RHEUMATOLOGY Flashcards

1
Q

Define rheumatoid arthritis

A

An autoimmune disease associated to Fc portion of IgG (RF) and anti-CCP

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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!)

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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
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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

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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.

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6
Q

Why is a WCC done in blood test for suspected RA?

A

Can be elevated due to complication of septic arthritis

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7
Q

Why is FBC carried out as an investigation for RA?

A

May show normocytic anaemia which is a feature of chronic disease

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8
Q

How is RA initially treated?

A

DMARD monotherapy - methotrexate

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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
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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

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11
Q

What features are characteristically seen in an XRAY of RA?

A
LESS 
Loss of joint space 
Erosions (periarticular)
Soft tissue swelling 
Subluxation
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12
Q

Define giant cell arteritis

A

Chronic vasculitis of large and medium sized vessels in individuals over 50yrs.

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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

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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

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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

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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
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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
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18
Q

What is your immediate managment in pt with suspected GCA?

A

Steroid therapy

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19
Q

How is polymyalgia rheumatica (PMR) characterised?

A

Pain and stiffness in shoulder, hip girdles and neck.

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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)

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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

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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

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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

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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

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25
Q

Give the medical term for describing the number of joints involved for the below:

(1) 1 joint
(2) 2-4 joints
(3) >4 joints

A

(1) monoarticular
(2) Oligoarticular / pauciarticular
(3) Polyarticular

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26
Q

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
A
  1. Inflammatory arthritis
    - RA
    - PsA
    - Reactive arthritis
  2. Autoimmune arthritis
    - SLE
    - Vasculitis
  3. Viral infection
    - HIV
    - Parovirus
    Chikungunya
  4. Crystal arthritis
    - UNcontrolled Gout
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27
Q

What are the causes of chronic monoarthritis? Use categories below to think of specific conditions

  1. Infections
  2. Inflammatory
  3. Non- inflammatory
  4. Tumours
A
  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!
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28
Q

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.

A

(1) PsA
(2) OA
(3) Heberden’s Nodes affecting the DIPJ in OA

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29
Q

What investigations may you consider for pt with suspected PMR?

A

Bloods - ESR, CRP, polycythemia vera

Temporal artery biopsyy if symptoms of GCA

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30
Q

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

A

__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.

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31
Q

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

A

Seronegative Spondyloarthropathy is associated with:

  • Anterior uveitis
  • Psoriasis
  • IBD

Hx of STI / Diarrhoea could indicate:

  • reactive arthritis
  • gonococcal arthritis
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32
Q

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

A

Smoking is implicated in causing and making ___RA____more severe

Patients with ____Raynaud’s ____ symptoms in the hands should be advised to stop smoking

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33
Q

Compare and Contrast the features of Inflammatory vs Mechanical disease.

E.g. Morning stiffness / effect of activity / effect of resting / Fatigue/ systemic involvement

A
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
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34
Q

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?

A

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

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35
Q

What are some extra - articular features of SLE?

LOADS IN THE BOOKLET - GUESS A FEW systems based!

A

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

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36
Q

What are some extra - articular features of RA?

A

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

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37
Q

What are some extra - articular features of RA?

A

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

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38
Q

What are some extra - articular features of Sjogrens Syndrome?

A

Mouth / Eyes
- Dry mouth / eyes

Skin
- Raynaud’s

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39
Q

What gene are the Spondyloarthropathies associated with?

A

HLA - B27

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40
Q

What gene are the Spondyloarthropathies associated with?

A

HLA - B27

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41
Q

How would a patient with Ankylosing Spondylitis Present?

  1. Typical pt
  2. Presenting symptoms
A
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
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42
Q

Examining a pt with Ankylosing Spondylitis

  1. Early examination vs later on
  2. What test might you perform and what is normal?
A
  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
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43
Q

Examining a pt with Ankylosing Spondylitis

  1. Early examination vs later on
  2. What test might you perform and what is normal?
A
  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.
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44
Q

What management for a pt with Ankylosing Spondylitis?

A

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
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45
Q

How would a pt with Psoriatic Arthritis present?

  1. Typical patient ?
  2. Pattern ?
A
  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
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46
Q

What signs might you see on a pt with Psoriatic Arthritis?

A
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

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47
Q

What signs might you see on a pt with Psoriatic Arthritis?

A
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

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48
Q

What screening test do patients with Psoriasis complete to see if need to be referred to a rheum?

A

PEST
Psoriasis Epidemiological Screening tool - asks questions about:

Joint pain
Swelling
Hx of Arthitis
Nail pitting

High score - get you to the rheumatologist.

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49
Q

What is arthritis Mutlians? How related to Psoriatic Arthritis?

A

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”

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50
Q

What is arthritis Mutlians? How related to Psoriatic Arthritis?

A

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”

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51
Q

How do you manage Psoriatic Arthritis?

A

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
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52
Q

What is reactive arthritis?

A

Synovitis in joints post an infective trigger

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53
Q

How does reactive arthritis typically present?

A

acute monoarthriits (often lower limb asymmetrical)

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54
Q

What are some triggers for reactive arthritis?

A

Z2F: Most common triggers: gastroenteritis and STI (chlamydia)

Distant infection

Gastroenteritis:
Camplyobacter
Shigella
Salmonella

STI 
Chlamydia Trachomatis (post urethritis / cervicitis)
(Gonorrhoea = gonococcal septic arthritis )
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55
Q

Associated symptoms with reactive Arthritis ?

A

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

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56
Q

Investigations for Reactive Arthritis?

A

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

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57
Q

Investigations for Reactive Arthritis?

A

Bloods:
Inflammatory markerts - CRP

Rule out:
Septic / crystal arthritis - aspirate to check for bacteria / crystals

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58
Q

Management for Reactive Arthritis?

A

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

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59
Q

What is Enteropathic arthritis?

A

10-20% of pts with IBD develop

2/3 get peripheral arthritis
1/3 get axial arthritis

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60
Q

With Enteropathic arthritis what are the 2 types of peripheral arthritis and how do they related to IBD flares?

A

Type 1: correlation with IBD flares - oligoarticular and asymmetric arthritis

Type 2 : NO correlation with IBD flares- poly articular symmetrical arthritis

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61
Q

How to treat Enteropathic arthritis? What be mindful of?

A

NSAIDs can cause IBD flare up.

use DMARDS

TNF inhibitors treat both the bowel disease and arthritis

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62
Q

Mnemonic for extra - articular Ankylosing Spondylitis features - 5 As

A
Anterior uveitis 
Aortic incompetence  
AV block 
Apical lung fibrosis 
Amyloidosis
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63
Q

What are features on inflammatory back pain? Mnemonic IPAIN

A
Insidious onset
Pain at night (getting up helps)
Age <40
Improves when exercise
No improvement with rest
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64
Q

What is Lupus (SLE)?

A

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

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65
Q

What are the common symptoms and signs with SLE?

SOAP BRAIN MD mnemonic

A

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

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66
Q

What investigations for SLE?

A

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

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67
Q

Why is ESR more useful than CRP in SLE?

A

Often ESR raised (+ plasma viscosity ) in pts where CRP can be normal

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68
Q

Why is FBC particularly useful for SLE?

A

Simple clue as abnormal in almost all patients

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69
Q

Common complications of SLE?

A

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
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70
Q

How to diagnose SLE?

A

SLICC or ACR criteria

confirm Antinuclear antibodies and establish clinical features suggestive of SLE

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71
Q

Treatment of SLE ?

A

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

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72
Q

What is Raynaud’s pnenomenon? What is the typical colour change

A

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

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73
Q

What diseases are associated with Raynaud’s?

A

Scleroderma
SLE
Dermatomyositis and polymyositis
Sjorgen’s syndrome

> 30 yrs when develop think underlying disease

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74
Q

Physical causes of Raynaud’s?

A

Physical cause:
heavy vibrating tools
sticky blood e..g cryoglobulinaemia

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75
Q

Drug cause of Raynaud’s?

A

Beta blockers

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76
Q

Treatment for Raynaud’s?

A

Non drug:
Stay warm
stop smoking

Drug:
Dihydropyridine CCB
Phosphodiesterase-5-inhibitors
Prostacylins

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77
Q

Raynaud’s episodes usually last ___(1)____
The pattern is ___(2)_____ and ____(3)____
___(4)____ is rare

A

Raynaud’s episodes usually last ___minutes____
The pattern is _bilateral__ and _symmetrical____
___Tissue Necrosis ____ is rare

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78
Q

What are some complications of Raynaud’s?

A

Digital ulcers
infection
gangrene

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79
Q

What is vasculitis ? How is this big group of conditions divided?

A

Inflammation of blood vessels.

Divided based on whether they affect :
Small vessels
Medium vessels
Large vessels

80
Q

What are some examples of Small vessel vasculitis?

A

Microscopic polyangitis (MPA)

Granulomatosis with polyangitis (GPA): resp + kidneys+cANCA

Eosinophillic granulomatosis with polyangitis: assoicated with asthma + eosinophils +pANCA

IgA vasculitis

81
Q

What are some examples of medium vessel vasculitis?

A
Kawasaki disease (KD)
Polyarteritis nodsa (PAN)
82
Q

What are some examples of large vessel vasculitis?

A
Giant cell arteritis (GCA) 
Takayasu arteritis (TAK)
83
Q

what can cause Secondary vasculitis? (mimics vasculitis )

A

Drugs
Infection
malignancy
connective tissue disorder

84
Q

General Presentation of Vasculitis - features that most types share

A
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
Q

Investigations for Vasculitis

A

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
Q

Management of Vasculitis?

A

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
Q

______(1)_____ is a common cause of skin vasculitis due to medication

_____(2)______ can present with vasculitis like syndrome. ECHO will exclude

A

_____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
Q

What is Dermatomyositis and Polymyositis? How would you distinguish them simply?

A

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
Q

Presentation of Dermatomyositis and Polymyositis?

A

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
Q

What is the relationship between Dermatomyositis and Polymyositis and malignancy?

A

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
Q

Diagnostic criteria for Dermatomyositis and Polymyositis?

A
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
Q

Key investigation result for diagnosing Dermatomyositis and Polymyositis? What other causes of a raised level of this enzyme?

A

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
Q

What are some of the features of Dermatomyositis skin rash?

A

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
Q

Patients with which _S_____ and __S_____ can develop myositis ?

A

Patients with which __Scleroderma__ and __SLE__ can develop myositis ?

95
Q

Treatment of Dermatomyositis and Polymyositis?

A

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
Q

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

A

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
Q

What are the two subtypes of Systemic sclerosis (SSc) aka scleroderma?

A

Limited SSc, diffuse SSc

98
Q

What other name is given to limited SSc?

A

CREST syndrome

99
Q

What does CREST in CREST syndrome (i.e. limited SSc) stand for?

A
Calcinosis Cutis 
Raynaud's phenomenon 
Eoesophageal dysmotility 
Sclerodactyly 
Telangiectasia
100
Q

What is the usual pattern of symptoms in Limited SSc/ crest syndrome?

A

Have Raynaud’s symptoms first, then get scleroderma (thickening of the skin). After about 10 years of these symptoms, get Pulmonary Arterial HTN.

101
Q

How is Diffuse scleroderma/SSc characterised?

A

Sudden onset of skin involvement. Proximal to the elbows and the knees (- so above elbow/nearer shoulder and above knee - so nearer thigh. )

102
Q

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?

A

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
Q

How is systemic sclerosis managed?

A

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
Q

Compare and contrast limited (CREST) and diffuse scleroderma

A

Limited - gradual onset. Diffuse - sudden onset

Limited - lower mortality. Diffuse - higher mortality

105
Q

What is the pathophysiology of Sjogren’s syndrome?

A

Chronic, autoimmune inflammatory disorder. Inflammation leads to diminished lacrimal and salivary gland secretion.

106
Q

What are the types of Sjogren’s syndrome?

A

Primary - not related to another disease

Secondary - related to an underlying disease

107
Q

Which gender most commonly presents with Sjogren’s syndrome?

A

Women - up to 80%

108
Q

How can Sjogren’s syndrome present?

A

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
Q

What investigations would you do for patient presenting with dry eyes, dry mouth and tiredness? You also note her parotid glands are enlarged.

A

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
Q

How is Sjogren’s managed?

A

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
Q

What other conditions may a patient with Sjogrens also have?

A

RA, SLE = v common

Other autoimmune conditions - coeliac disease, primary biliary cirrhosis, AI Thyroid disease

112
Q

A woman with Sjogrens is pregnant with her first child. What would you have to inform her of, regarding her condition and baby?

A

Anti Ro antibodies can:

  • increase the risk of foetal loss.
  • cause complete heart block in the foetus
  • cause neonatal lupus syndrome in newborn.
113
Q

A salivary gland biopsy is sometimes taken in suspected Sjogrens patients. What can be seen in the biopsy?

A

A characteristic picture of Sjogrens - focal lymphocytic infiltration of exocrine glands. Google this for histology slide

114
Q

What is hypermobility?

A

Where joints move beyond normal limits due to laxity of ligaments, capsules and tendons. Can affect many joints.

115
Q

Who does hyper mobility affect?

A

People with familial history (but there isn’t any genetic testing)
Women, Asian people

116
Q

What is the clinical presentation of hypermobility?

A

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
Q

What scoring system is used for Hypermobility?

A

Beighton score - a point is given for each manoeuvre a pt can do. Score is out of 9.

118
Q

What is the aim of Hypermobility syndrome treatment?

A

Treated based on improving pain and reducing disability

119
Q

What non-drug treatment is available for hyper mobility?

A

Strengthening exercises to reduce joint subluxation.
Posture and balance exercises
Splinting
Specialist pain management

120
Q

What pharmacological treatment is available for hyper mobility?

A

Paracetamol

121
Q

What is a DEXA scan?

A

Measures the amount of radiation absorbed by the bones - indicating bone mineral density - BMD

122
Q

Where should a DEXA scan reading be done to classify and manage OA?

A

At the hip - neck of femur to confirm OA and monitor treatment .

123
Q

What scores can bone density be represented as?

Which score is key for the WHO classification of OA?

A

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
Q

How is OA defined?

A

Degenerative joint disorder where there is progressive loss of articular cartilage accompanied by new bone formation and capsular fibrosis

125
Q

What are the aetiology possibilities for OA?

A

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
Q

What are the key features of cartilage in OA

A

Loss of elasticity with reduced tensile strength

Cellularity and proteoglycan content are reduced

127
Q

What are the RF for OA?

A
Age- over 65 
Women are more symptomatic than men 
Obesity- hand and knee 
Trauma and joint malalignment  
Fhx
128
Q

What are the most common joints to be affected by OA?

A

Hip, knee and spine

129
Q

What are the symptoms of OA?

A

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
Q

What are the xray features of OA?

A

LOSS

loss of joint space
osteophytes
subchondral scerlosis
subchondral cysts

131
Q

What is the aim of treatment?
(regarding osteoarthritis)

A

Pain improvement and reduce disability

132
Q

What non-drug therapy is recommended in patients with OA?

A

Hip and Knee- strengthening and range of movement exercises
Weight loss to reduce joint loading
Laterally wedged insoles or walking stick

133
Q

What pharmacological therapy is given for OA?

A

Paracetamol is first line
NSAIDs- short term
Topical NSAIDS, topical rubefacients and capsaicin can be used.
Intra- articular corticosteroids can be offered.

134
Q

What surgical therapy is offered in OA?

A

If physio and pharmatherapy is not helpful- joint replacement surgery can be offered

135
Q

What is fibromyalgia

A

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
Q

What is the pathogenesis of fibromyalgia?

A

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
Q

How would you assess a patient with osteoporosis?

A
  • 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
Q

What Management for osteoporosis ? Non-pharmacological?

A

Lifestyle:

Stop smoking 
Avoid falls
Activity and exercise 
Good Ca + Vit D intake 
Reduce alcohol 
Healthy weight
139
Q

What management for osteoporosis? Pharmacological?

A

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
Q

How do Bisphosphonates work? What are some side effects ?

A

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
Q

Treatment of fibromyalgia ?

A

Tailor to pain, function and associated symptoms : depression / fatigue / sleep disturbance

Drug:
Low dose Amitryptytline
Pregabalin
CBT

142
Q

What blood tests to rule out other causes of fibromyalgia ?

A

BLOODS:

ESR, CRP, FBC, U+E, LFT, Ca, CK, TFT

143
Q

What is Gout ?

A

Crystal arthropathy related to hyperuricemia .

Depositition of monosodium urate crystals in joints and soft tissues.

144
Q

How does Gout present?

A

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
Q

Typical joints affected by Gout?

A

Base of big toe - (metatarso-pharyhgeal joint)

Wrists

Base of thumb (carpo-metacarpal joint)

146
Q

Which type of Gout are older women with OA likely to get?

A

Pseudogout important DD in diagnosis of gout called by pyrophosphate crystals in older woman with OA

147
Q

What investigation and its result is patho-mnemonic for Gout?

A

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
Q

What would you seen on XRay of a joint with Gout?

A

Joint space is maintained

Lytic lesions in bone

Punched out erosions of bone

Erosions can have sclerotic borders with overhanging edges

Joint effusion

149
Q

What is management of acute flare of Gout?

A

NSAIDs e.g. ibuprofen- 1st line

Colchicine - 2nd line

Steroids - 3rd line

150
Q

What should be used for Gout if NSAIDS are not suitable?

Give an example of pt where NSAIDS not suitable?

A

Pts not suitable for NSAIDS :
Renal impairment
Significant heart disease

2nd line choice:
Colchicine

Side effects of Colchicine are GI upset and diarrohea

151
Q

What is the prophylactic treatment of Gout? When specifically can you start this treatment?

A

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
Q

How would you differenciate between Pseudo-Gout and Gout ?

A

Aspiration fluid

Pseudo gout: pyrophosphate rhomboid crystals

Gout
Needle shaped monosodium urate crystals

153
Q

What are some non-modifiable and modifiable risk factors for Gout?

A

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
Q

What is pathognomonic Xray finding in pseudo gout ?

A

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
Q

What is osteoporosis?

A

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
Q

Risk factors for osteoporosis?

A
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
Q

What is the triad of reactive arthritis?

A

Arthritis, urethritis, conjunctivitis

158
Q

What is the difference between GCA and trigeminal neuralgia?

A

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
Q

Reactive vs Septic arthritis?

A

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
Q

Name 2 differentials of poly myalgia rheumatica

A

Osteoarthritis, rheumatoid arthritis, SLE, myositis, cervical spondylitis, frozen shoulder (in both shoulders), hyper or hypo thyroidism, osteomalacia, fibromyalgia

161
Q

Never give methotrexate and which AB?

A

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
Q

You have prescribed a lady alendronic acid as a recent XR shows signs of osteoporosis. What advice would you give her about this drug?

A

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
Q

What drug(s) are used for acute attacks of gout or pseudogout?

A

NSAIDs
Colchicine
Intra articular steroid injections

164
Q

In what patients should colchicine be avoided?

A

Patients with renal failure

165
Q

What drug is used for prophylaxis of gout/pseudogout?

A

Allopurinol

166
Q

What is mechanism of action of methotrexate?

A

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
Q

What are ADRs of methotrexate?

A

V common: Nausea.

Other: oral ulcers, hair thinning, hepatitis, cirrhosis, pneumonitis, bone marrow suppression

168
Q

Who should not be prescribed methotrexate and why?

A

Pregnancy - teratogenic.
Severe renal impairment - it is renally excreted
Abnormal liver function - causes hepatotoxicity

169
Q

What is sulfalazine’s mechanism of action?

A

Release 5-aminosalicylic acid (5-ASA) which has anti-inflammatory and immunosuppressive effects

170
Q

What are ADRs of Sulfasalazine?

A

V common: GI upset

Other: rash, hepatitis, bone marrow suppression

171
Q

In which patient groups is sulfasalazine contraindicated and why?

A

In pts with aspirin hypersensitivity. Sulfasalazine and Aspirin are both part of the salicylates group

172
Q

What is the mechanism of action of azathioprine?

A

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
Q

Why is TMPT activity/phenotyping tested in patients before prescribing azathioprine?

A

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
Q

What are side effects of azathioprine?

A

GI upset - v common

Bone marrow suppression

175
Q

What are ADRs of cyclophosphamide?

A

Bone marrow suppression, infertility and increased cancer risk. Haemorrhagic cystitis

176
Q

What are ADRs of hydroxychloroquine?

A

V common: GI upset

Other: retinal pigmentation and loss of vision - rare but need to be screened.

177
Q

What mechanism of action do DMARDs Ciclosporin and tacrolimus have?

A

Calcineurin inhibitors - these are active against T helper cells.

178
Q

What blood results and other investigation results would you expect in a patient with osteomalacia?

A
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
Q

Characteristic gait of a patient with osteomalacia?

A

waddling gait

180
Q

Which medical conditions put patients at risk of developing Vit D deficiency and therefore osteomalacia?

A
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
Q

What risk factors for Vit D deficiency in population (therefore getting osteomalacia?)

A
Darker skin
Stay in doors a lot
colder / northern climates
Wear covered clothing 
low exposure to sunlight
182
Q
  1. Briefly explain relationship between Vit D, Ca, phosphate

2. How they become deranged in osteomalacia?

A
  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
Q

Symptoms of osteomalacia?

A
fatigue
Proximal myopathy - waddling gait 
Muscle /bone tenderness
Bone pain 
Pathological / abnormal fractures
184
Q

Treatment of osteomalacia?

A
Vitamin D (colecalciferol) e.g. 50,000 IU weekly for 6w
Vit D serum below 25 nmol/L is deficient
185
Q

Define osteomalacia

A

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
Q

what type of anaemia can sulfasalazine cause?

A

Heinz body

187
Q

What drug can cause gingival enlargement?

A

cyclosporin

188
Q

DMARD that causes myelosupression

A

Azthioprine

Methotrexate- worsened by renal impairment and certain drugs eg trimethoprim

189
Q

Felty’s syndrome?

A

triad of RA, low WCC and splenomegaly

190
Q

Xray changes in psoriatic arthiritis?

A

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
Q

What is scoring system for RA?

A
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
Q

Which drugs can increase risk of gout?

A

Diuretics- particularly thiazide like and loop
pyrazinamide
ciclosporin
tarcolimus

193
Q

Signs and symptoms of fibromyalgia?

A
Unrefreshed sleep 
Joint and muscle stiffness 
Profound fatigue 
Numbness 
Headaches 
IBS/ Bladder syndrome 
Depression and anxiety 
Poor concentration and memory fibrofog.
194
Q

Risk factors of fibromyalgia?

A

Female
Age - 40-50 usually
May have obvious trigger = emotional trauma, physical e.g. painful arthritis.

195
Q

Allopurinol - mechanism of action?

A

Xanthine oxidase inhibitor so reduces rate formation.

196
Q

Compare gout and pseudo gout?

A

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
Q

Late XR findings of gout?

A

Joint effusion
Punched out erosions
Reduced joint space