Rheumatology Flashcards
(194 cards)
1
Q
What is osteoarthritis?
A
- Degenerative change-> wear and tear of synovial joints
- Imbalance between cartilage wearing down + chondrocytes repairing
2
Q
What are the risk factors for osteoarthritis?
A
Obesity, age, occupation, trauma, female, family history
3
Q
What are the X-ray changes characteristic of osteoarthritis?
A
LOSS or no X-ray change
- Loss of joint space
- Osteophytes
- Subchondral sclerosis-> increased density of bone along joint line
- Subchondral cysts-> fluid filled holes in bone
4
Q
How does osteoarthritis present?
A
- Joint pain + stiffness-> worse on activity
- Deformity, instability, reduced function
- Joints affected-> MCP (thumb base), DIPJs, wrist, hips, knees, SIJs, cervical spine
- Hands-> Heberden’s nodes (DIPJ), Bouchard’s (PIPJ), squaring of thumb base, weak grip, reduced ROM
5
Q
What are Heberden’s nodes?
A
Deformity of DIPJs in osteoarthritis
6
Q
What are Bouchard’s nodes?
A
Deformity of PIPJs in osteoarthritis
7
Q
How is osteoarthritis diagnosed?
A
- Can make without investigations in >45s
- Symptoms-> activity related pain, no morning stiffness, no stiffness lasting <30 mins
8
Q
How is osteoarthritis managed?
A
- Weight loss
- Physio
- OT
- Analgesia-> paracetamol, NSAIDs (oral/topical), PPI, opiates
- Intra-articular steroid injections
- Joint replacement-> hip/knee
9
Q
What is rheumatoid arthritis?
A
- Autoimmune condition causing chronic inflammation of synovial lining, tendon sheaths and bursa
- Symmetrical polyarthritis
10
Q
What are the risk factors for rheumatoid arthritis?
A
- Women
- Any age but usually middle
- FH
- HLADR4 gene (in RF +ve patients)
11
Q
What is rheumatoid factor?
A
- Autoantibody-> usually IgM
- Targets Fc portion of IgG antibody-> systemic inflammation
- Present in 70% of RA patients
12
Q
What is anti-CCP?
A
- Antibody sometimes present in rheumatoid arthritis
- More sensitive than rheumatoid factor
13
Q
How does rheumatoid arthritis present?
A
- Pain, swelling + stiffness-> improves with activity
- Joints-> hands + feet (eg wrist, ankle, MCP, PIP, MTPJs)
- Can affect large joints-> knees, shoulders, elbows
- DIPJs hardly affected
- Atlantoaxial subluxation
- Hand signs-> Z-shaped deformity of thumb, Swan neck deformity, Boutonniere’s
- Extra-articular disease
14
Q
What hand signs can patients get in rheumatoid arthritis?
A
- Boggy inflammation + swelling
- Z-shaped deformity of thumb
- Swan neck deformity-> hyperextended PIPJ + flexed DIPJ
- Boutonniere’s deformity-> hyperextended DIPJ + flexed PIPJ
15
Q
What extra-articular disease can present in rheumatoid arthritis?
A
- Caplan’s syndrome-> pulmonary fibrosis + nodules
- Bronchiolitis obliterans
- Felty’s syndrome-> rheumatoid arthritis + neutropaenia + splenomegaly
- Sicca syndrome-> secondary Sjogren’s
- Anaemia
- CVD
- Carpal tunnel
- Episcleritis + scleritis
- Rheumatoid nodules
- Amyloidosis
16
Q
What is Felty’s syndrome?
A
rheumatoid arthritis + neutropaenia + splenomegaly
17
Q
How is rheumatoid arthritis investigated?
A
- Clinical
- Rheumatoid factor
- Anti-CCP
- CRP
- ESR
- X-rays-> joint destruction, bony erosions etc
- US-> synovitis
- Referral criteria
18
Q
What X-ray changes might be present in rheumatoid arthritis?
A
- Joint destruction + deformity
- Soft tissue swelling
- Periarticular osteopenia
- Bony erosions
19
Q
What is the referral criteria for suspected rheumatoid arthritis?
A
- Adult with persistent synovitis regardless of markers
- Urgent-> small joints of hands + feet, multiple joints, symptoms for 3+ months
20
Q
What is the diagnostic criteria for rheumatoid arthritis?
A
American College of Rheumatology + European League Against Rheumatism-> score added up and 6+ indicates
- More joints-> higher
- Small joints-> higher
- RF and anti-CCP
- ESR and CRP
- Symptoms for 6+ weeks
21
Q
What is the DAS28 score?
A
For rheumatoid arthritis
- Disease activity-> assess 28 joints for swelling + tenderness
- Use ESR + CRP too
22
Q
What affects prognosis in rheumatoid arthritis?
A
Young, male, more joints, RF and anti-CCP positive, erosions on X-ray-> worse prognosis
23
Q
What is used for rheumatoid arthritis treatment?
A
- NSAIDS, COX-2 inhibitors, steroids (+PPI cover)
- DMARDs-> methotrexate, leflunomide, sulfasalasine, hydroxychloroquinine
- May use infliximab or etanercept or rituximab
- CRP + DAS28 to monitor treatment success
24
Q
What is the mechanism of action of methotrexate?
A
Interferes with folate metabolism + suppresses immune system-> use in rheumatoid arthritis
25
How is methotrexate taken?
- 1 tablet/injection once weekly
| - Folic acid 5mg on different day
26
What are the side effects of methotrexate?
Pulmonary fibrosis, teratogenic, liver toxicity, mouth ulcers, mucositis, low WBCs
27
What is leflunomide?
Interfered with pyrimidine production (RNA + DNA)-> used in rheumatoid arthritis
28
What are some side effects of leflunomide?
Hypertension and peripheral neuropathy
29
What is sulfasalazine?
Immunosuppressant + anti-inflammatory-> related to folate metabolism
30
What is a side effect of sulfasalazine?
Reduced sperm count
31
What is hydrochloroquine?
Antimalarial but immunosuppressant too-> use in rheumatoid arthritis
32
What are some side effects of hydrochloroquine?
Nightmares + reduced visual acuity
33
How do anti-TNF drugs (eg etanercept) work?
Block TNF cytokines + reduce inflammation
34
What are some side effects of anti-TNF drugs (eg etanercept)?
reactivation of TB + Hep B
35
What is rituximab?
Monoclonal antibody-> can use in RA
36
What are some side effects of rituximab?
Low platelets, night sweats
37
What is psoriatic arthritis?
- Inflammatory arthritis associated with psoriasis
| - Seronegative spondyloarthropathy
38
How many patients with psoriasis have psoriatic arthritis?
10-20%
39
What are the different patterns of psoriatic arthritis?
- Symmetrical polyarthritis-> hands, wrists, ankles, DIPJ
- Asymmetrical pauciarthritis-> digits + few joints
- Spondylitis pattern-> back, sacroiliitis etc
- Other-> spine, achilles, plantar fascia
40
What are the signs of psoriatic arthritis?
- Nails-> psoriasis, nail pitting, onycholysis
- Dactylitis-> full finger inflammation
- Enthesitis-> tendon inserting to bone tender
- Eyes-> conjunctivitis, anterior uveitis
- Aortitis
- Amyloidosis
41
What is the PEST screening tool?
Psoriasis Epidemiological Screening Tool-> if high score get rheum referral
42
What are the X-ray changes classical of psoriatic arthritis?
- Pencil in cup-> central erosion to bone beside joint, one bone hollow + other narrow, in hands + feet
- Periostitis-> periosteum inflammed
- Ankylosis-> bones join + stiffen
- Osteolysis-> destroy bone
- Dactylitis-> soft tissue swelling
43
What is the 'pencil in cup' X-ray sign?
Sign of psoriatic arthritis
- central erosion to bone beside joint
- one bone hollow + other narrow
- in hands + feet
44
What is arthritis mutilans?
- Severe psoriatic arthritis
- Osteolysis of phalanxes + progressive shortening
- Telescopic finger
45
How is psoriatic arthritis managed?
- NSAIDs
- DMARDs
- Anti-TNF
- Ustekinumab-> monoclonal antibody
46
What is reactive arthritis (Reiter syndrome)?
- Acute monoarthritis
- Synovitis in reaction to recent infective trigger
- Often GE or STI
- Seronegative spondyloarthopathy
47
What usually triggers reactive arthritis (Reiter syndrome)?
- Gastroenteritis
| - STI-> chlamydia
48
What gene is seronegative spondyloarthropathy associated with?
HLAB27
49
What symptoms is reactive arthritis (Reiter syndrome) associated with?
Can't pee, see or climb a tree
- Bilateral conjunctivitis
- Anterior uveitis
- Circinate balantitis
- Arthritis
50
How is reactive arthritis (Reiter syndrome) managed?
- Hot joint policy-> antibiotics until septic excluded
- Aspirate-> MC&S to exclude septic + crystal exam (gout or pseudogout)
- NSAIDs
- Steroid injections
- Systemic steroids in multiple joints
- Most resolve in 6 months
- Recurrent-> DMARDs or anti-TNF
51
What is ankylosing spondylitis?
- Seronegative spondyloarthropathy
- Progressive stiffness + pain in spine-> SIJ + vertebral column joints
- Fusion in joints
52
What is the classic X-ray finding in ankylosing spondylitis?
Bamboo spine
53
How does ankylosing spondylitis present?
- Typically young male with symptoms for 3 months
- Lower back pain + stiffness in buttocks
- Improves with movement
- Worse at night + morning (may wake)
- Takes 30 minutes to improve on a morning
- Vertebral fractures
- Lots of systemic associations eg weight loss, chest pain, dactylitis
54
What other systemic/extra-articular symptoms can ankylosing spondylitis present with?
- Weight loss
- Fatigue
- Chest pain-> costovertebral + costosternal joints
- Enthesitis
- Dactylitis
- Anaemia
- Anterior uveitis
- Aortitis
- Heart block
- Restrictive lung disease
- Pulmonary fibrosis
- IBD
55
What is Schober's test?
- Stand straight + find L5 vertebrae
- Mark 10cm above + 5cm below joint
- Ask to bend forwards + measure distance
- Different of <20cm-> restriction of lumbar movements
- Supports diagnosis of ankylosing spondylitis
56
What investigations are done in ankylosing spondylitis?
- CRP + ESR
- HLAB27 genetics
- X ray of spine and sacrum-> bamboo spine
- MRI-> bone marrow oedema
57
What X-ray findings might be present in ankylosing spondylitis?
- Bamboo spine
- Squaring of vertebral bodies
- Subchondral sclerosis + erosions
- Syndesmophytes-> bone growth where ligaments insert
- Ossification-> ligaments/discs turn to bone
- Fusion-> SI, facet or costovertebral joints
58
How is ankylosing spondylitis managed?
- NSAIDs
- Steroids in flares
- Anti-TNF-> etanercept or infliximab
- Secukinumab
- Physio
- Exercise
- Avoid smoking
- Bisphosphonates for osteoporosis
- Surgery
59
What is Systemic Lupus Erythematosus (SLE)?
- Inflammatory autoimmune connective tissue disease
- Affects multiple organs + systems (systemic)
- Red malar rash (erythematosus)
- Relapsing remitting course
60
Who is Systemic Lupus Erythematosus (SLE) more common in?
- Women
- Asians
- Young to middle ages adults
61
What is the pathophysiology of Systemic Lupus Erythematosus (SLE)?
- Anti-nuclear antibodies-> to cell nuclei
- Immune response to target proteins-> inflammatory response
- Chronic + against body tissues
62
How does Systemic Lupus Erythematosus (SLE) present?
- Photosensitive malar rash-> butterfly rash on nose + cheeks worsened by sunlight
- Fatigue
- Weight loss
- Arthralgia
- Myalgia
- Fever
- SOB
- Pleuritic chest pain
- Mouth ulcers
- Hair loss
- Raynaud's
- Lymphadenopathy
- Splenomegaly
63
How is Systemic Lupus Erythematosus (SLE) investigated?
- Autoantibodies-> anti-nuclear (ANA), anti-double stranded DNA (anti-dsDNA), antiphospholipid (secondary)
- FBC-> normocytic anaemia
- CRP + ESR
- C3 + C4 decreased
- Urine protein:creatinine + renal biopsy-> nephritis
64
What diagnostic criteria can be used in Systemic Lupus Erythematosus (SLE)?
SLICC or ACR-> ANA + clinical features
65
What auto-antibodies are usually present in Systemic Lupus Erythematosus (SLE)?
- anti-nuclear (ANA)
- anti-double stranded DNA (anti-dsDNA)
- antiphospholipid (secondary)
66
What are the complications of Systemic Lupus Erythematosus (SLE)?
Chronic inflammation...
- CVD-> often cause death
- Infection
- Anaemia of chronic disease
- Pericarditis
- Pleurisy
- Interstitial lung disease
- Lupus nephritis
- Neuropsychiatric
- Recurrent miscarriage
- IUGR
- Pre-eclampsia
- Preterm
- Anti-phospholipid syndrome
- VTE
67
How is Systemic Lupus Erythematosus (SLE) managed?
- Symptom control not sure
- NSAIDs, prednisolone, hydroxychloroquinine, suncream + sun avoidance
- Immunosuppressants when severe-> MTX, azathioprine etc
- Biological when severe/resistance-> rituximab etc
68
What is Discoid Lupus Erythematosus?
Non-cancerous skin condition associated with risk of SLE + squamous cell cancers
69
Who is Discoid Lupus Erythematosus more common in?
- Women
- Age 20-40
- Darker skinned
- Smokers
70
How does Discoid Lupus Erythematosus present?
- Lesions on face/ears/scalp-> inflamed, dry, erythematous, patchy, crusty/scaling, photosensitive
- Scarring alopecia-> hair doesn't grow back
- Hyper/hypopigmented scars
71
How is Discoid Lupus Erythematosus managed?
- Skin biopsy to confirm
- Sun protection
- Topical steroids
- Intralesion steroid injections
- Hydroxychloroquine
72
What is systemic sclerosis?
- Autoimmune inflammatory + fibrotic connective tissue disease
- Affects skin + internal organs
- Scleroderma-> hardening of skin
73
What are the different types of systemic sclerosis?
- Limited cutaneous systemic sclerosis-> CREST syndrome
| - Diffuse cutaneous systemic sclerosis-> CREST features + internal organ problems
74
What are the features of limited cutaneous systemic sclerosis (CREST syndrome)?
- Calcinosis
- Raynaud's
- Oesophageal dysmotility
- Sclerodactyly
- Telangiectasia
75
What are the features of Diffuse cutaneous systemic sclerosis?
- Calcinosis
- Raynaud's
- Oesophageal dysmotility
- Sclerodactyly
- Telangiectasia
- HTN + CAD
- Pulmonary HTN + fibrosis
- Glomerulonephritis
- Scleroderma renal crisis
76
What are the features of systemic sclerosis?
- Scleroderma-> tight + shiny skin
- Calcinosis-> deposits under skin
- Raynaud's
- Oesophageal dysmotility
- Sclerodactyly
- Telangiectasia
- Systemic and pulmonary HTN
- Pulmonary fibrosis
- Scleroderma renal crisis
77
What is Raynaud's phenomenon?
- Fingers completely white/blue in response to cold
- Vasoconstriction of vessels
- Common without underlying disease but may indicate systemic sclerosis
78
What auto-antibodies may be present in systemic sclerosis?
- ANA
- Anti-centromere
- Anti-Scl-70
79
What is nailfold capillaroscopy?
- Examine where skin meets fingernails at base
- Peripheral capillaries-> abnormal, avascular, microhaemorrhages etc
- Look for systemic sclerosis in Raynaud's
80
What is used to investigate for systemic sclerosis?
Nailfold capillaroscopy
- Examine where skin meets fingernails at base
- Peripheral capillaries-> abnormal, avascular, microhaemorrhages etc
- Look for systemic sclerosis in Raynaud's
81
How is systemic sclerosis diagnosed?
American College of Rheumatology + European League Against Rheumatism criteria-> clinical + antibodies + nailfold capillaroscopy
82
How is systemic sclerosis managed?
- Steroids + immunosuppressants
- Skin stretching
- Emollients
- Cold avoidance
- PT + OT
- Nifedipine-> Raynaud's
- PPIs
- Analgesia
- Anti-HTNs
83
What is polymyalgia rheumatica?
Inflammatory condition causing pain and stiffness in the shoulders, pelvic girdle and neck
84
What is polymyalgia rheumatica strongly associated with?
Giant cell arteritis
85
Who is polymyalgia rheumatica most common in?
- Women
- Caucasians
- 50+
86
What are the core features of polymyalgia rheumatica?
- Symptoms for 2+ weeks
- Bilateral shoulder pain + can radiate to elbow
- Bilateral pelvic girdle pain
- Worsened-> movement
- Interferes with sleep
- 45 min+ of morning stiffness
- Systemic-> weight loss, low mood
- Upper arm tenderness
- Carpal tunnel
- Pitting oedema
87
What the the differentials for polymyalgia rheumatica?
- OA
- RA
- SLE
- Myositis
- Cervical spondylosis
- Adhesive capsulitis
- Hyper or hypothyroidism
- Osteomalacia
- Fibromyalgia
88
How is polymyalgia rheumatica diagnosed?
- Clinical + response to steroids
- Inflammatory-> CRP + ESR
- FBC, U+Es, LFTs, TFTs, CK (raised)
- RF
- Calcium-> other causes
- Urine dip
- Rule out-> ANA (SLE), anti-CCP (RA), Bence-Jones (myeloma), CXR
89
How is polymyalgia rheumatica managed?
- Oral prednisolone-> 15mg OD
- Assess after 1 week + stop if no response
- Assess after 3-4 weeks-> should improve
- Good response-> reducing regime long term
- Recurrence-> increase dose + can take 1-2 years
- Rheumatology referral-> if need steroids for 2+ years
90
How long does it take for someone to become dependent on steroids?
3 weeks
91
What extra precautions should be done when someone is on long-term steroid therapy?
- Sick day rules-> increase dose when unwell
- Treatment card-> alert when unresponsive
- Osteoporosis prevention-> bisphosphonates + calcium + vitamin D
- PPI
92
What is giant cell arteritis?
Systemic vasculitis of medium + large arteries-> commonly temporal arteritis
93
What is giant cell arteritis associated with?
Polymyalgia rheumatica-> high risk when female + 50+
94
What is a key complication of temporal arteritis?
Irreversible vision loss
95
What are the symptoms of temporal arteritis?
- Headache-> severe, unilateral, temple/forehead
- Scalp tenderness-> when brush hair etc
- Jaw claudication
- Blurred/double vision-> can get rapid complete painless sight loss
- Systemic-> fever, muscle ache, fatigue, weight loss, loss of appetite, peripheral oedema
96
How is temporal arteritis diagnosed?
- Raised ESR
- Temporal artery biopsy-> multinucleated giant cells + beware of skip lesions
- FBC, LFTs, CRP
- Duplex US of temporal artery-> hypoechoic halo sign
97
How is temporal arteritis managed?
- Steroids immediately-> 40-60mg prednisolone a day + review response in 48 hours
- Steroids till symptoms resolve-> can take few years
- Aspirin 75mg OD-> if suspect visual loss + stroke
- PPI + osteoporosis prevention
98
What are the complications of temporal arteritis?
- Neuro-opthalmic-> visual loss + stroke
- Relapses
- Steroid related SEs
- CVA
- Aortitis-> aneurysm + dissection
99
What is polymyositis?
Chronic inflammation of muscles
100
What is dermatomyositis?
Connective tissue disorder in which skin + muscles are chronically inflamed
101
What is the key investigation for polymyositis & dermatomyositis?
Creatinine kinase-> can be >1000u/L (normal- <300)
102
What can cause polymyositis & dermatomyositis?
Malignancy-> paraneoplastic syndrome of lung, breast, ovarian or gastric cancers
103
How does polymyositis present?
- Muscle pain + weakness-> bilateral, proximal, shoulders, pelvic girdle, over a few weeks
- No skin involvement
- Fatigue
104
How does dermatomyositis present?
- Muscle pain + weakness-> bilateral, proximal, shoulders, pelvic girdle, over a few weeks
- Fatigue
- Photosensitive erythematous rash-> back, shoulders, neck
- Purple rash on face + eyelids
- Periorbital oedema
- Subcutaenous calcinosis
- Gottron lesions-> scaly + erythematous
105
How is polymyositis diagnosed?
- Clinical
- High CK
- Autoantibodies-> anti-Jo-1
- Electromyography (EMG)
- Muscle biopsy
106
How is dermatomyositis diagnosed?
- Clinical
- High CK
- Autoantibodies-> anti-Mi-2, ANA
- Electromyography (EMG)
- Muscle biopsy
107
How is polymyositis managed?
- Assess for underlying cancers
- PT + OT
- Corticosteroids, azathioprine, IV Igs, biologicals
108
How is dermatomyositis managed?
- Assess for underlying cancers
- PT + OT
- Corticosteroids, azathioprine, IV Igs, biologicals
109
What is the pathophysiology if antiphospholipid syndrome?
- Antiphospholipid antibodies
- Interfere with coagulation + make more prone-> hypercoagulable
- Primary or secondary (eg SLE)
110
What are the associations/complications of antiphospholipid syndrome?
- VTE, stroke, MI
- Pregnancy-> recurrent miscarriage, stillbirth, pre-eclampsia
- Thrombocytopaenia
- Livedo reticularis-> purple rash + mottled
- Libmann-Sacks endocarditis
111
How is antiphospholipid syndrome diagnosed?
History of thrombosis or pregnancy complications + antibodies (antiphospholipid)
112
How is antiphospholipid syndrome managed?
- Rheumatology, haematology + ostatrics involvement
- Warfarin-> INR range 2-3
- LMWH + aspirin in pregnancy
113
What is Sjogren's syndrome?
- Autoimmune disease of exocrine glands
- Dry mucous membranes-> mouth, eyes, vagina
- Primary or secondary (eg SLE)
114
What antibodies are present in Sjogren's syndrome?
Anti-ro + anti-la
115
What is the Schirmer test?
- Folded filter paper under lower lid + strip hand out over eyelid
- 5 mins-> measure distance along strip
- Normal-> >15mm
- Sjogren's syndrome-> <10mm
116
How is Sjogren's syndrome managed?
- Artificial tears and saliva
- Vaginal lubricants
- Hydroxychloroquine
117
What are the common complications of Sjogren's syndrome?
- Conjunctivitis
- Corneal ulcers
- Dental cavities
- Candida infections
- Sexual dysfunction
118
What is vasculitis?
Inflammation of blood vessels-> can be small, medium or large
119
What are some types of small vessel vasculitis?
- Henoch-Schoenlein Purpura
- Churg-Strauss
- Wegener's granulomatosis
- Microscopic polyangiitis
120
What are some types of medium vessel vasculitis?
- Polyarteris nodosa
- Churg-Strauss
- Kawasaki's disease
121
What are some types of large vessel vasculitis?
- Giant cell arteritis
| - Takayasu's arteritis
122
How does vasculitis present (in general)?
- Systemic-> fatigue, weight loss, anaemia, fever
- Purpura-> purple + non-blanching
- Joint + muscle pain
- Peripheral neuropathy
- Renal impairment
- Uveitis + scleritis
- HTN
123
What investigations are done in vasculitis?
- CRP
- ESR
- Anti-neurophil cytoplasmis (ANCA)
- P-ANCA + C-ANCA
124
How is vasculitis managed?
- Depends on type
- Rheum referral
- Steroids-> oral, IV, nasal, inhaled
- Cyclophosphamide
- MTX
- Azathioprine
125
What is Henoch-Schonlein Purpura?
- IgA vasculitis-> deposits in blood vessels
| - Small vessel vasculitis
126
How does Henoch-Schonlein Purpura present?
- Purpuric rash on legs/bum
- Joint pain
- Abdominal pain
- Renal involvement
- Often kids <10 years
127
What can trigger Henoch-Schonlein Purpura?
- Upper airway infection
| - GE
128
How is Henoch-Schonlein Purpura managed?
Supportive-> simple analgesia
129
How often does Henoch-Schonlein Purpura recur?
In 1/3 patients within 6 months
130
What is Churg-Strauss?
- Eosinophilic Granulomatosis with Polyangiitis
| - Type of vasculitis-> small + medium vessel
131
How does Churg-Strauss present?
- Severe asthma in late teens/adults
| - High eosinophils on RBC
132
How does microscopic polyangiitis present?
- Renal failure
- SOB
- Haemoptysis
133
How does Wegener's granulomatosis present?
- Upper resp-> epistaxis, crusty secretions, saddle nise
- Hearing loss
- Cough, wheeze, haemoptysis
- Glomerulonephritis
134
What is polyarteritis nodosa associated with?
Hep B/C or HIV
135
How does polyarteritis nodosa present?
- MI
- Renal impairment
- Livedo reticularis-> mottles purple lace-like rash
136
What is Kawasaki disease?
Medium vessel vasculitis
137
What are the features of Kawasaki disease?
- High fever for 5+ days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema + desquamation of palms + soles
- Strawberry tongue
138
What is the main complication of Kawasaki disease?
Coronary artery aneurysm
139
How is Kawasaki disease managed?
- Aspirin
| - IV Igs
140
How does Takayasu's arteritis present?
- Age <40
- Fever
- Malaise
- Claudication
- Syncope
141
What is Takayasu's arteritis?
- Large vessel vasculitis
- Aorta, branches + pulmonary arteries
- Aneurysms + blockages
142
How is Takayasu's arteritis diagnosed?
CT, MRI angiography, carotid doppler US
143
What is Bechet's syndrome?
Complex inflammatory condition causing recurrent oral + genital ulcers
144
What gene is Bechet's syndrome associated with?
HLA B51
145
What are the features of Bechet's syndrome?
- 3+ ulcers a year taking 2-4 weeks to heal-> genital + oral
- Erythema nodosum
- Uveitis
- Morning stiffness + arthralgia
- CNS-> headaches + memory problems
- Pulmonary artery aneurysms
- DVT
146
How is Bechet's syndrome investigated?
Pathergy test-> subcut abrasion + measure size in 24-48 hours
147
How is Bechet's syndrome managed?
- Steroid tablets
- Colchicine
- Lidocaine ointment
- Immunosuppressants
148
What is gout?
- Crystal arthropathy
| - High uric acid causes urate crystals in joints
149
What are gouty tophi?
- Subcutaneous deposits of uric acid
- Often in hands, elbows, ears and DIPJs
- Need to rule out septic arthritis if hot + swollen
150
What differential for gout is important to rule out?
Septic arthritis
151
What are the risk factors for gout?
- Male
- Obese
- High purine-> meat + seafood
- Alcohol
- Diuretics
- CVD
- Kidney disease
- Family history
152
What joints are typically affected in gout?
- MTPJ
- Wrists
- Base of thumb (CMJs)
153
How is gout diagnosed?
- Clinical + exclude septic joint
- Aspirate-> needle shaped negatively birefringent crystals (monosodium urate)
- Joint X ray-> lytic lesions, punched out erosions, sclerotic borders, overhanging edges to erosions
154
What will be found on a joint aspirate in gout?
needle shaped negatively birefringent crystals (monosodium urate)
155
How is gout managed acutely?
- NSAIDs 1st line
- Colchicine
- Steroids considered
156
How is gout managed long term?
- Allopurinol after acute attack settled
- Weight loss
- Minimise seafood + alcohol
157
What is allopurinol?
- Xanthine oxidase inhibitor
| - Used in gout (long term)
158
What is pseudogout?
- Crystal arthropathy
- Calcium pyrophosphate crystals in joints
- AKA chondrocalcinosis
159
How does pseudogout present?
- Hot, swollen, stiff, painful joint(s)
- Affects knee, shoulders, wrists, hips
- Can be chronic
160
How is pseudogout diagnosed?
- Rule out septic
- Aspiration-> no bacteria, rhomboid shaped positive birefringent crystals (calcium pyrophosphate)
- X ray shows LOSS + chondrocalcinosis-> thin white line in middle of joint space (calcium deposits)
161
What does LOSS stand for (in X-ray signs)?
- Loss of joint space
- Osteophyte formation
- Subchondral sclerosis
- Subarticular sclerosis
162
What will be found on joint aspiration in pseudogout?
rhomboid shaped positive birefringent crystals (calcium pyrophosphate)
163
What X-ray sign is pathognomonic for pseudogout?
chondrocalcinosis-> thin white line in middle of joint space (calcium deposits)
164
How is pseudogout managed?
- Nothing if chronic + asymptomatic
- May spontaneously resolve over few weeks
- NSAIDs
- Colchicine
- Joint aspiration
- Steroids
- Arthrocentesis (washout)
165
What is osteoporosis?
Reduction in bone density leading to weakness and fractures
166
What is osteopenia?
Less severe than osteoporosis-> reduction in bone density
167
What are the risk factors for developing osteoporosis?
- Older
- Female
- Reduced mobility/activity
- Low BMI
- RA
- Alcohol
- Smoking
- Long term steroids
- Other meds-> SSRIs, PPIs, anti-epileptics, anti-oestrogens
- Post-menopausal (as oestrogen protective)
- Previous fracture
- Falls
- Endocrine disorders
168
What can protect against osteoporosis?
- Oestrogen therapies-> HRT
| - Pre-menopausal
169
What is the FRAX scoring tool?
Risk of fragility fracture (major osteoporotic and hip fractures) over the next 10 years
- Age
- Sex
- BMI
- Co-morbidities-> RA, secondary osteoporosis, glucocorticoids
- History of fracture
- Smoking
- Alcohol (>3 units/day)
- Family history
- BMD
170
How is bone mineral density measured?
- DEXA scan
- Measure how much radiation absorbed by bones + indicate how dense
- Measured at hip
- T-score-> number of std devs below mean for healthy young adult
171
What is a normal BMD score?
-1 or more
172
What BMD score indicates osteopaenia?
-1 to -2.5
173
What BMD score indicates osteoporosis?
-2.5 or less
174
What BMD score + features indicate severe osteoporosis?
-2.5 or less + fracture
175
When is FRAX score measured?
- When at risk
- Women >65
- Men >75
- Younger with risk factors
176
How is osteoporosis managed?
- Activity
- Stop smoking
- Fall avoidance
- Calcium + Vitamin D
- Bisphosphonates eg alendronate
- Denosumab-> monoclonal antibody
- HRT
- Follow up-> repeat FRAX + DEXA every 3-5 years
- Can have 'treatment holiday' if no fractures for 18 months to 3 years
177
How do bisphosphonates work?
Interfere with osteoclast activity-> prevent resorption of bone (not replace lost bone)
178
What are some examples of bisphosphonates and how are they taken?
- Alendronate + risondronate-> PO + need to take on empty stomach then sit upright for 30 mins before moving or eating
- Zolendronic acid-> IV once yearly
179
What are the side effects of bisphosphonates?
- Reflux + oesophageal erosions
- Atypical fractures
- Osteonecrosis of the jaw + external auditory canal
180
What is osteomalacia?
Vitamin D deficiency leading to defective bone mineralisation and 'soft' bones
181
What is Rickets?
Vitamin D deficiency leading to defective bone mineralisation and 'soft' bones-> in kids + before growth plates close
182
What can cause Vitamin D deficiency + osteomalacia?
- Poor dietary intake
- Darker skin-> need longer sun exposure to get same level
- Malabsorption disorder (eg IBD)
- CKD as less metabolised in kidneys
183
What is the normal physiology and role of Vitamin D?
- Created from cholesterol by skin in response to UV radiation
- Metabolised in kidneys
- Needed to absorb calcium + phosphate from intestines + kidneys
- Regulates bone turnover + promotes bone reabsorption to boost serum calcium
184
What are the effects of low Vitamin D on blood components and bones?
- Low calcium and phosphate-> defective bone mineralisation
- Secondary hyperparathyroidism-> response to low calcium so tries to stimulate increased reabsorption from bones-> further mineralisation problems
185
How does osteomalacia present?
- Asymptomatic
- Fatigue
- Bone pain
- Muscle weakness + aches
- Pathological fractures
186
How is osteomalacia investigated?
- Serum 25-hydroxyvitamin D-> <25nmol/L counts as diagnostic (25-50nmol/L is insufficiency)
- Low calcium, low phosphate, high ALP, high PTH
- X-ray-> osteopenia
- DEXA-> may have low BMD
187
How is osteomalacia treated?
- Vitamin D supplements-> high dose short term (eg 4000IU daily for 10 weeks) then maintenance of 800IU+ for life
- Insufficiency-> maintenance regime only
188
What is Paget's disease of the bone?
Bone turnover excessive and unco-ordinated due to osteoclast and osteoblast activity balance
189
What is the pathophysiology of Paget's disease of the bone?
- Bone turnover excessive and unco-ordinated due to osteoclast and osteoblast activity balance
- High (sclerosis) + low (lysis) density areas in patches
- Causes enlarged, misshapen bones that easily fracture
- Particularly axial skeleton (head + spine)
190
How does Paget's disease of the bone present?
- Older adults
- Bone pain + deformity
- Fractures
- Hearing loss
191
What are the biochemical findings in Paget's disease of the bone?
- Raised ALP
- Other LFTs normal
- Normal calcium + phosphate
192
What are the X-ray findings in Paget's disease of the bone?
- Bone enlargement and deformity
- Osteoporosis circumscripta-> lytic lesions + well definted
- Cotton wool skull appearance-> sclerosis + lysis
- V-shaped defects in long bones-> osteolytic
193
How is Paget's disease of the bone managed?
- Bisphosphonates
- NSAIDs for pain
- Calcium + Vitamin D
- Surgery
- Monitoring-> symptoms + ALP
194
What are the serious complications of Paget's disease of the bone?
- Osteosarcoma
| - Spinal stenosis (canal narrowing due to deformity)
