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Phase 2a Annabel > Msk + Rheumatology > Flashcards

Msk + Rheumatology Flashcards

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1
Q

What is the MC arthritis?

A

Osteoarthritis

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2
Q

Define oseoarthritis (OA)

A

Progressive synovial joint damage ‘wear and tear’
Effectively non inflammatory degeneration

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3
Q

Who is commonly affected by OA?

A

> 55
Women

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4
Q

What are the risk factors of OA?

A

Increased age
Female
Obesity
Manual labour
Genetics

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5
Q

What gene can predispose OA?

A

COL2A1 (type 2 collagen)

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6
Q

What cartilage is most commonly affected by OA?

A

articular cartilage

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7
Q

Outline the pathophysiology of OA

A

Destruction and breakdown of cartilage -> imbalanced collagen breakdown and repair -> chondrocytes secreted -> degrade collagen -> bone attempts to replace -> abnormal bony growths

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8
Q

What are the symptoms of OA?

A

Transient morning pain worsening throughout the day
Bouchard and hebderen nodes on fingers
Asymmetrical hard joints

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9
Q

Where are Bouchard nodes located?

A

proximal interphalangeal joints (PIPJs)

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10
Q

Where are Hebderen nodes located

A

Distal interphalangeal joints (DIPJs)

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11
Q

What is the way to remember the location of Hebderen and Bouchard nodes?

A

B is before H so Bouchard’s are more proximal than Heberden’s

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12
Q

How is OA diagnosed?

A

X-ray: (LOSS) Loss of joint space, Osteophytes, subchondral sclerosis and cysts

Rheumatoid factor and Antinuclear antibodies are NEGATIVE

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13
Q

How is OA treated?

A

Weight loss and physio
NSAIDs
Steroid injections
Surgery (arthroplasty- hip or knee replacement)

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14
Q

Define rheumatoid arthritis

A

autoimmune disorder causing symmetrical polyarthritis and destruction of the synovial joints

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15
Q

What are the risk factors of RA?

A

Smoking
Women 30-50
FHx
Autoimmune disease

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16
Q

Outline the pathophysiology of RA

A

Overproduction of TNF-alpha -> synovitis and joint destruction -> leukocytes forced into synovial -> inflammation -> damages cartilage -> bone exposed

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17
Q

What are the symptoms of RA?

A

Pain worse in morning but easier as day goes on
Symmetrical joints
Extraarticular symptoms
Swollen,tender and warm
Hand deformities

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18
Q

What are 3 hand deformities in RA?

A

Ulnar deviation
Swan neck/Z thumb
Boutonniere deformity

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19
Q

What genes predispose RA?

A

HLA DR4
HLA DR1

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20
Q

How is RA differentiated from PA?

A

DIP spared in RA

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21
Q

How is RA differentiated from Spondylarthropathies?

A

RA spares lumbar spine

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22
Q

What is ulnar deviation?

A

Fingers deviate medially towards the ulna at MCP

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23
Q

What is Bouttonieres deformity?

A

PIP flexion + DIP hyperextesnion

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24
Q

What is swan neck deformity?

A

PIP hyperextension + DIP flexion

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25
What 6 other organs can be affected by RA?
Lungs Heart Eyes Brain and CNS Kidneys Skin
26
What does splenomegaly indicate in RA?
Feltys syndrome
27
What is Feltys syndrome?
Triad of RA Splenomegaly Neutropenia
28
How is RA diagnosed?
ESR and CRP raised Positive rheumatoid factor (RF) Positive anti-CCP X-ray
29
What are the signs of RA on X-ray?
LESS Lost joint space Bony Erosion Soft tissue swelling Periarticular osteopenia
30
How is RA treated?
Urgent referral to prevent deformity DMARDs (GS) NSAID analgesia Intraarticular steroid injections Biologicals
31
What is an example of a DMARD?
methotrexate
32
What are 2 biological treatments of RA?
Infliximab Rituximab
33
What type of drug is infliximab?
TNF-a inhibitor
34
What type of drug is rituximab?
CD40 B cell inhibitor
35
What are 3 differences between RA and OA?
RA pain eases with use, OA gets worse RA is hot and red RA usually presents younger and is more genetic OA affects knees RA responds to NSAIDs, OA not as much RA has faster onset
36
What are 2 crystal arthropathies?
Gout Pseudogout
37
What is the MC inflammatory arthritis in the UK?
Gout
38
Define gout
Inflammatory arthritis caused by hyperuricaemia and intraarticular monosodium urate crystals
39
Define hyperuricaemia
High levels of uric acid
40
Define gouty tophi
nodular masses of monosodium urate crystals deposited in the soft tissues of the body
41
Who is most affected by gout?
Overweight middle aged men
42
Why are women less likely to develop gout?
Oestrogen promotes uric acid excretion in kidneys
43
What are the risk factors of gout?
High alcohol intake (especially beer) Purine rich foods (red meat) diuretics High fructose intake FHx Obesity
44
Outline the pathophysiology of gout
Purines ->uric acid via xanthine oxidase -> monosodium urate crystals -> symptomatic gout and pain
45
Will everyone with hyperuricaemia develop gout?
No- only round 1/5
46
What are the symptoms of gout?
Sudden very painful onset and swelling and redness in big toe (MTP) Can also affect ankle, base of thumb Gouty tophi (chronic)
47
How is gout diagnosed?
Joint fluid aspiration and light microscopy -negatively bifringent needle shaped urate crystals - no bacterial growth Increased urate on blood test Joint X-ray: punched out erosions and lytic lesions
48
How are acute gout flare ups treated?
NSAIDs Colchicine (targets acid crystalisation) IM injections (eg Prednisolone)
49
When should gout prophylaxis be initiated?
1-2 weeks post flare up
50
What is used in gout prophylaxis?
1.Allopurinol 2. Febuxostat Reduce red meat and eat more dairy!
51
How does allopurinol work?
Inhibits xanthine oxidase = less uric acid production
52
How does febuxostat work?
Non-purine xanthine oxidase inhibitor- basically same as allopurinol but used if contraindicated?
53
What are negatively bifringent needle shaped urate crystals indicative of?
Gout
54
Define pseudogout
Deposition of calcium pyrophosphate crystals on joint surface
55
Who is mostly affected by pseudogout?
Elderly women
56
What are 3 risk factors of pseudogout?
Old age DM Osteoarthritis Hyperparathydroidism
57
What are the symptoms of pseudogout?
Hot swollen tender joint Usually knee or wrist Fever
58
What is the differential diagnosis of pseudogout?
Septic arthritis
59
How is pseudogout diagnosed?
Joint aspiration: - positively bifringent rhomboid shaped crystals (Positive = Pseudo) X-ray: chondrocalcinosis Rule out septic arthritis
60
How is pseudogout treated?
1. NSAIDs 2. Colchicine 3. Corticosteroid eg. Prednisolone Joint aspiration can be very helpful
61
What are 3 differences between gout and pseudogout?
Pseudogout patients tend to be older Pseudogout affects larger joints Swelling worse in gout Gout = uric acid deposition, pseudo = calcium pyrophosphate Gout= negatively bifringest, pseudo= positively
62
What do positively bifringent rhomboid shaped crystals indicate?
Pseudogout
63
What is deposited in gout?
Uric acid/ urate crystals
64
What is deposited in pseudogout?
Calcium pyrophosphate
65
Define osteoporosis
Bone mineral density (BMD) >2.5 standard deviations below the young adult mean value
66
Define osteopenia
Precursor to oesteoporosis BMD between 1-2.5 standard deviations below the young adult mean value
67
Define osteomalacia
Poor bone mineralisation causing soft bone due to lack of Ca2+, vitamin D and phosphate
68
What is a DEXA scan?
Dual energy X-ray absorptiometry Used to calculate T score
69
What does a T score of >-1 indicate?
Normal
70
What does a T score of -1 to -2.5 indicate?
Osteopenia
71
What does a T score of >-2.5 indicate?
Osteoporosis
72
Who is most commonly affected by osteoporosis?
Post menopausal women >50
73
What are the risk factors of osteoporosis?
SHATTERED Steroid use Hyper(para)thyroidism Alcohol and tobacco Thin Testosterone decreased Early menopause Renal or liver failure Erosive/inflammatory bone disease Dietary calcium low
74
What are the symptoms of osteoporosis?
Only fracture!
75
What are 3 mc fractures in oesteoporosis?
Proximal femur Vertebral crush fracture Colles fracture of the wrist
76
How is osteoporosis diagnosed?
DEXA scan (GS) FRAX score
77
What is FRAX score used for?
Uses data to calculate risk of fracture over 10 years
78
How is osteoporosis treated?
1. Bisphosphonates (sit for 30 mins after) 2. Vitamin D and calcium supplements -HRT in women -Denosumab - Recombinant PTH
79
What are 2 biphosophate drugs?
MC: Alendronate (once weekly) Risedronate (once weekly) Zolendronic acid (once yearly) IV
80
How do bisphosophates work?
Inhibit bone breakdown by decreasing osteoclast activity
81
How does denosumab work?
Inhibits RANK so osteoclasts can not be activated
82
What is an example of a recombinant PTH drug?
Teriparatide
83
How do recombinant PTHs work?
Increases osteoblast activity and bone formation
84
Define fibromyalgia
Widespread MSK pain for 3+ months after all other diseases have been excluded - Basically MSK version of IBS
85
Who is most commonly affected by fibromyalgia?
Usually women Any age (usually 20-50) Depression and stress
86
What are the symptoms of fibromyalgia?
Chronic widespread pain Aggravated by stress, cold and activity Fatigue and irritability Waking up in the night
87
What are 2 differential diagnoses of fibromyalgia?
PMR SLE Hypothyroidism Low vitamin D RA
88
What pain pathway is affected by fibromyalgia?
Non-nocioceptive
89
How is fibromyalgia diagnosed?
Exclude everything else (TFT, ESR+CRP, low vit D) Pain in 11/18 tender points on body on palpation
90
How is fibromyalgia treated?
Educate patient and family Physio Low dose antidepressants and anticonvulsant
91
What are the complications of fibromyalgia?
Really bad quality of life :( Opiate addiction Anxiety and depression
92
Define polymyalgia rheumatica (PMR)
Large vessel vasculitis presenting as chronic pain syndrome
93
Who is mc affected by PMR?
Women ALWAYS >50
94
What are the symptoms of PMR?
Similar to fibromyalgia Sudden onset of severe pain and stiffness of joints, hips, and lumber spine Worse in morning Fatigue, weight loss, depression
95
How is PMR diagnosed?
ESR and CRP raised Clinical history May have anaemia of chronic disease Temporal artery biopsy may show GCA
96
How is PMR treated?
Oral Prednisolone or corticosteroids help a lot -Prevent osteoporosis
97
Define Sjögren’s syndrome
Chronic t4 inflammatory autoimmune mediated destruction of epithelial exocrine glands
98
What are the 2 categories of Sjögren’s syndrome?
Primary: MC in females Secondary: secondary to connective tissue disease eg. RA, SLE
99
What are the risk factors of sjogrens?
Female 7x more likely with first degree relative >50
100
What gene is associated with primary Sjögren’s?
HLA-B8/ DR3
101
What are the symptoms of Sjögren’s?
Keratoconjunctivitis sicca (dry eyes) Xerostomia (dry mouth) Dryness of skin and vagina Some have systemic symptoms
102
What is xerostomia?
Dry mouth due to decreased saliva production
103
What is keratoconjunctivitus sicca?
Dry eyes
104
How is Sjögren’s diagnosed?
Anti-Ro and anti LA Schirmer tear test Rose bengal staining
105
What is a schirmer tear test?
Strip of test paper put in lower eyelid - wetting of <10mm in 5 mins indicates Sjögren’s
106
What is rose bengal staining?
Staining of eyes shows keratitis in sjogrens
107
What are anti-Ro and anti-La antibodies specific to?
Sjögren’s
108
How is sjogrens treated/managed?
Artificial tears and saliva replacement NSAIDs and hydrxychloroquine Vaginal lube
109
What are the complications of Sjögren’s?
Eye infections Vaginal problems Oral problems Vasculitis Neuropathy
110
What are 3 categories of vasculitis?
Large vessel Medium vessel Small vessel
111
Define vasculitis
Inflammation of vessel wall
112
What is the main treatment of vasculitis?
Corticosteroids
113
What are 2 types of large vessel vasculitis?
Giant cell arteritis/ polymyalgia rheumatica (MC) Takayasu’s arteritis
114
What are 2 types of medium vessel vasculitis?
Polyarteritis nodosa / PAN (MC) Kawasaki’s disease
115
What are 2 types of small vessel vasculitis?
Granulomatosis with polyangitis/ Wegners/GPA (MC) Eosinophilia granulomatosis with polyangitis/ churg Strauss(EGPA)
116
What do ANCAs indicate in vasculitis?
Small cell vasculitis
117
Define giant cell arteritis (GCA)
Inflammatory granulomatous arteritis of large cerebral arteries and other large vessels such as the aorta
118
Who is commonly affected by GCA?
>50 Women Northern European
119
What are the symptoms of GCA?
Unilateral temple headache Tenderness of scalp Claudication of jaw when eating Visual disturbances
120
How is GCA diagnosed?
Raised ESR Temporal artery biopsy (GS) - patchy so take a big chunk
121
What artery is mc affected by GCA?
External carotid
122
How is GCA treated?
Rapid corticosteroids (eg Prednisolone) - give GI (PPI) and bone (Ca2+) protection
123
What is the complication of GCA?
Sudden painless vision loss in one eye - can be permanent EMERGENCY
124
How is the sudden painless vision seen sometimes in GCA treated?
Methycprednisolone
125
Who is affected by Takayatsu’s arteritis?
Asian/Japanese women
126
Who is affected by Kawasaki disease?
Children
127
What does Kawasaki disease cause?
Coronary artery aneurysm
128
What are the risk factors of polyarteritis nodosa (PAN)?
Hep B Middle age Male
129
What are the symptoms of PAN?
Subcutaneous nodules Numbness and tingling, loss of function (mononeuritis vasculitis) GI bleeds Prerenal AKI and HTN Livedo reticularis Gangrene
130
How is PAN diagnosed?
CT contrast angiogram “BEADS ON A STRING” Raised ECR and CRP Biopsy
131
How is PAN treated?
Control BP Corticosteroids Treat Hep B after steroids
132
What are the symptoms of Kawasaki disease?
Strawberry tongue Bilateral conjunctivitis Erythema and desquamation
133
How are EGPA and GPA/Wegners differentiated?
EGPA = p-ANCA positive and eosinophilia GPA = c-ANCA positive
134
What 2 organs are affected by GPA/Wegners?
Respiratory tract Kidneys
135
Who is most affected by GPA/Wegners?
Late teens Early adulthood
136
What are the symptoms of GPA/Wegners?
Saddle shaped nose Epistaxis (nosebleed) Cough, wheeze and haemoptysis GLOMERULONEPHRITIS
137
How is GPA/Wegners diagnosed?
c-ANCA positive Granulomas on histology Biopsy
138
How is GPA/Wegners treated?
Nasal corticosteroids Cyclophosphamide
139
What is the main complication of GPA/Wegners?
Glomerulonephritis
140
What are 2 inflammatory markers?
ESR CRP
141
What does ESR stand for?
Erythrocyte sedimentation rate
142
What produces CRP?
Liver
143
What does CRP stand for?
C-reactive protein
144
Why is CRP produced?
In response to IL-6
145
Define Paget’s disease
Focal disorder of bone remodelling causing patchy bone
146
Outline the pathophysiology of Pagets
Excessive osteoblast and osteoclast activity -> excessive bone turnover -> formation of weaker new bone -> enlarged and deformed bones -> increased risk of fracture
147
Who is most affected by Pagets?
Increases with age Europeans and northern English people MC in UK! Females
148
What causes Pagets?
Idiopathic! May be linked to latent viral infection FHx
149
What are the symptoms of Pagets?
Bone pain Bone deformity Neurological complications (deafness, paraparesis) Osteosarcoma Fracture
150
How is Pagets diagnosed?
X-ray: cotton wool skull, osteolysis Urinary hydroxyproline
151
How is Pagets treated?
Biphosphonates NSAIDs Surgery
152
What gene is associated with spondyloarthropathies (SpA)?
HLA-B27 - MHC 1 serotype
153
What are the general features of SpA?
SPINEACHE Sausage digit (dactylitis) Psoriasis Inflammatory back pain NSAIDs good response Enthesitis (plantar fasciitis) Arthritis Crohn’s, colitis, elevates CRP HLA-B27 Eye (uveitis)
154
What are 4 SpAs?
Ankylosing spondylitis Psoriatic arthritis Reactive arthritis Enteric arthritis
155
Define ankylosing spondylitis
Chronic inflammatory disorder of the spine, ribs, and sacroiliac joints
156
Define ankylosis
Abnormal stiffening and immobility of joint due to new bone formation
157
Who is affected by ankylosing spondylitis?
Mc in males and also more severe (women under diagnosed) Presents at 16 or <30 HLA-b27 positive Native Americans suffer a lot
158
Outline the pathophysiology of ankylosing spondylitis
Sydesmophyte replaces spinal bone damaged by inflammation -> heals in weird places -> reduced mobility
159
What are the symptoms of ankylosing spondylitis?
Young male with progressively worsening back stiffness made better by exercise Question mark back Loss of lumbar lordosis Wakes up second half of night but goes back to sleep fine Anterior uveitis (inflammation of middle layer of eye)
160
How is Ankylosing spondylitis diagnosed?
ESR! And CRP raised X-ray: **bamboo spine, sydesmophytes**, fusion of sacroiliac spine MRI: shows sacrolitis before seen on X-ray Schober’s test: <20cm Genetic test
161
How is ankylosing spondylitis treated?
Lots of exercise and physiotherapy 1. NSAIDs 2. DMARDs 3. TNF-a (infliximab)
162
What are 2 TNF-alpha blockers?
Infliximab Adalimumab
163
Does psoriasis have to be present for the patient to develop psoriatic arthritis?
No
164
How many people with psoriasis develop psoriatic arthritis?
10-40% within 10 years Can present before skin changes
165
Define psoriatic arthritis
Inflammatory arthritis associated with psoriasis
166
What are the symptoms of psoriatic arthritis?
DIPJs involved Dactylitis (sausage fingers) Oncholysis (separation of nail from nail bed) + pitting Arthritis mutilans Psoriasis
167
What is arthritis mutilans?
Affects around 5% of psoriatic arthritis patients Causes bone shortening and destruction of small bones in the hands and feet Pencil in cup deformity
168
How is psoriatic arthritis diagnosed?
Exclude RA (negative anti CCP and RF) PEST screening in people with psoriasis X-ray: pencil in cup deformity in IPJ, erosion in DIPJ, periostitis
169
How is psoriatic arthritis treated?
DMARDs Anti TNFs Ustekinumab
170
Define reactive arthritis
Sterile inflammation in the synovium/joint triggered by a distant infection (usually GI or STI)
171
Are men or women MC affected by reactive arthritis?
Men
172
What are 3 causes of reactive arthritis?
GI: salmonella, shingella, c.jejuna STI: chlamydia trachomatis (MC) , gonorrhoea
173
Outline the pathophysiology of reactive arthritis
Bacterial antigens found in inflamed synovium suggesting the antigenic material drives the inflammatory response
174
What are the symptoms of reactive arthritis?
Days-weeks post infection Can’t see, cant wee, cant climb a tree - acute anterior uveitis, circinate balanitis (ulceration of penis), arthritis and enthesitis Asymmetrical lower limb arthritis
175
How is reactive arthritis diagnosed?
ESR and CRP raised Find cause Aspirated fluid is sterile and no crystallography
176
How is reactive arthritis treated?
NSAIDs and corticosteroids Treat infection with Abx Relapse = methotrexate Last line = TNF alpha blockers
177
What is the differential diagnosis of reactive arthritis?
Septic arthritis
178
What is enteric arthropathy associated with?
Crohn’s IBD Coeliac Basically any IBD before after or during
179
Define enteric arthropathy
Arthritis secondary to IBD
180
Who is affected by enteric arthropathy?
Males more 10-20% IBD patients
181
What are the symptoms of enteric arthropathy?
Asymmetric joint involvement Synovitis in peripheral joints Sacroilitis Mouth ulcers Signs of IBD
182
How is enteric arthropathy treated?
No NSAIDs! DMARDs
183
What are 2 types of infective arthritis?
Septic arthritis Osteomyelitis
184
Define septic arthritis
Infection of the joints by direct action or hematogenous spread
185
What joint is most commonly affected by septic arthritis?
Knee
186
What are the symptoms of septic arthritis?
Medical emergency! Extremely painful tender hot and swollen Fever Children may avoid using joint Usually only affects one joint
187
What is the complication of septic arthritis?
Joint can be destroyed in <24 hours
188
What is the MC cause of septic arthritis?
Staph aureus
189
What is the MC cause of septic arthritis in children?
Haemophilius influenzae Now rare due to vaccines :)
190
What are 3 other causes of septic arthritis?
Strep N. Gonorrhoea E. Coli
191
What are the risk factors of septic arthritis?
Pre existing joint disease Recent joint surgery Prosthetic joints IVDU >80 and infants DM Immunosuppression
192
How is septic arthritis diagnosed?
Urgent joint aspiration, microscopy, culture and sensitivity - thick fluid due to WCC High CRP, ESR, WCC
193
How is septic arthritis treated?
Aspirate to drain IV Abx NSAID analgesia STOP methotrexate and anti-TNF Double Prednisolone if on it long term
194
What is the prognosis of septic arthritis?
10% mortality
195
Define osteomyelitis
Inflammation/ infection in bone and bone marrow due to haematogenous spread or local infection
196
Who is usually affected by haematogenous spread osteomyelitis?
Children (long bones)
197
Who is most commonly affected by contiguous/ direct osteomyelitis?
Adults (adjacent soft tissue)
198
Why are different bones affected by osteomyelitis in adults and children?
Long bones have faster blood flow in children Vertebrae affected in adults (haematogenous) due to increased vascularisation
199
What is the MC cause of osteomyelitis?
Staphylococcus aureus
200
What are 3 ways osteomyelitis is spread haematogenously?
Catheter IV Sickle cell
201
What are 2 ways osteomyelitis is spread non-haematogenously?
Direct innoculation from trauma/ medial procedures Continuous spread from directly infected tissue
202
What are 3 risk factors of osteomyelitis?
DM PVD Decreased immunity Sickle cell Trauma
203
What are the symptoms of osteomyelitis?
Children refuse to use limb Fever, sweats Tenderness, warmth, erythema, swelling
204
How is osteomyelitis diagnosed?
BM biopsy and culture to find cause Raised ESR, CRP, WCC (acute) X-ray for chronic
205
What is the main differential diagnosis of osteomyelitis?
Charcot joint (damage due to sensory nerves affected by DM)
206
How is osteomyelitis treated?
Flucloxacillin and rifampicin >6 weeks Debridement or affected implant Immobilise
207
What should be ruled out in osteomyelitis?
Tuberculosis osteomyelitis (biopsy)
208
Define systemic lupus erythematosus (SLE)
Inflammatory autoimmune connective tissue disease
209
What type of hypersensitivity reaction is SLE?
Type 3
210
Who is commonly affected by SLE?
Females >>>>> males 20-40 Afro-carribeans and Asians
211
What are the risk factors of SLE?
Female Polymygia Rheumatica FHx HLA genes and DR2/3 Drugs EBV
212
What are 3 drugs that can cause SLE?
Isoniazid Penicillamine Hydralazine
213
Outline the pathophysiology of SLE
Impaired apoptotic debris presented to TH2 -> B cell activation -> antigen antibody complexes form -> ANA and aDsDNA autoantibodies attack
214
What are the symptoms of SLE?
Photosensitive malar butterfly rash on cheeks and bridge of nose Alopecia Ulcers Seizures and psychosis Glomerulonephritis with proteinuria Raynaud’s
215
How is SLE diagnosed?
1. ANA positive Anti-dsDNA positive (GS) Anti Smith positive ESR raised but CRP normal Prolonged PTT
216
How is SLE treated?
Suncream and avoid sun 1. NSAIDS + hydroxychloroquine (helps skin) + steroids (prednisolone) 2. Azathioprine
217
What is used for SLE patients in remission?
Hydroxychloroquine
218
Define anti phospholipid syndrome (APS)
blood more prone to clotting ‘hyper-coagulable state’ due to APL antibodies activating platelets
219
What are the 2 categories of APS?
Primary: on its own Secondary: SLE! ESP ect
220
Is APS more common in males or females?
Females
221
Outline the pathophysiology of APS
Antiphospholipid antibodies (aPL) bind to phospholipid normally and cause thrombosis
222
What are symptoms of APS?
CLOTs Coagulation defect Livedo reticularis (lace like purple discolouration on skin) Obstetric issues- ie miscarriage Thrombocytopenia (low platelets)
223
How is APS diagnosed?
Hx of thrombo events and miscarriage 1. Lupus anticoagulant (LA) + 2. Anticardiolipin test (aCL) + 3. Anti-B2-glycoprotein I + Made after 2 abnormal tests more than 12 weeks apart
224
How is APS treated?
Long term warfarin if had a thrombosis Pregnant: aspirin and SC heparin Prophylactic: aspirin
225
What is the difference between polymyositis (PM) and dermatomyositis (DM)?
PM = skeletal muscle fibres DM = skin involved
226
Define DM/PM
Rare muscle disorder of unknown aetiology where there is inflammation and necrosis of skeletal muscle/skin
227
Define myositis
Proximal muscle weakness + myalgia (muscle pain)
228
What happens when PM/DM affects the lungs?
Interstitial lung disease
229
What are the symptoms of PM?
Symmetrical progressive muscle weakness and wasting affecting the proximal muscles of the shoulder and pelvis Can’t raise arms above head Can’t rise from chair Dysphagia, dysphonia, respiratory failure
230
What are the symptoms of DM?
Heliotrope (purple) discolouration of eyelids Gottrons papules Raynauds Dysphagia SC calcitonis Shawl sign
231
What are gottrons papules?
Scaly erythematous plaques over knuckles
232
What are 3 risk factors of PM/DM?
Coxsackie virus Rubella Influenza HLA-B8/DR3
233
How is DM/PM diagnosed?
Rise in creatine kinase EMG Muscle biopsy shows necrosis (GS)
234
What autoantibodies are specific to PM?
Anti-Jo-2 antibodies
235
What 2 autoantibodies are specific to DM?
anti-nuclear antibodies Anti-Mi-2 antibodies
236
How is PM/DM treated?
1. Oral Prednisolone Bed rest with exercise programme Screen for malignancy Early intervention with immunosuppressant (azathioprine)
237
Define scleroderma / systemic sclerosis
Fibrosis of small vessels, and thickening and tightening of skin due to excess collagen production
238
Who is scleroderma most common in?
Females 30-50 Rare in children
239
What are 2 types of scleroderma?
Limited: only CREST Diffuse: CREST and affects internal organs
240
What are 3 causes of scleroderma?
Vinyl chloride Silica dust Bleomycin Genes
241
What are the symptoms of scleroderma/systemic sclerosis?
CREST Calcinosis (calcium deposition in SC) Raynauds Eosophageal strictures or dysmotility Sclerodactyly (thickening of skin on fingers and toes) Telenagiectasia (spider veins) + scleroderma
242
What are some diffuse symptoms of scleroderma?
CKD Atony of oesophagus, SI, colon (obstructed) Lung fibrosis Myocardial fibrosis
243
How is scleroderma diagnosed?
Limited: Anti centromere bodies (ACA) Diffuse: anti-sci-70, anti-RNA polymerase ANA positive in almost all Nailfold capilaroscopy
244
How is scleroderma treated?
Physio and gentle stretching Symptomatic management: PPI, ACE-inhibitor, CCB, hand warmers for Raynauds
245
What are red flags for back pain?
TUNA FISH Trauma- osteoporosis Unexplained weight loss- cancer Neurological symptoms- cauda equina Age >50 or <20- Ankylosing spondylitis, herniated disc Fever- infection IVDU- infection Steroid use- infection History of cancer- metastasis to spine
246
Define lumbar spondylitis
Degradation of IV disc over time
247
Where does lumbar spondylitis most commonly affect?
L4-L5 L5/S
248
What is Ricket’s?
Defective mineralisation before fusion of the epiphyses
249
What is dietary vitamin D found in?
Oily fish Egg yolks Margarine
250
What are the causes of osteomalacia?
Hyperparathydroidism (hypophosphatemia) Vitamin D deficiency Renal disease Drugs Liver disease
251
What are the symptoms of oestomalacia?
Fatigue and bone pain Fractures Muscle weakness (waddling gait)
252
What are the symptoms of Rickets?
In kids only! Growth retardation and hypotonia Knock knees and bowed legs Widened epiphyses at wrist Hypocalcaemic tetany (muscle spasms) if severe
253
How is osteomalacia diagnosed?
GS: biopsy shows incomplete mineralisation X-ray shows loss of cortical bone and incomplete mineralisation and Loosers zone Low Ca2+ and phosphate Low 25-hydroxy vitamin D*
254
How is osteomalacia treated?
Calcitriol (vit D replacement) Increase dietary intake
255
What are 4 primary bone tumours?
Osteosarcoma Firbosarcoma Chondrosarcoma Ewings tumour
256
What are 5 locations of cancers that metastasise to bone?
KP BLT Kidney Prostate Breast Lungs (bronchus) Thyroid
257
Who do primary bone tumours commonly affect?
Young people- rare
258
What are the symptoms of primary bone tumours?
Pain worst at night Bony masses Inflammatory symptoms Motility issues
259
How are primary bone tumours diagnosed?
1. X-ray GS: bone biopsy Skeletal isotope scan CT Hypercalcaemia
260
How are primary bone tumours treated?
Chemo and radiotherapy Biphosphonates Analgesia
261
How are bone tumours staged?
Enneking staging G0= benign G1= low grade malignant G2= high grade malignant
262
Where does Ewing’s sarcoma arise from?
Neuroectoderm blue cells
263
Who is most commonly affected by Ewing’s sarcoma?
Usually 15 <30
264
Where does Ewing’s sarcoma most commonly affect?
Femur (MC) Pelvis Distal tibia
265
What are the findings of Ewing’s sarcoma on X-ray?
Onion skin appearance
266
What is the MC primary bone tumour?
Osteosarcoma
267
What disease is osteosarcoma associated with?
Pagets
268
What is the peak onset age of osteosarcoma?
15-19
269
Where do osteosarcomas most commonly occur?
Knee (MC) Proximal humerus
270
Where does osteosarcoma usually metastase to?
Lungs
271
What are the signs of osteosarcoma on X-ray?
Sunburst appearance Codmans triangle
272
Define chondrosarcoma
Cancer of the cartilage
273
What age group is mc affected by chondrosarcoma?
Middle aged
274
What are the appearances of chondrosarcoma on X-ray?
Popcorn calcification Moth eaten in high grade
275
What are 2 connective tissue disorders?
Marfans syndrome Ehlers-danlos
276
What gene is mutated in Marfans syndrome?
Autodom fibrillin 1 mutation
277
Outline the pathophysiology of Marfans syndrome
Gene involved in fibrilin (in connective tissue) -> abnormal connective tissue -> decreased tensile strength
278
What are the symptoms of Marfans syndrome?
Tall Long neck, limbs, and fingers Hyper mobility Pectus carinatum/excavatum (protruding/indented chest) Aortic complications (AAA, regurgitation, dissection) High arch palate Eye issues
279
How is Marfans diagnosed?
Physical exam Ghent criteria Genetic test
280
What are the complications of Marfans?
AAA Lens dislocation Pneumothorax GORD Scoliosis
281
How is Marfans managed?
BBs Physio to strengthen joints Yearly echo
282
What is the MC cause of death in Marfans?
CVD complications (aortic rupture)
283
What causes Ehlers-Danlos syndrome (EDS)?
Autodom mutations affecting type III collagen
284
How many types of EDS are there?
Around 13 MC = hyper mobile
285
What are the symptoms of EDS?
Joint hyper mobility Easily stretched skin (hyper extensibility) Chronic joint pain Reoccurring dislocation CVD complications
286
How is EDS diagnosed?
Beighton score to assess hypermobility (max 9) Exclude Marfans
287
How is EDS managed?
Fitness Physio Occupational therapy Psychological support
288
What is Raynaud’s phenomenon associated with?
Systemic sclerosis! SLE RA DM/PM
289
Define Raynaud’s
Intermittent spasm of arteries in the fingers and toes caused by cold and relieved by heat
290
What is the difference between Raynaud’s disease and Raynaud’s phenomenon?
No underlying cause = disease Underlying cause = phenomenon
291
What are the symptoms of Raynauds?
Skin pallor -> cyanosis -> redness Numbness and burning as fingers warm up
292
How is Raynauds treated conservatively?
Avoid cold Hand warmers Wear gloves and warm clothes Stop smoking Stop beta blockers
293
How is Raynaud’s treated pharmacologically?
CCB- nifedipine Vasodilators
294
What can cause a false positive for APS?
Syphilis
295
What are 2 pieces of advice given to people taking bisphosphonates?
Doses should be taken while sitting or standing. On an empty stomach / at least 30 minutes before breakfast Patient should stand or sit upright for at least 30 minutes after administration.
296
What chromosome is HLA B27 found on?
6

Phase 2a Annabel (12 decks)

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