heart block (only non-reversible Sx)
2% offspring of mothers who Anti-Ro and Anti-La experience congenital heart block

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