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Rheumatology Flashcards

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1
Q

What are the cardinal features of Scleroderma

A

excessive collagen production
vascular damage
immune system activation by autoantibody production

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2
Q

What is localised scleroderma?

A

Causes one of more hard patches of skin - thickened, discoloured, hair loss over patch

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3
Q

What is the pathophysiology behind localised scleroderma

A

Fibroblasts make too much collagen
Plaque morphea - oval patches
Linear - in some severe cases just beneath the skin, it can cause scarring

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4
Q

Which creams are used in localised scleroderma

A

Cream containing calcipotriol
Tacromilus ointment
Steroid treatments/ointments

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5
Q

Which autoantibodys are associated with limited systemic scleroderma

A

Anti-centromere

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6
Q

Where does limited scleroderma affect predominantly

A

Affects the face and distal limbs predominantly

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7
Q

What is CREST

A

Calcinosis - calcium deposits usually on fingers
Raynauds Phenomenon - treated by Nifedipine (calcium channel blocker)
oEsophageal dysmotility - collagen deposition and loss of smooth muscle function
Sclerodactyly - thickening of the skin
Telangiectasisa - red spots

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8
Q

Which type of scleroderma has the poorer prognosis

A

Diffuse

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9
Q

Which antibodies are assciated with diffuse scleroderma

A

Scl-70 antibodies

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10
Q

Which other body systems are involved in diffuse scleroderma

A

Heart –> myocardial fibrotic change - secondary to Pulmonary artery hypertension
Blood vessels - Hypertension
Lungs - lung fibrosis –> pulmonary artery hypertension causing
Exertional Dyspneoa
syncope
RV strain
Renal –> due to vascular complications e.g. hypertension, can go into renal crisis with apparently normal BP
MSK - flexion contractures, arthralgia, myalgia and stiffness

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11
Q

Which parts of the skin are involved in diffuse scleroderma

A

Skin of the trunk
upper arms
thighs
Skin changes within 1 year of Reynauds

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12
Q

What is a renal crisis in scleroderma patients

A 
Characterised by acclerated BP with symptoms 
- oliguria 
- headache 
- fatigue 
- oedema 
Investigations show 
- riase in creatinine 
- proteinuria 
- microscopic haematuria
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13
Q

Why is regular BP monitoring important in diffuse scleroderma

A

Important to monitor it so its known what normal is for the patient as a scleroderma renal crisis can occur in a apparently normal BP but is higher than baseline

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14
Q

How is a scleroderma renal crisis treated

A

ACE inhibitors

Dialysis if needed

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15
Q

Which investigations would be done in suspected Scleroderma

A 
Bedside: BP, Urine dip - if presenting with renal crisis 
Bloods 
FBC 
U+Es
LFTs
ESR and CRP 
Autoantibodies - anti-centromere (limited)
Scl-70 (diffuse) 
Barium swallow 
Endoscopy - if presenting with dysphagia 
CXR
Lung function tests
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16
Q

Which investigations are done to monitor scleroderma

A 
Regular BP monitoring 
Renal function 
BNP
Lung function 
CT chest 
ECG
Endocscopy - dilatation of strictures
Cardiac MRI 
Oesophageal manometry +24hr pH studies
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17
Q

What is psoriatic arthropathy

A

A condition that affects both the skin and joints
Referred as one of the seronegative spondyloarthropathy - inflammatory arthritis that is negative for RF
Often precedes the development of skin lesions

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18
Q

What are the clinical features of psoriatic arthritis

A

Oligoarthritis - 2-5 joints affected, usually asymmetrical and weight bearing
Sometimes spinal involvement - typically at the sacrum
Osteolysis - as bone in fingers are lost - telescoping on X ray –> pencil in cup
Plaques on exstensor surfaces - check scalp, natal cleft and umbilicus
Nail changes
- discoloured
- ridging
- onycholysis
- psoriatic nail dystrophy –> pitting, leukonychia

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19
Q

How is psoriatic arthropathy treated

A

DMARDs - methotrexate and leflunomide
- same as RA
NOT hydroxychloroquine as this exacerbates skin conditions
Anti-TNF alpha

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20
Q

What is rheumatoid arthritis

A

Systemic inflammatory disorder - affects multiple joints usually 5 or more symmetrically
Autoimmune
causes progressive symmetrical joint destruction esp in hands

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21
Q

Where else in the body can be affected in rheumatoid arthritis

A

Brain - interleukin 1 and 6 –> fever
Skeletal muscle - protein breakdown
Liver - hepicidin produced due to inflammatory cytokines causing a decrease in iron absorption
Blood vessels - atheroma - strokes, MI and ischaemia
Skin - rheumatoid nodules, necrosis centre surrounded by macrophages (found at pressure points)
Lungs - interstitium –> fibroblasts proliferate causing fibrosis and decrease in gas exchange
PLeural cavity - becomes inflamed and causes pleural effusion (exudative) - SOB

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22
Q

How does RA typically present

Study These Flashcards
A

Swollen, tender painful joints in hands and feet
Stiffness worse in morning which lasts longer than 30 mins
Systemic symptoms: tiredness, depression
typical patient in a young woman

23
Q

What is the pathophysiology of RA

Study These Flashcards
A

Immune cells - activated T cells and plasma cells with antigens reach the joint space
T cells secrete cytokines and recruit macrophages which produce more cytokines and causs granular cells to proliferate
causes thick swollen synovial membrane - which damage cartilage and erodes bone
Joints are red due to increased blood flow
Swollen due to increased blood flow and inflammatory process
painful due to the nerve endings in the joint being irritated by the cytokines

24
Q

Which specific deformities are associated with RA

Study These Flashcards
A

Ulnar deviation
Boutonnieres deformity - extensor tendon splits (PIP flexion and DIP hyperextension)
Swan neck deformity - PIP hyperextended and DIP flexion
Bakers cysts - synovial sac bulges into popliteal fossa

25
Which Score is used in to determine the severity of RA
DAS-28
26
What is Felty syndrome
Rheumatoid arthritis Splenomegaly Agranulocytosis increased risk of infections - life threatening
27
How is rheumatoid arthritis investigated
``` Bedside: BP Bloods - FBC - U+Es - LFTs - CRP - Rheumatoid factor - Anti CCP - May test for other Rheumatological conditions such as SLE e.g anti dsDNA ``` Imaging Xray - different to OA
28
What are the features of RA on XRay
``` Narrowing of joint space Bony erosions - periarticular erosions Soft tissue swelling Decreased bone density subluxation ```
29
How is newly diagnosed RA treated
2 DMARDs - MTX and other | Short course of prednisolone
30
Which drugs are used to treat RA
DMARDS - Methotrexate --> pulmonary toxicity, weekly dose - Hydroxychloroquine - can result in severe and permanent retinopathy, also exacerbates skin conditions - Sulfasalazine - can be used during pregnancy, give folate Biologics e.g. Infliximab TNF inhibitor
31
Which drugs are used in acute flares of RA
NSAIDs | Glucocorticoids - short term use
32
When are Biologics used
if there is an inadequate response to at least 2 DMARDs including Methotrexate
33
How else is RA managed other than DMARDs
Physiotherapy Analgesia Surgery
34
What are some of the ocular manifestations of RA
Keratoconjunctivitis sicca - dryness of the conjunctiva and cornea Episcleritis Scleritis - erythema and pain Corneal ulceration Keratitis Iatrogenic: Steroid induced Cataracts and chloroquine retinopathy
35
What are the risk factors for RA
Smoking | Family history
36
What are the differentials for RA
Psoriatic Arthropathy Reactive arthritis - would only affect one or two joints, post infection, uvetitis and urethritis SLE - other systemic symptoms Polymyalgia rheumatica - stiffness in proximal muscles rather than joints, rapid onset Gout - usually seen in first metatarsal Osteoarthritis - usually weight bearing joints and stiffness wears off <30mins and worse at end of day
37
What are the side effects of Methotrexate
``` Anti-folate Myelosuppression Liver cirrhosis Pneumonitis Lung fibrosis ```
38
What are the side effects of Sulfalazine
Rashes Oligospermia Heinz body anaemia Interstitial lung disease
39
What are the side effects of leflunomide
Liver impairment Interstitial lung disease Hypertension
40
What are the side effects of Hydroxychloroquine
Retinopathy Corneal deposits Skin condition exacerbation
41
What is SLE
An inflammatory autoimmune disease in which any tissue can be targeted by autoantibodies It has a relapsing and remitting course
42
What are the clinical features of SLE
MD SOAP N HAIR Malar rash Discoid rash Serositis - pleuritis and pericarditis Oral ulcer Arthritis Photosensitivity Neurological abnormality --> psychosis and seizures Haematological abnormalities --> Anaemia, thrombocytopenia and leukopenia ANA positive, Anti ds DNA positive, anti smith - last two are more specific Immunological abnormality Renal involvement - abnormal urine protein,, diffuse proliferation, glomerulonephritis
43
Describe the malar rash seen in patients with SLE
It is across the cheeks but spares the naso-labial folds and appears after sun exposure
44
What is the pathophysiology behind SLE
Damage done to cells so parts of the nucleus (histones, DNA, proteins) go into circulation B cells then create antibodies against nuclear antigens Antinuclear antibodies formed genetic factors then cause patients body to recognise the ANA as foreign and when they deposit there is an inflammatory reaction Complement system activated Type 3 hypersensitivity reaction occurs
45
Which drugs can cause drug induced SLE
Hydralazine Isoniazid Procainimide Anti-histone antibodies seen
46
How is SLE managed
``` Lifestyle: avoid UV exposure Patient education minimise cardiovascular risk factors Pharmacological - Hydroxychloroquine - reduces flares and improves fatigue - Paracetamol/NSAIDs for analgesia - Steroids - in the short term to treat flares - Methotrexate for chronic arthritis - Aziothioprine - moderate disease CNS or renal involvement requires aggressive treatment - high dose steroids - azathioprine or mycophenalate - cyclophosphomide - ciclosporin (may require multiple agents) ``` Biologics - used for refractory disease
47
What are the complications of SLE
Cardiovascular disease Infection Antiphospholipid Syndrome Osteoporosis
48
How does polymyalgia rheumatica present
Proximal muscle aching and stiffness in the morning bilateral shoulder pain with bilateral arm weakness - may be difficult to raise arms to brush hair May have pain in neck or pelvis girdle also Rapid onset Typically > 60 years old mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats flu like symptoms
49
What is the pathophysiology of polymyalgia rheumatica
Histology shows vasculitis with giant cells, characteristically skips certain sections of affected artery whilst damaging others Muscle bed arteries mostly affected
50
Which investigations are done in polymyalgia rheumatica
Bloods - FBC - reduced CD8+ T cells - U+Es - LFTs - ESR >40mm/hr - CK - normal Imaging rule out OA
51
What are the differentials for Polymyalgia rheumatica
``` Inflammatory disorders: Rheumatoid arthritis Ankylosing Spondylitis Psoriatic arthritis SLE Scleroderma Vaculitis Dermatomyositis polymyositis ``` ``` Non-inflammatory disorders: OA Drug induced myalgia e.g. statins Rotator cuff disease Infections Malignancy ```
52
What is the treatment in CREST syndrome
supportive endothelin receptor antagonists phosphodiesterase 5 inhibitors ACE inhibitors in renal crisis
53
Which antibodies is CREST syndrome associated with
Anticentromere antibodies
54
What is the colour change in Reynauds syndrome
White to blue to red

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