Infection and Rheum Flashcards
How does lupus present?
Skin: - Butterfly, malar rash with naso-labial fold sparing - Discoid scaly rash on sun exposed areas - Livedo reticularis - Raynauds - Alopecia Renal: - Proteinuria - Glomerulonephritis Chest: - Pleurisy, pneumonitis - Pericarditis, myocarditis, endocarditis MSK: - Arthralgia General: - Fever - Fatigue Neuro: - anxiety/depression - seizures - psychosis
What would you find on investigation with lupus?
Raised ANA (highly sensitive but less specific)
Raised dsDNA (highly specific, less sensitive)
Raised anti-Smith
Raised ESR
CRP may be normal in active disease
Complement C3/C4 low
How is lupus monitored?
Anti-dsDNA titre
Describe the epidemiology of lupus
Females (9:1)
Afro-Caribbeans
20-40yo
Increasing incidence
How is lupus managed?
- hydroxychloroquine
- steroids
+/- methotrexate, mycophenolate mofetil, cyclophosphamide and many others
+ NSAIDS for stiffness
What is ankylosing spondylitis?
Degenerative spondylarthopathy associated with HLAB27
How does ankylosing spondylitis present?
<30yo and typically male
Sacroiliitis
Stiffness and low back pain worse at rest - improve with exercise
Kyphosis of the spine
Apical lung fibrosis Anterior Uveitis Aortic regurgitation AV node block Amyloidosis Cauda equina Achilles tendonitis
How does ankylosing spondylitis appear on clinical examination?
Reduced lateral flexion
Reduced forward flexion - positive Schober’s test
Reduced chest expansion
How is ankylosing spondylitis investigated?
Inflammatory markers are typically raised X-Ray of sacroiliac joints: - subchondral erosions and sclerosis - bamboo spine (fusion of bones) - squaring of lumbar vertebrae - syndesmophytes CXR - apical fibrosis Spirometry may show restrictive defect
How is ankylosing spondylitis managed?
NSAID’s for pain
Encourage regular exercise
Physiotherapy
Sulfasalazine for peripheral joint involvement
Anti-TNF if persistently high disease activity (etanercept)
What antibody is drug induced lupus associated with?
Anti-histone
How does polymyositis present?
Symmetrical proximal muscle weakness Raynaud's Respiratory muscle weakness Interstitial lung disease Dysphagia and dysphonia
If associated skin manifestation –> dermatomyositis
Typically Middle Aged
More females
What investigations would you request if you suspect polymyositis and what would you expect to find?
- CK - elevated
- Lactate dehydrogenase, AST and ALT also elevated
- EMG - spontaneous bursts of low amplitude
- Muscle biopsy
- Anti Jo antibody –> if lung involvement, fever and Raynaud’s
- ANA often also high - esp dermatomyositis
What skin manifestations are seen in dermatomyositis?
Heliotrope purple discolouration around orbit with oedema
Raised purple red scaly patches over extensors
Macular rash over back and shoulders
Gottron’s papules - rough red papule on extensors of hands
Mechanic’s hands - extremely dry scaly hands with cracks
What is antiphospholipid syndrome?
Acquired predisposition to arterial and venous thromboses
Associated with Lupus
What are the features of antiphospholipid syndrome?
Thrombus Recurrent miscarriage Livedo reticularis Thrombocytopaenia Prolonged APTT
Pre-eclampsia and pulmonary hypertension
What antibodies is antiphospholipid syndrome associated with?
Lupus anticoagulant
Anticardiolipin
How is antiphospholipid syndrome managed?
Primary thrombophylaxis - low dose aspirin
Secondary thrombophylaxis:
- Initial venous/arterial event –> lifelong warfarin INR 2-3
- Recurrent - lifelong warfarin (+aspirin) INR 3-4
What is Behcet’s syndrome?
Triad
- oral ulcers
- genital ulcers
- anterior uveitis
What are the features of behcet’s syndrome?
Classic triad Thrombus Arthritis Neurological - aseptic meningitis GI - abdo pain, diarrhoea, colitis Erythema nodosum
What blood results are seen in osteomalacia?
Low calcium
Low phosphate
High ALP
High PTH
What blood results are seen in primary hyperparathyroidism?
High Ca
Low PO4
High ALP
High PTH
What blood results are seen in chronic kidney disease (–> secondary hyperparathyroidism)?
Low Ca
High PO4
High ALP
High PTH
What blood results are seen in Paget’s disease of the bone?
Normal Ca, PO4 and PTH
High ALP
What blood results are seen in osteopetrosis?
Normal Ca, PO4, ALP and PTH
What are the types of bone tumour?
Benign = osteoma, osteochondroma, giant cell tumour
Malignant - Osteosarcoma, Ewing’s, Chondrosarcoma
What is important to know about osteoma’s?
Overgrowth
Associated with Gardner’s syndrome - FAP
What is important to know about osteochondroma?
Most common benign tumour
More in males, <20yo
Cartilage capped bony projections on extensors
What is important to know about giant cell tumours?
Epiphyses of long bones
Xray show double bubble or soap bubble appearance
What is important to know about osteosarcomas?
Most common primary malignant bone tumour
Mostly children and adolescents
Metaphyseal region of long bones
X-ray –> codman triangle and sunburst pattern
Due to mutation of Rb gene
Other predisposing factors are Paget’s disease and radiotherapy
What is important to know about Ewing’s sarcoma?
Mainly children and adolescents Small round blue cell tumour Pelvis and long bone Severe pain! Onion skin on xray
What is important to know about chondrosarcoma?
Malignant tumour of cartilage
Most commonly affects axial skeleton
Middle age
Which antibody is associated with rheumatoid arthritis?
Anti-CCP
Which antibody is associated with Sjogren’s?
How else would you diagnose it?
Anti-Ro and Anti-La
ANA+ - 70%
RF - 50%
Schirmer’s test
Hypergammaglobulinaemia and low C4
What does a raised ANCA antibody indicate?
Vasculitis
What does CREST syndrome stand for and which antibody is raised?
Calcinosis Raynaud's oEsophageal dysmotility Sclerydactyl Telangiectasia
Anti-centromere
What is Rheumatoid Arthritis?
autoimmune symmetrical destruction of the joint leading to persistent synovitis and systemic inflammation
What antibody tests would you do for suspected Rheumatoid Arthritis? In which order would you do these tests?
RF first
if this is negative do anti-CCP
Although Rheumatoid Arthritis is a clinical diagnosis, what diagnostic criteria define Rheumatoid Arthritis?
- Joint involvement (more likely if multiple joints, more likely if small joints)
- RF and anti-CCP
- ESR and CRP
- Symptoms >6 weeks
How does Rheumatoid Arthritis present?
Joint pain
Swelling
Morning stiffness
Progressively gets worse with involvement of large joints
What are some late deformities seen in the hands in Rheumatoid Arthritis?
Swan neck (PIP extension and DIP flexion) Boutonnieres (PIP flexion and DIP extension)
What is palindromic rheumatism?
relapsing and remitting monoarthritis of differing large joints
How is the response to treatment monitored in Rheumatoid Arthritis?
CRP
Questionnaires (DAS28 score)
How are Rheumatoid Arthritis flares managed?
Steroids - can be oral or IM
What is the stepwise management of Rheumatoid Arthritis?
1) methotrexate +/- bridging prednisolone
2) switch to another DMARD such as sulphasalazine, hydroxychloroquine or leflunomide
3) TNF-inhibitors (Etanercept or infliximab)
What is an important ADR of TNF inhibitors?
Reactivation of TB
What are some respiratory manifestations of Rheumatoid Arthritis?
Pulmonary fibrosis
Pleural effusions
Methotrexate associated pneumonitis
What are some ocular manifestations of Rheumatoid Arthritis?
Keratoconjunctivitis sicca (dry)
Episcleritis
Scleritis
Name some side effects of the DMARDs
METHOTREXATE - pneumonitis, myelosupression, liver cirrhosis SULPHASALAZINE - ILD, rash, oligospermia, heinz body anaemia HYDROXYCHLOROQUINE - retinopathy LEFLUNOMIDE - ILD, HTN, liver impairment
What is Feltys syndrome?
RA + low WCC + splenomegaly
What are some x-ray findings of Rheumatoid Arthritis?
reduced joint space
juxta-articular osteoporosis
soft tissue swelling
Late: periarticular erosions
What is chronic fatigue syndrome?
> 4 months disabling fatigue affecting physical and mental function and more than >50% of time
Diagnosis of exclusion
Sleep disturbance, muscle pains, headaches, sort throat, cognitive dysfunction etc.
How is chronic fatigue managed?
CBT - very effective
Graded exercise therapy - formally supervised program
‘Pacing’ - organise activities to avoid tiring
Low dose amitriptyline
Pain management
What can cause dactylics?
Spondyloarthritis - psoriatic or reactive
Sickle cell disease
Rare - TB, sarcoid, syphilis
How is osteoporosis managed?
Bisphosphonates first line - alendronate
Alternatives after: strontium ranelate and denosumab
What is an important thing to be aware of with denosumab?
Atypical femoral fractures
What commonly causes drug induced lupus and how does it vary from SLE?
Procainamide and hydralazine
No renal or nervous system involvement normally
What is Ehler-Danlos syndrome?
Autosomal dominant condition affecting type III collagen Features: - joint hypermobility - elastic, fragile skin - easy bruising - aortic regurgitation, mitral valve prolapse, aortic dissection - subarachnoid haemorrhage - angioid retinal streaks
What is fibromyalgia? What is the epidemiology?
Syndrome of wisespread pain throughout body with specific tender points
No known aetiology
More common in women 30-50yo
What are the features of fibromyalgia?
Chronic pain at multiple sites - sometimes all over Lethargy Cognitive impairment Sleep disturbance Headache Dizziness
How is fibromyalgia managed?
Biopsychosocial approach
Explanation
Aerobic exercise
CBT
Medication - pregablin, duloxetine, amitriptyline
What is gout?
Inflammatory arthritis with flares and symptom free intervals
Monocrystal synovitis due to deposited of urate monohydrate secondary to chronic hyperuricaemia
Acute episodes of intense pain, swelling and erythema for around 12 hours
What joints are commonly affected in gout?
1st metatarsophalangeal
Others - ankle, wrist, knee
What happens if gout is untreated?
Damage joints leading to chronic joint problems
What are the radiological features of gout?
- Joint effusion - early
- Well-defined punched out erosions with sclerotic margins in a junta-articular distribution
- Preservation of joint space until late
- Eccentric erosions
- No periarticular osteopenia (RA does)
- Soft tissue tophi
What can predispose someone to gout?
Decreased uric acid excretion - diuretics, CKD, lead toxicity
Increased uric acid - myeloproliferative disorder, cytotoxic drugs, severe psoriasis
Lesch-nyhan syndrome
How is gout managed?
Acute attack:
- NSAID or Colchicine in acute attacks
- Oral/IA steroids if both CI for acute attack
- Continue allopurinol if on it in acute attack, don’t start it
Urate lowering therapy:
- After first attack
- Esp. if >=2 attacks, top, renal disease, uric acid renal stones, or on cytotoxics/diuretics
- Offer allopurinol first line and titrate, colchicine cover
- Febuxostat if allopurinol ineffective/not tolerated
What lifestyle modifications should be considered for gout?
- Reduced alcohol intake and avoid in acute attack
- Lose weight if obese
- Avoid purine high food: liver, kidneys, seafood, oily fish, yeast products
- Consider Vit C supplementation
- Avoid precipitating drugs. Losartan okay in gout
What is the main side effect of colchicine?
Diarrhoea
How is gout investigated and what is seen?
Arthrocentesis with synovial fluid analysis
Negatively birefringent needle shaped crystals
Also - serum uric acid, Xray, ultrasound, DECT
How do pseudogout crystals appear?
Positively birefringent rhomboid shaped crystals
What causes pseudo gout?
Calcium pyrophosphate deposition in synovium
Increased age Haemochromatosis Hyperparathyroidism Low magnesium, low phosphate Acromegaly Wilson's disease
What are the features of pseudo gout?
Knee, wrist and shoulders affected
Chondrocalcinosis on Xray - linear calcifications of the meniscus and articular cartilage
How is pseudogout managed?
Aspirate joint fluid - exclude septic arthritis
NSAID’s or steroids (IA/IM/oral)
What is Marfan’s disease?
Autosomal dominant disorder.
Defect in fibrillin 1
What are the features of Marfan’s disease?
- Tall with large arm span>height ratio
- High arched palate
- Arachnodactyly
- Pectus excavatum
- Pes planus
- Scoliosis >20 degrees
- Dilation of aortic sinuses in heart –> aneurysm/dissection/regurg/mitral valve prolapse
- Lungs - repeated pneumothoraces
- Upward lens dislocation in eye, blue sclera
- Ductal ectasia
How are patients with Marfan’s managed?
Regular echo monitoring
beta blocker/ACEi therapy
What is the prognosis for Marfan’s?
40-50 yo
Mostly due to aortic dissection and other cvs problems
What is McArdle’s disease and how does it present?
Autosomal recessive type V glycogen storage disease leading to reduced muscle glycogenolysis
- Muscle pain and stiffness following exercise
- Muscle cramps
- Myoglobinuria
- Low lactate during exercise
What is polyarteritis nodosa?
Vasculitis affecting medium sized arteries
Necrotising inflammation –> aneurysm formation
More common in middle aged men and Hep B
What are the features of polyarteritis nodosa?
Fever, malaise, anaemia Weight loss HTN Sensorimotor polyneuropathy Testicular pain Livedo reticular Haematuria and renal failure ANCA in 20% HepB in 30%
What are the features of polymyagia rheumatica?
- > 60 yo
- Rapid onset
- Aching and morning stiffness in proximal limbs
- NO WEAKNESS
- Mild polyarthralgia, lethargy, depression, low grade fever, anorexia and night sweats
- Associated with giant cell arteritis
How do you investigate polymyalgia rheumatica?
Inflammatory markers - ESR - raised (>40mm/hr)
CK and EMG normal
How is polymyalgia rheumatica managed?
Prednisolone
Regular review
If it doesn’t respond to steroids, its not PMR
What is Raynaud’s phenomenon?
Exaggerated vasoconstrictive response to digital arteries and cutaneous arterioles to the cold or emotional stress
Primary - raynaud’s disease
Secondary - raynaud’s phenomenon
What are some secondary causes of Raynaud’s?
Connective tissue disorders - scleroderma, RA, SLE Leukaemia T1 cryoglobulinaemia Use of vibrating tools COCP Cervical rib
How is Raynaud’s managed?
Refer to secondary care if suspect secondary
First line - Ca2+ blocker like nifedipine
IV prostacyclin infusion - last several weeks/months
What are the features of Sjogren’s?
Dry eyes Dry mouth Vaginal dryness Arthralgia Raynaud's Sensory polyneuropathy Recurrent episodes of parotitis Renal tubular acidosis
++ risk of Lymphoma (often NH)
How is sjogrens managed?
Artificial tears and saliva
Pilocarpine - stimulate saliva production
What is Sjogrens?
Autoimmune disorder affecting exocrine glands leading to dry mucosal surfaces
What is Still’s disease?
Arthralgia Raised serum ferritin Salmon pink maculopapular rash Pyrexia in early afternoon Lymphadenopathy
Negative for RF and ANA
How is still’s disease managed?
NSAIDs - fever, joint pain, serositis
Steroids - symptoms
If remain - methotrexate, IL1 or anti-TNF
What is systemic sclerosis? Which antibodies are they associated with?
3 patterns of disease:
- Limited cutaneous - CREST is a subtype
- Diffuse
- Scleroderma
ANA 90%
RF 30%
anti-scl70 - diffuse
anti-centromere - limited
What are the symptoms of limited cutaneous systemic sclerosis?
Raynaud’s first sign
Scleroderma - face and distal limbs
Anti centromere
CREST
What are the features of diffuse systemic sclerosis?
- Scleroderma on trunk and proximal limbs
- scl-70 antibody
- Common cause of death due to resp involvement - ILD/pulmonary artery hypertension
- Renal disease
- HTN
- Poor prognosis