Rheumatology Flashcards
What is rheumatoid arthritis?
Chronic systemic inflammatory disorder of unknown cause with characteristic joint involvement
Diagnostic criteria for rheumatoid arthritis?
4/7 of:
morning stiffness >1 hour Arthritis of 3 or more joints Arthritis of hand joints Symmetrical arthritis Rheumatoid nodules Rheumatoid factor positive X-ray changes
Clinical features of rheumatoid arthritis - hands?
Symmetrical poly arthritis sparing the DIPS
Ulnar deviation and prominent ulnar styloid Swan neck deformity Boutonniere deformity Z-thumb Subluxation at MCP's and wrist
Clinical features of rheumatoid - not hands?
Atlanto-axial instability due to weakening of transverse ligament holding odontoid of C2 against arch of C1
- diagnosed when odontoid > 3mm from anterior arch
Anaemia of chronic disease
Scleritis
Interstitial fibrosis, pulmonary nodules
X-ray changes in rheumatoid?
BENJ
Bone cysts
Erosions
Narrowing of joint space
Juxta-articular osteoporosis
What markers should you look for in rheumatoid arthritis?
Rheumatoid factor = 70%
- IgM to Fc of IgG
Anti-CCP, much more specific but less sensitive
Management of Rheumatoid arthritis?
Conservative = info/counselling, OT.
Medical:
Analgesics and NSAIDs
1st line = Methotrexate 7.5mg PO once weekly + 1 other DMARD e.g. Sulfasalazine
2nd line = biologicals, only if you have tried two DMARDs one being methotrexate, and DAS score >5.1 twice
Steroids used for bridging when starting DMARDs + for systemic flair ups
Examples of DMARDs and their SE’s in rheumatoid arhtirits?
Methotrexate = Pulmonary fibrosis, BM suppression
Sulfasalzine = reduced sperm, Heinz body anaemia, BM supression
Leflunomide = HTN and interstitial lung disease
Hydroxychloroquinine = Rash, retinopathy
Examples of biologicals and their SE’s in rheumatoid arthritis?
Anti-TNF e.g. Etanercept and infliximab = BM suppression and hair loss
Anti-B cell e.g. Rituximab = Cytokine release syndrome, infusion reaction
Anti-IL6 e.g. tocilizumab = BM suppression and mouth ulcers
CTLA4-Ig fusion e.g. Abatacept = GI and BM suppression
Monitoring in rheumatoid?
LFT’s and FBCs every 1-2 months early doors then every 3-4 months once stable
DAS score = disease activity score
What is osteoarthritis?
The degenerative loss of articular cartilage
Clinical features of osteoarthritis?
Pain on activity and worse at end of day / night
morning stiffness <45 minutes
Functional limitation
Affects weight bearing joints = knees, hips
Hands DIP
Spine lumbar affected most
X-ray changes in OA?
Loss of joint spaces
Osteophytes
Subchondral cysts
Sclerosis
Management of osteoarthritis?
Conservative = weight control, exercise and appropriate orthotics
Medical = analgesia:
1st line = paracetamol
Then NSAIDs
Then Opioids
Intra-articular steroid injections
Surgery = Replace joints
What is septic arthritis?
Inflammation of the joint due to the presence an MO
Common MO’s in septic arthritis?
Staph aureus = 60%
S. Pyogenes = 15%
N. Gonnorhoea in sexually active patients
Diagnosis of septic arthritis?
Joint aspiration prior to antibiotics:
Gram stain
WCC > 50,000/mm3
+ve culture
Management of septic arthritis?
Local guidelines = IV for two weeks or until improvement then four weeks orally
No RF’s for atypical = Vancomycin 1g IV BD for two weeks, then clindamycin
If high risk G-ve in elderly / UTI / recent abdo surgery = Ceftriaxone 2g IV OD for 2 weeks, then cefalexin
What is gout?
Disorder of purine metabolism, characterised by hyperuricaemia and the deposition of monosodium rate crystals in joints
Precipitating factors for acute gout?
Starvation or alcohol excess
Surgery
drugs = Thiazides, furosemide, high dose salicylates
Reduced excretion in renal failure
What does synovial fluid show in gout?
-vely birefringent needle shaped crystals
Management of acute gout?
NSAIDS e.g.naproxen 500mg PO BD 2 weeks
2nd line = prednisolone
3rd line = colchicine
- useful if contraindication to NSAIDS e.g. GI bleed
Prophylaxis for gout?
Conservative = weight loss, dietary modifications and avoid alcohol
Medical prophylaxis if recurrent attacks, tophi and erosive disease
Xanthine oxidase inhibitor = allopurinol 100mg PO OD
- don’t start within 2 weeks of attack
2nd line = Probenecid = increased renal excretion
Refractory = pegolticase
What is pseudogout?
Deposition of calcium pyrophosphate crystals in joint
Pseudogout associations?
4 H’s
Hypoparathyroid
Haemochromatosis
Hypomagnesaemia
Hypophosphatia
Synovial fluid findings in pseudogout?
+vely birefringent rhomboid crystals
X-ray change in gout vs pseudogout?
Gout = punched out erosions
Pseudogout = linear calcium deposition in cartilage
Management of pseudogout?
Accessible joints = IA corticosteroids
Inaccessible = Colchicine + NSAIDs
If refractory = systemic corticosteroids
What is a seronegative spondyloarthritis
Any joint disease of the vertebral column that is seronegative i.e. RF -ve
Types of seronegative spondyloarthritis?
Anyklosing spondylitis = HLA-B27
Psoriatic arthritis
Reactive arthritis
Enteropathic arthritis
What is ankylosing spondylitis?
Chronic inflammation of the spine, sacra-iliac joint and axial joints
Strongly HLA-B27
Clinical features of ank spond?
Insidious back pain > 3 months
Early morning stiffness, and bad at night
Relived by exercise
Enthesitis is common = heel and knee
Extra articular signs of ank spond?
AAAA
Anterior uveitis
Aorititis = aortic regurgitation
Amylodosis
Apical lung fibrosis
X-ray findings in ankylosing spondylitis?
Sacra-Iliac fusion
Squaring of vertebral bodies
Bamboo spine
Syndesmophytes = Ossification of annulus fibrosis (the tough circular exterior of the intervertebral disc
Management of ankylosing spondylitis?
Conservative = educate and physio
Medical = NSAIDs for pain
IA injection of intra-articular disease / entheisits
Peripheral joint involvement = sulfasalazine
If refractory:
- continue NSAIDs
- TNF alpha inhibitor e.g. Adalimumab or Etanercept
What is psoriatic arthritis?
Inflammatory arthritis in association with psoriasis
Clinical features of psoriatic arthritis?
Psoriasis generally precedes arthritis
Some may have nail dystrophy = pitting and onycholysis
Can have multiple patterns, but most common is polyarthritis mimicking RA
X-ray changes in psoriatic arthritis?
May see axial changes like in ankylosing spondylitis
Pencil cup deformity
Management of psoriatic arthritis?
If limited peripheral joint disease = Naproxen 500mg PO BD + physio ± steroid injections
Progressive peripheral joint disease:
Methotrexate
2nd line = Etanercept or adalimumab
What is enteropathic arthritis?
Arthropathy associated with pathology in large / small bowel e.g. IBD, coeliacs
Clinical features of enteropathic arthritis?
May only have arthralgia
Asymmetrical oligoarticular disease
May see associated skin lesions e.g. pyoderma gangrenous and erythema nodosum
Management of enteropathic arthritis?
Treat the IBD
Symptomatic = NSAIDsand steroids for flares
Sulfasalazine as treats the joints and the IBD
What is reactive arthritis?
Sterile inflammation of joint, initiated by infection e.g. Salmonella / shigella / yersinia
Classic triad of reactive arthritis?
Arthritis
Conjuctivitis
Urethritis
Clinical features of reactive arthritis?
Arthritis weeks after urethritis / dysentry
Often self limiting
Associated:
- Conjunctivitis
- Uveitis
- Oral ulcers
- Keratoderma blennorhagica
Management of reactive arthritis?
NSAIDs and steroids acutely
Ongoing = sulfasalazine
What is osteoporosis?
Predisposition to fractures due to low bone mass and micro-architectural deterioration of bone tissue
What is the bone mineral density of osteoporosis and osteopenia?
Osteoporosis is
RF’s for osteoporosis?
Post-menopause Previous # Steroids Low BMI Malabsorption Alcoholic / smoker
Pathology of osteoporosis?
Increased osteoclast activity = reduced density
Investigations for osteoporosis?
FRAX tool for 10 year fracture risk - used in all women >65, men >75
Younger if RF’s
Bloods NORMAL
DEXA scan
How does FRAX score affect management?
Low risk = conservative
Intermediate = DEXA
High risk = Medical therapy
Management of osteoporosis?
Conservative = manage RF’s, dietary advice, exercise, falls prevention with OT and physio.
Medical = Calcium and vitamin D
Anti-resorptive agents = bisphosphonates e.g. alendronate 10mg PO OD
OR
RANK-L antibodies = Denosumab 60mg SC twice yearly
What is osteomalacia?
Inadequate bone mineralisation due to vitamin D deficiency.
Can be due to poor absorption or poor metabolism
Biochem of osteomalacia?
X-ray signs?
Low vitamin D
Low calcium and phosphate = PTH raised = secondary hyperPTH
High alkaline phosphatase
Loosers zones
Management of osteomalacia?
Vitamin D supplementation
Anti-Scl 70?
Systemic sclerosis
Anti-centromere?
CREST
Anti-jo 1?
Polymyositis
C-ANCA?
GPA / Wegeners
P-ANCA?
Microscopic polyangitis and EGPA/Churg Strauss
Anti-ro/la?
Sjogrens
Anti-CCP?
RA
Anti dsDNA or Anti-Sm?
SLE
Clinical features of SLE?
Skin features: MALAR (rash) Mouth ulcers Alopecia Livedo reticularis Abnormal sensitivity to light Raynauds
Arthritis
Pericarditis, pneumonitis, nephritis
Blood disorders e.g. anaemia, leucopenia, thrombocytopaenia
Diagnostic criteria for SLE?
4 of: BD PANORAMAS….
Blood disorder
Discoid rash
Photosensitive Arthritis Neuro disease Oral ulcers Renal disease ANA = 99% +ve Malar rash Autoantibodies to dsDNA or Sm = very specific but not that sensitive Serositis
Management of SLE?
Conservative = avoid sun, stop smoking, healthy
Medical:
Mucocutaneous = eye drops and sunscreen
Joints = Naproxen 500mg BD
+ hydroxychloroquinine if severe
Steroids if refractory and severe
If nephritis may need immunosuppressants e.g. cyclophosphamide
What is drug induced lupus?
Clinical signs?
Variant on SLE that resolves within days to months on drug withdrawal
Mostly skin and lung signs
Most common drugs causing drug induced lupus?
Anti-hypertensives = Hydralazine
Anti-arrhythmic = Procainamide
Antibiotics = Minocycline and isoniazid
What are the antibodies for drug induced lupus?
ANA+ve 100%, anti-histone 95%
what is anti-phospholipid syndrome?
Due to anti-phospholipid antibodies against anti-cardiolipin and lupus anticoagulant.
mainly primary, but can be secondary due to SLE
Diagnostic criteria for antiphospholipid syndrome?
Vascular thrombosis in any organ OR a pregnancy event
+
Persistently positive for antibodies
Clinical features of antiphospholipid syndrome?
Clotting
Miscarriage
Livedo reticularis
Cardiac valve lesions
If due to lupus anticoagulant = prolonged APTT
Management of antiphospholipid syndrome?
Initially LMWH e.g. Dalteparin
Then lifelong warfarin INR 2-3
If pregnant LMWH only
What is systemic sclerosis?
Disease of unknown aetiology characterised by fibrosis of skin, vessels and internal organs
Classification of systemic sclerosis?
Diffuse = 30% = early and severe organ involvement
Limited = 70% = CREST, skin limited to face, hands and feet.
Clinical features of limited vs diffuse sclerosis?
Limited = CREST + 15% have pulmonary HTN
Calcinosis Raynauds oEsophageal dismotility Sclerodactyly Telangiectasia
Diffuse: Widespread skin involvement Organ fibrosis: GI = GORD, aspiration and incontinence Lung fibrosis Cardiac = arrhythmias Renal = acute HTN crisis
Management in systemic sclerosis?
Conservative = exercise, stop smoking, skin emollients
Hand warmers for Raynauds
Medical = immunosuppressants e.g. methotrexate or mycophenolate
then treat symptomatically…..
Calcinosis = excision Raynauds = Nifedipine 10mg PO TDS GORD = PPI's
Renal crisis = ACEI Captopril 12.5mg PO OD
What is Sjogrens?
Autoimmune condition characterised by inflammation and destruction of the exocrine glands
Clinical features of sjogrens - glandular?
Dry itchy eyes = Keratoconjunctivitis sick and dry mouth
Other glands = dry skin, otitis media, chest infection and pancreatic insufficiency
Clinical features of sjogrens - extra glandular?
Fatigue and arthralgia Peripheral neuropathy RTA Vasculitis LN's and hepatosplenomegaly
Serology for sjogrens?
RF+ve
ANA speckled +ve
Antibodies to ro and la
Management of sjogrens?
Dry eyes = artificial tears, ophthalmic ciclosporin
Dry mouth = artificial saliva
Arthritis = Naproxen
2nd line = hydroxychloroquinine
Vascultitis = corticosteroids
What is myositis?
Group of disorders with immune mediated muscle injury causing proximal muscle weakness
four types of myositis?
Polymyositis
Dermatomysositis
Inclusion body myositis
Necrotising myopathy
Women aged 50-70, symmetrical proximal muscle weakness, dysphagia, systemic upset and arthralgia
Myositis
What is dermatomyositis?
Myositis + skin signs
Dermatomyositis skin signs?
FACE:
Heliotrope rash = lilac rash affecting eyelids and nasolabial folds
HANDS:
Gottrons papules = purple erythematous flat lesions over interphalangeal region of fingers
Mechanic hands = cracking and fissuring over skin pads
Nailfold abnormalities = periungual erythema, dilated capillary loops
BODY:
V-sign rash = Confluent erythematous rash over anterior chest and neck
Macular rash = shawl like erythematous over shoulders
Who does anti-synthetase syndrome affect?
30% of patients with polymyositis and dermatomyositis
What are the features of anti-sdnthetase syndrome?
MARIMBA
Myositis Acute onset Raynauds Interstitial lung disease Mechanic hands B-symptoms Arthritis
CK raised, anti-jo1 and EMG characteristic findings
Myositis
Management of myositis?
Physio and rehab
Medical = immunosuppression
High dose steroids for 1 week then taper
2nd line = azathioprine / methotrexate
What is polymyalgia rheumatica?
Clinical syndrome of proximal limb girdle pain and stiffness, associated with rapid response to steroids
70 years old female
Clinical features of polymyalgia rheumatica?
Acute onset, bilateral proximal muscle pain = Neck, shoulder and hip
Early morning stiffness
Normal muscle strength but hard to assess due to pain
Investigations In polymyalgia rheumatica?
Management?
CRP and ESR raised
USS for bursitis and effusions = commonly trochanteric
Prednisiolone high dose then taper to response
Will see rapid response in 48 hours
Usually on low dose for prednisolone for a year
What is a vasculitis?
Inflammation of the blood vessels with narrowing / occlusion = tissue ischaemia or necrosis
What is the Chapel-Hill Classification of vasculitides?
By size:
Large = Giant cell arteritis and Takayasu’s
Medium = Polyarteritis nodosa and Kawasakis
Small = GPA, EGPA and microscopic polyangitis
HSP
What is giant cell arteritis associated with?
Polymyalgia rheumatica
Clinical features of GCA?
Commonly affects temporal and ophthalmic = unilateral headache, temporal
Scalp tenderness and jaw claudication
Visual phenomenon
May have proximal weakness due to PMR
Investigations for GCA?
Raised CRP
Gold standard = temporal artery biopsy
Management of GCA?
Do not delay steroids as blindness risk
High dose 60mg PO OD for 4 weeks then taper down
IV steroids if impending visual loss
Aspirin 75mg + GI and bone protection
What is takayasus arteritis?
Granulomatous panarteritis of the aorta and great vessels causing stenosis / occlusion / aneurysm
Affects young Asian females
Clinical features of takayasus - systemic stage?
Systemic stage = fever, fatigue, weight loss and arthralgia
Clinical features of Takayasus - Occlusive stage?
Vascular = claudication, HTN and ABSENT PERIPHERAL PULSES
Neuro = Syncope, TIAs
Cardiac = Angina and dyspnoea
- aortic regurgitation secondary to root dilation
Investigations for Takayasus?
Bloods = inflammatory markers raised
Non-invasive angiography e.g. MRA CTA
Management of Takayasus?
Medical = Steroids, Aspirin and GI and bone protection
If cannot tolerate steroids = methotrexate
May need surgical intervention if significant limb ischaemia - immunosuppress prior to revascularisation
What is polyarteritis nodes (PAN)
Vasculitis of medium sized vessels with aneurysm formation
Often middle aged men with Hep B
Clinical features of PAN?
Fever and weight loss, arthralgia
GI = perforation / haemorrhage
Renal = Haematuria, HTN
Testes = pain
Investigations for PAN?
Angiography, biopsy affected organs
Management of PAN?
Non-hepatitis:
Poor prognosis = Prednisolone, cyclophosphamide and azathioprine
Good prognosis = Prednisolone
Hepatitis related:
Prednisolone
What is Kawasakis?
An acute febrile self limiting vasculitis of medium vessels
Asian children
Clinical features of Kawasaki’s?
Fever > 5 days plus 4 of:
- Bilateral conjucntivitis
- Cervical lymophadenopathy
- Oral mucositis = strawberry tongue
- Hand/ feet erythema followed by desquamation
- Blotchy red rash
20% will develop coronary vasculitis / aneurysm
Management of Kawasakis?
IVIG 2g/kg as single dose
The aspiring 80mg/kg / day
What is GPA?
Granulomatosis with polyangitis
Necrotising granulomatous vasculitis of small vessels
Whats the classic triad of GPA?
URT, LRT and glomerulonephritis
Clinical features of gPA?
ENT = rhinorrhoea, sinusitis, saddle nose due to nasal ulceration, otitis media
LRT = cough and haemoptysis
Renal = Glomerulonephritis
Investigations forn GPA?
cANCA +ve
Urinalysis
CXR
Biopsy affected tissue = necrotising granulomas
Management of GPA?
Organ threatening = Methylprednisolone IV, followed by oral + cyclophosphamide
Non-Organ threatening = steroids and methotrexate
Maintenance of remission = Steroids and methotrexate
What is eGPA?
Also a necrotising granulomatous vasculitis
Rare, but prevalent in ASTHMATICS
Classic triad of eGPA?
Eosinophilia, granulomatous inflammation and vasculitis
Clinical features of eGPA?
Lungs = Asthma, rhinitis or sinusitis
Skin = nodules and purpura
Neuro = neuropathy
Investigations eGPA?
pANCA +ve
CXR = infiltrates
Biopsy = eosinophilic necrotising granulomas
Management of eGPA?
Prednisolone
If steroids not sufficient = Methotrexate
Severe = Cyclophosphamide and rituximab
Clinical features of microscopic polyangitis?
Fever and malaise
Lung = pulmonary infiltrates
Renal = Glomerulonephritis
Neuropathy
Investigations for MPA?
pANCA
biopsy = necrotising granulomas
Management of MPA?
Steroids and methotrexate
Cyclophosphamide if more severe
What is HSP?
IgA mediated autoimmune hypersensitivity vasculitis
Most common vasculitis of childhood. Males
Clinical features of HSP?
Hx of URTI
Palpable non blanching rash over lower limb extensor surfaces
Symmetrical large joint arthralgia / arthritis = Knees and ankles
Glomerulonepgirits (IgA nephropathy )
Investigations for HSP?
Urinalysis = RBC’s, casts and protein
Elevated IgA in serum
biopsy = Immunofluorescence show IgA and C3
Management of HSP?
Steroids
If severe add in cyclophosphamide
Prognosis excellent, 1 in3 have relapses
Pain all over, with multiple trigger points. Normal joints…
Fibromyalgia
Management of fibromyalgia?
Information and self care
Analgesia = Pregabalin or duloxetine
Physio, accupuncture
CBT
Assess for psychiatric disturbances
