MDEMO - rheumatology and orthopaedics Flashcards
Give a quick summary of Polymyalgia rheumatica
A specific disease (autoimmune) causing early morning prolonged proximal muscle stiffness (shoulder and pelvic girdle), but not arthritis. It tends to occur in people over 60, and sometimes in association with temporal (or giant cell) arteritis (when younger people might be affected). Note that it is stiffness and not weakness.
Does creatine kinase tend to be raised in polymyalgia rheumatica?
No, CK levels tend to be normal, and this helps to distinguish it from myositis/ myopathies
What is the treatment for polymyalgia rheumatica?
Prednisolone 15mg / day = expect a dramatic response within 1 week and if not consider an alternative diagnosis (as steroids work amazingly for PMR). Provide bone protection. NSAIDS are not effective.
What is the difference between osteomalacia and osteoporosis
In osteomalacia, there is a normal amount of bone, but its mineral content is low (there is excess uncalcified osteoid and cartilage). In osteoporosis, the mineralisation is unchanged, but there is overall loss of bone.
List some causes of osteomalacia
Vitamin D deficiency/ resistance Renal osteodystrophy Liver disease Drug induced Tumour induced osteomalacia
What might you find on x-ray in a patient with osteomalacia?
Looser’s zones - since osteomalacia affects cortical bone you see apparent partial fractures without displacement, and these may typically be seen on the lateral border of the scapula, inferior femoral neck and medial femoral shaft
Recap the 4 radiographic features of OA
Loss of joint space (joint space narrowing)
Osteophytes
Subarticular sclerosis
Subchondral cysts
What are Heberden’s and Bouchard’s nodes?
Heberden’s nodes = DIP joints
Bouchard’s nodes = PIP joints
In OA what tissue type is primarily damaged?
Cartilage (focal loss in early OA), in end stage OA some parts total loss of cartilage. Patchy cartilage death and local bony overgrowth
What is the difference between primary and secondary OA?
Primary OA is degeneration of the articular cartilage and surfaces of a joint with no predisposing factors- in this sense the disease is idiopathic. In secondary OA, there is an underlying precipitation to the degenerative process e.g. post-traumatic, post-operative, post-infective etc.
What is the classic pattern of joint involvement in RA?
Classically a symmetrical distal small joint polyarthropathy, often affecting the hands and feet, but later stage can affect any joint. MCP joints predominantly affected. PIP joints also affected (sparing of DIP joints)
Roughly what percentage of RA patients will have a positive rheumatoid factor?
Around 70%
What are the two markers of RA that tend to be measured in the blood?
Rheumatoid factor and anti-CCP
What are the x-ray findings in RA?
Soft tissue swelling, juxta-articular osteopenia and decreased joint space. Later on, there may be bony erosions, subluxation or complete carpal destruction
What tool is used to measure disease activity in RA?
DAS-28
What is involved in the early inflammatory arthritis protocol?
Identify and treat early and aggressively
DMARDs- methotrexate is usually first line unless contraindicated
Biologics- e.g. anti-TNF alpha, anti-IL6 etc
Patients have to have tried at least 2 DMARDs before going onto biologics
What are the key clinical features of anti-phospholipid antibody syndrome?
Can be remembered as ‘CLOT’: Coagulation defects (arterial/venous), Livedo reticularis, Obstetric (recurrent miscarriage), Thrombocytopenia. Also, cerebral disease (e.g. migraine/seizure)
What is antiphospholipid syndrome also known as and with what other disease may it be associated?
Also known as Hughes’ syndrome. Was initially described in SLE and can be associated with SLE in 20-30% of cases
What are the autoantibodies associated with antiphospholipid syndrome?
Antiphospholipid antibodies (anticardiolipin, lupus anticoagulant, anti-Beta 2 glycoprotein 1)
In SLE, patients may rarely develop a symmetrical small joint polyarthropathy similar to that seen in rheumatoid arthritis. What is this called in the context of SLE and what is the main difference between it and RA?
It is called Jaccoud’s arthropathy and the difference is that it tends to be non-deforming
What is the typical epidemiology of SLE?
Prevalence of ~0.2%, F:M = 9:1, tends to affect women of child-bearing age, commoner in Afro-Caribbean and Asian populations. Associated genetic factors: HLA DR2, DR3 and B8
What are some key signs/ symptoms to ask about in a history if suspecting SLE?
Rashes, photosensitivity, alopecia, mucosal ulceration, Raynaud’s phenomenon
What are the three best ways of monitoring disease activity in SLE?
- anti-DNA antibody titres, 2. Complement (reduced C3 and C4 and increased C3d and C4d degradation products- as complement is used up), 3. ESR. Note that CRP tends to be normal
What are the key autoantibodies involved in SLE?
Anti-dsDNA, anti-Ro, anti-La.
As well as anti-Sm, anti-RNP.
40% are rheumatoid factor positive and antiphospholipid antibodies may also be positive.
What are the treatment principles for SLE?
Avoidance of sun exposure where possible and high factor suncream for photosensitive rashes. For skin flares, try topical steroids first. NSAIDs are useful for joint pains (caution in patients with renal disease). Hydroxychloroquine is used to alleviate symptoms mediate term. Immunosuppressants such as azathioprine, cyclophosphamide and methotrexate are sometimes used too. For severe flares urgent high-dose steroids, mycophenolate, Rituximab and cyclophosphamide
What are the shared features of the spondyloarthropathies?
Absence of rheumatoid factor (hence seronegative)
HLA-B27 association
‘Axial arthritis’ pathology in the spine and sacroiliac joints
Peripheral arthropathy- asymmetrical large joint oligoarthritis or monoarthritis
Enthesitis: e.g. plantar fasciitis, Achilles tendinitis
Extra-articular manifestations e.g. anterior uveitis
What tends to be the first line and the gold standard investigations for the spondyloarthropathies?
MRI tends to be gold standard, but x-rays tends to be first line
The ‘5A’s’ of ankylosing spondylitis is a way to remember some of the extra-articular manifestations. What are they?
Anterior uveitis, Achilles tendinitis, Apical lung fibrosis, Aortic regurgitation, amyloidosis
What tends to be the only radiological investigation required to assist in a diagnosis of ankylosing spondylitis?
A plain x-ray
What is the epidemiology of ankylosing spondylitis?
Prevalence 0.25-1%. Men present earlier : M:F 6:1 at 16 years, 2:1 at 30 years old. ~90% are HLA-B27 positive.
What is the typical presentation of a patient with ankylosing spondylitis?
A man <30 years old with gradual onset of low back pain, worse during the night with spinal morning stiffness relieved by exercise. Pain radiates from sacroiliac joints to hips/ buttocks and usually improves towards the end of the day. There is progressive loss of spinal movement in all directions.
What tends to be one of the first areas affected by inflammation in ankylosing spondylitis?
The sacroiliac joints. What initially starts as joint space widening eventually leads to sclerosis and joint fusion, visible on x-ray.
Briefly what is enteropathic arthritis and what is it’s epidemiology?
Enteropathic arthritis is acute-onset inflammatory oligoarthritis which is associated with IBD. Approximately 10% of those with UC, and twice as many with Crohn’s suffer from enteropathic arthritis. Men and women are affected equally. Exacerbations of arthritis tend to occur simultaneously with flare ups of bowel disease.
What are the two patterns of joint disease in IBD associated arthritis?
Peripheral oligoarthritis- generally affects the joints of the lower limb such as the hips and knees and is usually non-erosive. Enthesitis such as plantar fasciitis may occur. More commonly associated with Crohn’s disease than UC, and usually flares with the activity of the bowel disease, and resection of the colon in UC may cure the joint symptoms. This form is not associated with HLA-B27.
Sacro-iletis with spondylitis- clinically indistinguishable from ankylosing spondylitis and is associated with HLA-B27 in 60-70% of cases. The joint disease may predate the intestinal disease by some years.
Radiological findings in IBD associated arthropathy (remember there are 2 clinical patterns)…
The changes affecting the sacroiliac joints and the lumbar spine are indistinguishable from those seen in ankylosing spondylitis.
The peripheral joints will show soft tissue swelling, but generally an absence of erosions.
What about treatment options for IBD associated arthropathy?
NSAIDs are effective, but use limited because they often cause a flare of colitis. Simple analgesics provide pain relief, but do not treat the inflammatory component. Exercise, physio and hot and cold packs should be encouraged. Second line treatments: The only drug of any proven benefit is sulphasalazine.
What are the 5 different patterns/forms of psoriatic arthritis?
Symmetrical polyarthritis (like RA) DIP joints Asymmetrical oligoarthritis Spinal (similar to AS) Psoriatic arthritis mutilans
Monoarthritis means one affected joint, how many joints are affected in each of oligoarthritis and polyarthritis?
Oligoarthritis is 2-5 joints and polyarthritis is >5 joints
What radiological feature might you see in severe psoriatic arthritis?
Erosive changes such as the ‘pencil in cup’ deformity
Other associated changes in psoriatic arthritis?
Associated with nail changes in 80%, dactylitis, acneiform rashes and palmo-plantar pustulosis
Are men or women more affected by psoriatic arthritis?
Affected equally (as is IBD-associated arthropathy)
How might you distinguish between psoriatic arthritis and RA?
Psoriatic arthritis is seronegative and usually follows a relapsing and remitting course. Furthermore, psoriatic arthritis rarely leads to severe joint destruction, with the exception of ‘arthritis mutilans’
Give some examples of the nail changes seen in psoriatic arthritis
Pitting, horizontal ridges, onchylosis and subungual hyperkeratosis
How might you distinguish between sacroiliac involvement in ankylosing spondylitis and that of psoriatic arthritis? (Remember that spinal involvement is one of the 5 patterns of psoriatic arthritis)
Sacroilitis is more likely to be bilateral in AS and unilateral in psoriatic arthritis
What is the triad of conditions in Reiter’s syndrome?
Conjunctivitis, urethritis and arthritis (can’t see, pee, or climb a tree)
Give some brief information about reactive arthritis i.e. what it is and why it happens and more common in men/women?
Reaction to infection (usually GI, STI/GU or URTI). When the immune system is up regulated by an infection antigens in the synovium can look quite similar and as a result you can get inflammation of the synovium ‘reactive arthritis’. It is the most common cause of inflammatory arthritis in men aged 16-35 and is more common in men than women
How does reactive arthritis tend to present and thus what 2 other conditions might it mimic?
Tends to present with 1 hot, big, swollen joint after an infection a few weeks before (so can present quite similarly to septic arthritis and gout- so would want to rule these out)
Which two of the spondyloarthropathies is HLA-B27 more closely associated with?
Ankylosing spondylitis and reactive arthritis
What is often the key presenting symptom in systemic sclerosis/ scleroderma?
Raynaud’s phenomenon
What is systemic sclerosis?
A group of disorders characterised by the development of hardened, sclerotic skin. They are part of the spectrum of connective tissue diseases and as such they are multi system disorders- involvement such as the lungs and GI tract is common. Classified into: limited cutaneous systemic sclerosis (formerly CREST), diffuse cutaneous systemic sclerosis, environmentally induced scleroderma and mixed connective tissue disease/ overlap syndromes
What does CREST stand for in the setting of limited cutaneous systemic sclerosis?
Calcinosis, Raynaud’s, (O)esophageal dysmotility, Sclerodactyly, Telangiectasia
Which antibodies may be associated with systemic sclerosis?
Anti-centromere and anti-SCL 70 antibodies
What is the pathogenesis of systemic sclerosis and what are the hallmark clinical features?
Inflammation is not usually a feature. Pathologically there is over-production of connective tissue (notably collagen), with fibrosis and widespread micro vascular damage. The hallmarks are Raynaud’s phenomenon, and tight, thickened skin over the hands. However there is a spectrum of clinical manifestations and various disease subtypes exist
What is Raynaud’s syndrome and what does it encompass?
It is peripheral digital ischaemia due to paroxysmal vasospasm precipitated by cold or emotion. Fingers or toes ache and change colour: pale (ischaemia) -> blue (deoxygenation) -> red (reactive hyperaemia). It may be idiopathic (Raynaud’s disease) or have an underlying cause (Raynaud’s phenomenon)
What is seen under polarised light microscopy of synovial fluid in gout ?
Negatively birefringent urate crystals (needle shaped)
What is podagra?
Gout which occurs at the metatarsophalangeal joint of the big toe
What is the acute treatment of gout?
NSAIDs or colchicine (if NSAIDs contraindicated)
What medication is typically given for gout prophylaxis and under what circumstances is this drug started?
Allopurinol - if patient has >1 attack in 12 months, has tophi or renal stones.
What is seen under polarised light microscopy of synovial fluid in pseudogout?
Weakly positive birefringent crystals (rhomboid shaped)
How does pseudogout tend to present?
Acute monoarthropathy usually of larger joints in the elderly.
How is chronic fatigue syndrome defined?
Persistent disabling fatigue lasting >6 months, affecting mental and physical function, present >50% of the time, plus 4 or more of: myalgia, polyarthralgia, reduced memory, unrefreshing sleep, fatigue after exertion >24hr, persistent sore throat, tender cervical/axillary lymph nodes
Are there any investigation results for helping to diagnose fibromyalgia?
No, in fibromyalgia all investigation results are normal. Diagnosis is clinical.
What management measures could you recommend to a patient with fibromyalgia?
Graded exercise programmes (both aerobic and strength training). Relaxation, rehabilitation and physiotherapy may also help. CBT can also help patients to develop coping strategies and set achievable goals. Pharmacotherapy: low-dose amitriptyline (e.g. 10-20mg at night) has been shown to help relieve pain and improve sleep. Pregabalin can be used if amitriptyline is ineffective. Also SSRIs if comorbid anxiety and depression
What tends to characterise fibromyalgia? (Clinical presentation)
Pain that is chronic (>3 months) and widespread. Profound fatigue is almost universal with complaint of unrefreshing sleep, and significant fatigue and pain with small increases in physical exertion. Additional features: morning stiffness, paraesthesia, headaches, poor concentration, low mood and sleep disturbance.
What % of all GP consultations are for musculoskeletal complaints?
About 12%
Remember that one way to look at the different types of arthritis is to divided them into 4 major groups related to the underlying pathology. What are the 4 main groups and what is the most common arthropathy within each?
Cartilage death (osteoarthritis)
Synovial inflammation and overgrowth (RA)
Inflammation and new bone formation at entheses (Ankylosing spondylitis).
Crystals in synovial fluid (gout)
Why is septic arthritis so important to investigate for in any acutely inflamed joint?
Because it can destroy a joint in under 24 hours and has a mortality rate of up to 11%.
What joint is affected in >50% of cases of septic arthritis?
The knee
What is the key investigation when suspecting septic arthritis?
Urgent joint aspiration for synovial fluid microscopy and culture is the key investigation- quickest to ask for a gram stain. Plain x-ray and CRP may be normal. Blood cultures are essential (prior to antibiotics).
What are the common bacterial causes of septic arthritis in the UK?
Staphylococcus (50%) e.g. staph aureus, Streptococci (25%), approx 25% other bacterial cause e.g. Neisseria gonorrhoea and gram negative bacilli. In the USA half of cases are caused by gonococcus
What is the predominant organic component of bone tissue?
Type 1 collagen
What is the pathophysiology of Paget’s disease of bone?
There is increased bone turnover associated with increased numbers of osteoblasts and osteoclasts with resultant remodelling, bone enlargement, deformity and weakness.
What is the treatment for Paget’s disease?
Bisphosphonates
What are some of the clinical features of Paget’s disease?
Bone pain, bone swelling and deformity, increased head circumference, increased incidence with age
What are some of the complications of Paget’s disease?
Long bone #s, osteosarcomas, nerve compression which can cause deafness, osteoarthritis
What do the investigations for Paget’s disease tend to show?
Calcium and phosphate are normal.
ALP is elevated.
X-ray show localised enlargement of bone and patchy cortical thickening with sclerosis, osteolysis and deformity. Isotope bone scans are useful for investigating the extent of the disease
How does WHO define osteoporosis?
A bone density more than 2.5 standard deviations below the young adult mean value (T-score less than -2.5)
What are the three common sites for fractures in osteoporotic bones?
Distal radius, neck of femur, vertebrae
What is the garden classification for neck of femur fractures?
The garden classification assesses the severity of intracapsular neck of femur fractures. I = incomplete and impacted, II = complete but undisplaced, III = partially displaced, the capsule may be intact IV- complete, fully displaced and the capsule is not intact
What are the clinical features of polymyositis/ dermatomyositis?
Proximal muscle weakness (upper arms and thighs). Myositis, joint pains.
Constitutional- fatigue
Cutaneous- heliotrope rash, Gottron’s papules, calcinosis, Raynaud’s phenomenon
Respiratory- respiratory muscle weakness, interstitial lung disease
Cardiac- myocarditis
Pharyngeal- dysphasia
What are the vasculitides?
A group of multisystem inflammatory diseases that are characterised by inflammation of blood vessel walls (= vasculitis). Can happen to any size blood vessel.
Treatment for GCA?
Start prednisolone 60mg/day PO immediately
What is polyarteritis nodosa?
A rare systemic illness characterised by necrotising inflammation of medium-sized arteries leading to aneurysm formation. It principally affects viscera, and may lead to organ infarction if left untreated.
