Rheumatology Flashcards

(212 cards)

1
Q

What is rheumatism?

A

Rheumatism or rheumatic disorder is an umbrella term for conditions causing chronic, often intermittent pain affecting the joints and/or connective tissue.

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2
Q

What is ankylosing spondylitis?

A
  • Chronic seronegative sponyloarthopathy

- Affects the axial skeleton

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3
Q

Who usually develops ankylosing spondylitis?

A
  • Young males (teenage or early 20s)

- Family history present (ass. with HLA B27)

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4
Q

How does ankylosing spondylitis present?

A
  • Morning stiffness/pain in the back

- Relieved with exercise and simple NSAIDs

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5
Q

Where does the pathology usually begin in ankylosing spondylitis?

A
  • Usually starts at the sacroilliac joints.

- ->causes diffuse non-specific buttock pain

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6
Q

What is the typical posture for a patient with chronic ank. spond?

A
  • Question mark posture

- -> loss of lumbar lordosis, buttock atrophy, exaggerated thoracic kyphosis, stooped forward neck posture

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7
Q

How can you measure the extent of ank.spond posture?

A
  • Occiput to wall measurement

- Lumbar spine side flexion test

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8
Q

What 4 extra-articular manifestations associated with ank. spond? (4As)

A
  • Acute Achilles tendonitis
  • Aortic regurgitation
  • Apical (pulmonary) fibrosis
  • Anterior uveitis
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9
Q

What are the diagnostic criteria for ank. spond?

A

1 radiological criteria (+/- clinical criteria) OR 3 clinical criteria = DIAGNOSTIC

  • Clinical criteria:
  • ->Lower back pain >3/12 (improved with exercise, worse with rest)
  • ->Limitation of lumbar spine motion
  • ->Limitation of chest expansion (in relation to others of same sex/age)
  • Radiological criteria
  • -> Sacroiliitis on x ray
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10
Q

What classic finding is found on X ray of ank. spond?

A

-Bamboo spine (only seen in advanced disease)

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11
Q

What are some differential diagnosis of ank. spond?

A
  • Mechanical back pain
  • Inflammatory conditions ie RA, Reactive arthritis
  • Degenerative conditions ie OA
  • Infection
  • Neoplasms
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12
Q

What are some investigations for ank. spond?

A
  • Blood tests: exclude other disease ie RA
  • ->Raised inflammatory markers
  • Imaging
  • ->Xray (bamboo spine)
  • ->MRI/CT (Enthesitis and sacroiliitis)
  • Confirmation of clinical diagnosis -> MRI of sacroiliac joints
  • ->DEXA (osteoporosis)
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13
Q

What is the management for ank. spond?

A
  • Non-pharmacological
  • ->Physiotherapy (+/-hydrotherapy)
  • ->Firm mattress
  • Drugs
  • ->NSAIDs
  • ->Biologics (adalimumab)
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14
Q

What other conditions need monitoring for in a patient with ankylosing spondylitis?

A
  • Peripheral arthritis
  • Osteoporosis
  • Increased risk of fractures
  • Renal disease (rare)
  • Neurological disease (secondary to spine fusing)
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15
Q

What would you find on examination of the spine in a patient with ankylosing spond?

A
  • Tenderness over the spinous processes

- Schober’s test: <5cm change

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16
Q

What is osteoporosis?

A

A progressive systemic skeletal disease characterised by reduced bone mass and mirco-architectural deterioration of bone tissue

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17
Q

What is a fragility fracture?

A

A fracture sustained from falling from a standing level height or less

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18
Q

What T score suggests osteoporosis?

A

-2.5

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19
Q

What is a T score?

A

The standard deviations of a patient away from a healthy young adult

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20
Q

How is the T score measured?

A

DEXA scan

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21
Q

How many men and women develop osteoporosis in their life time?

A
  • 1 in 2 women

- 1 in 5 men

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22
Q

What are some risk factors for osteoporosis?

A

ACCESS

  • Alcohol use
  • Corticosteroid use
  • Calcium low
  • oEstrogen low
  • Smoking
  • Sedentary lifestyle

*Family history, history of parental hip fracture

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23
Q

What are some of the secondary causes of osteoporosis?

A
  • RA and other inflammatory arthropathies
  • Diabetes
  • Hyperthyroidism (thyrotoxicosis)
  • CKD
  • Primary parathyroidism
  • Premature menopause
  • Gastro disease ie Crohn’s
  • Primary hypogonadism
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24
Q

How do patients with osteoporosis usually present?

A
  • Usually asymptomatic until they fall and sustain a fracture
  • Usually the fall is low trauma
  • Common fractures:
  • ->Spine, neck of femur and wrist
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25
What investigations should be done for someone with suspected osteoporosis?
- X ray - DEXA scan - Bloods: FBC, CRP, U&E, LFT, TFT, testoserone, gonadotrophins, serum immunoglobulins and paraprotein (Bence-Jones' proteins)
26
What is the management for osteoporosis?
- Lifestyle advice - ->Adequate nutrition - ->Weight bearing exercise - ->Smoking and alcohol cessation - Adequate calcium and Vit D supplementation - Bisphosphonates - Denosumab - Analgesia and treatment for fractures
27
What is gout?
- An arthritis caused by deposition of MONOSODIUM URATE (MSU) crystals within joints - ->causes acute inflammation and eventual tissue damage
28
Who are the most likely group to develop gout?
Men. 30-60 years old. Usually overweight, drinkers
29
What are risk factors for developing gout?
- Male - Diet - -> increased meat and seafood consumption - Alcohol - Diuretics - Obesity - Hypertension - CHD - DM - Heart failure
30
How does gout present?
- Acute fast onset pain, heat, swelling, erythema in a joint - Typically in the first metatarsal phalangeal joint at night - Wakes patient from sleep at night
31
What sites does gout most commonly affect?
- 1MTPJ (also known as podagra when affected) - Knee - Midtarsal joint - Wrists - Ankles - Small hand joints - Ankles
32
What are the signs of gout?
Acute attack - >Extreme tenderness - >Florid synovitis and swelling - Chronic tophaceous gout - >irregular firm nodules (deposits of crystal) around the extensor surfaces of the fingers, hands, forearms, elbows, achiles tendons - >Tophi (chalky white - becomes very white when the skin is stretched)
33
Investigations for gout?
- Clinical diagnosis in presence of hypercalcaemia - Joint aspiration - ->monosodium urate crystals in the synovial fluid or tophi - Imaging for chronic gout and identification of other sites of deposition
34
What is the management for acute gout?
- NSAIDs and colchicine | - RICE
35
What is prophylactic treatment for gout? (Non-pharmacological)
- Lifestyle modification - >alcohol cessation - >healthy eating - >avoid dehydration - >increase exercise - >weight loss - >smoking cessation
36
What is prophylactic pharmacological treatment for gout?
Xanthine-oxidase inhibitors ie allopurinol
37
Why should allopurinol not be started straight away when a pt has an acute attack of gout?
Allopurinol lowers urate in the blood which results in extra urate crystals being shed from articular cartilage into the joint space, resulting in further acute inflammation.
38
What should be given to a patient in an acute attack of gout when NSAIDs and colchicine is contraindicated?
-Oral corticosteroids
39
What are some important differentials for gout?
- Septic arthritis - RA - Pseudogout
40
What is morphoea (localised scleroderma)?
-A disorder of excessive collagen deposition leading to a thickening of the dermis and the subcutaneous tissues
41
What is the epidemiology of localised scleroderma?
- Usually affects children - Adults affected are usually in their 50s - More common in women
42
What is the aetiology of localised scleroderma?
- Unknown. - Genetic predisposition. There is usually a family history of autoimmune disease. - Environmental trigger: - >tick bites (Lyme disease) - >Measles and other viral infections - >Localised injury - >Pregnancy - >Autoimmune disease - ->>vitiligo, diabetes, lichen sclerosus, lichen planus
43
How does localised scleroderma present?(plaque morphoea)
- Plaque morphoea - >most common type - >1-20cm thickened oval patches - >hairless, smooth, shiny - >asymmetrical distribution over limbs and trunk
44
How does localised scleroderma present? (superficial morphoea)
- middle aged women - symmetrical mauve coloured patches under skin folds - -> groin, arm pit, under the breast
45
How does localised scleroderma present? (Linear morphoea)
- Most common in children - affects the scalp, foreheads and limbs - Long narrow plaques with underlying contractures
46
What are the investigations required for suspected localised scleroderma?
- Autoantibodies (RF +ve, lack of SSc-specific antibodies) - Clinical diagnosis - Biopsy
47
What is the management for localised scleroderma?
- Phototherapy - Immunosuppression - >Methotrexate +/- corticosteroids for involvement of underlying tissues
48
What is pseudogout also known as?
-Acute calcium pyrophosphate crystal arthritis
49
What is pseudogout?
-Inflammation of a joint caused by deposition of calcium pyrophosphate crystals in the articular and periarticular joints
50
Who commonly gets pseudogout?
-The elderly
51
What are risk for pseudogout?
- Dehydration - Intercurrent illness - Hyperparathyroidism - Chronic steroid use - Hypothyroidism - Arthritis - Haemachromatosis - Wilson's disease - Dialysis
52
How do patients with pseudogout present?
``` Similar presentation to gout but slightly milder ->Acute joint pain and swelling ->Warmth ->Tenderness ->Pain on movement ->Effusion Most commonly affects the knees Sometimes a fever and raised WCC is present ```
53
What are some differentials of pseudogout?
- Acute gout - Septic arthritis - OA - RA
54
What are the investigations for pseudogout?
- X ray - Dual energy CT - identifies other calcium pyrophosphate deposition - Aspiration of joint fluid - Exclusion of differentials
55
What will pseudogout crystals look like under microscopy?
+vely birefringent crystals | Calcium pyrophosphate
56
How is pseudogout treated?
- Symptomatic treatment - >RICE - >aspiration of the joint - >NSAIDs - >Intra-articular steroids - >Systemic steroids - Treat the underlying cause
57
What is psoriatic arthritis?
- Seronegative spondyloarthropathy | - An inflammatory arthritis affecting the joints, connective tissue and is associated with nail and hand changes
58
What is psoriasis?
An autoimmune disease characterised by red scaly patches on the skin and affects the nails
59
What are risk factors for psoriatic arthritis?
- Western White population - Women - 35-55 years of age - HLA B27 gene alteration with an environmental trigger (unknown ?physical trauma or infection)
60
What is the pathology leading to psoriasis?
- Unregulated immune response to a trigger causing inflammatory cytokines -->activation of TNF and interleukins - Causes kertianocytes and fibroblasts to form psoriatic plaques
61
How does psoriatic arthritis present?
- Rash usually precedes arthritis by couple of years - >Affects the scalp, extensor aspects of forearms and elbows, umbilicus, natal cleft - Nail changes - >Pitting, yellowing, tranverse ridges, oncyholysis - Tenosynovitis
62
What are some typical patterns of presentation for a patient with psoriatic arthritis?
- Symmertical - Asymmetrical oligoarticular - Lone DIP disease - Arthritis mutilans - Spondylitic pattern + sacroiliitis - Juvenille onset
63
Which sites does a symmetrical psoriatic arthritis commonly affect?
- Wrists - Hands (DIPJ) - Feet - Ankles
64
How does an asymmetric oligoarticular psoriatic arthritis present?
- Involvement of the hands and feet initially | - Dactylitis
65
How dose lone DIP disease present (psoriatic arthritis)?
- Nail and paronychial tissue involvement - Looks like an infection of hammer blow appearance - Commonly seen in males
66
What is arthritis mutilans?
- Rare variation of DIP disease - Teloscopic digit appearance caused by the resorption of the phalynx - >pencil in water appearance on radiograph
67
What is the spondylitic pattern presentation in psoriatic arthritis?
- More common in men - Morning stiffness - Limitation of back movement - Asymmetrical vertebrae involvement
68
What is the juvenille onset of psoriatic arthritis?
- Starts as monoarthritis, but DIP disease may be seen - Tenosynovitis - Nail changes - Growth affected if there is epiphyseal involvement - Onset of rash and arthritis occurs at same time
69
What investigations are needed to investigate psoriatic arthritis?
- Clinical and radiographic impressions in presence of a classical rash - Blood tests: ^ESR + ^CRP. RF-ve - Xray
70
What X ray changes are seen in psoriatic arthritis?
- Mild bony erosions at edge of cartilage - Asymmetric erosive changes in hands or feet - DIP or PIP involvement - >erosion or deformity - >bony alkalosis of the joint - >subluxation - Erosion of distal tuft of distal phalynx
71
How is psoriatic arthritis managed?
- NSAIDs to relieve MSK symptoms - Corticosteroid injections - DMARDs - TNF inhibitors - Ustenkinumab
72
What are some examples of DMARDs that are used to treat psoriatic arthritis?
- Methotrexate (cutaneous psoriasis) - Leflunomide (peripheral active psoriatic arthritis) - Sulfasalazine (alt. to leflunomide)
73
When should TNF inhibitors be considered for treating psoriatic arthritis?
- When there is not an adequate repsonse to at least one synthetic DMARD, NSAID or local steroid injection - When the pt has arthritis with >3 tender joints
74
What are some examples of TNF inhibitors?
- Adalimumab - Etanercept - Golimumab - Infliximab
75
When us ustekinumab used?
- Used alone or in combination with methotrexate in adults only - when treatment with TNF inhibitors is contraindicated and the pt would need advanced treatment
76
What is reactive arthritis?
A seronegative spondyloarthritis associated with inflammatory back pain and oligoarthritis with extra-articular symptoms -Usually follows a GI or GU infection
77
What is a clinical subtype of Reactive arthritis?
- Reiter's syndrome | - >large joint oligoarthritis, urogenital infection and uveitis
78
What gene is Reactive arthritis associated with?
-HLA B27 mutation
79
What infective agents are associated with reactive arthritis?
- Post enteric: campylobacter, salmonella, shigella | - Post veneral: chalmydia, HIV
80
Who is commonly affected by reactive arthritis?
-young adults with exposure to chlamydia (most common causative organism)
81
How does reactive arthritis present?
- Develops 2-4 weeks post GI/GU infection - Acute onset, malaise, fever, fatigue - Asymmetrical, lower extremity oligoarthritis - Lower back pain
82
What are the extra-articular features found in reactive arthritis?
- Eyes: uveitis, episcleritis, keratitis, corneal ulceration - GI: abdo pain, diarrhoea - CV: aortitis +/- AR, conduction defects - Skin: erythema nodosum, circinate balanitis - Nails: dystrophic changes - Mucous membranes: mouth ulcers
83
What are some differential diagnosis of reactive arthritis?
- ankylosing spondylitis - gonococcal arthritis - gout - IBD - psoriatic arthritis - rheumatic fever - RA - septic arthritis
84
What investigations should be done for suspected reactive arthritis?
- Bloods: FBC (anaemia, leukocytosis, thrombocytosis) ESR/CRP^. HLA B27 +ve - Joint aspiration: exclude gout - Blood cultures - Chalmydia swabs - Xray: identify chronic disease - ECG: monitoring for conduction disturbance with suspected chronic disease
85
What is the management of reactive arthritis?
- Acute phase: - >Rest, aspirate synovial effusions - >Antibiotics - >NSAIDs - Other: - >Physiotherapy - >Steroids
86
What is rheumatoid arthritis?
- Chronic inflammatory autoimmune disease causing a symmetrical polyarthritis - Inflammation of synovial joints = joint and periarticular destruction
87
What are examples of systemic features of RA?
- Fever - Malaise - Decreased appetite - Weakness
88
What is the pathology behind RA?
An immune response of cytokines, interkeukins etc --> proliferation of synovial membrane. -Formation of granulation tissue (thick, swollen) --> Pannus formation = damage to cartilage, bone and other soft tissues
89
What is the epidemiology of RA?
Female > male | Peak age of onset: 30-50 years
90
What are risk factors for RA?
-Genetic susceptibility and environmental factor interaction
91
What are some genetic risk factors for RA?
- High birth weight - Rheumatoid factor - Anti-citrullinated protein antibody - HLA DR1/4 - Female sex
92
What are some environmental risk factors for RA?
- Smoking - Silica exposure - Alcohol abstention - Obesity - Diabetes Mellitus
93
How does RA present?
- Begins with an insidious symmetrical polyarthritis - Joint inflammation: - >heat - >redness - >swelling - >pain - >stiffness (early morning >30 mins)
94
What joints may be affected in someone with RA?
- Symmetrical, distal, small joints affected - >Proximal interphalangeal joints - >Metacarpophalangeal - >Wrist - >Ankle - >Knee - >Cervical spine joints
95
What hand deformities may be seen in RA?
- Boutonniere's deformity - Swan neck deformity - Ulnar deviation from the metacarpalphalangeal joint - Muscle wasting - Tendon rupture
96
What are some other extra-articular manifestations of RA?
- Eyes: ->sjogren's sydnrome ->scleritis ->episcleritis - Skin: ->leg ulcers ->Felty's syndrome ->Rheumatoid nodules - Neuro: ->Nerve entrapment ->Polyneuropathy - Resp system: ->Pulmonary fibrosis - CV system: -> pericardial involvement ->valvulitis - Other: -> thyroid disorders -> osteoporosis -> depression
97
How is a diagnosis of Ra made?
- Clinical | - Investigations are used to exclude differentials
98
What are specific investigations to confirm RA?
- Rheumatoid factor - Anti-CCP - Xray: soft tissue swelling, loss of joint space, erosions, difformity, periarticular osteopenia, decreased bone density
99
What are some differential diagnosis of RA?
- Arthritis mutilans - SLE - Acute viral polyarthritis
100
When is an urgent referral needed for suspected RA?
- Involvement of the small hands or feet - More than one joint affected - >3 months of symptoms
101
What simple analgesics can be used to manage pain?
NSAIDs and simple analgesics
102
Who makes up the MDT in RA?
- GP - Physio - Rheumatologist - Nurse specialist - OT - Dietician - Podiatrist - Pharmacist
103
What is 1st line RA treatment?
1. Methotrexate (2.5mg-20mg) | - With folic acid
104
What is 2nd/3rd line treatment?
- Sulfasalazine - Hydroxychloroquine - Leflunonamide
105
How often do blood tests need to be done when a patient is on methotrexate?
-3 months
106
What are some examples of DMARDs?
- Methotrexate - Sulfasalazine - Hydroxychloroquine
107
What are some examples classes of biologic therapies?
- TNF inhibitors - Anti-CD20 therapy - Anti-interleukin therapy
108
What are examples of TNF inhibitors?
- Adalimumab - Etanercept - Infliximab
109
What is an example of anti-CD20 therapy?
-Rituximab
110
What is an example of an anti-interleukin therapy?
-Tocilizumab
111
How does Abatercept work?
-Suppresses T cells
112
What are the Xray signs for OA?
- Loss of joint space - Osteophyte formation - Subchondral cysts - Subchondral sclerosis
113
What are the Xray signs for RA?
- Loss of joint space - Erosions - Soft tissue swelling - See-through bone (osteopenia)
114
What is scleroderma?
-A multisystem disease where there is increased fibroblast activity resulting in abnormal growth of connective tissue
115
Where does fibrosis occur in the body in scleroderma?
- Skin - GI tract - Heart - Lungs - Vasculature
116
How is scleroderma classified?
- Limited cutaneous systemic sclerosis - >Used to be known as CREST syndrome - >> Calcinosis, raynaud's disease, oEsophageal dysmotility, sclerodactyly, telangiectasia - Diffuse cutaneous systemic sclerosis - >more fatal, less common
117
What is the stereotypical group affected with scleroderma?
Women> men | 40-50 year olds
118
What are the cardinal features of scleroderma?
- Excessive collagen deposition - Vascular damage - Immune system activation
119
What is the aetiology of scleroderma?
``` -Unknown: genetic predisposition with an environmental trigger >>Infectious agent >>Chemicals >> Drugs >>Radiation therapy >>Physical trauma >>Vitamin D deficiency ```
120
What type of scleroderma has the best prognosis?
- Limited systemic sclerosis - >milder disease - >less skin involvement - >slow onset and progression - Diffuse systemic sclerosis - >rapid onset - >internal organ involvement is common
121
What are common presenting features for a patient with limited cutaneous systemic sclerosis?
- Raynaud's phenomenon - Skin hardening in hands or face - Oesophageal symptoms - fatigue - MSK pain - Hand swelling
122
What are some skin features of scleroderma?
- Sclerodactyly - Digital ulcers - Raynaud's phenomenon - Calcinosis - Dryness and itchiness
123
What are some MSK features of scleroderma?
- Joint pain and swelling - Myalgia - Restriction of joint movements - Tendon friction rubs
124
What are some GI features of scleroderma?
- Heartburn and reflux - Oesophageal dysphagia - Reduced small bowel motility - Watermelon stomach - >gastric antral vascular ectasia - Constipation - Anorectal dysfunction
125
What are some pulmonary features of scleroderma?
- Pulmonary fibrosis - Pulmonary arterial hypertension - Aspiration pneumonia - Resp. muscle weakness - Pneumothorax
126
What are some cardiac features of scleroderma?
- Microvascular coronary artery disease - Atherosclerosis - Myocardial fibrosis - Pericarditis - Pericardial effusion - Arrhythmias and conduction defects
127
What are some renal features of scleroderma?
- ANCA associated glomerulonephritis | - Reduced renal function reserve
128
How might a scleroderma renal crisis present?
Affects from accelerated hypertension. - Oliguria - Headache - Fatigue - Oedema - Rising creatinine levels - Proteinuria
129
What are some GU features of scleroderma
- Erectile dysfunction in males | - Dyspareunia in women
130
What are some investigations for scleroderma?
- Routine bloods - Autoantibodies: - >Anti-toposomerase 1 - >Anti-centromere antibody - >Anti RNA polymerase 111 antibody - Urine screen - Hand x ray - Endoscopy (if GI symptoms)
131
What is anti-topoisomerase 1 associated with?
- Scleroderma with following organs involved: - >lung fibrosis - >renal disease - Poor prognosis
132
What is anti-centromere antibody associated with?
Localised scleroderma with pulmonary hypertension
133
What is anti-RNA polymeraseIII antibody associated with?
Diffuse scleroderma with kidney involvement
134
Diagnosis of scleroderma criteria?
-Skin thickening extending to proximal MCP joints -Skin thickening of fingers -Fingertip lesions -Telangiectasia -Abnormal nailfold capillaries -Pulmonary arterial hypertension +/- ILD -Raynaud's SSc-related autoantibodies
135
What is the management of scleroderma?
-Manage symptoms of the complex disease
136
What is some non-pharmacological treatments for scleroderma?
- Physio - Smoking cessation - Healthy weight - Avoid harsh skin irritants - Keeping warm - OT assessment - Laser therapy for telangiectasia
137
What immunotherapy is used for scleroderma?
- Methotrexate - Mycophenolate mofetil - Cyclophosphamide
138
What is septic arthritis?
An infection causing inflammation in a native or prosthetic joint
139
What is the most common causative organism causing septic arthritis?
-Staph. aureus
140
Which joint is most commonly affected in septic arthritis?
The knees
141
What are risk factors for septic arthritis?
- Increasing age - DM - Prior joint damage - >RA, gout - Joint surgery - Hip/knee prosthesis - Skin infection with prosthesis - Immunodeficiency
142
What is the presentation of septic arthritis?
- Single, hot, swollen, extremely painful joint - >unable to move the joint - Fever and rigors
143
How would children present with septic arthritis?
- Mainly affects preschool infants - Fever, unwillingness to move joint - Loss of appetite - Oliguria - Drinking less
144
What are signs of a septic joint?
- Swollen - Warm - Tender - Extremely painful on movement - Effusion
145
In which groups might signs of septic arthritis be less obvious?
- Elderly - Immunocomprimised - IVDU - Infections of the hip, spine and shoulders
146
What investiations should be done for suspected septic arthritis?
- CRP - FBC - Blood cultures - Synovial fluid aspirate
147
What form of imaging would detect osteomyelitis?
- MRI | - CT
148
What is the management for septic arthritis?
- Antiboiotics - >Treat empirically before cultures return. FLUCLOXACILLIN - Joint drainage - Splinting
149
If a patient has MRSA septic arthritis, what is the antibiotic required?
Vancomycin
150
If septic arthritis is caused by a gonooccal strain, what is the abx of choice?
-Cefotaxime
151
How long should antibiotics be given for in a patient with septic arthritis?
- IV: 2-3 weeks | - Then switch to PO for 2-4 weeks
152
What is systemic lupus erythematous?
- A heterogenous, inflammatory, mutlisystem autoimmune disease - Presence of Antinuclear Antibodies
153
What are genetic risk factors for SLE?
- HLA DR3/DR2 - Defective C4 complement gene - Oestrogen
154
What are some environmental risk factors for SLE?
- UV light - Cigarettes - Viruses eg. EBV - Drugs - >Chlorpromazine, Methyldopa , Isoniazid, D-penicillamine
155
What is the pathology causing SLE?
- Environmental factors damage cells - Exposure of susceptible DNA from cells - Causes activation of the immune system - Susceptible DNA prevents effective clearance - Increased susceptible DNA presence - Increases the inflammatory system
156
What type of hypersensitivity reactions is involved in SLE?
- Type III sensitivity (immune-complex mediated) | - Type II sensitivity (cytotoxic, antibody dependent mediated)
157
What disease pattern does SLE follow?
-Relapsing and remitting
158
What are some non-specific symptoms of SLE?
- Fatigue - Malaise - Fever - Splenomegaly - Lymphadenopathy - Weight loss
159
What is the typical pattern of arthralgia in SLE?
- Early morning stiffness - Peripheral - Symmetrical - Flitting - Polyarthritis
160
What oral symptoms might someone experience with SLE?
- Mouth ulcers | - >Big and painful
161
What is a classic skin symptom sign in SLE patients?
- Photosensitive skin rashes - >Malar (butterfly rash) - ->raised and pruritic. spares the naso-labial folds - >Discoid lupus erythematous - ->well demarcated, erythematous, scaling lesion in sun exposed areas - >Livedo reticularis - >Diffuse alopecia - >Vasculitic rashes
162
What pulmonary complications may be present in SLE?
- Pleurisy - Fibrosing alveolitis - Obliterative bronchiolitis - PE
163
What cardio complications may be caused by SLE?
- Pericarditis - Hypertension - Increased risk of ACS
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What is a common renal complication from SLE?
-Glomerulonephritis
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What are some neuropsychiatric complications of SLE?
- Anxiety and depression - Psychosis - Seizures - Neuropathy - Meningitis - Organic brain syndrome
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What condition is seen in the hands as a result of SLE?
-Raynaud's
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What happens to the eyes and mouth in a patient with SLE?
-Dry mouth and eyes
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What is the diagnostic criteria for SLE? How many criteria need to be met
-4 out of 11 criteria (Don't all need to be present at the same time) >Malar rash >Discoid rash >Photosensitivity rash >Oral or nasopharyngeal ulcers >Non-erosive arthritis >2 joints >Pleuritis or pericarditis >Renal involvement: persisten proteinuria/cast cells >Seizures or psychosis >Haematological disease >Immunological disorder (presence of antibodies)
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What antibodies are specfic to SLE?
- Anti-DNA antibody - Anti-SM antibody - Antiphospholipid antibodies - Positive antinuclear antibody
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What investigations should be done for suspected SLE?
- Urinalysis: proteinuria/haematuria - FBC and ESR - Autoantibodies
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What is ANA?
- An antibody which is found in 95% of patients with symptoms of SLE. - Screening test!
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What is anti-dsDNA?
- Antibody in SLE which reflects the level of disease activity. - Can guide changes in therapy
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What is anti-Sm?
-The most specific antibody to SLE. But not found in all patients
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What are some autoantibodies found in SLE?
- ANA - Anti-dsDNA - Anti-Sm - Anti-SSA (Ro) - Anti-SSB (La) - Anti-RNP - Anti-histone - Antiphospholipid antibody
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What happens in c3 and c4 levels in SLE?
-They both decrease
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What types of conditions are patients with SLE more at risk of?
- Antiphospholipid syndrome - Other connective tissue disease - Other autoimmune conditions - Cardivascular disease - Malignancies ie NHL
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What is the main goal of managing SLE?
-Prevention of flares and reduction of the severity of the flares
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What is the management of patients with SLE?
- Pain relief - Reduction of rashes - Consider corticosteroids and DMARDs for severe disease - Consider adjunctive therapy and biologics for uncontrolled disease - Plasma exchange for life threatening disease - Patient counselling
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What DMARDs should be considered in SLE?
- Cyclophosphamide - Mycophenolate motefil - Azathioprine - Methotrexate - Ciclosporin
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What biologics can be used for SLE?
- Rituximab - Abatercept - Tocilizumab
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What is an example of adjunctive therapy for SLE?
-Belimumab
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What advice should be given to patients with SLE wishing to become pregnant?
- Wait until disease control is maximal - Avoid oestrogen pill whilst trying to gain disease control (oestrogen causes flare ups) - Increased risk of intrauterine deaths, thrombosis, pre-eclampsia, miscarriages
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What is temporal arteritis?
- A systemic immune mediated vasculitis affecting medium and large sized vessels - Mainly affects the carotid arteries - Strong association with polymyalgia rheumatica
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What is the epidemiology of temporal artitis?
- Women | - Northern European ethnicities
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What are risk factors for temporal arteritis?
- European background - Peak incidence: 60-80 years - Genetic factors
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How does GCA present?
- Sudden onset temporal headaches - >scalp tenderness (combing hair) - >transient visual symptoms: blurred vision, amaurosis fugax, visual loss, diplopia - >facial pain - Jaw/tongue claudication (chewing/talking) - Systemic features: anorexia, myalgia, malaise, fever, fatigue, depression
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How is GCA diagnosed?
- Development of symptoms >50 - New headache: new onset - Temporal artery tenderness on palpation - Elevated ESR >50mm/hr - Abnormal artery biopsy
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What is the management for GCA?
- Steroids (40mg pred) - Low dose aspirin - Tocilizumab
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Why is GCA a medical emergency?
-In elderly, may cause permanent visual loss
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What are some complications of GCA?
-Loss of vision -Aneurysms, dissections, stenotic lesions -CNS disease -Steroid related complications: >osteoporosis, myopathy, bruising, hypertension, DM, fluid retention, psychosis
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What is polymyalgia rheumatica?
-An inflammatory condition of unknown cause causing severe bilateral pain. >Morning stiffness of the shoulder, neck and pelvic girdle
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What is the epidemiology of PMR?
- Usually occurs in the 50+ age group | - Female>male
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How does PMR present?
- Non-specifically | - Using the core inclusion criteria
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What is the core inclusion criteria for PMR?
- Age >50 years - Bilateral, severe and persistent pain in the neck, shoulders and pelvic girdle - >Initial flu like symptoms - Morning stiffness >45 minutes - Raised CRP
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What diseases need to be excluded before a diagnosis of PMR can be given?
- Active infection - Cancer - GCA
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What are some inflammatory differential diagnoses for PMR?
- RA - Late onset spondyloarthropathy - SLE and other connective tissue disease
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What are some non-inflammatory differential diagnoses for PMR?
- Degenerative disease: OA, spinal spondylosis - Rotator cuff disease - Drug induced myalgia - Infections - Malignancy - Amyloidosis - Parkinsonism - Chronic pain syndrome
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What investigations are needed before diagnosing PMR?
- Exclusion of other disease - FBC, CRP^, bone profile - Urinalysis - Urinary Bence-Jones protein - Autoantibodies - >ANA and anti-CCP (should both be -ve)
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What is the management of PMR?
- Physiotherapy - Occupational therapy - Prednisolone (weened after 2 years) - Regular reviews - Prevention of osteoporosis with bisphosphonates
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What is vasculitis?
-A series of conditions in which there in inflammation of the vessels
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What are the most common types of vasculitis?
1. PMR | 2. GCA
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What are the causes of vasculitis?
- Idiopathic - Infection - >HSP, Septic arthritis, URT flares of wegners arteritis - Inflammatory disease - >SLE, RA, crohns, UC - Drug induced ie sulfonamides - Neoplastic
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How can vasculitis be classified?
- Infective - Non-infective - >Large vessel ie GCA - >Medium vessel ie Kawasacki disease - >Small vessel ie immune complex mediated - >Variable vessel ie Bechet's disease
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How does small vessel vasculitis present?
- Palpable purpura 1-3 mm (may join to form plaques ± ulcer) - Tiny papules - Splinter haemorrhages - Urticaria - Vesicles - Livedo reticularis (rare)
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How does medium vessel vasculitis present?
- Ulcers - Digital infarcts - Nodules - Livedo reticularis - Papulo-necrotic lesions - Hypertension (damage to the renal vessels)
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How does large vessel vasculitis present?
- End-organ ischaemia (eg, TIA/CVE) - Hypertension - Aneurysms - Dissection ± haemorrhage or rupture
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What investigations should be done for suspected vasculitis?
- FBC, U&E, LFTs, Inflammatory markers - Urine culture and microscopy - Complement levels - Hepatitis serology - Rheumatoid factor - CXR - Echocardiogram and blood cultures if there is cardiac murmur present. - Antinuclear antibodies (ANAs) if there is medium-sized vessel involvement and any suggestion of connective tissue disease. - Skin biopsy. - Imaging - PET scan, MRI and colour Doppler ultrasonography
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What is acute management of vasculitis?
-Corticosteroids
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What is long term management of vasculitis?
-Immunosuppression: cyclophosphamide, azathioprine, methotrexate -Plasmapharesis for refractory disease
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How should vasculitis be monitored?
- ESR | - c-ANCA elevation indicates disease activity
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Why does hydroxychloroquine need monitoring?
- Causes bull's eye maculopathy | - Causes central vision disturbance
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Why might liver disease occur in rheumatology patients?
- Methotrexate - Psoriasis disease - Obesity