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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
Polyarthritis JIA (RF positive) clinical features
``` Onset 9-12 years 9:1 females <10% all JIA Aggressive symmetric polyarthritis Rheumatoid nodules 10% (more severe disease course) Low grade fever ```
26
Polyarthritis JIA (RF positive) Rx
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
Intra-articular steroids
Trimenoclone | Should result in improvement for 4 months if effective- can then be repeated as needed
28
TNFa inhibitors
Etanercept, adalimumab Monitoring: FBC, creatinine, LFTs Before starting, Q3-6 months ?TB screening
29
Psoriatic arthritis classification
``` 6 weeks duriation, <16 years Arthritis + psoriasis OR Arthritis and 2 of: - Dactylitis - Nail pitting and onycholysis - Psoriasis in a 1st degree relative ```
30
Psoriatic arthritis clinical features
``` Peak age 2-4 and 9-11 2:1 females Asymmetric arthritis of small/medium joints Uveitis 10% Psoriasis 50% ANA positive 50% ```
31
Psoriatic arthritis Mx
NSAIDs IA steroids MTX Anti-TNFa
32
Enthesitis-related arthritis classification
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
Enthesitis-related arthritis clinical features
``` Peak age 9-12 years 7:1 boys Lower limb joints and axial skeleton Acute anterior uveitis 80% pos HLA-B27 ```
34
Enthesitis-related arthritis Rx
NSAIDs IA steroids Sulfasalazine
35
Undifferentited arthritis classification
6 weeks, < 16 years | Fulfils no other JIA category or 2 or more categories
36
Pathogenesis JIA
T lymphocytes - release proinflammatory cytokines favoring TH1 response
37
Uveitis in JIA
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
Short stature in JIA
Initially arthritis in large joints esp knees will accelerate linear growth, but continued inflammation then closes growth plate Can have limb length discrepancy
39
Seronegative spondylarthropathies
``` Ankylosing spondylathritis Reactive arthritis Psoriatic arthritis Arthritis assoc with IBD Isolated anterior uveitis ``` = RF negative, often ANA negative, often HLA-B27 positive
40
Reactive arthritis
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
SLE genetics
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
Typical SLE patient
90% female Reproductive age Higher risk in Black, Asian, Hispanic, Native American, Pacific Islander patients
43
SLICC classification - diagnostic criteria for SLE
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
SLICC classification- clinical criteria for SLE (11)
``` 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
SLICC classification- immunological criteria for SLE (6)
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
Cutaneous lupus
Malar rash Bullous lupus TEN variant SLE Photosensitive lupus rash ``` Chronic: Discoid rash Lupus panniculitis Mucosal lupus Chillblains ```
47
Serositis in lupus
Pleurisy or pericardial pain >= 1 day Pleural effusion or rub Pericardial effusion or rub ECG evidence of pericarditis
48
Renal involvement in lupus
RBC casts >500mg/24hrs urine protein Most common- nephrotic syndrome
49
Neurological involvement in lupus
``` Seizures Psychosis Mononeuritis multiplex Myelitis Peripheral or cranial neuropathy Acute confusional state ```
50
Drug induced lupus
MC drugs: minocycline, AEDs, sulfonamides, antiarrhythmics Usually anti-histone Ab positive Recover over months-years once drug ceased
51
Antiphospholipid antibody syndrome
Positive antibody + arterial or venous clotting event | Can occur in SLE or independent of SLE
52
SLE management
``` 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
Neonatal lupus syndrome
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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