Top cases Flashcards
Rheumatoid Hands - acing a station
Is inflammation active? Functional status Features of other CTD - SS, dermatomyositis, SLE --> for a diagnosis of mixed connective tissue disease Multisystem involvement Differentials -
RA - pattern
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!
Diagnostic criteria RA
4 from: Morning stiffness >1h Symmetrical joint involvment Arthritis >3 joints Small joints of hand \+ve RF Rheumatoid nodules Radiographic evidence
Tests for RA
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
Poor prognostic markers for RA
\+ve RF \+ve anti-CCP Early radiographic changes Impaired functional status Persistant synovitis
Anaemia and eyes with RA
AoCD, IDA (GIB 2* to NSAIDS), B9 def 2* to methotrexate, BM supression 2* DMARDS, Felty’s
Eyes - Episcleritis, scleritis, Sjorgems, steroid induced cataracts
Managing RA
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)
Psoriatic Arthropathy - patterns
Asymetrical oligoarthritis Symmetrical polyarthritis (PIP & MCP, rheumatoid pattern) DIP arthritis Arthritis mutilans Spondylitis +/- sacroilitis
Genetics of psoriatic arthropathy
HLA-B27 association but not as strong as other (eg reactive arthropathy or ank spond) - 60-70% HLA B27
RA vs psoriatic arthropathy
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
Enthesitis
Inflammation of tendon insertion site (most commonly achilles tendonitis or plantal fascitis)
Treating psoriatic arthropathy
General - educiton, physio, OT
Analgesia - NSAIDS (can worsen psoriasis)
Steroids - intraarticular
DMARDS - methotrextea, sulphasalazine, cyclosporin
Biological - anti TNFa
Assessing an Ank Spond
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
Diagnosing ank spond
HLA-B27 95% (usually not assessed)
Sacroilitis (XR usually sensitive 0(nil) to 5(fused) , MRI)
ESR/CRP only up in 50% pts
DDx seronegative arthritis
Asymmetrical oligoarthritis Ank Spond Psoriatic arthropathy Reactive arthropathy Enteropathic arthritis HLA-B27 + anterior uveitis Absence RF
Gout on examination
Tophi (hands, forearms, ears) and olecranon bursae
Tophi are an accumulation of uric acid crystals surrounded by histiocytes, giant cells and fibrosis.
Treatment of acute gout and recurrent gout
Rest and rehydration
NSAIDS or colchicine
Intraarticular steroid injection
Recurrent - allopurinol with nsaids/colchicine against an acute attack
Examining marfans
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)
Pathogenesis of marfans
Fibrillin-1 gene (extracellular matrix protein), AD with 25% de novo lesions
Diagnosing marfans disease
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
High arched palate
Marfanrs
Turner’s
Friedrich ataxia
Tuberous sclerosis
Manage marfans
Fibrillin-1 genetic testing
Echo (annual, lifeling B blockade, prophylactic surgery to replace aortic root before 5cm
Ocular evaluation
MRi ductal extasia
Examining scleroderma
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
Systemic sclerosis organ involement
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
