Rheumatology Flashcards
What are the 3 components of Lesch-Nyhan syndrome?
Lesch-Nyhan syndrome:
- Hyperuricaemia - gout
- Behavioural – self-mutilation/harm
- Neurological – cerebral palsy like motor deficits
Which 3 drugs can cause drug induced lupus?
Drug-induced lupus (HIP): hydralazine, isoniazid, procainamide
What is the treatment of PMR? Are relapses common?
20mg prednisolone for 1 month, slow taper to achieve lowest dose to keep symptoms at bay, may require 1-2 years of treatment, relapses are common.
True/False: PMR often presents with muscle weakness or tenderness.
False: PMR does not have objective muscle weakness or tenderness.
True/False: PMR is more likely in a patient < 50yrs.
False.
True/False: synovitis is less likely in PMR
True.
What are 3 important auto-antibodies in the diagnosis of anti-phospholipid syndrome? What is the regimen for detecting these Abs?
- Anti-cardiolipin Abs
- Lupus anticoagulant
- Anti-beta-2 glycoprotein 1 (AB2GP) Abs
For Dx; Abs need to be positive on 2 occasions > 12 weeks apart.
Patient has 2 week history of lower limb rash associated with arthralgia. On examination you note palpable purpura in both legs. Diagnosis?
Henoch-Schonlein purpura.
What does the biopsy reveal in Henoch-Schlonlein purpura?
Fibrinoid necrosis and neutrophils within the walls of dermal capillaries.
Vasculitis affects mostly postcapillary venules within the papillary dermis with inflammatory infiltrate (neutrophils and monoctyes)
What does immunofluorescence of a Henoch-Schlonlein purpura (HSP) biopsy of vessel walls reveal (3)? Which part of this differentiates it as a diagnosis from other forms of vasculitis?
FAC:
- Fibrin
- IgA - specific to HSP
- Complement C3
Patient has a rash, biopsy reveals neutrophils within the walls of medium sized arteries in the subcutis. Likely diagnosis?
Vasculitis of medium-sized vessels.
Most likely PAN (polyarteritis nodosa).
Whilst walking downstair a patient with OA will likely experience pain in which part of the body?
Patella-femoral compartment.
Aside from ESR and CRP which other sign of inflammation correlates well with disease activity in RA?
Platelet - thrombocytosis, acute inflammatory marker.
How do you differentiate between lower limb claudication due to peripheral vascular disease vs. spinal canal stenosis?
In spinal canal stenosis pain is improved with walking downstairs or leaning forward (relieves spinal pressure).
What major muscle groups are implicated in the Trendelenberg test?
Remember rhyme:
‘Tensor fascia latae, glut med and min
ABduct the thigh and rotate it in’
Main thigh ABductor is gluteus medius
Explain the Trendelenberg test/sign.
Test for weakness in the hip ABductors.
When walking - higher hip is the abnormal hip.
When standing on the abnormal hip it is higher, and the normal (contralateral) side sags.
What are the clinical features of inclusion body myositis (IBM)?
Compare this to dermatomyositis (DM) and polymyositis (PM)
DISTAL myopathy with atrophy in quadriceps.
DM and PM cause PROXIMAL weakness.
What is inclusion body myositis?
Which sex does it affect most?
Inflammation of the ‘endomysium’.
Affect mostly men.
Endomysium is the areolar connective tissue that ensheaths each muscle fibre/cell. Includes blood vessels, nerves and lymphatics.
What are the biochemical and muscle biopsy findings of IBM?
CK normal (or mildly raised)
Muscle Bx: inclusion bodies (obviously)
What is the treatment of IBM?
Prednisone + immunosuppressant (MMF / cytotoxics / AZA / Rituximab)
What is fibromyalgia?
What are the examination findings?
What is the CRP and ESR?
How is it diagnosed?
Non-inflammatory chronic pain syndrome with generalised pain and no diurnal variation.
Reproducible muscle and joint pain.
Normal CRP/ESR.
Clinical diagnosis - no specific investigations.
What is Sjogren’s syndrome?
What is the presentation?
Chronic inflammatory autoimmune disorder that is characterised by Sicca complex (decreased lacrimal and salivary gland secretion)
Presents with fatigue, dry eyes (keratoconjunctivitis Sicca) and dry mouth (xerostomia)
Which 4 blood tests aid the diagnosis of Sjogrens syndrome?
S-SEA!
‘Sjogren went to Sea, Sea, Sea’
Positive: SSA/Ro SSA/La ENA ANA
What is the treatment of Sjogren’s syndrome?
Mainly symptomatic.
Patient has gout, which is the BEST antihypertensive to use?
‘GO LO! = GOut LOsartan’
Losartan has hypouricaemic properties.
What is Prof. Barnsley’s approach to interpreting rheumatological X-rays?
JABS:
Joint space and margins: chondrocalniosis, erosions, osteopenia, sclerosis
Alignment
Bones: normal, thick, thin, trabecular pattern
Soft tissue: swollen, calcification, tophus
Which joints in the hands are spared in RA?
DIP
What is the most specific test for RA?
What is the sensitivity for this test?
Is it a useful prognostic marker
anti-CCP (anti-cyclic citrullinated peptide_ has 98% specificity.
Poor sensitivity - 50%
Predicts rapid disease and risk of bony erosions.
What are the ‘juxta-articular’ changes on X-ray in RA?
Narrowed joint space
Osteopenia
Erosion
Which of the following are contraindications to starting TNFi treatment:
- Treated Hep C
- NYHA Class IV
- Multiple Sclerosis
- Latent TB treatment 1 yr ago
- Breast cancer treated 10 yrs ago
Contraindications:
Multiple sclerosis: TNFi may cause demyelination and irreversible neurological damage.
CCF:
infliximab contraindicated (or must be used at low doses)
etanercept may be used
The rest are NOT contraindications.
Which rheumatological disorder is Reynaud’s phenomena is most closely associated with?
Scleroderma
Give 4 abnormal X-ray findings for Gout.
What 2 joint features are preserved?
‘STOP JaB’:
- Subchondral cysts
- Tophi
- Overhanging edges
- Punched-out biopsies
Preserved:
- Joint space
- Bone (no periarticular osteopenia)
What is the prototypical form of IgG4-related disease?
Autoimmune pancreatitis.
True/False: IgG4 related disease often implicates joints.
False.
Ironic that rheumatologists look after this disorder.
What are typical biochemical and biopsy findings of a patient with IgG4 related disease?
Biochemistry: elevated serum levels of IgG4
Biopsy: infiltration of IgG4 bearing plasma cells in organ.
Give 7 diseased that fall under IgG4 related diseases.
SPLARTS:
Sclerosing cholangitis (not PSC) Pancreatitis (autoimmune) Lung / Lacrimal (Mikulicz) Aortitis Retroperitoneal fibrosis Thyroiditis (Reidels) Salivary (Kuttners/Mikulicz)
