RHEUMATOLOGY Flashcards
1
Q
Define rheumatoid arthritis
A
An autoimmune disease associated to Fc portion of IgG (RF) and anti-CCP
2
Q
Describe the pathogenesis of RA in as much detail as you can
A
1) Citrullination of self antigens. These are recognised by T and B cells. T and B cells produce antibodies aka RF and anti-CCP.
2) Macrophages and fibroblasts get stimulated and release TNFalpha
3) Inflammatory cascade starts - causes proliferation of synoviocytes = these will grow over cartilage and cut off nutrition to it = damages cartilage !
4) Macrophages also stimulate osteoclast = get bone damage
Note: citrullination is just where amino acid arginine is converted to citrulline. This is v important bc citrulline is not one of the 20 amino acids in our DNA code - so leads to modification (here it causes RA!)
3
Q
What is clinical presentation of RA?
A
Female gender (3:1). 30-50yrs Symptoms are progressive, peripheral and symmetrical polyarthritis Affects MCPs,PIPs, MTPs - does NOT affect DIPs Affects hips, knees, shoulders, c-spine History over 6 weeks Morning stiffness for over 30 mins. Commonly have fatigue, malaise
4
Q
What can be found on examination in a pt with RA?
A
Soft tissue swelling and tenderness.
Ulnar deviation, or palmar subluxation of MCPs
Swan neck or/and Boutonniere deformity to digits
Rheumatoid nodules (usually on elbow)
Median N - carpal tunnel association
5
Q
Name three investigations (or more!) you would consider for a pt with suspected RA
A
RF, anti-CCP, FBC, WCC, inflammatory markers, X ray or can do MRI or USS in early disease.
6
Q
Why is a WCC done in blood test for suspected RA?
A
Can be elevated due to complication of septic arthritis
7
Q
Why is FBC carried out as an investigation for RA?
A
May show normocytic anaemia which is a feature of chronic disease
8
Q
How is RA initially treated?
A
DMARD monotherapy - methotrexate
9
Q
Describe treatment you would discuss with patient recently diagnosed with RA
A
- DMARD such as methotrexate. Can discuss use of combination
- Steriods to be used acutely both orally or intra-articular
- NSAIDs + PPI to aid with symptom control
- Non drug options - OT/PT, podiatry, psychological
10
Q
What are the extra-articular features of severe RA?
A
Remember with mnemonic CAPS: (come in 3s)
C - carpal tunnel, CVD, cord compression
A - anaemia, amyloidosis, arteritis
P - pericarditis, pleural dosease, pulmonary disease
S - Sjögren’s, scleritis, Splenic enlargement
11
Q
What features are characteristically seen in an XRAY of RA?
A
LESS Loss of joint space Erosions (periarticular) Soft tissue swelling Subluxation
12
Q
Define giant cell arteritis
A
Chronic vasculitis of large and medium sized vessels in individuals over 50yrs.
13
Q
Why is giant cell arteritis an emergency?
A
Occlusive arteritis can lead to anterior ischaemic optic neuropaty and acute visual loss. The visual symptoms are an opthalmoc emergency
14
Q
What are the risk factors for GCA aka Temporal arteritis?
A
Age
White ethnicity
PMH of polymyalgia rheumatica
Genetic predisposition with HLA-DR4
15
Q
How does GCA present?
A
Presentation is acute
Headaches in 70% of presentations
Localised, unilateral, piercing or stabbing over the temple
Tongue and/or jaw claudication upon mastication
Constitutional symptoms
Visual symptoms
Scalp tenderness, especially over temporal artery
16
Q
How is giant cell arteritis diagnosed?
A
- Over 50
- The presence of two or more of these symptoms:
Raised ESR, CRP or PV
New onset of localised headache
Tenderness or decreased pulsation of temporal artery
New visual symptoms
Biopsy of necrotizing arteritis
17
Q
How is GCA treated?
A
- Prednisolone 60-100mg PO per day for at least 2 weeks then slowly reduce
- if visual symptoms are present - 1g methylprednisolone IV pulse therapy for 1-3 days
- low dose aspirin therapy to reduce thrombotic risks
18
Q
What is your immediate managment in pt with suspected GCA?
A
Steroid therapy
19
Q
How is polymyalgia rheumatica (PMR) characterised?
A
Pain and stiffness in shoulder, hip girdles and neck.
20
Q
Who does PMRF usually affect?
A
The elderly - incidence increases with age. Average age is 70.
Affects patients with GCA (there’s evidence that PMR and GCA are associated to one another)
21
Q
How does PMR present (just from a history)?
A
Sudden onset of pain in proximal limbs - so the neck, shoulders and hips.
Difficulty getting up from chair (hip pain), and combing hair (shoulder pain).
Pain at night time
Systemic symptoms - fatigue, weight loss, low grade fever
From Z2F:
Presence of symptoms for at least 2 weeks
Bilateral shoulder pain that radiates to the elbow
Bilateral pelvic girdle pain
Worse with movement
Interferes with sleep
Stiffness for at least 45 minutes in the morning
May also have systemic symptoms - weight loss, fatigue, low grade fever, low mood Upper arm tenderness Carpal tunnel syndrome Pitting oedema 
22
Q
Patterns of Joint / Muscle involvement:
If joint involvement was symmetrical it would suggest ____(1)____
Whereas, asymmetrical joint involvement would suggest ___(2)_____ or ____(3)_____
A
(1) RA
(2) Gout
(3) Psoriatic Arthritis
23
Q
What investigations may you consider for pt with suspected PMR?
A
Bloods - ESR, CRP, polycythemia vera
Temporal artery biopsy if symptoms of GCA
24
Q
Patterns of Joint / Muscle involvement:
Small joint only would suggest___(1)_____
Large joints only would suggest ___(2)____
Large and small joints would suggest ____(3)______
A
(1) Early stages of RA
(2) OA
(3) Late stages of RA
25
Give the medical term for describing the number of joints involved for the below:
(1) 1 joint
(2) 2-4 joints
(3) >4 joints
(1) monoarticular
(2) Oligoarticular / pauciarticular
(3) Polyarticular
26
List some causes of acute polyarthritis. Use categories below to give specific conditions
1. Inflammatory arthritis
2. Autoimmune arthritis
3. Viral infection
4. Crystal arthritis
1. Inflammatory arthritis
- RA
- PsA
- Reactive arthritis
2. Autoimmune arthritis
- SLE
- Vasculitis
3. Viral infection
- HIV
- Parovirus
Chikungunya
4. Crystal arthritis
- UNcontrolled Gout
27
What are the causes of chronic monoarthritis? Use categories below to think of specific conditions
1. Infections
2. Inflammatory
3. Non- inflammatory
4. Tumours
1. Infections
- TB
2. Inflammatory
- Psoriatic arthritis
- Reactive arthritis
- Foreign body
3. Non- inflammatory
- OA
- Trauma (meniscal tear)
- Osteonecrosis (prednisolone use)
- Neuropathic ( Charcots arthropathy )
4. Tumours
- he says v rare!
28
What 2 conditions __(1)____ and ____(2)______can cause arthritis of the DIPJs?
In ___(1)___ changes will also be seen on the nail of the digit.
___(2)___ is the most common disease affecting this joint. ____(3)____ nodes can be seen on the DIPJ in this disorder.
(1) PsA
(2) OA
(3) Heberden's Nodes affecting the DIPJ in OA
29
What investigations may you consider for pt with suspected PMR?
Bloods - ESR, CRP, polycythemia vera
| Temporal artery biopsyy if symptoms of GCA
30
History taking - DRUG HISTORY of a rheum patient
____(1)____ causes cutaneous hypersensitivity vasculitis
____(2)___ cause Lupus skin rashes
____(3)___ worsen Raynaud's symptoms
____(4)____ reduce uric acid excretion and can lead to gout.
HINTS (1) AB, (2) DMARD (3) HEART (4) WATER
__Penicillin____ causes cutaneous hypersensitivity vasculitis
___Sulfasalazine___ cause Lupus skin rashes
____ Beta Blockers___ worsen Raynaud's symptoms
____Diuretics_(thiazides)___ reduce uric acid excretion and can lead to gout.
31
History taking - PMHS HISTORY of a rheum patient
Seronegative Spondyloarthropathy is associated with which 3 conditions?
A history of STI / diarrhoea could indicate what 2 types of arthiris
Seronegative Spondyloarthropathy is associated with:
- Anterior uveitis
- Psoriasis
- IBD
Hx of STI / Diarrhoea could indicate:
- reactive arthritis
- gonococcal arthritis
32
History taking - Social HISTORY of a rheum patient
SMOKING:
Smoking is implicated in causing and making ___(1)____more severe
Patients with ____(2)____ symptoms in the hands should be advised to stop smoking
Smoking is implicated in causing and making ___RA____more severe
Patients with ____Raynaud's ____ symptoms in the hands should be advised to stop smoking
33
Compare and Contrast the features of Inflammatory vs Mechanical disease.
E.g. Morning stiffness / effect of activity / effect of resting / Fatigue/ systemic involvement
```
Inflammatory disease:
> 1 hour morning stiffness
Activity - improves
Resting - worsens
Fatigue - profound
Systemic symptoms - yes
```
```
Mechanical Disease
< 30 mins morning stiffness
Activity - worsens
Resting - improves
Fatigue - minimal
Systemic symptoms - no
```
34
History taking - Constitutional symptoms
What are some examples of constitutional symptoms?
What do they indicate? (3)
What are some conditions which may present with constitutional symptoms?
Constitutional symptoms
- Fever
- Weight loss
- Night sweats
- Loss of appetite
What do they indicate?
1. Inflammation
2. Infection
3. Neoplasia
What are some conditions which may present with constitutional symptoms?
Ankylosing spondylitis (all)
GCA
SLE - fever
35
What are some extra - articular features of SLE?
LOADS IN THE BOOKLET - GUESS A FEW systems based!
Mouth / Eyes
- mouth uclers
Skin
- Digital ulcers
- Malar flush / photosensitivity
- Alopecia
- Raynaud's
Genito-urinary
- renal failure / hypertension
- Micro -haematuria / proteinuria
Obstetric
-Miscarriage / pre-eclampsia
Neuro
- headache
- seizures
- psychosis
- TIA / CVA
Cardio- Resp
SOB - PE / Pul effusion / Pulmonary HTN / alveolitis/ pleuritic chest pain
36
What are some extra - articular features of RA?
Mouth / Eyes
- scleritis
- Dry mouth / eyes
Skin
- subcutaneous nodules
Genito-urinary
- renal failure / hypertension
- Micro -haematuria / proteinuria
Neuro
- Compressive - e.g. carpal tunnel syndrome
Cardio- Resp
SOB - alveolitis
37
What are some extra - articular features of RA?
Mouth / Eyes
- scleritis
- Dry mouth / eyes
Skin
- subcutaneous nodules
Genito-urinary
- renal failure / hypertension
- Micro -haematuria / proteinuria
Neuro
- Compresive - e.g. carpal tunnel syndrome
Cardio- Resp
SOB - alveolitis
38
What are some extra - articular features of Sjogrens Syndrome?
Mouth / Eyes
- Dry mouth / eyes
Skin
- Raynaud's
39
What gene are the Spondyloarthropathies associated with?
HLA - B27
40
What gene are the Spondyloarthropathies associated with?
HLA - B27
41
How would a patient with Ankylosing Spondylitis Present?
1. Typical pt
2. Presenting symptoms
```
1. Typical pt
Young male (teens - mid 30s)
```
```
2. Presenting symptoms
Gradual development over 3 months
Bilateral buttock pain (sacroilliac)
lower back pain/ stiffness - wakes pt up in morning- improve with activity
Chest wall pain
Thoracic pain
Often 'flare ups' of symptoms
```
42
Examining a pt with Ankylosing Spondylitis
1. Early examination vs later on
2. What test might you perform and what is normal?
1. Early examination - often normal
Later examination
- reduced chest wall expansion
- loss of lumbar lordosis and increased thoracic kyphosis
2. Schober's test - to see if decreased lumbar spine range of movement
Mark skin 10cm above and 5cm below the L5 vertebrae. Ask pt to bend forward with straight legs. If distance increases < 20 cm supports diagnosis as restricted movement
43
Examining a pt with Ankylosing Spondylitis
1. Early examination vs later on
2. What test might you perform and what is normal?
1. Early examination - often normal
Later examination
- reduced chest wall expansion
- loss of lumbar lordosis and increased thoracic kyphosis
2. Schober's test - to see if decreased in lumbar spine range of movement
Mark skin 10cm above and 5cm below PSIS. Ask pt to bend forward with straight legs. Distance should increase >20cm = normal test.
44
What management for a pt with Ankylosing Spondylitis?
Drug:
NSAIDS
TNF inhibitors - e.g. infliximab (Monoclonal AB)
IL-17 inhibitors e.g. Secukinumab (Monoclonal AB) if TNF and NSAIDs don't work
```
Non Drug:
Physio
stop smoking
Bisphosphonates if osteoporosis
exercise
treat complications
```
45
How would a pt with Psoriatic Arthritis present?
1. Typical patient ?
2. Pattern ?
1. 10 % pts with hx of psoriasis
- often middle aged.
- Male and females equally affected.
- Nail
- Psoriasis extensor
2. Different patterns! can be symmetrical like RA. Hands/ wrists / ankles /DIP (not MCP like in RA)
- OFTEN Asymmetrical olgio / arthritis- swollen feet / fingers. "Dactylitis" = swollen fingers
46
What signs might you see on a pt with Psoriatic Arthritis?
```
Nail pitting
Onycholysis - nail coming away from bed
Psoritatic plaques
Dactylitis (inflammation of full finger)
Enthesitis (inflammation where tendon inserts into bone)
```
Associated :
EYe - uveitis / conjunctivitis
Aortitis - inflammation of aorta
Amyloidosis
47
What signs might you see on a pt with Psoriatic Arthritis?
```
Nail pitting
Onycholysis - nail coming away from bed
Psoritatic plaques
Dactylitis (inflammation of full finger)
Enthesitis (inflammation where tendon inserts into bone)
```
Associated :
EYe - uveitis / conjunctivitis
Aortitis - inflammation of aorta
Amyloidosis
48
What screening test do patients with Psoriasis complete to see if need to be referred to a rheum?
PEST
Psoriasis Epidemiological Screening tool - asks questions about:
Joint pain
Swelling
Hx of Arthitis
Nail pitting
High score - get you to the rheumatologist.
49
What is arthritis Mutlians? How related to Psoriatic Arthritis?
most severe form of psoriatic arthritis.
Osteolysis of bones around the joints in the phalanxes.
Causes digit to get shorter and skin to fold over the shortened finger - "telescopic finger"
50
What is arthritis Mutlians? How related to Psoriatic Arthritis?
most severe form of psoriatic arthritis.
Osteolysis of bones around the joints in the phalanxes.
Causes digit to get shorter and skin to fold over the shortened finger - "telescopic finger"
51
How do you manage Psoriatic Arthritis?
Rheum and Derm together.
```
Drugs:
NSAID (pain)
DMARDS e.g. methotrexate / sulfasalazine
Anti-TNF eg.g. infliximab
Last line is a IL12/23 Inhibitor Ustekinumab
```
52
What is reactive arthritis?
Synovitis in joints post an infective trigger
53
How does reactive arthritis typically present?
acute monoarthriits (often lower limb asymmetrical)
54
What are some triggers for reactive arthritis?
Z2F: Most common triggers: gastroenteritis and STI (chlamydia)
Distant infection
Gastroenteritis:
Camplyobacter
Shigella
Salmonella
```
STI
Chlamydia Trachomatis (post urethritis / cervicitis)
(Gonorrhoea = gonococcal septic arthritis )
```
55
Associated symptoms with reactive Arthritis ?
Eye: Bilateral conjunctivitis / uveitis
Skin: Circinate Balanitis (dermatitis on head of penis) and urethritis
"can't SEE, can't PEE, cant CLIMB A TREE" as arthritis, eye prob, balanitis
56
Investigations for Reactive Arthritis?
Bloods:
Inflammatory markerts - CRP
Rule out:
Septic arthritis- aspirate, gram stain, culture + sensitivities. GIVE AB until excluded
Crystal arthritis - aspirate to check for gout / pseudo gout
57
Investigations for Reactive Arthritis?
Bloods:
Inflammatory markerts - CRP
Rule out:
Septic / crystal arthritis - aspirate to check for bacteria / crystals
58
Management for Reactive Arthritis?
Treat infection (may not help arthritis)
NSAIDs
Steroid injection to joint
most resolve within 6 months
If reoccurs DMARDS / Anti-TNFA drugs especially likely if HLA-B27 +ve
59
What is Enteropathic arthritis?
10-20% of pts with IBD develop
2/3 get peripheral arthritis
1/3 get axial arthritis
60
With Enteropathic arthritis what are the 2 types of peripheral arthritis and how do they related to IBD flares?
Type 1: correlation with IBD flares - oligoarticular and asymmetric arthritis
Type 2 : NO correlation with IBD flares- poly articular symmetrical arthritis
61
How to treat Enteropathic arthritis? What be mindful of?
NSAIDs can cause IBD flare up.
use DMARDS
TNF inhibitors treat both the bowel disease and arthritis
62
Mnemonic for extra - articular Ankylosing Spondylitis features - 5 As
```
Anterior uveitis
Aortic incompetence
AV block
Apical lung fibrosis
Amyloidosis
```
63
What are features on inflammatory back pain? Mnemonic IPAIN
```
Insidious onset
Pain at night (getting up helps)
Age <40
Improves when exercise
No improvement with rest
```
64
What is Lupus (SLE)?
Inflammatory autoimmune connective tissue disease.
Characterised by inadequate T cell suppressor activity and increased B cell activity.
Anti-nuclear antibodies target proteins in own cell nucleus
Remissions and flares
complex multi organ involvement / varied presentations
65
What are the common symptoms and signs with SLE?
| SOAP BRAIN MD mnemonic
SOAP BRAIN
Serositis -pleurisitis / pericarditis
Oral ulcers - esp palatte, painless
Arthritis - small joint non-erosive
Photosensitivity - malar / discoid rash
Blood disorders - Low WCC, lymphopenia
Renal involvement - glomerulonephritis
Autoantibodies - ANA +ve in 90%
Immunological - low complements e..g C3 C4 low
Neurologic - seizures / psychosis
M - malar rash
D - discoid rash
66
What investigations for SLE?
Bloods:
Raised ESR / plasma viscosity
FBC - normocytic anaemia of chronic disease / leukopenia
Autoantibodies
ANA +ve (90% are)
Anti dsDNA (specific to SLE) rises with active disease
Antiphospholipid antibodies can occur in SLE - VTE risk
Complement
C3 C4 decreased in active disease
Urinanalysis and protein: creatinine ration for proteinuria in lupus nephritis
Skin biopsy / renal biopsy can be diagnostic
67
Why is ESR more useful than CRP in SLE?
Often ESR raised (+ plasma viscosity ) in pts where CRP can be normal
68
Why is FBC particularly useful for SLE?
Simple clue as abnormal in almost all patients
69
Common complications of SLE?
page 220 of Z2F
```
CVD- chronic inflammation in vessels - HTV- CAD
Anaemia of chronic Disease
Pericarditis
Plueritis
Interstitial lung disease
Lupus nephritis
Neuro psychiatric SLE
Recurrent miscarriage
VTE
```
70
How to diagnose SLE?
SLICC or ACR criteria
confirm Antinuclear antibodies and establish clinical features suggestive of SLE
71
Treatment of SLE ?
Drug:
NSADIS
Steroids (prednisolone) short courses for flares
DMARD : hydroxychloroine (rash and arthralgia)
Can move onto Methotrexate / Mycophenolate mofetil / Azathoprine
Biologics for severe disease e.g. Rituximab
Non drug:
Sun avoidance - malar rash
Lifestyle advice - CVD
72
What is Raynaud's pnenomenon? What is the typical colour change
Painful vasospasm of digits. Idiopathic, possibly familial, women ++
Colour change in response to cold stimulus:
White = reduced blood flow
Blue = venous stasis
Red = rewarming hyperaemia
73
What diseases are associated with Raynaud's?
Scleroderma
SLE
Dermatomyositis and polymyositis
Sjorgen's syndrome
>30 yrs when develop think underlying disease
74
Physical causes of Raynaud's?
Physical cause:
heavy vibrating tools
sticky blood e..g cryoglobulinaemia
75
Drug cause of Raynaud's?
Beta blockers
76
Treatment for Raynaud's?
Non drug:
Stay warm
stop smoking
Drug:
Dihydropyridine CCB
Phosphodiesterase-5-inhibitors
Prostacylins
77
Raynaud's episodes usually last ___(1)____
The pattern is ___(2)_____ and ____(3)____
___(4)____ is rare
Raynaud's episodes usually last ___minutes____
The pattern is _bilateral__ and _symmetrical____
___Tissue Necrosis ____ is rare
78
What are some complications of Raynaud's?
Digital ulcers
infection
gangrene
79
What is vasculitis ? How is this big group of conditions divided?
Inflammation of blood vessels.
Divided based on whether they affect :
Small vessels
Medium vessels
Large vessels
80
What are some examples of Small vessel vasculitis?
Microscopic polyangitis (MPA)
Granulomatosis with polyangitis (GPA): resp + kidneys+cANCA
Eosinophillic granulomatosis with polyangitis: assoicated with asthma + eosinophils +pANCA
IgA vasculitis
81
What are some examples of medium vessel vasculitis?
```
Kawasaki disease (KD)
Polyarteritis nodsa (PAN)
```
82
What are some examples of large vessel vasculitis?
```
Giant cell arteritis (GCA)
Takayasu arteritis (TAK)
```
83
what can cause Secondary vasculitis? (mimics vasculitis )
Drugs
Infection
malignancy
connective tissue disorder
84
General Presentation of Vasculitis - features that most types share
```
Purpura (leaking blood vessels)
joint and muscle pain
peripheral neuropathy
ask about Raynaud's
Renal impairment h
GI disturbance (Abdo pain, PR bleeeding, diarrhoea)
Anterior uveitis and scleritis
Hypertension
```
```
Systemic :
Fatigue
fever
weight loss
anorexia
weight loss
```
85
Investigations for Vasculitis
ALERT
Renal involvement - urine dipstick +/- microscopy - glomerulonephritis
Bloods:
FBC, U&Es, LFts, PV, CRP, ESR
serum antibodies :
ANCA - anti neutrophil cytoplasmic antibodies test for vasculitis - p-ANCA and c-ANCA
ANA
RF
p33 of rheum workbook has lots more detail of other tests
86
Management of Vasculitis?
Depends on type - see pg 234 of Z2F for details.
General :
Rule out infection
stop offending drug e.g. secondary cause
1st line : Corticosteroids for target area
e.g. oral prednisolone/ IV hydrocortisone/ nasal/ inhaled
2nd line: Cyclophosphamide
Methotrexate
Azathioprine
rituximab
87
______(1)_____ is a common cause of skin vasculitis due to medication
_____(2)______ can present with vasculitis like syndrome. ECHO will exclude
_____Hypersensitivity vasculitis_____ is a common cause of skin vasculitis due to medication
_____Atrial myxoma (tumour)______ can present with vasculitis like syndrome. ECHO will exclude
88
What is Dermatomyositis and Polymyositis? How would you distinguish them simply?
Autoimmune inflammation of striated muscle. M:F similar peak onset is 40-50 yrs.
Dermatomyositis - muscle weakness and skin rash
Polymyositis - proximal muscle weakness (muscles closest to trunk)
89
Presentation of Dermatomyositis and Polymyositis?
Insidious onset over weeks muscle pain, fatigue and weakness (can be painless)
Bilateral affecting proximal muscles
Shoulder and pelvic girdle affected
Presentation may be accompanied with:
SOB
Rash (Dermatomyositis only)
Raynaud's common
90
What is the relationship between Dermatomyositis and Polymyositis and malignancy?
Can be caused by underlying malignancy (paraneoplastic syndromes). Risk is greatest in 2-3 yrs before and after diagnosis.
common causes:
Lung
Breast
Ovarian
Gastric
91
Diagnostic criteria for Dermatomyositis and Polymyositis?
```
Symmetrical muscle weakness
raised serum enzymes e.g. CK, ALT (muscle w/o other LFTS raised)
Electromyographic changes
Biopsy muscle
Rash of dermatomyositis
```
PM if >3 of first 4
DM if >/= 2 of first 3
Autoantibodies :
Anti-Jo- 1 AB - PM
Anti- Mi-2- AB - DM
ANA AB - DM
92
Key investigation result for diagnosing Dermatomyositis and Polymyositis? What other causes of a raised level of this enzyme?
Creatinine Kinase blood test will be high. Inflammation of muscle cells - released.
>1000 U/L (usually less than 300 U/L)
```
Other causes:
Rhabdomyolysis
AKI
MI
Statins
Strenuous exercise
```
93
What are some of the features of Dermatomyositis skin rash?
Photosensitive rash on light exposed places e.g. scalp, neck, back, shoulders, face - lead to post-inflammation hypo/ hyper pigmentation
Gottron lesion - linear erythematous plaques on back of hands
Purple rash on face and eyelids
Periorbital oedema
Subcutaneous calcinosis ( ca deposits in subcut tissue)
'Mechanics hands' dry cracked palms and fingers
94
Patients with which _S_____ and __S_____ can develop myositis ?
Patients with which __Scleroderma__ and __SLE__ can develop myositis ?
95
Treatment of Dermatomyositis and Polymyositis?
DRUG 1st Line:
Corticosteroids- main in first weeks
DRUGS Long term / steroids not working:
Immunosuppressants :Methotrexate / Azathioprine
IV immunoglobulins
Biologics: infliximab
Monitoring:
CK levels, EMG studies, MRI, Biopsy
NON DRUG:
Physio / Occupational health - muscle strength / function
Sun protection - HCQ may help with rash
Long term condition - help with this
96
A patient is diagnosed with systemic sclerosis. A medical student sat with you in clinic wants to know what that condition is. Summarise the condition for her
It is an autoimmune disease which affects multi-systems of the body.
There is increased activity of fibroblasts (which usually make connective tissue), so you get ABNORMAL growth of connective tissue.
This causes vascular damage and fibrosis.
97
What are the two subtypes of Systemic sclerosis (SSc) aka scleroderma?
Limited SSc, diffuse SSc
98
What other name is given to limited SSc?
CREST syndrome
99
What does CREST in CREST syndrome (i.e. limited SSc) stand for?
```
Calcinosis Cutis
Raynaud's phenomenon
Eoesophageal dysmotility
Sclerodactyly
Telangiectasia
```
100
What is the usual pattern of symptoms in Limited SSc/ crest syndrome?
Have Raynaud's symptoms first, then get scleroderma (thickening of the skin). After about 10 years of these symptoms, get Pulmonary Arterial HTN.
101
How is Diffuse scleroderma/SSc characterised?
Sudden onset of skin involvement. Proximal to the elbows and the knees (- so above elbow/nearer shoulder and above knee - so nearer thigh. )
102
What investigations would you do for pt attending rheumatology clinic with thickening of the skin and PMH of fingers that turn very pale with cold and stress?
They might have CREST syndrome - as have scleroderma and raynauds features. Investigations to do include:
XR of hands - check for calcinosis (the c in crest!)
CXR, PFT, high resolution CT of lungs - check for pulmonary disease
ECG and ECHO - check for PA HTN, HF, myocarditis,arrythmias
Antibody screen - check for positive ANA (+ve in 90% of patients), Anti-centromere antibody in limited SSc, Scl-70 and anti-RNA polymerase III antibodies in diffuse SSc
103
How is systemic sclerosis managed?
No cure for this condition - may need psych support to deal with this.
Manage the symptoms:
For Raynaud's symptoms - calcium antagonist, sildenafil, iloprost infusion
For skin thickening - methotrexate and mycophenolate can reduce this.
HTN crisis - ACEi
Renal failure (related to scleroderma renal crisis) - ACEi
Flare ups - prednisolone
GI symptoms - PPIs
104
Compare and contrast limited (CREST) and diffuse scleroderma
Limited - gradual onset. Diffuse - sudden onset
| Limited - lower mortality. Diffuse - higher mortality
105
What is the pathophysiology of Sjogren's syndrome?
Chronic, autoimmune inflammatory disorder. Inflammation leads to diminished lacrimal and salivary gland secretion.
106
What are the types of Sjogren's syndrome?
Primary - not related to another disease
| Secondary - related to an underlying disease
107
Which gender most commonly presents with Sjogren's syndrome?
Women - up to 80%
108
How can Sjogren's syndrome present?
Common presentation is common! Most common are called sicca symptoms - dry eyes (xerophthalmia), dry mouth (xerostomia) and fatigue.
```
MADFRED mnemonic for all common symptoms
Myalgia
Arthralgia
Dry eyes
Fatigue
Raynaud's phenomenon
Enlarged parotids
Dry mouth
```
109
What investigations would you do for patient presenting with dry eyes, dry mouth and tiredness? You also note her parotid glands are enlarged.
This is a presentation of Sjogren's ! Investigations would include:
Antibody screen - Anti Ro and Anti La antibodies common. May also have RF and anti ds-DNA
Schirmer's test - measure tear volume (would be reduced if pt has Sjogrens where the lacrimal glands are diminished)
Salivary gland biopsy
110
How is Sjogren's managed?
Treated based on symptoms
- educational = avoid dry or smokey atmospheres.
- symptomatic = artificial tears, artificial saliva, skin emollients, vaginal lubricants
- autoimmune profile = immunosuppressants and steroids are rarely needed.
111
What other conditions may a patient with Sjogrens also have?
RA, SLE = v common
| Other autoimmune conditions - coeliac disease, primary biliary cirrhosis, AI Thyroid disease
112
A woman with Sjogrens is pregnant with her first child. What would you have to inform her of, regarding her condition and baby?
Anti Ro antibodies can:
- increase the risk of foetal loss.
- cause complete heart block in the foetus
- cause neonatal lupus syndrome in newborn.
113
A salivary gland biopsy is sometimes taken in suspected Sjogrens patients. What can be seen in the biopsy?
A characteristic picture of Sjogrens - focal lymphocytic infiltration of exocrine glands. Google this for histology slide
114
What is hypermobility?
Where joints move beyond normal limits due to laxity of ligaments, capsules and tendons. Can affect many joints.
115
Who does hyper mobility affect?
People with familial history (but there isn't any genetic testing)
Women, Asian people
116
What is the clinical presentation of hypermobility?
PC: Presents in childhood, or young adulthood. Pain around the joints, worse after activity. Pain is generalised. Fatigued
PMH: Recurrent subluxations and recurrent dislocations
On examination:
- Soft tissue rheumatism, abnormal skin - thin, hyper extensible, striae, marfanoid habits, arachnodactyly, drooping eyelids, myopia, hernias, prolapses or uterine or rectal contents
117
What scoring system is used for Hypermobility?
Beighton score - a point is given for each manoeuvre a pt can do. Score is out of 9.
118
What is the aim of Hypermobility syndrome treatment?
Treated based on improving pain and reducing disability
119
What non-drug treatment is available for hyper mobility?
Strengthening exercises to reduce joint subluxation.
Posture and balance exercises
Splinting
Specialist pain management
120
What pharmacological treatment is available for hyper mobility?
Paracetamol
121
What is a DEXA scan?
Measures the amount of radiation absorbed by the bones - indicating bone mineral density - BMD
122
Where should a DEXA scan reading be done to classify and manage OA?
At the hip - neck of femur to confirm OA and monitor treatment .
123
What scores can bone density be represented as?
Which score is key for the WHO classification of OA?
Z score ( how much bone mineral density falls below mean of pts age)
T score (how much bone mineral density falls below mean of healthy young person)
T SCORE - CLINICALLY IMPORTANT
124
How is OA defined?
Degenerative joint disorder where there is progressive loss of articular cartilage accompanied by new bone formation and capsular fibrosis
125
What are the aetiology possibilities for OA?
Failure of normal cartilage subject to abnormal or incongruous loading for long periods
Damaged or defective cartilage failing under normal conditions of loading
Break up of cartilage due to defective stiffened subchondral hone passing more load to it
126
What are the key features of cartilage in OA
Loss of elasticity with reduced tensile strength
| Cellularity and proteoglycan content are reduced
127
What are the RF for OA?
```
Age- over 65
Women are more symptomatic than men
Obesity- hand and knee
Trauma and joint malalignment
Fhx
```
128
What are the most common joints to be affected by OA?
Hip, knee and spine
129
What are the symptoms of OA?
Pain provoked by movement and weight bearing
Pain starts off intermittent but as it progresses becomes constant
Knee-inactivity gelling and feeling that joint will give way is common
130
What are the xray features of OA?
LOSS
loss of joint space
osteophytes
subchondral scerlosis
subchondral cysts
131
What is the aim of treatment?
(regarding osteoarthritis)
Pain improvement and reduce disability
132
What non-drug therapy is recommended in patients with OA?
Hip and Knee- strengthening and range of movement exercises
Weight loss to reduce joint loading
Laterally wedged insoles or walking stick
133
What pharmacological therapy is given for OA?
Paracetamol is first line
NSAIDs- short term
Topical NSAIDS, topical rubefacients and capsaicin can be used.
Intra- articular corticosteroids can be offered.
134
What surgical therapy is offered in OA?
If physio and pharmatherapy is not helpful- joint replacement surgery can be offered
135
What is fibromyalgia
A common disorder of central pain processing characterized by chronic widespread pain in all 4 quadrants of the body (both sides and above and below the waist)
Allodynia, a heightened and painful response to innocuous stimuli, is often present.
136
What is the pathogenesis of fibromyalgia?
It can be induced by deliberate sleep deprivation.
Reduced REM and delta wave sleep -->causes hyper-activation in response to noxious stimulation, and neural activation in brain regions associated with pain perception in response to non-painful stimuli.
137
How would you assess a patient with osteoporosis?
* Patients are categorised as low, intermediate or high risk based on the risk calculator. For QFracture, this is based on the percentage, and patients above 10% are considered for a DEXA scan.
* For FRAX, this is based on the NOGG guideline chart (linked to on the online FRAX tool), which advises whether to arrange a DEXA scan or start treatment.
These suggestions do not apply to specific groups. For example, NICE CKS (April 2023) suggest:
* A DEXA may be arranged without calculating the risk in patients over 50 with a fragility fracture
* Treatment may be started without a DEXA in patients with a vertebral fracture
In FRAX: Using the T score (Bone mineral density score) use the FRAX assessment tool (plug in RF -AR, sex, age, steroid use) p242 Z2F
138
What Management for osteoporosis ? Non-pharmacological?
Lifestyle:
```
Stop smoking
Avoid falls
Activity and exercise
Good Ca + Vit D intake
Reduce alcohol
Healthy weight
```
139
What management for osteoporosis? Pharmacological?
Vit D + Ca supplements - Calcichew-D3
1st line for osteoperosis
Bisphosphonates e.g. Alendronate 70mg weekly
If above not tolerated :
Denosumab - monoclonal AB that blocks ostoeclasts
HRT for early menopause women
140
How do Bisphosphonates work? What are some side effects ?
MOA: reduce activity of osteoclasts stopping resorption of bone
1. Reflux and oesophageal erosions (empty stomach and sit upright for 30 mins)
2. Atypical fractures e.g. femoral
3. Osteonecrosis of the jaw
4. Osteonecrosis of the external auditory canal
141
Treatment of fibromyalgia ?
Tailor to pain, function and associated symptoms : depression / fatigue / sleep disturbance
Drug:
Low dose Amitryptytline
Pregabalin
CBT
142
What blood tests to rule out other causes of fibromyalgia ?
BLOODS:
ESR, CRP, FBC, U+E, LFT, Ca, CK, TFT
143
What is Gout ?
Crystal arthropathy related to hyperuricemia .
Depositition of monosodium urate crystals in joints and soft tissues.
144
How does Gout present?
The monosodium urate crystals that deposit in soft tissues and joints cause acute and chronic arthritis.
Hot swollen joint
Can affect >1 joint but most commonly 1st metatarsal (podagra)
Soft tissue deposits of uric acid - tophi
urate nephropathy + uric acid stones.
145
Typical joints affected by Gout?
Base of big toe - (metatarso-pharyhgeal joint)
Wrists
Base of thumb (carpo-metacarpal joint)
146
Which type of Gout are older women with OA likely to get?
Pseudogout important DD in diagnosis of gout called by pyrophosphate crystals in older woman with OA
147
What investigation and its result is patho-mnemonic for Gout?
Aspiration of joint will reveal:
```
Monosodium Urate Crystals are PM
also will find:
No bacterial growth
MSU crystals = needle shaped
-ve befringement of polarised light
```
148
What would you seen on XRay of a joint with Gout?
Joint space is maintained
Lytic lesions in bone
Punched out erosions of bone
Erosions can have sclerotic borders with overhanging edges
Joint effusion
149
What is management of acute flare of Gout?
NSAIDs e.g. ibuprofen- 1st line
Colchicine - 2nd line
Steroids - 3rd line
150
What should be used for Gout if NSAIDS are not suitable?
Give an example of pt where NSAIDS not suitable?
Pts not suitable for NSAIDS :
Renal impairment
Significant heart disease
2nd line choice:
Colchicine
Side effects of Colchicine are GI upset and diarrohea
151
What is the prophylactic treatment of Gout? When specifically can you start this treatment?
Allopurinol - urate lowering
Only start once flare up has stopped. Once started can continue through subsequent flares
```
Lifestyle changes:
lose weight
hydration
reduce alcohol
reduce purine based food e.g. meat / seafood
```
152
How would you differenciate between Pseudo-Gout and Gout ?
Aspiration fluid
Pseudo gout: pyrophosphate rhomboid crystals
Gout
Needle shaped monosodium urate crystals
153
What are some non-modifiable and modifiable risk factors for Gout?
Nonmodifiable
* Male sex
* Age over 50 years
* Family history of gout
* Inherited syndrome with uric acid overproduction (eg. Lesch–Nyhan syndrome)
Modifiable
* Obesity
* Hypertension
* Chronic kidney disease
* Diabetes
* Metabolic syndrome
* Medications (eg. thiazide diuretics, ACE inhibitors and aspirin)
154
What is pathognomonic Xray finding in pseudo gout ?
Chondrocalcinosis - thin white line in middle of joint space
Severe cases - joint washout (arthrocentesis)
Joint changes similar to )A
LOSS
L- loss of joint space
O- osteophytes
S- sub articular sclerosis
S- subchondral cysts
155
What is osteoporosis?
low bone mass, deterioration of bone tissue, and disruption of bone architecture that leads to compromised bone strength and an increased risk of fracture
156
Risk factors for osteoporosis?
```
Advanced age (>65 years)
Female gender
Caucasian or south Asians
Fhx
Low body weight (58 kg or body mass index
[BMI] <21)
early menopause (age<45)
- Calcium/vitamin D deficiency
- Inadequate physical activity
- Cigarette smoking
- Excessive alcohol intake (>3 drinks/day)
- Iatrogenic: e.g. corticosteroids, aromatase
inhibitors
```
157
What is the triad of reactive arthritis?
Arthritis, urethritis, conjunctivitis
158
What is the difference between GCA and trigeminal neuralgia?
GCA- chronic vasculitis of large and medium sized vessels in the head whereas TRIGEMINAL NEURALGIA- compression of trigeminal nerve
GCA- presents with headaches, stabbing facial pain and visual disturbances
TRIGEMINAL NEURALGIA- electric shock and knife pain unilaterally
159
Reactive vs Septic arthritis?
Reactive- response to a systemic infection, or post systemic infection e.g. gastroenteritis or chlamydia. No infection in joint
Septic- infection is in the joint e.g staphylococcus aureus
160
Name 2 differentials of poly myalgia rheumatica
Osteoarthritis, rheumatoid arthritis, SLE, myositis, cervical spondylitis, frozen shoulder (in both shoulders), hyper or hypo thyroidism, osteomalacia, fibromyalgia
161
Never give methotrexate and which AB?
Never give methotrexate and trimethoprim
Causes: Bone marrow suppression and severe or fatal pancytopaenia
Pt might present with : infection, bleeding anaemia
Adverse effect made worse by renal impairment
162
You have prescribed a lady alendronic acid as a recent XR shows signs of osteoporosis. What advice would you give her about this drug?
Should take on an empty stomach.
Other drugs and food should be avoided for at least 30mins after taking it.
This is to maximise gut absorption.
Swallow whole with water and avoid bending down for 30mins - reduce indigestion SE.
163
What drug(s) are used for acute attacks of gout or pseudogout?
NSAIDs
Colchicine
Intra articular steroid injections
164
In what patients should colchicine be avoided?
Patients with renal failure
165
What drug is used for prophylaxis of gout/pseudogout?
Allopurinol
166
What is mechanism of action of methotrexate?
Inhibits dihydrofolate reductase.
Relevance - this ^ converts folic acid to tetrahydrofolate which is usually needed for DNA and protein synthesis. - so without it, cells can't replicate (v important in cells actively dividing, e.g. cancer cells).
167
What are ADRs of methotrexate?
V common: Nausea.
| Other: oral ulcers, hair thinning, hepatitis, cirrhosis, pneumonitis, bone marrow suppression
168
Who should not be prescribed methotrexate and why?
Pregnancy - teratogenic.
Severe renal impairment - it is renally excreted
Abnormal liver function - causes hepatotoxicity
169
What is sulfalazine's mechanism of action?
Release 5-aminosalicylic acid (5-ASA) which has anti-inflammatory and immunosuppressive effects
170
What are ADRs of Sulfasalazine?
V common: GI upset
| Other: rash, hepatitis, bone marrow suppression
171
In which patient groups is sulfasalazine contraindicated and why?
In pts with aspirin hypersensitivity. Sulfasalazine and Aspirin are both part of the salicylates group
172
What is the mechanism of action of azathioprine?
Azathioprine is a prodrug (so when metabolised it makes substances that are pharmacologically active). It is metabolised to 6-MP which is further metabolised.
These breakdown products inhibit the synthesis off purines, so inhibit DNA replication.
173
Why is TMPT activity/phenotyping tested in patients before prescribing azathioprine?
1) Levels of TPMT vary amongst people.
2) Metabolism and elimination of azathioprine and its metabolites requires TPMT.
3) If you don't have TPMT, metabolites convert to another substance which can cause myelosuppression - not v good.
174
What are side effects of azathioprine?
GI upset - v common
| Bone marrow suppression
175
What are ADRs of cyclophosphamide?
Bone marrow suppression, infertility and increased cancer risk. Haemorrhagic cystitis
176
What are ADRs of hydroxychloroquine?
V common: GI upset
| Other: retinal pigmentation and loss of vision - rare but need to be screened.
177
What mechanism of action do DMARDs Ciclosporin and tacrolimus have?
Calcineurin inhibitors - these are active against T helper cells.
178
What blood results and other investigation results would you expect in a patient with osteomalacia?
```
Low Ca
Low Vit D
Low Phosphate
High ALP
High PTH (2nd hyperparathyroidism)
```
Other :
Xray - looser lines - radiolucent bones and osteopenia
DEXA: low mineral bone density
179
Characteristic gait of a patient with osteomalacia?
waddling gait
180
Which medical conditions put patients at risk of developing Vit D deficiency and therefore osteomalacia?
```
Malabsorption disorders e.g. IBD Chrons
Lack of sunlight
diet
CKD - kidneys needed to metabolise to active form
Drug - anticonvulsants
Inherited - hypophosphatemic rickets
liver disease - cirrhosis
```
181
What risk factors for Vit D deficiency in population (therefore getting osteomalacia?)
```
Darker skin
Stay in doors a lot
colder / northern climates
Wear covered clothing
low exposure to sunlight
```
182
1. Briefly explain relationship between Vit D, Ca, phosphate
| 2. How they become deranged in osteomalacia?
1. Vit D essential for Ca and Phos absorption from intestines and kidney.
2. Low vit D leads to lack of Ca and Phos in blood causing defective bone mineralisation.
Low Ca causes increased PTH (2nd hyperparathyroidism) which makes mineralisation problem worse as it stimulates Ca resorption from bones.
183
Symptoms of osteomalacia?
```
fatigue
Proximal myopathy - waddling gait
Muscle /bone tenderness
Bone pain
Pathological / abnormal fractures
```
184
Treatment of osteomalacia?
```
Vitamin D (colecalciferol) e.g. 50,000 IU weekly for 6w
Vit D serum below 25 nmol/L is deficient
```
185
Define osteomalacia
Defective bone mineralisation causing soft bones in adults due to low vitamin D. When this happens in children before growth plates have closed it is called Rickets.
186
what type of anaemia can sulfasalazine cause?
Heinz body
187
What drug can cause gingival enlargement?
cyclosporin
188
DMARD that causes myelosupression
Azthioprine
Methotrexate- worsened by renal impairment and certain drugs eg trimethoprim
189
Felty’s syndrome?
triad of RA, low WCC and splenomegaly
190
Xray changes in psoriatic arthiritis?
Dactylitis - soft tissue swelling as whole digit is inflammed
'Pencil in cup' appearance
Osteolysis - destruction of bone
Periostitis - inflammation of periosteum thick border
Ankylosis - bones join together - stiff
191
What is scoring system for RA?
```
DAS28 is disease activity score:
1. Swollen joints
2. Tender joints
3. Raised ESR / CRP
Used to monitor progression and response to treatment
```
Diagnostic score is ACR score:
1. Joint involvement (lots of small joints score higher)
2. Presence of AB - RF / Anti-CCP
3. Inflammatory markers - raised ESR / CRP
4. Duration of symptoms >6 weeks
Score > or = to 6 = diagnose RA
192
Which drugs can increase risk of gout?
Diuretics- particularly thiazide like and loop
pyrazinamide
ciclosporin
tarcolimus
193
Signs and symptoms of fibromyalgia?
```
Unrefreshed sleep
Joint and muscle stiffness
Profound fatigue
Numbness
Headaches
IBS/ Bladder syndrome
Depression and anxiety
Poor concentration and memory fibrofog.
```
194
Risk factors of fibromyalgia?
Female
Age - 40-50 usually
May have obvious trigger = emotional trauma, physical e.g. painful arthritis.
195
Allopurinol - mechanism of action?
Xanthine oxidase inhibitor so reduces rate formation.
196
Compare gout and pseudo gout?
Gout = male, needle shaped negatively birefringent monosodium rate crystals (under polarised light)
Pseudogout = women, usually with PMH of OA. Weakly positive birefringent rhomboid crystals made of calcium pyrophosphate
197
Late XR findings of gout?
Joint effusion
Punched out erosions
Reduced joint space
