Rheumatology Flashcards
Common antibodies in SLE
ANA
Anti-smooth muscle
Anti-double stranded DNA
Antiphospholipid
Common antibodies in RA
Rheumatoid factor
Anti CCP
Common antibodies in Sjogren’s
Anti Ro
Anti La
Common antibodies in Systemic Sclerosis
Limited = Anti Centromere
Diffuse = Anti Scl 70
Polymyositis antibodies
Anti Synthetase
Anti-Jo1
Rheumatoid Arthritis
FM = 3:1, usually 30-50 years old at onset
Associated with HLA-DR4
Symmetrical, deforming, polyarthropathy
- Small joints mainly (70% have RA in wrist or hands)
- Spares DIPs
Morning stiffness
Slow onset
Outline the American Classification Criteria for RA
= 4 components, A to D
A = joint involvement (type and number)
B = Serology (RF and anti CCP)
C = Acute phase reactants (CRP, ESR)
D = duration of symptoms (>6 weeks)
Score >6/10 = suggestive of RA
How would you investigate a patient in whom you suspect RA?
Bloods
- Routine FBC (?Felty’s), inflam markers, baseline U&Es
- RF (positive in 70%)
- anti CCP (positive in 70-80%, more specific)
Xrays of affected joints
- Loss of joint space, osteopaenia, subluxation, erosions
CXR –> HRCT if suspicious for fibrosis/nodules
Lung function tests
Poor Prognostic Features for RA
RF positive
Anti-CCP antibodies
Poor functional status at presentation
HLA DR4 positive
Early erosions on xrays (<2 years)
Extra-articular features e.g. fibrosis
Insidious onset
Possible Extra-Articular Features of RA
Can have multisystem involvement!
NEURO - peripheral neuropathy, mononeuritis multiplex
OCULAR - episcleritis, scleritis, keratoconjunctivitis
CARDIO - IHD, peri/myocarditis
RESP - pulmonary fibrosis (lower), effusions, nodules
HAEMATO - anaemia (secondary to chronic disease/NSAIDs), Felty’s syndrome, bone marrow suppression (secondary to MTX)
SKELETAL - osteoporosis
Increased risk of amyloidosis!
How would you manage a patient with RA?
MDT approach - rheumatologists, PT/OT
Exercise programme
Initial Rx = DMARD monotherapy (e.g. MTX) +/- prednisolone
Flares = PO/IV steroids
Biologic therapy e.g. Infliximab
- Indicated if on >2 DMARDs and 2 x DAS28 scores >5.1 at least 2 months apart
How can you monitor response to treatment in RA?
CRP &ESR
Disease Activity Score 28
How does MTX toxicity present? What can you give to treat this?
Bone Marrow Suppression
= symptoms of anaemia
= increased risk of infections (neutropaenia)
= bleeding/bruising (thrombocytopaenia)
Can also cause hepatitis
Rx = folinic acid
What are the main differences between Psoriatic Arthropathy and RA?
Psoriatic Arthropathy =
1) Asymmetrical (typically)
2) Negative RF, negative anti CCP
3) Nail changes e.g. pitting, oncholysis
4) Dactylitis
5) FHx/PMHx of psoriasis
6) F:M = 1:1
7) Skin change e.g. psoriatic plaques, koebner’s phenomenon
- Poor correlation with cutaneous psoriasis
8) Associated with HLA B27
9) More likely to affect DIPs
How would you manage a patient with Psoriatic Arthopathy?
MDT approach - rheum, derm, OT/PT
Mild Disease = NSAIDs
Moderate disease/little response to NSAIDs
= DMARDS e.g. MTX or sulfasalazine
= TNF inhibitors
Which DMARD should you avoid using in Psoriatic Arthopathy?
Hydroxychloroquinne - can exacerbate psoriatic skin lesions
Differentials for Shortness of Breath in SLE / CTD
Multisystem disorders!
CARDIAC - Pericarditis, pericardial effusion, IHD, cardiomyopathy
RESP - Pneumonia (increased risk of infections), pulmonary fibrosis, PE, pleural effusion
- Renal failure –> pulmonary oedema
- ARDS reaction to biologics
NEUROMUSCULAR - Myositis, GBS
Screening Questions for Connective Tissue Disease
1) Any rashes? Do they worsen in the sun? (PHOTOSENSITIVITY!)
2) Any hair loss?
3) Have you noticed any changes to your hands?
4) Do you suffer from dry eyes / dry mouth / ulcers?
5) Are you SoB?
6) Any difficulty swallowing? Any heartburn?
7) Any diarrhoea / weight loss?
8) Any muscle pain or weakness?
9) Any difficulty getting out of a chair/car?
10) How is this affecting your everyday life?
SLE
Multisystem autoimmune disorder
Relapsing-remitting
F:M = 9:1, 20-40 year olds
Features (ACR diagnostic criteria =>4/11 present)
- Malar Rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Non-erosive arthritis (symmetrical, hands & knees commonly)
- Serositis e.g. pericarditis
- Renal disorder
- CNS disorder e.g. psychosis, seizures, GBS
- Haematological
- ANA +ve
How would you investigate a patient in whom you suspect SLE?
Bedside
- BP
- Urine dip, ACR and red cell casts
Bloods
- Routine FBC, U&Es, LFTs
- Inflammatory markers
- ANA (+ve in 99%)
- Anti dsDNA, ENAs, Antiphospholipid
- Complement
- CK (exclude myositis)
CXR
Echocardiogram
?Skin biopsy
?Renal biopsy
How would you manage a patient in whom you suspect SLE?
MDT approach as multisystem disorder - rheum/derm/renal/cardio
First line = hydroxychloroquine +/- steroids
If internal organ involvement = IV cyclophosphamide
If lupus nephritis = MMF
If severe flare = ?IVIG
Managing Complications of SLE
- Antihypertensives (ACEIs/ARBs)
- Statins
- Warfarin/LMWH if secondary APS
How can you monitor for disease activity in SLE?
Urinary protein + blood
Urinary ACR
Urinary red cell casts
ESR
Anti dsDNA
Sjogren’s Syndrome
Autoimmune disorder of exocrine glands
Can be primary or secondary to rheumatoid arthritis/other CTDs
F:M = 9:1
Increased risk of lymphoid malignancy (B-cell) - >40x!
Features
- Dry eyes
- Dry mouth (xerostomia)
- Vaginal dryness
- Arthralgia
- Recurrent parotitis –> bilateral enlarged parotid glands
- Raynaud’s
- Myalgia
- Sensory polyneuropathy
How would you investigate a patient in whom you suspect Sjogren’s?
Bedside
- Schrimer’s test (litmus paper on lower eyelid)
Bloods
- Routine FBC, U&Es, LFTs
- RF (positive in 100%)
- Anti Ro (70% of primary SS)
- Anti La (30% of primary SS)
Salivary gland biopsy (definitive test)
How would you manage a patient with Sjogren’s?
Artificial saliva/tears
Long term f/u due to risk of malignancy (B cell lymphoma!!)
What is Raynaud’s disease?
= exaggerated vasoconstrictive response of digital arteries to cold/stress
Can be primary or secondary to underlying CTD (scleroderma, SLE, RA)/leukaemia/drugs e.g. COCP
Features
- Painful cold hands & extremities
- White –> blue –> red
- Digital ulcers (if severe)
Outline features which are more suggestive of Raynaud’s secondary to underling CTD
Onset > 40
Unilateral symptoms
Presence of autoantibodies
Features of systemic CTD e.g. joint pain, rashes, SoB
How would you manage a patient with Raynaud’s disease?
Gloves/hand warmers
1st line = CCBs (nifedipine)
2nd line = sildenafil
IV prostacyclin infusions if severe e.g. digital ulcers
Systemic Sclerosis
Connective tissue disorder, characterised by thickening and fibrosis of the skin +/- systemic involvement
F:M = 3:1
3 patterns of disease
1) Scleroderma = localised skin thickening
2) Limited Cutaneous Systemic Sclerosis
= distal skin involvement, facial involvement, trunk spared
= Anti-Centromere
{CREST is a subgroup of this}
3) Diffuse Cutaneous Systemic Sclerosis
= Proximal skin involvement (above elbows and knees), trunk involved
= Anti Scl 70 abs
Features of Systemic Sclerosis
Scleroderma
CREST
- Calcinosis
- Raynaud’s
- oEsophageal dysmotility
- Sclerodactyly
- Telangiectasia
Microstomia
Pulmonary involvement - ILD, pulmonary HTN
What medical emergency are patients with Systemic Sclerosis at risk of? What can precipitate it, and what do we use to treat it?
Scleroderma renal crisis
= acute HTN and AKI
Can lead to pulmonary oedema, encephalopathy and renal failure
Common precipitant = steroids!
Rx = ACEIs
How would you investigate a patient in whom you suspect Systemic Sclerosis?
Bedside
- BP
- Urine dip
- Lung function tests
Bloods
- Routine FBC, U&Es, inflammatory markers
- Autoantibodies
– ANA positive in 90%
– Anticentromere and anti Scl70
– Anti RNA polymerase III (increased risk of renal crisis)
Oesophageal studies/OGD (if features of oesophageal dysmotility)
CXR & HRCT (if features of ILD)
Echo
How would you manage a patient with Systemic Sclerosis?
No curative treatment = symptomatic management mainly
Raynauds = CCBs etc
Cyclophosphamide has been shown to improve skin thickening, stabilise lung function and improve survival
PPIs
AVOID steroids!! (can precipitate a renal crisis)
Causes of Gout
Main causes =
1) Diuretics
2) Alcohol
3) Trauma
4) CKD
Gout is secondary to increased serum urate concentration = either due to
1) Reduced uric acid excretion e.g. CKD, hyperparathyroidism, alcohol
2) Increased uric acid production e.g. MPD, cytotoxic drugs
Clinical Features of Gout
Asymmetrical
70% 1st presentation = swollen, hot 1st MTP = Podagra
Other common sites = knee/ankle/wrist
“Flares” of joint pain and swelling, lasting 3-10 days
Often demonstrate diurnal pattern (worst at night!)
Tophi! e.g. ears
Monosodium urate crystals on joint aspirate (negatively bifringent)
How would you manage a patient in whom you suspect gout?
Acute Rx
- NSAIDs or colchicine
- 2nd line = steroids
- Continue allopurinol if already on it
- Stop precipitating medications e.g. diuretics
Long-term Urate Lowering Therapy
- Offer if >2 attacks/year OR presence of erosions/tophi OR renal impairment secondary to calculi
- Offer for prophylaxis if on cytotoxics
= Allopurinol –> Febuxostat
Lifestyle Modifications
- Alcohol abstinence
- Weight loss
- Avoid high purine food e.g. seafood
Key Questions for Acute Hot Joint
Onset - acute vs insidious
SOCRATES
Systemic features
Skin lesions
Previous history
Preceeding trauma
Preceeding infection e.g. UTI, STI, gastroenteritis
DHx
- Anticoagulation
- Recent course of steroids (AVN!!!)
Pseudogout
Synovitis secondary to calcium pyrophosphate dihydrate crystal deposits
Associated with haemochromatosis, hyperparathyroidism, acromegaly
Knee most common joint affected
Weakly positive bifringent rhomboid crystals on aspirate
Rx = NSAIDs or IA/PO steroids
How would you investigate a patient with an acutely swollen red hot joint?
Full history
Bedside
- BP, temp (?fever)
- Urine dip
Bloods
- FBC, U&Es, LFTs,
- CRP
- Urate, LDH
- RF, anti CCP
- Blood cultures if suspect septic arthritis
Joint Xrays
Joint Aspirate
- WBC > 50,000 = treat as septic arthritis
- MC&S
- Cytology for crystals
?MRI of joint
Contraindications to a joint aspirate
Prosthetic joint - aspirate in theatre
Overlying celluitis
Overlying psoriatic plaque (increased risk of septic arthritis)
Excess anticoagulation e.g. INR >3 / coagulopathy
Ankylosing Spondylitis
HLA B27 associated spondyloarthropathy
Axial arthropathy - affects sacroiliac joints & spine
Lower back pain and stiffness
Worse in morning
Improves with exercise
Associated with IBD & psoriasis
Extra-articular features of Ankylosing Spondylitis
The 8 As
Anterior Uveitis
Apical fibrosis
AV block
Aortic regurgitation
Amyloidosis
Arthritis
Achilles tendonitis
and cauda equina syndrome
Clinical Tests for Ankylosing Spondylitis
Schober’s Test
= reduced lumbar spine forward flexion
- Mark 5cm below line between PSIS, and mark 10cm above
- Measure distance when bending forwards
- Diagnostic if <5cm increase in distance between 2 points (<20cm distance)
On forward flexion, >10cm distance between fingers and floor
Occiput to wall distance > 0cm
Management of Ankylosing Spondylitis
Conservative
- Regular exercise & physio
Medical
- NSAIDS
- DMARDs only useful if peripheral joint involvement = sulphasalazine
- Anti TNF therapy e.g. adalimumab, etanercept
— Indicated if axial disease refractory to 2 different NSAIDs with 2 active disease flares 3 months apart
— Symptomatic relief, does not affect progression
HLA B27 Associated Arthropathies
Ankylosing Spondylitis
Reactive Arthritis
Psoriatic Arthritis
Reactive Arthritis
HLA B27 associated arthopathy
“Post dysenteric” e.g. shigella, salmonella, campylobacter
“Post STI” e.g. chlamydia
Occurs 2-6 weeks post infection
Asymmetrical oligoarthritis (<4 joints)
Urethritis
Dactylitis
Conjunctivitis/anterior uveitis
“Can’t pee, can’t see, can’t climb a tree”
Management = pain relief, usually resolves within 12 months
Kocher Criteria for Septic Arthritis
1) Fever > 38.5
2) Non weight bearing
3) Raised ESR
4) Raised WBC
Giant Cell Arteritis
Systemic granulomatous arteritis
Large-to-medium vasculitis
Usually > 50 years old, F>M
Associated with PMR
Rapid onset headache (unilateral) -Often localised to temporal region
Scalp tenderness
Jaw claudication
Diplopia –> visual disturbance
Painless loss of vision
Systemic features e.g. fever
How would you investigate and manage a patient in whom you suspect GCA?
Bedside
- BP
- Fundoscopy
Bloods
- FBC, U&Es, LFTs
- ESR!
- Vasculitis screen e.g. ANA
?Temporal artery USS/biopsy - should NOT delay Rx
Treatment = high dose steroids to prevent blindness e.g. 60mg pred OD
Refer to rheumatology and ophthalmology
Paget’s Disease
Disease of accelerated bone turnover
Typically affects skull, spine, pelvis and long bones of legs
Typical presentation = isolated raised ALP in older men with bone pain
Spontaenous fractures, bone deformities e.g. bowing of tibia
Complications of Paget’s Disease
Bone deformities e.g. bowing of tibia, skull thickening
CN palsies due to bone thickening e.g. CN VIII compression –> deafness
Headaches
HTN, IHD, high output heart failure
Hypercalcaemia
How would you investigate someone in whom you suspect Paget’s disease?
Bloods
- Routine FBC, U&Es, LFTs - looking for raised ALP
- Calcium profile (normal)
- C-telopeptide = marker of bone turnover and progression of disease
Plain xrays
Technetium bone scan (high uptake areas)
Management of Paget’s
Analgesia
Bisphosphonates if
- Bony pain
- Skull/long bone deformity
- Fractures
Calcitonin can be used if refractory to bisphosphonates/contraindicated
Side Effects of Bisphosphonates
Bone pain
Hypocalcaemia
Oesophagitis/GI ulcers
Fever/flu-like symptoms
Osteonecrosis of the jaw!
Sarcoidosis
Multisystem granulomatous disorder
Non caseating granulomas on biopsy
KEY FEATURES
- Fever, malaise, weight loss
- Erythema nodosum
- Lupus pernio
- Heerford’s syndrome = PUFF
– Parotid enlargement
– Uveitis
– Fever
– Facial nerve palsy
- SoB (pulmonary involvement)
MULTISYSTEM = neuro to cardiac to lung to renal involvement
Poor prognostic features of Sarcoidosis
Insidious onset > 6 months
Absence of erythema nodosum
Extrapulmonary features
CXR stage III-IV
Afro-Caribbean
Increasing age
How would you investigate someone in whom you suspect Sarcoidosis?
Bedside
- BP
- Urine dip
Bloods
- FBC, U&Es, LFTs
- Calcium profile
- ACE
CXR - ?hilar lymphadenopathy ?features of fibrosis
?HRCT
Biopsy of LNs
Spirometry
How would you manage a patient with Sarcoidosis?
MDT approach, refer to rheumatology
If asymptomatic, no Rx required
Analgesia
Steroids if:
- Progressive CXR/PFT changes
- Cardiac or neuro involvement
- Hypercalcaemia
- Uveitis
Ehler Danlos Syndrome
Autosomal dominant CTD, mostly affecting type III collagen
= increased elasticity of skin and muscle tissue
Features
- Elastic, fragile skin
- Atrophic scars
- Hypermobility and joint dislocation
- Easy bruising
- Aortic regurg/dissection
- MVP
- Angioid retinal streaks
- Poor wound healing
- Prolapses/hernias
Increased risk of SAH
Polymyositis
Inflammatory myopathy
Causes = idiopathic, CTD or malignancy
F:M = 3:1
Features
- Symmetrical proximal muscle weakness
- Raynaud’s
- Resp muscle weakness
- ILD
- Dysphagia
- Dysphonia/dysarthria
Anti-Jo1 antibodies
Rx = Steroids
Dermatomyositis
Inflammatory myopathy
Causes = idiopathic, CTD or malignancy (lung, breast, ovarian)
Skin Features (usually precedes myopathy)
- Periorbital heliotrope rash
- Gottron’s papules (red papules on dorsal aspect of fingers)
- Photosensitivity rash
Features
- Symmetrical proximal muscle weakness
- Raynaud’s
- Resp muscle weakness
- ILD
- Dysphagia
- Dysphonia/dysarthria
Anti-Mi2 antibodies
Rx = Steroids
How would you investigate a patient in whom you suspect myositis?
Blood
- FBC, U&Es
- LFTs - AST and ALT often raised (markers of muscle)
- CK
- ANA
- Anti-Jo1 and Anti Mi-2
EMG
Muscle biospy
Consider investigating for underlying malignancy e.g. CT TAP, tumour markers
