Musculoskeletal Flashcards
(141 cards)
1
Q
Osteoarthritis definition
A
Non-inflammatory degenerative arthritis. The result of mechanical and biological events that destabilise the normal process of degradation and synthesis of articular cartilage chondrocytes, extracellular matrix and subchondral bone. It involved the entire joint, including the articular cartilage, subchondral bone, pericapsular muscles, capsule and synovial, The condition leads to loss of cartilage, sclerosis and eburnation (degeneration of bone) of the sunchondral bone, osteophytes, subchondral cysts. It is clinically characterised by joint pain, stiffness and functional limitation.
2
Q
Osteoarthritis epidemiology
A
Ver common condition
3
Q
Osteoarthritis aetiology
A
No single cause. High bone mineral density and low oestrogen, such as in post-menopausal women.
Primary (idiopathic): no preceding injury to the joint, further categorised into localised OA, mostly affecting the hands, hip or foot or generalised OA, usually affecting the hands and another joint.
Secondary: a previous insult to the joint such as a congenital abnormality, trauma, inflammatory arthropathies and ongoing strenuous physical activities.
4
Q
Osteoarthritis risk factors
A
Age, female sex, obesity, genetic factors
5
Q
Osteoarthritis signs and symptoms
A
Pain: usually associated, pain at rest is unusual except in advanced OA
Functional difficulties: for example a knee giving way to locking. Can reflect internal derangement such as partial meniscus tear or a loose body within the joint
Knee, hip, hand or spine involvement: commonly involved joints are the knee, hip, hands and lumbar and cervical spine.
Hand OA spares the metacarpophalangeal (CP) joints and the proximal interphalangeal (PIP) and distal interphalangeal joints, which helps distinguish it from rheumatoid arthritis.
Bones deformities: particularly in the hands and leads to enlargement of proximal interphalangeal (PIP) joints (Bouchard’s nodes) and distal interphalangeal (DIP) joints (Heberden’s nodes)
Limited range of motion
Malalignment
6
Q
Osteoarthritis 1st line investigations
A
X-ray of affected joints: LOSS
- loss of joint space
- osteophytes
- subarticular sclerosis
- subchondral cysts
Serum CRP and ESR: normal, should be ordered if inflammatory arthritis suspected
Rheumatoid factor (RF): if RA suspected
7
Q
Osteoarthritis management
A
‘Analgesic ladder’
Knee replacement
8
Q
Rheumatoid arthritis definition
A
Rheumatoid arthritis is a chronic inflammatory condition affecting around 1% of the population. It primarily affects the small joints of the hands and feet and can cause major work loss, decreased quality of life, need for joint replacement surgery and mortality
9
Q
Rheumatoid arthritis aetiology
A
Unknown. DRw4 antigen is more common in RA patients. Infection may be a triggering factor in genetically susceptible individuals.
10
Q
Rheumatoid arthritis risk factors
A
50-55yrs, female sex
11
Q
Rheumatoid arthritis pathophysiology
A
Inflamed synovial showing increased angiogenesis, cellular hyperplasia, influx of inflammatory cells, changes in adhesion molecules and cytokines. Cytokines such as TNF, IL-1 and IL-6 are abundant in the joints.
12
Q
Rheumatoid arthritis signs and symptoms
A
Active symmetrical arthritis lasting > 6 weeks
Joint pain and swelling: commonly bilateral MCP, PIP and MTP joints are involved. Painful to touch and when range of motion exercises are performed.
Morning stiffness (may be present with OA but lasts longer in RA)
Less common but specific:
- ulcer deviation
- boutonniere deformity
- swan neck/Z-thumb
- extra-articular involvement - eg lung (pulmonary nodules), eyes (scleritis) and cord compression
Important to note onset can be at any time and progress quickly compared to OA. There will often by systemic symptoms unlike in OA
13
Q
Rheumatoid arthritis 1st line investigations
A
Bloods: CRP and/or ESR raised
Rheumatoid factor: positive in about 60-70% of patients
Anti-CCP antibody: positive in about 70% of patients. Helpful in RF-negative patients because it may be positive in these patients
X-ray: LESS
- L-loss of joint space
- E-erosion (periarticular)
- S-soft tissue swelling
- S- soft bones (osteopenia)
14
Q
Rheumatoid arthritis management
A
Joint stiffness/pain - NSAIDs and aspirin
Analgesic ladder
Suppress disease - corticosteroid
DMARDS - methotrexate
15
Q
Rheumatoid arthritis complications
A
Extra-articular involvement - lung (pulmonary nodules), eye (scleritis) and cord compression
16
Q
Crystal arthropathy - gout definition
A
A syndrome characterised by hyperuricemia and deposition of rate crystals causing attacks of acute inflammatory arthritis; top around the joints and possible joint destruction, renal glomerular, tubular and interstitial disease and uric acid urolithiasis
17
Q
Crystal arthropathy - gout aetiology
A
Under excretion of uric acid
- diabetes - nephropathy
- chronic kidney disease
- drugs - aspirin (decreases renal excretion) and diuretic eg thiazides
- dehydration
Overproduction of uric acid
- high purine diet - alcohol, purine rich food (red meat and shellfish), fructose sweetened drinks
- increased cell turnover - leukaemia, lymphoma, psoriasis
18
Q
Crystal arthropathy - gout risk factors
A
More common in middle aged males
19
Q
Crystal arthropathy - gout pathophysiology
A
Purines from diet
Purines -> hypoxanthine -> xanthine
Xanthine -> uric acid (catalysed xanthine oxidase) and excreted in kidneys
Process not efficient so excess uric acid can be converted to monosodium crystals
20
Q
Crystal arthropathy - gout key presentations
A
Rapid onset of severe pain
Obese man with a toe pain who had an alcohol and shellfish
21
Q
Crystal arthropathy - gout investigations
A
Joint aspiration - rule out septic arthritis
Polarised light microscopy
- negatively birefringent
- needle-shaped monosodium crystals
Bloods - raised WBC, ESR and urate
X-ray
22
Q
Crystal arthropathy - gout management
A
Weight loss, less alcohol, hydration and dairy products - protective
NSAIDs - naproxen, ibuprofen or colchicine if contraindicated
Prophylaxis - allopurinol - xanthine oxidase inhibitor
23
Q
Crystal arthropathies - pseudogout definition
A
Calcium pyrophosphate deposition (pseudo gout) is associated with both acute and chronic arthritis. Acute CPP crystal arthritis occurs in one more joints.
Chronic CPP arthritis mimics OA or RA and is associated with variable degrees of inflammation
24
Q
Crystal arthropathies - pseudogout aetiology
A
Possible causes:
- direct trauma to the joint
- intercurrent illness
- hypothyroidism
- hyperparathyroidism - more calcium production
- surgery - especially parathyroidectomies
- hypercalcaemia
- blood transfusions - excess iron
25
Crystal arthropathies - pseudogout risk factors
Predominantly a disease of the elderly (70+), typically older females
26
Crystal arthropathies - pseudogout 1st line investigations
Joint aspiration - rule out sceptic arthritis
Polarised light microscopy
- positively birefringent
- rhomboid-shaped calcium pyrophosphate crystals
Xray: evidence of chonedrocalcinosis
Bloods: raised WBCs and calcium
27
Crystal arthropathies - pseudogout management
NSAIDs, colchicine
| No current medical prophylaxis
28
Osteoporosis definition
A systemic skeletal disease characterised by low bone mass and micro architectural deterioration of bone tissue, with consequent increase in bone fragility and susceptibility to fracture
29
Osteoporosis aetiology
Primary - menopause and age - oestrogen protects the bone
Secondary - disease or drugs which increase bone turnover - SHATTERED
- S-steroids (prednisolone)
- H-hyperthryroidism/hyperparathyroidism
- A-alcohol/smoking
- T-thin (low BMI)
- T-testosterone low
- E-early menopause
- R-renal or liver failure
- E-erosive/inflammatory bone disease eg RA, myeloma
- D-dietary calcium low, malabsorption
30
Osteoporosis risk factors
Age, female sex, low oestrogen/testosterone (hypogonadism, anorexia, menopause)
31
Osteoporosis pathophysiology
Mismatch of bone remodelling - osteoclastic bone resorption not compensated by osteoblastic bone formation.
Bone remodelling is regulated by various cytokines.
32
Osteoporosis key presentations
Asymptomatic until fracture
- hip - neck of femur after fall onto side or back
- wrist - distal radius = Colle's/Smith's fractures after fall on outstretched arm
- Vertebra - shorter and stooping posture - sudden onset of severe spine pain radiating to front
33
Osteoporosis investigations
Gold standard: DEXA Bone Mineral Density (BMD) scan - T score. Based off mean BMD of a 30 year old
- more than 1.0 below T score = normal
- between 1-2.5 SD below T score = osteopenia
- less than -2.5 SDs = osteoporosis
- less than -2.5SDs and fracture = severe osteoporosis
X-ray
FRAX - risk score to predict fracture risk
Raised alkaline phosphatase due to bone breakdown
34
Osteoporosis 1st line management
Oral bisphosphates eg alendronate (careful of oesophageal risks)
Lifestyle; smoking cessation, alcohol abstinence, regular weight-breaking exercise
Vitamin D supplements
Calcium rich diet
Give HRT - oestrogen replacement)
Anabolic
35
Osteoporosis 2nd line management
Denosumab - monoclonal antibody against RANKL to prevent osteoclast production
36
Osteoporosis 3rd line management
Daily submit Teriparatide injection (PTH receptor agonist) - chronically elevated PTH decreases bone density by stimulating osteoclast activity, however intermittent exposure to PTH (or teriparatide) activated osteoblasts more than osteoclasts, therefore increasing bone density
37
Ankylosing spondylitis definition
Seronegative spondyloarthropathies are a group of autoimmune conditions with similar features that includes: ankylosing spondylitis psoriatic arthritis, reactive arthritis. They are all associated with the following features:
- axial inflammation - spine and sacroiliac joints
- asymmetrical peripheral arthritis
- absence of RF
- strong association with HLA-B27
Chronic progressive inflammatory condition in which the spine and other areas of the body become inflamed.
38
Ankylosing spondylitis epidemiology
Men more than women
| Late adolescence/early adulthood
39
Ankylosing spondylitis aetiology
No known specific cause, though genetic factors seem to be involved.
In particular, people who have a gene called HLA-B27
40
Ankylosing spondylitis risk factors
- endoplasmic reticulum aminopeptidase (ERAP1) and interleukin 23 receptor (IL23R) genes
- positive family hisotry of AS
- Klebsiella pneumoniae
41
Ankylosing spondylitis clinical manifestations
Generic for Seronegative Spondyloarthropathies: (SPINE ACHE)
- S-sausage digits (dactylitis) -inflammation of tendon sheaths and joints in fingers
- P-psoriasis
- I-inflammatory back pain
- N-NSAIDs good response
- E-Enthesitis (inflammation of the entheses, where tendons or ligaments inset into the bone, in these conditions it normally occurs in the heels)
- A-arthritis
- C-Crohn's/UC/elevated CRP
- H-HLA-B27
- E-eye (uveitis)
42
Ankylosing spondylitis signs and symptoms
Symptoms: weight loss, fever, fatigue, buttock/thigh pain - sacroiliac joints, neck or back pai/stiffness - cervical/thoracic region can cause SOB
Signs: SPINE ACHE, slow onset of monitoring stiffness, severe kyphosis of thoracic and cervical spine
43
Ankylosing spondylitis investigations
Bloods: HLA-B27, raised ESR/CRP
X-ray: sacroilitis, syndesmophytes fusing (bamboo spine, seen below)
MRI: more sensitive
Diagnosis is made in patients with over 3 months of back pain and age of onset below 45 with sacroilitis as well as at least one SPINE ACHE feature
44
Ankylosing spondylitis management
Non-pharmacological management
- exercised - reduce pain and stiffness
- physiotherapy
Pharmacological management
- NSAIDs
- DMARDs - methotrexate
- Anti TNFs eg etanercept
45
Ankylosing spondylitis complications
New bone forms (body's attempt to heal) and bridge gap between vertebrae/eventually fuses sections of vertebrae
Eye inflammation (uveitis)
Compression fractures
Heart problems
46
Psoriatic arthritis definition
Immune system attacks healthy cells and tissue
| Abnormal immune response causes inflammation in joints and over production of skin cells
47
Psoriatic arthritis aetiology
Psoriasis
48
Psoriatic arthritis risk factors
- FHx if psoriasis or psoriatic arthriits
- Hx of joint or tendon trauma
- HIV infection
49
Psoriatic arthritis pathophysiology
T cell mediated attack of joints in people with psoriasis
50
Psoriatic arthritis clinical manifestations
Symptoms:
- joint pain and stiffness
- peripheral arthritis
- pain at site of tendon attachment
- spinal stiffness
- reduction of cervical spine mobility
Signs:
- dactylitis
51
Psoriatic arthritis 1st line investigations
Plain film x-rays of the hands and feet
- erosion distal interphalangeal (DIP) joint and periarticular new-bone formation
ESR and C-reactive protein - normal/elevated
Rhematoid factor negative
Anti cyclic citrullinated peptide antibody
- negative
52
Psoriatic arthritis other investigations
Plain film x-ray of the spine and pelvis
| MRI scan of sacroiliac joints
53
Psoriatic arthritis differential diagnosis
Polyarticular PA distinguished from R. arthritis by:
Presence of:
- catylitis - a fusiform swelling of an entire digit, and sacroilitis are manifestations not observed in RA
Absence of anticlyclic citrullinated peptide antibodies
- frequent oligoarticular or monoarticular initial pattern of join involvement
- distal interphalangeal joint (DIP) involvement
54
Psoriatic arthritis management
NSAIDS/physiotherapy
Crticosteroids
Early intervention with disease modifying antirheumatic drugs (DMARDs)
- methotrexate, leflunomide, sulfasalazine
Anti-TNF drugs
IL 12/23 blockers
IL 17 blockers
55
Psoriatic arthritis complications
- diabetes
- eye health issues
- cardiovascular problems
- depression
- lung health problems
- stomach and digestive issues
- liver and kidney issues
56
Reactive arthritis definition
Joint pain.swelling triggered by an infection in another part of the body - most often your intestines, genitals or urinary tract
57
Reactive arthritis epidemiology
Young adults around 20-40 age group,
men more than women (3:1)
Risk 50 times greater in HLA B27 positive people
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Reactive arthritis aetiology
GI: campylobacter, salmonella, shigella
GU: chalmydia, gonorrhoea
59
Reactive arthritis pathophysiology
Joint inflammation 2-6 weeks following a bacterial GI/GU infection
60
Reactive arthritis clinical manifestations
Reiter's triad: conjunctivitis/uveitis, urethritis, olioarthritis/polyarthritis
Psoriatic-like skin lesions: keratoderma blennorrgagica (rash on feet), circinate balanitis (skin inflammation around the penis)
SPINE ACHE features
61
Reactive arthritis 1st order investigations
- ESR/CRP: elevated
- antinuclear antibody (ANA): negative
- rheumatoid factor: negative
- urogenital/stool cultures: negative (unless just after infection)
- arthrocentesis with synovial fluid analysis (for DDx vs gout or pseudogout)
- x-ray (looking for sacroilitis)
62
Reactive arthritis management
Treat causative agent with ABx
NSAIDs
Corticosteroid joint injections (eg prednisolone)
If chronic: DMARDs (eg sulfasalazine)
63
Reactive arthritis key presentations
Hot, swollen, painful, restricted joint - usually only in one joint, most common site is the knee
Fever
Unusually severe symptoms for underlying disease
64
Reactive arthritis 1st line investigations
Aspiration of synovial fluid: microscopy with gram staining, cultures, WCC
Blood cultures
Calprotein (emerging but not widely available test): raised
65
Reactive arthritis differential diagnosis
Calprotein forms DDx for septic vs aseptic arthritis
66
Reactive arthritis management
Standard sepsis treatment (IV Abx, fluids, ect)
Aspiration of the joint
NSAIDS
67
Systemic lupus erythematosus (SLE) definition
A chrnoic multisystem disorder that most commonly affects women during their reproductive years
68
Systemic lupus erythematosus aetiology
Aetiology is not fully known (common for autoimmune disorders) SLE is common in those with complement deficiency
69
Systemic lupus erythematosus risk factors
Strong female preponderance
Age 15-45 years
African/Asian descent in Europe and US
70
Systemic lupus erythematosus pathophysiology
An antigen-driven immune-mediated disease characterised by high affinity IgG antibodies.
T-cell dysregulation of B cells may arise, resulting in autoimmunity. This is why monoclonal antibodies may be useful in treatment
71
Systemic lupus erythematosus key presentations
Malar (butterfly rash
Photosensitive rash (rash occuring after sun exposure)
Discoid rash
72
Systemic lupus erythematosus signs and symptoms
Signs: oral ulcers, alopecia, Raynaud's phenomenon (colour changed of the digits induced by cold or emotion), HTN, signs of nephrosis such as oedema, peripheral lymphadenopathy
Symptoms: fatigue, weight loss, fibromyalgia, chest pain and SOB, abdo pain
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Systemic lupus erythematosus 1st line investigations
FBC: anaemia, leukopenia (normally lymphopenia), thrombocytopenia and rarely pancytopenia
U&Es: elevated urea and creatinine
ERS and CRP: may be raised
Antibodies: ANA antibodies almost always present but not specific. Anti-dsDNA and anti-Smith antbodies are highly specific for SLE
Urinalysis: (assess renal involvement) haematuria, or proteinuria
74
Systemic lupus erythematosus management
1st line:
- hydroxychloroquine
- NSAIDS and corticosteroids (prednisolone)
- methotrexate and folinic acid
Lupus nephritis
- add immunosuppression such as cyclophosphamide
75
Systemic lupus erythematosus complications
Anaemia, leukopenia, thrombocytopenia and corticosteroid related complications
76
Osteomyelitis definition
Inflammatory condition of bone caused by an infecting organism, either by local infection or via blood
77
Osteomyelitis epidemiology
Acute occurs predominantly in children whereas chronic is more common in elderly
78
Osteomyelitis aetiology
Infecting organism, most commonly Staphylococcus aureus followed by Staphylococcus Epidermis
Salmonella as a complication of sickle cell
79
Osteomyelitis risk factors
- previous osteomyelitis
- penetrating injury
- IV drug misuse
- diabetes
- HIV infection
- recent surgery
- distant or local infection
- sickle cell disease
- rheumatoid arthritis
- chronic kidney injury
- immunocompromising condition
80
Osteomyelitis pathophysiology
Severity staged depending on aetiology of infection,
- pathogenesis
- extent of bone involvement
- duration
- host factors particular to individual patient
Broadly, bone infection is either
- haematogenous
- contiguous-focus
Necrotic bone sequestra may be present in chronic disease
81
Osteomyelitis clinical manifestations (acute)
Pyrexia
Swelling and extreme tenderness over site
- erythema and warmth
Severe pain, exacerbated by movement
Effusion into neighbouring joints, can result in septic arthritis
82
Osteomyelitis clinical manifestations (chronic)
Erythema, tenderness and swelling over site
Non-healing ulcer
Draining sinus tracts (ie tunnelling wound)
Neighbouring joints have decreased ROM
83
Osteomyelitis investigations
Imaging
- X-ray
- MRI may show marrow oedema
Bloods
- raised WCC
- raised CRP and ESRs
- blood cultures
Gold standard: bone biopsy and cultures
84
Osteomyelitis management
Immobilisation of the bone to aid healing
Antimicrobial therapy
- IV flucloxacillin is common treatment
Surgical debridement and removal of sequestra (dead bone)
85
Primary and secondary bone tumours definition
Primary bone tumours: osteosarcomas, fibromas, sarcomas
Secondary: most common places to metastasise to bone: lung, breats, prostate, thyroid, kidney
86
Primary and secondary bone tumours clinica maifestations
Pain at tumour location associated with localised bone pain. Malaise, pyrexia, aches, pains and hypercalcemia
87
Primary and secondary bone tumours 1st line investigations
Skeletal isotope scan
88
Primary and secondary bone tumours management
Analagesics and antiinflammatory drugs
Radiotherapy, chemotherapy, hormonal therapy
Bisphosphates
89
Fibromyalgia definition
A chronic pain syndrome disgnosied by the presence of widespread body pain
90
Fibromyalgia aetiology
Aetiology and pathophysiology are unknown. Associated with abnormalities in the stress response and triggering events. More common in females.
91
Fibromyalgia diagnosis
Clinical diagnosis: presence of >3 months of widespread pain and associated symptoms
92
Fibromyalgia management
Non-pharmacological: exercise, relaxation therapies, CBT
Pharmacological: analgesia (paracetamol, weak opioids), antidepressants (amitriptyline or duloxetine)
93
Osteomalacia definition
Poor bone mineralisation due to a lack of Ca++ but the amount/density is normal.
Rickets is osteomalacia but before the fusion of the epiphyseal plates (ie growth plates)
94
Osteomalacia aetiology
Vitamin D deficiency is the main cause (poor diet, lack of sun)
Renal/liver disease
Drug-induced such as anticonvulsants
Tumour induced
95
Osteomalacia presentation
Muscle weakness and pain: characteristic waddling gait, can't get out of chair
Bone pain: widespread, dull ache, worse on weight bearing
Fractures
Imparied growth
Bowed legs
Hypocalcemic tetany in severe cases
96
Osteomalacia diagnosis
Bloods:
- U&Es: low Ca++, high phosphate
- PTH - low
- alkaline phosphatase (usually raised)
- 25-hydroxyvitamin D - low
Gold standard: biopsy showing incomplete mineralisation
X ray: defective mineralisation
97
Osteomalacia management
Vitamin D replacement
- poor diet - calcium D3 Forte
- malabsorption - calcitriol
- renal - calcitriol
98
Paget's disease of bone definition
A disorder in which there is a focal disruption of bone remodelling. There is increased osteoclastic bone resorption followed by formation of weaker new bone. This bone is disorganised making it mechanically weaker as well as being less compact and more vascular. This process predisposes patients to deformity and fractures.
99
Paget's disease of bone aetiology
Unknown
100
Paget's disease of bone presentation
Most are asymptomatic and discovered on x-ray incidentally
Bone pain
Deformity - bowing of tibias, kyphosis
Fractures
Can affect a single bone (monostotic) or multiple bones (polyostotic)
Deafness/tinnitus due to compression of CNVIII due to deformity
101
Paget's disease of bone diagnosis
1st line: x-ray - localised bony distorrtion and enlargement, sclerotic changes (increased density), osteolytic areas (reduced density)
Bloods
- high alkaline phosphatase (increase bone turnover)
- normal Ca++, phosphate and PTH
Gold standard: isotope bone scan, unable to distinguish between Paget's and secondary metastatic bone carcinoma so will need biopsy
102
Antiphospholipid syndrome definition
Antiphospholipid antibodies (aPL) to phospholipid-binding proteins leads to a hyper coagulable state and an increased risk of venous and arterial thrombosis
103
Antiphospholipid syndrome aetiology
Aetiology is unclear. More common in white female young adults. Associated with SLE (30-40% with SLE)
104
Antiphospholipid syndrome presentation
```
Recurrent thrombosis (DVT, PE, storke, MI)
Pregnancy complications (recurrent miscarriage, stillbirth, preeclampsia)
Arthralgia, lived reticularis
```
105
Antiphospholipid syndrome diagnosis
Presence of aPL, thrombocytopenia, history of thrombosis
106
Antiphospholipid syndrome management
LMWH and warfarin
107
Sjogren syndrome definition
Chronic systemic auto-immune disorder characterised by diminished lacrimal and salivary gland secretion
108
Sjogren syndrome aetiology
Lymphocytic infiltration into lacrimal and salivary glands leading to dry eyes and mouth
Secondary Sjogren's: secondary to SLE, systemic sclerosis, RA (60% of Sjogren's diagnosed)
109
Sjogren syndrome presentation
Fatigue, dry eyes, dry mouth
110
Sjogren syndrome diagnosis
Schirmer's test: filter paper in lower conjunctival sac
| Autoantibodies: anti-Ro and anti-La
111
Sjogren syndrome management
Artificial tears, salivary substitutes
112
Marfan syndrome definition
An inherited connective tissue disorder
113
Marfan syndrome aetiology
75% of cases are inherited (autosomal domiant), 25% spontaneous mutation
Mutations in fibrillin-1 genes, production of abnormal fibrillin protein, connective tissue abnormalities
Affects both men and women equally
114
Marfan syndrome presentation
Tall stature, wide arm span, arachnodactyly (long and slender fingers), high arched palate, positive thumb and wrist sign, pectus excavatum/carinatum, hypermobility
Subluxed eye lens, aortic/mitral valve murmur
115
Marfan syndrome diagnosis
Echocardiogram
CT thorax
Slit - lamp eye exam
USS, CT, MRI abdomen
116
Marfan syndrome management
BBs to reduce MAP and pulse rate
| Surgical interventions: aortic repair, orthopaedic bracing, correction of ectus excavatum/carinatum
117
Vasculitis definition
Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow to end organs
118
Vasculitis aetiology
This may be tirggered by an infection or a medicine, although often cause is unknown
119
Vasculitis pathophysiology
Combination of:
- vessel wall destruction > perforation and haemorrhage into tissues
- endothelial damage predisposing patient to thrombosis and subsequent ischaemia/infarction on dependent tissues
Classification done by:
- size of vessel affected (small, medium, large)
- target organs
- presence/absence of anti-neutrophil cytoplasmic antibodies (ANCA)
It can also be classified into
- primary, spontaneous development on its own
- secondary - to another disease process (eg cancer, infection, other autoimmune conditions)
120
Vasculitis clinical manifestations
```
Constiutional symptoms:
- fever, athralgia
- arthritis
- rash
- weight loss
- headache
- foot drop
Major event such as:
- stroke
- bowel infarction
```
Large vessel vasculitis associated with
- vision loss
- upper extremity claudication
- asymmetric brachial pulses
- bruits
Medium vessel
- abdo pain
- foot drop
- cutaneous ulcers
Small vessel
- haematuria
- palpable purpura
- otorrhoea, ear pain
- nasal symptoms
- sinus pain
- wheeze
121
Vasculitis investigations
ESR = >100 mm/hour
CRP = elevated
ANCA = positive
- antibodies present in patients with small/medium (not large) vessel vasculitis
- directed against specific antigens in cytoplasmic granules of neutrophils and monocyte lysosomes
Srum urea and creatinine = norm/elevated
Urinalysis = haematuria, proteinuria, red blood cell casts
Biopsy
122
Vasculitis management
Medication
- corticosteroid drug eg prednisone
- medication used depends on type of vasculitis
- methotrexate, azathioprine, mycophenolate, cyclophosphamide, tocilizumab or rituximab
Surgery
123
Avascular necrosis definition
Death of bone tissue due t an interruption of the blood supply
124
Avascular necrosis aetiology
The neck of femur is the classic location as the artery supplying the femoral head obliterates by adulthood meaning if the neck is fractured the blood supply is cut off
125
Avascular necrosis resentation
Usually there is pain and joint discomfort that progresses over time, however often it is associated with traumatic fractures to certain long bone
126
Avascular necrosis diagnosis
Often done by x-ray but MRIs can confirm but not usually needed
127
Avascular necrosis management
Almost always surgical and usually requires a replacement of the joint
128
Myositis - polymyositis and dermatomyositis definition
Chronic inflammation of muscles (polymyositis) and skin (dematomyositis)
129
Myositis presentation
Slow onset proximal muscle weakness, fatigue, weight loss
| Dermatomyositis only: gottron's papules, photosensitive erythematous rash, periorbital oedema
130
Myositis diagnosis
Raised creatinine kinase: enzyme found in muscle cells
Muscle biopsy, electromyogram (EMG)
Antibodies: anti-Jo-1 (both), anti-Mi-2, ANA (dermatomyositis only)
131
Myositis management
Steroids (prednisolone)
Sun protection
IV immunoglobulin in severe disease
132
Systemic sclerosis (scleroderma) definition
Multisystem autoimmune disease characetrised by vascular damage and fibrosis.
Types:
- limited cutaneous (CREST syndrome): milder, less skin involvement, slow progression
- diffuse cutaneous: faster onset, internal organ involvement more common
133
Systemic sclerosis (scleroderma) aetiology
Increased fibroblast activity = abnormal growth of connective tissue. Excessive collagen production and deposition
Very rare condition, more common in females, peak onset 40-50yrs
134
Systemic sclerosis (scleroderma) presentation
Pulmonary: pulmonary hypertension, interstitial lung disease
Cardio: Raynaud's, CAD, pericarditis, arrhythmias
GI: GORD, oesophageal scarring, reduced small bowel motility
MSK: skin thickening, sclerodactyly (hardening of skin of the hands)
135
Systemic sclerosis (scleroderma) diagnosis
Nailfold capillaroscopy
| Autoantibodies: 90% have ANA
136
Systemic sclerosis (scleroderma) management
Avoid cold exposure, gentle stretching, smoking cessation, occupational therapy as needed
PPIs for GORD, ACEi for HTN, emollient/steroid for skin
137
Ehlers-Danlos syndromes definition
A group of connective tissue disorders
138
Ehlers-Danlos syndromes aetiology
Inherited. Hypermobile EDS is the most common form. More common in females
Genetic mutation leads to production of faulty connective tissue proteins (eg collagen) leading to hypermobility or fragility
139
Ehlers-Danlos syndromes presentation
Skin elasticity/fragility, hypermobility, fatigue, easy bruising, striae
140
Ehlers-Danlos syndromes diagnosis
Clinical diagnosis, genetic testing
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Ehlers-Danlos syndromes management
Physiotherapy, CBT, analgesia
