Rheumatology Flashcards
Which drugs are associated with drug-induced lupus?
- Procainamide
- Hydralazine
- Isoniazid
- Minocycline
- Phenytoin
Which anitbodies are associated with drug induced lupus?
100% of these patients are ANA postiive:
- 80-90% anti-histone
- 5% anti-Ro and anti-Sm
What is the triad of Felty’s Syndrome?
- Rheumatoid arthritis
- Splenomegaly
- Low white cell count
What do you need to check for before starting a patient on azathioprine?
Thioipurine methyltransferase deficiency.
If this enzyme is deficnet, the patient will accumulate toxic amounts fo the drug leading to pancytopenia
What abnormality in the hands can occur with ractive arthritis?
Dactylitis.
The spondyloarthropathies can all cause dactylitis (e.g. psoriatic)
What is the characteristic skin changes seen in dermatomyositis?
- Gottron’s Papules: roughened red papules over extensor surfaces of fingers
- Heliotrope rash in the periorbital region
- Macular rash over back and shoulder
What is dermatomyositis?
An inflammatory disorder causing symmetrical, proximal muscle weakness + characteristic skin lesions.
This condition is very much associated with malginancy - 25% have underlying malignancy (more if older).
Other than muscle and skin features, what other features are there in dermatomyositis?
- Rexnaud’s
- Respiratory muscle weakness
- Interstitial lung disease (fibrosing alveolitis or organising pneumonia)
- Dysphagia, dysphonia
What serology doe dermatomyositis patients usually have
Majority is ANA positive (80%):
- anti-Jo-1
- anti-SRP
- Anti-Mi-2
Which antibiotics should be avoided in patients taking methotrexate?
The folate antagonising ABx, trimethoprim and co-trimoxazole
Where is Gout most likely to attack?
E
This is the metatrsophalangeal joint
What are risk factors for osteoporosis considered in FRAX?
- History of glucocoirticoid use
- Rheumatoid arthritis
- Alcohol excess
- Parentl hip fracture
- Low BMI
- Smoking
What are Raynaud’s phenomena?
Raynaud’s phenomena can be primary (Raynaud’s disease) or secondary (Raynaud’s phenomenon).
Primary:
- Onset usually in females around 30
- Bilateral
Secondary:
- Underlying CTD:
- Scleroderma
- Rheumatoid arthritis
- SLE
- Leukaemia
- Cryoglobulinaemia, cold agglutins
- Use of vibrating tools
- Cervical rib
Describe the management of Raynaud’s phenomena.
Conservative:
- Wear warm protection such as gloves
Medical:
- Nifedipine (first-line)
- Prostacycline infusion
- Sildenafil
Surgical: (only used in severe disease)
- Sympathectomy (removing part of the sympatheic nervous system)
What is the difference between osteomalacia and osteoporosis?
In osteoporosis, there is normal mineralisation, but the overall bone mass is decreased.
In osteomalacia, the bone is demineralised.
What do blood investigations show in osteomalacia and osteoporosis?
Osteomalacia:
- Vitamin D low
- ALP raised
- Calcium may be low
- Phosphate may be low
Osteoporosis:
- All Ix normal
What are potential X-ray findings in osteomalacia?
Adults:
- Looser’s zones aka Pseudofractures (transverse lucencies)
What is ankylosing spondylitis?
Ankylosing spondylitis is an HLA-B27 associated spondyloarthropathy.
It leads to enthesitis, i.e. insertion of tendons into bones, particularly in the spine (intervertebral joints, fact joints, sacroiliac joints) - a process which eventually leads to ossification. This makes the spine immobile.
Other joints affected are achilles tendons and plantar fasciitis.
What are some extra-articular features of ankylosing spondylitis?
Skin:
- Keratoderma blenorrhagicum
Eyes:
- Uveitis
Cardiovascular system:
- Aortitis -> anaeurysms at the root (can lead to aortic regurgitation)
- Direct inflammation of the aortic valve leading to aortic regurgitation
- AV node block
Lungs:
- Apical lung fibrosis
Summarise the managment of ankylosing spondylitis.
Conservative:
- Exercise regime: regular exercise (swimming)
- Phsyiotherapy
Medical:
- NSAIDs are used first-line (give with gastro-protection)
- DMARDs: sulphasalazine, MTX
- Biologics: etanercept and adalimumab
Surgical:
- Reparation of damaged joints
- Spinal surgery is risky, and so only rarely performed
Summarise investigations and findings for ankylosing spondylitis.
Bloods
- Test for rheumatoid factor (to proove it is seronegative)
- ESR/CRP - can be a marker of disease severity
Imaging:
- X-Ray spine (not very sensitive):
- Sacroiliitis, showing subchondral erosions and sclerosis
- Squaring of lumbar vertebrae
- Syndesmophytes - ossification of the annulus fobrosus (the outer part of the spinal discs)
- Bamboo spine (late complication)
- MRI (much better at showing inflammation and more sensitive)
What is the scoring system used for ankylosing spondylitis?
The BASDAI score - Bath AnkSpon Disease Activity index.
What is fibromyalgia?
Fibromyalgia is a syndrome characterised by widespread pain throughout the body with tender points at specific anatomical sites.
Cause is unknown.
Clinical features:
- Chronic pain: at multiple sites, somtimes “all over”
- Lethargy
- Cognitive impairment (Fibro Fog)
- Sleep disturbance, headaches, dizziness
Which test can be used to test for likelyhodd of fibromyalgia?
The ACR has classification criteria that lists 9 points on each site of the body; if ≥11/18 are tender, fibro is likely.
Summarise the management apporach for fibromyalgia.
Management is difficult and often unsuccessful.
- Explanation
- Exercise (especially aerobic)
- Cognitive behavioural therapy
- Medical:
- Pregabalin
- Duloxetine
- Amitriptyline
In what demographic does Ankylosing spondylitis most commonly present?
Men (ratio 3:1) in their late teens to early 30s.
Which disease is most associated with cANCA?
What is the targe of these Ab?
Cytoplasmic anti-neutrophil cytoplasmic antibodies - cANCA - is associated with Granulomatous Polyangiitis.
The target is Proteinase 3.
Which disease is most associated with pANCA?
What is the targe of these Ab?
Perinuclear anti neutrophil cytoplasmic antibodies - pANCA - are found in eosinophilic granulomatous polyangiitis and micorscopic polyangiitis.
The antibody binds to MPO, myeloperoxidase.
Other non-vasculitic diseases associated with pANCA include:
- Primary sclerosing cholangitis
- IBD (UC >> Crohn’s)
- Connective tissue diseases (RA, SLE, Sjögren’s)
- Autoimmune hepatitis
What is the definition of osteoporosis?
Osteoporosis is defined as a T-score of -2.5 (osteopenia -1 to -2.5).
This is due to decreased bone material (as opposed to decreased mineralisation in osteomalacia).
Summarise the treatment of osteoporosis.
All women over 65, all men over 75 + women 50-64/men 50-75 with risk factors should be assessed for osteoporosis.
Use a combination of FRAX or QFracture score, ± DEXA scan to estimate risk for patients. (unecessary complex algorithm by NICE)
Some patients, e.g. those with previous fragility fractures, may be started on medication without DEXA.
Medical:
-
Oral bisphosphonates (e.g. alendronate, risendronate)
- Oral on empty stomach whilst erect, in the morning
- If not tolerated, refer to specialist for:
- IV bisphosphonates
- Strontium
- Raloxifen (SERM)
- Denosumab (stops RANK-L and therefore blocks osteoclast activity)
- Teriparatide (PTH agonist - increases osteoblasts more than osteoclasts)
What are the clinical features of polymyalgia?
Clinical features:
- > 60 years old
- Rapid onset of symptoms (< 1 month)
- In proximal limb muscles:
- Aching muscles
- Morning stiffness
- Mild polyarthraliga, lethargy, low-grade fever, night sweats
Note that there is an overlap with temporal arteritis - histology shows vasculitis with giant cells.
What is the treatment for polymyalgia rheumatica?
Prednisolone 15mg/od. (cf. the 40-60mg used in GCA)
What is the buzzword frequnetly used for Radiograph findings of psoriatic arthritis?
“Pencil-in-cup appearance”
(literally looks nothing like this wtf)
What are the types of arthritis that can be seen in psoriatic arthropathy?
- Rheumatoid-like polyarthritis (30-40%)
- Asymmetrical oligoarthritis of hands and feet (20-30%)
- Sacroiliitis
- DIP joint disease (10%)
- Arthritis mutilans (telescoping of the fingers - very severe disease)
What is the material that the crystals in pseudogout are made of?
Calcium pyrophosphate
What are the risk factors for pseudogout?
- Haemochromatosis
- Hyperparathyroidism
- Acromegaly
- Low magensium, low phosphate
- Wilson’s disease
Which organisms are associated with reactive arthritis?
- Campylobacter
- Shigella
- Salmonella
- Yersinia
- E. Coli
- Chlamydia
Note that there is also a post-infection/infection-associated arthritis which is different from reactive arthritis. This can occur after streptococcal infection, mumps, Hep C, HIV or Lyme’s disease.
What is reactive arthritis?
What are clinical features?
This is an HLA-B27 associated seronegative spondyloarthropathy. Potentially due to molecular mimicry.
Clinical features include:
- ~4 weeks after 1° infection
- Arthritis (oligoarthritis - typically of lower limbs)
- Dactylitis
- Urethritis (irrespective of STI origin)
- Conjunctivitis or anterior uveitis
- Skin:
- Circinate balanitis (painless vesicles on the coronal margin of prepuce)
- Keratoderma blenorrhagica
What is this condition called?
Keratoderma blenorrhagicum
What is the mode of inheritance for Marfan’s Syndrome?
Autosomal dominant
What are the clinical features of Marfan’s Syndrome?
Clinical features:
- Tall stature increased arm span to height ratio.
- High-arched palate
- Arachnodactyly (long fingers)
- Pectus excavatum (funnel chest)
- Pes planus
- Scoliosis
- Cardiac:
- dilation of aortic root -> aortic aneurysms, aortic dissection, aortic regurgitation
- Mitral valve prolapse
- Eyes: upwards lens dislocation
What are adverse effects of bisphosphnates?
- Gastrointestinal:
- Oesophageal reactions: oesophagitis, ulceration
- Musculoskeletal:
- Osteonecrosis of the jaw
- Atypical stress fractures on proximal femoral shaft
- General:
- Acute phase reaction with fever, myalgia and arthralgia for 2-4 days after starting (especially with IV)
- Hypocalcaemia
What instruction should you give for taking oral bisphosphonates?
‘Tablets should be swallowed whole with plenty of water while sitting or standing; to be given on an empty stomach at least 30 minutes before breakfast (or another oral medication); patient should stand or sit upright for at least 30 minutes after taking tablet’
Which antibodies are most specific for limited systemic sclerosis?
anti-centromere antibodies
Which antibodies are positive in diffuse cutaneous systemic sclerosis?
anti-scl-70 antibodies.
What are the clinical features of limited and diffuse cutaneous systemic sclerosis?
Limited cutaneous systemic sclerosis
- Raynaud’s may be first sign
- scleroderma affects face and distal limbs predominately
- a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia
Diffuse cutaneous systemic sclerosis
- scleroderma affects trunk and proximal limbs predominately
- the most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
- other complications include renal disease and hypertension
- poor prognosis
What is scleroderma?
Scleroderma is the tightening and fibrosis of the skin. May be manifest as plaques (morphea) or linear.
What are risk factors for Dupuytren’s contractures?
- Family History
- Manual labour
- Alcholic liver disease
- Phenytoin treatment
- Diabetes mellitus
- Trauma to the hand
When should surgery for Dupuytren’s contractures be considered?
Consider surgical treatment of Dupuytren’s contracture when the metacarpophalangeal joints cannot be straightened and thus the hand cannot be placed flat on the table
What is the management of ractive arthritis?
Acute reactive arthritis can be treated with NSAIDs (unless contraindicated).
Steroids can be an option in some cases and DMARDs are usually only used if the disease becomes chronic.
What could be a potential cause ofa seronegative asymmetrical polyarthritis in a woman from India?
Tuberculosis.
This is an extrapulmonary presentation of TB.
What spine abnormalities are seen in ankylosing spondylitis?
- Reduced lateral flexion of the lumbar spine
- Loss of lumbar lordosis
- Accentuated thoracic kyphosis
What does the APTT do in anti-phospholipid syndrome?
What happens to platelets?
It paradoxically prolongs, due to an ex-vivo reaction of the lupus anticoagulatn autoantibodies with phospholipids involved in the coagulation cascade.
There is a thrombocytopenia.
What is Still’s disease?
This is a systemic autoinflammatory disease characterised by:
- Fevers (typically rise in the late afternoon/early evening)
- Joint pain
- Salmon-pink maculopapular rash
Other features include elevated serum ferritin and lymphadenopathy.
How is Still’s disease managed?
- NSAIDs (try these for a week before moving on)
- Steroids
- MTX, IL-1, anti-TNF
What is Ehler-Dahnlos Syndrome?
Ehler-Danlos syndrome is an autosomal fominant connective tissue disorder that mostly affects type III collagen.
This results in tissue being more elastic than normal, leading to joint hypermobility and increased elasticity of the skin.
What are potential complications of Ehler-Danlos syndrome?
Features and complications
- elastic, fragile skin
- joint hypermobility: recurrent joint dislocation
- easy bruising
- aortic regurgitation, mitral valve prolapse and aortic dissection
- subarachnoid haemorrhage
- angioid retinal streak
How long does MTX need to be discontinued before contraception is no longer required?
6 months for both men and women.
What are potential adverse effects of methotrexate?
What is done to avoid these?
- Myelosuppression and subsequent infection
- Pneumonitis
- Pulmonary fibrosis
- Muositis
- Liver fibrosis
Therefore, FBC, U&E, LFTs need to be monitored every 2-3 months whilst on MTX.
Also, folix acid should be prescribed with the MTX.
What needs to be monitored in a patient on hydroxychloroquine?
Visual acuity is checked regularly, as hydroxycholoquine can cause Bull’s eye retinopathy.
Generally, baseline ophthalmic examination followed by yearly examinations is recommended.
What are the potential adverse effects of sulfasalazine?
Caution in patients with G6PD deficiency or allergy to aspirin or sulphonamides.
Adverse effects:
- Oligospermia
- Stevens-Johnson’s Syndrome
- Pneumonitis + Lung fibrosis
- Myelosuppression
- Heinz body anaemia, megaloblastic anaemia
Which 2 DMARDs are considered safe to use in pregnancy?
Hydroxycholorquine and sulfasalazine.
What are the CK levels like in PMR?
Normal. This helps to distinguish it from myositis.
What are the clinical features for Behçhet’s disease`
- Ulceration in mouth, genital area
- Anterior uveitis
- Arthritis
- Neurological involvement (aseptic meningitis)
- GI: abdominal pain, diarrhoea, colitis
- Erythema nodosum
what are the diagnostic test for Behçhet’s disease?
There is no definitive test. And the diagnosis is based on clinical findings.
Pathargy test (puncture site following needle prick becomes inflamed with pustules forming) is positive.
Associated with HLA-B51
What infection is associated with polyarteritis nodosa.
Hepatitis B - this is positive in 30% of patients.
Summarise the management of rheumatoid arthritis.
Initial therapy:
- DMARD monotherapy ± sort course of steroids
- DARDS:
- MTX (most common)
- Sulfasalazine
- Lefluamide
- Hydroxychloroquine
- DARDS:
Regular monitoring with CRP and a disease activity score (e.g. DAS28) is important. Also monitor bloods (LFTs, FBC) with MTX.
If fialure to respond to two DMARDs including MTX, a biologic(JAKi can be used:
- Ant-TNF (infliximab, adalimumab, etanercept)
- Rituximab
- Abatacept
- Tocilizumab
- Baricitinib
What are poor prognositc features for rheumatoid arthritis?
Poor prognostic factors for RA are:
- RF positive
- Anti-CCP positive
- Poor functional status at preentationHLA-DR-4 positive
- X-Ray erosions early (after < 2 years)
- Extra articular lesions (e.g. nodules)
- Insiduous onset
When can bisphosphonates be stopped?
5 years after oral bisphosphonates, or 3 years after IV, treatment should be reasessed with an updated FRAX score and DEXA. THen put into high or low-risk
High risk:
- Age >75
- Steroid therapy
- Previous hip or vertebral fracture
- Fractures whilst on treatment
- High-risk on FRAX scoring
- T-score <-2.5 after treatment.
Low risk can go off treatment and be reassessed in 2 years.
High-risk should have treatment again.
What is the Z-score on DEXA?
Z score is adjusted for age, gender and ethnic factors
What are the features on fundoscopy you might expect in temporal arteritis?
Pale and oedemtous optic disc.
What dose of glucocorticoids is thought to significantly increase the risk of fractures?
A dose of 7.5mg prednisolone a day for > 3 months is linked to significant increases in fracture risk.
What is the management of patients at risk of corticosteroid-indeuced osteoporosis?
Patients < 65: offer bone density scan:
- ≥0 reassure
- 0- -1.5: Repeat scan after 1-3 years
- < -1.5: offer bone protection
Patients > 65 (or if previous fragility fracture): offer bone protection.
An allergy to which medication means sulfasalazine is contraindicated?
Aspirin - sulfasalazine is also a salicylate, and therefore there is a degree of crossreactivity.
How would you treat a GCA patient who has a negative biopsy?
The vasculitis comes with skipped lesions, leading to false-negatives.
Therefore, patients should remain on steroids despite negative biopsy results.
What are some clinical features of ankylosing spondylitis?
- Typically a young man
- Lower back pain and stiffness of insidious onset
- Pain at night which improves when getting up
Describe how Schober’s test should be carried out if done properly.
A line is drawn 10 cm above and 5 cm below the line of the 2 iliac crests. The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
What score assesses hypermobility?
The Beighton score.
What imaging should rheumatoid arthritis have before any surgery?
AP and lateral cervical spine radiographs.
This is due to atlantoaxial subluxation - a rare complication of RA that can lead to cervical cord compression.
This ensures the patient wears a C-spine collar and neck is not hyperextended on intubation.
What is the chelating agent used to treat Wilson’s Disease?
What is a complication of this treatment?
Penicillamine.
This can cause membrannous glomerulonephropathy - presents with nephrotic syndrome.
If a patient suffers from complications with alendronate (e.g. severe upper GI disturbances), what do you do next accoriding to NICE?
If alendronate can’t be continued, switch to risendronate or etidronate. After that, IV options such as zoledronate or denosumab can be considered by a specialist.
What are the features of IgA vasculitis?
IgA vasculitis aka. Henoch-Schönlein Purpura:
- Palpable purpuric rash (due to immune complexes damaging the vessels and blood leaking)
- Abdominal pain
- Polyarthritis
- Features of IgA nephropathy (haematuria, renal failure)
Note that there is NO thrombocytopenia, as the rash is not due to DIC/platelet consumption.
How can you distinguish between discoid lupus and lupus pernio?
Lupus pernio is a complication of sarcoidosis:
- Raised purple plaque of indurated skin that affects the face, frequently the nose
- Not itchy
- Not painful
Discoid lupus:
- Raised patches, red in colour
- Scar
- Rash is uncomfrotable for patients
What is the managment for Goodpasture’s syndrome?
- Plasmapharesis
- Steroids
- Cyclophosphamide
What are the eye symptoms associated with RA?
- Episcleritis (non-painful)
- Scleritis
- Keratoconjuncitivits Sicca (dry eye)
- Keratitis (corneal inflammation)
- Cataracts from steroids
- Bull’s Eye retinopathy from hydroxycholorquine
What are the respiratory complications of RA?
- Interstitial lung disease (PF) (basal)
- Pulmonary nodules
- Pleural Effusion
- Bronchiolitis Obliterans
- MTX related pneumonitis and pulmonary fibrosis
What are cardiovascular complications of rheumatoid arthritis?
- Increased risk of atherosclerosis
- Cornary artery disease
What are the renal complications of RA?
- AA amyloid
- Membranous glomerulonephritis
- Tubulo-interstitial nephritis
What are the haematological complications of RA?
- Anaemia of chronic disease
- Felty’s Syndrome (RA, splenomegaly, neutropaenia)
- Thrombocytosis
When do you give steroids in sarcoidosis?
Indications for steroids
- patients with chest x-ray stage 2 or 3 disease who are symptomatic. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment
- hypercalcaemia
- eye, heart or neuro involvement
What dermatological condition is associated with Sarcoidosis?
- Erythema nodosum
- Lupus pernio (see image - affects nose and face)
