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Top cases Flashcards

1
Q

Rheumatoid Hands - acing a station

A 
Is inflammation active?
Functional status
Features of other CTD - SS, dermatomyositis, SLE --> for a diagnosis of mixed connective tissue disease
Multisystem involvement 
Differentials -
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2
Q

RA - pattern

A

Symmetrical deforming arthropathy involveing small joints of hand (PIP & MTC with DIP sparing). Ulnar deviation, Z thumb, swan neck, boutonnieres.
Nodules, olecranon bursae, psoriatic plaques
Earlobe for tophi, hairline for psoriasis
Median or ulnar entrapment!

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3
Q

Diagnostic criteria RA

A 
4 from:
Morning stiffness >1h
Symmetrical joint involvment
Arthritis >3 joints
Small joints of hand 
\+ve RF
Rheumatoid nodules
Radiographic evidence
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4
Q

Tests for RA

A

Rheumatoid factor –> 70-75%, Ab against Fc portion of own IgG (non specific - can be +ve in almost all other CTD, and also 5% population)
Anti-CCP –> more specific but only 60% +ve.
Both only diagnostic use, titres do not vary with disease activity

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5
Q

Poor prognostic markers for RA

A 
\+ve RF
\+ve anti-CCP
Early radiographic changes
Impaired functional status
Persistant synovitis
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6
Q

Anaemia and eyes with RA

A

AoCD, IDA (GIB 2* to NSAIDS), B9 def 2* to methotrexate, BM supression 2* DMARDS, Felty’s
Eyes - Episcleritis, scleritis, Sjorgems, steroid induced cataracts

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7
Q

Managing RA

A

General - eduction, physio, OT, bone protection, Vaccination
Steroids
DMARDS - methotrexate (with folate), sulphasalazine, azathioprine, leflunomide, GOld, Hydroxychloroquine
Biologics - Anti-TNFa (infliximab, adalimumab, etanercept), IL-1 (anakira), CD-20 (rituximab)

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8
Q

Psoriatic Arthropathy - patterns

A 
Asymetrical oligoarthritis 
Symmetrical polyarthritis (PIP & MCP, rheumatoid pattern)
DIP arthritis 
Arthritis mutilans 
Spondylitis +/- sacroilitis
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9
Q

Genetics of psoriatic arthropathy

A

HLA-B27 association but not as strong as other (eg reactive arthropathy or ank spond) - 60-70% HLA B27

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10
Q

RA vs psoriatic arthropathy

A

RA - more in women, sparing distal joints, symmetrical, tenderness then deformity, no enthesopathy
Psoriatic arthropathy - equal sex, commonly involves distal joints, assymetry, less pain and therefore more deformity earlier, enthesis, spinal involement, dactylitis

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11
Q

Enthesitis

A

Inflammation of tendon insertion site (most commonly achilles tendonitis or plantal fascitis)

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12
Q

Treating psoriatic arthropathy

A

General - educiton, physio, OT
Analgesia - NSAIDS (can worsen psoriasis)
Steroids - intraarticular
DMARDS - methotrextea, sulphasalazine, cyclosporin
Biological - anti TNFa

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13
Q

Assessing an Ank Spond

A

Stand, look from side, then back
Cervical - look down, up, left, right - mobility symmetrically decrease
Occiput-wall distance
Thorax - chest expansion
Sacrum - palpate SI joints (or not, ask) for tenderness
Other - nails, hands, scalp ?psoriasis
Systemic - heart (AR, ECG for AV conduction, note PPM), eyes (uveitis), lung (fibrosis)
Modified Shobers test - mark PSIS +10 and -5. Bend maximally forwards, should be >+5cm
Ask - atlanto-axial subluxation, enthesitis, knees, hips

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14
Q

Diagnosing ank spond

A

HLA-B27 95% (usually not assessed)
Sacroilitis (XR usually sensitive 0(nil) to 5(fused) , MRI)
ESR/CRP only up in 50% pts

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15
Q

DDx seronegative arthritis

A 
Asymmetrical oligoarthritis
Ank Spond
Psoriatic arthropathy
Reactive arthropathy 
Enteropathic arthritis
HLA-B27 + anterior uveitis 
Absence RF
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16
Q

Gout on examination

A

Tophi (hands, forearms, ears) and olecranon bursae

Tophi are an accumulation of uric acid crystals surrounded by histiocytes, giant cells and fibrosis.

17
Q

Treatment of acute gout and recurrent gout

A

Rest and rehydration
NSAIDS or colchicine
Intraarticular steroid injection
Recurrent - allopurinol with nsaids/colchicine against an acute attack

18
Q

Examining marfans

A

Tall, skinny, disproportionately long limbs (arm span>height), kyphscoliosis, pectus excavatum (or evidence of surgical corrrection), arachnodactyly (wrist sign, thumb sign) with hyperextansibility of joints, high arched palate, pes planus, heterochromia (DDx homocysteinuria)

19
Q

Pathogenesis of marfans

A

Fibrillin-1 gene (extracellular matrix protein), AD with 25% de novo lesions

20
Q

Diagnosing marfans disease

A

Ghent criteria
Cardiovascular - aortic root aneurism, AR, aortic dissection, mitral disease. Annual surveillance
Ocular - lens displacement, heterochromia, myopia, blue sclerae, lens dislocation
MSK - dural ectasia (neural canal enlargement - back pain), hypermobility
Resp - pectus excavatum , spont ptx, apical bullae
Hernia

21
Q

High arched palate

A

Marfanrs
Turner’s
Friedrich ataxia
Tuberous sclerosis

22
Q

Manage marfans

A

Fibrillin-1 genetic testing
Echo (annual, lifeling B blockade, prophylactic surgery to replace aortic root before 5cm
Ocular evaluation
MRi ductal extasia

23
Q

Examining scleroderma

A

Thickening and fibrosis of skin - face, fingers - smooth, shiny, tight, loss of facial wrinkles, restricted mouth opening
Sclreodactyly of hands with flexion deformitie.
Dilated nail fold capillaries with dystrophic nails
Digital ulceration
Palpable calcinosis nodules
Raynauds phenomenon
Oedema, livedo reticularis, scarring alopecia, vitiligo
CV –> PTH, restrictive CM
Resp –> ILD
GI –> PBC, dysphagia

24
Q

Systemic sclerosis organ involement

A

Skin - Raynauds, sclerodacily, calcinosis, nail fold dystrophy, ulceration, telangectasia
MSK - arthritis, myositis, myopathy, osteopenia
GI - dysphagia, GORD, hypomotility, obstruciton
Renal - malignany HTN, glomerulonephritis, renal crisis
Resp - ILD, effusions
CV - restrictive cardiomyopathy, pericarditis, effusions, PTH, conduction defects

25
Q

Classifing systemic sclerosis

A

Diffuse scleroderma - skin / trunk / extemities, early involement of lung, kidney, gut and heart
Limited - skin only at face or extremities, CREST, late lung/pth/renal
Malignant - rapid progression, often elderly men

26
Q

Diagnosing SS

A

Major - skin sclerosis of arms, face and/or neck
Minor - sclerodactyly, pulp atrophy, pulmonary fibrosis
1 major or 2+ minor

27
Q

CREST syndrome

A 
Calcinosis
Raynauds
Eosophageal dysmotility 
Sclerodactyly 
Telangectasia
28
Q

Autoantibiodies in SS

A

Limited systemic sclerosis –> Anti-centromere, ANA, RF
Diffuse systemic sclerosis –> Anti-ScL 70 (topoisomerase), AntiRNA polymerase 1, 2 and 3

In mixed CTD anti-ribonuclear protein +ve

29
Q

Managing SS

A

General - education and counselling, PT/OT, analgeisa, MDT
Raynauds - smoking cessation, hand warmds, dilators (ACEi, Ca antagonists, prostacyclin analogues)
GI - PPI / prokinetics
Renal - ACEi (agressive HTN management)
Resp - vasodilatorsfor PTH

30
Q

Examining NF

A

Cafe-au-lait spots 6+
Neurofibromata (anywhere along peripheral nerves) - S/C (well circumscribed), cutaneous (button-hole invagination when pressed, non specific NF
Axillary / inguinal freckling
Associated: BP (RAS / pheochromocytoma), CN (hearing aid, for acoustic neuroma / CP angle lesions), Eye (cataracts, optic glioma, lisch nodules), CV (constrictive cardiomyopathy, Resp (fibrosis, PTX),

31
Q

Subtypes, diagnosis, and genetics of NF

A

1 - von Recklinghausen, most common, AD, chr 17 neurofibromin protein (Tumour Supressor)
6+ cafe-au-lait spots; 2+neurofibromata; 2+Lisch nodules (iris hamartoma, slit lamp), axillary/inguinal freckling, optic glioma; FH (1st* relative)
2 - central chr 22
B/L acoustic neuroma, meningioma, optic glioma, cataracts

PACES (6 decks)

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