Rheumatology Flashcards

1
Q

Radiological features of OA

A

 Loss of joint space
 Osteophytes
 Subchondral cysts
 Subchondral sclerosis

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2
Q

Radiological features of RA

A
 Loss of joint space
 Soft tissue swelling
 Peri-articular osteopenia
 Deformity
 Subluxation
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3
Q

Radiological features of Gout

A

 Normal joint space
 Soft tissue swelling
 Periarticular erosions

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4
Q

Red flags for back pain

A
 Age <20 or >55yrs
 Neurological disturbance (inc. sciatica)
 Sphincter disturbance/ urinary retention
 Saddle anaesthesia
 Bilateral or alternating leg pain/ Leg claudication
 Current or recent infection
 Fever, wt. loss, night sweats
 History of malignancy
 Thoracic back pain
 Morning stiffness
 Acute onset in elderly people
 Constant or progressive pain
 Nocturnal pain
 Abdo mass
 Immunosupressed
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5
Q

Causes of back pain

A
Mechanical
 Strain/idiopathic
 Trauma
 Pregnancy
 Disc prolapse 
 Spondylolisthesis (forward shift of one vertebra)

Degenerative: spondylosis, vertebral collapse, stenosis

Inflammatory: Ank spond, Paget’s

Neoplasm: Mets, myeloma

Infection: TB, abscess

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6
Q

Describe what a disc prolapse is and the presenting features

A

Herniation of nucleus pulposus through annulus fibrosus
- often L4/5 or L5/S1
- severe pain on sneezing, coughing or twisting a few days after low back strain
 Lumbago: low back pain
 Sciatica: shooting radicular pain down buttock and thigh

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7
Q

Signs of disc prolapse

A
 Limited spinal flexion and extension
 Free lateral flexion
 Pain on straight-leg raise
 Lateral herniation → radiculopathy
 Central herniation → corda equina syndrome
L4/5 → L5 Root Compression
 Weak hallux extension ± foot drop
 weak inversion (tib. post.) helps distinguish from
peroneal N. palsy.
 ↓ sensation on inner dorsum of foot
L5/S1 → S1 Root Compression
 Weak foot plantarflexion and eversion
 Loss of ankle-jerk
 Calf pain
 ↓ sensation over sole of foot and back of calf
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8
Q

Ix and Rx of Disc Prolapse

A

Ix: MRI (emergency if cauda equina)

Rx
 Brief rest, analgesia and mobilisation effective in ≥90%
+/- steroid injection
 Surgical: discectomy or laminectomy (microscopic-resection of nucleus pulposus) may be needed in cauda-equina syndrome, continuing pain or muscle
weakness.

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9
Q

Presenting features of spondylolisthesis

A

 Displacement of one lumbar vertebra on another
 Usually forward
 Usually L5 on S1
 May be palpable

Presentation
 Onset of pain usually in adolescence or early adulthood
 Worse on standing
 ± sciatica, hamstring tightness, abnormal gait

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10
Q

Causes of spondylolisthesis

A

 Congenital malformation
 Spondylosis
 Osteoarthritis

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11
Q

Dx and Rx of spondylolisthesis

A

Dx
 Plain radiography

Rx
 Corset
 Nerve release
 Spinal fusion

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12
Q

Features and presentation of Spinal Stenosis

A

 Developmental predisposition ± facet joint OA
→ generalized narrowing of lumbar spinal canal.
Presentation
 Spinal claudication
 Aching or heavy buttock and lower limb pain on walking
 Rapid onset
 +/- paraesthesiae/numbness
 Pain eased by leaning forward (e.g. on bike)
 Pain on spine extension

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13
Q

Ix and Rx 0f Spinal Stenosis

A

Ix –> MRI

Rx
 Corsets
 NSAIDs
 Epidural steroid injection
 Canal decompression surgery
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14
Q

How does presentation of nerve root lesions differ from L2-S1

A

L2 - weak Hip flexion + adduction
L3 - weak Knee extension +
Hip adduction
–> ↓ Knee Jerk
L4 - weak Foot inversion + dorsiflexion + Knee extension
–> ↓ Knee Jerk
L5 - weak - Great toe dorsiflexion + Foot inversion + dorsiflexion + Knee Flexion
Hip extension + abduction
S1- weak - Foot eversion +
Foot and toe plantarflexion + Knee flexion
–> ↓ Ankle Jerk

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15
Q

Ix for back pain

A

IF RED FLAGS
FBC, ESR, CRP , ALP , se electrophoresis, PSA

MRI

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16
Q

General Mx of back pain

A

Conservative
 Max 2d bed rest
 Education: keep active, how to lift / stoop
 Physiotherapy
 Psychosocial issues re. chronic pain and disability
 Warmth

Medical
 Analgesia: paracetamol ± NSAIDs ± codeine
 Muscle relaxant: low-dose diazepam (short-term)
 Facet joint injections

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17
Q

Acute cord compression presentation

A

 Bilateral pain: back and radicular
 LMN signs at compression level
 UMN signs and sensory level below compression
 Sphincter disturbance

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18
Q

Acute Cauda Equina Compression presentation

A

 Alternating or bilateral radicular pain in the legs
 Saddle anaesthesia
 Loss of anal tone
 Bladder ± bowel incontinence

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19
Q

Ix Mx of acute cord/ cauda equina compression

A
  • bldder scan, MRI,

 Large prolapse: laminectomy / discectomy
 Tumours: radiotherapy and steroids (if hx cancer) - and stabilise spine
 Abscesses: decompression

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20
Q

Causes of acute cord/ cauda equina compression

A
  • bony met
  • large disc protrusion
  • myeloma
  • cord/ paraspinal tumpour
  • TB
  • Abscess
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21
Q

Yellow flags for back pain

A
Attitudes
Beliefs
Compensation
Diagnosis
Emotions
Family hx

Work

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22
Q

UMN v LMN lesions

A

UMN

  • hypertonia
  • hyperreflexia
  • clonus
  • +ve babinski
  • clasp - knife relflex
  • +/- weakness

LMN =

  • Hypotonia
  • hyporeflexia
  • wasting
  • fasciculations
  • weakness
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23
Q

Define OA

A

Degenerative joint disorder in which there is
progressive loss of hyaline cartilage and new bone
formation at the joint surface and its margin.

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24
Q

Risk factors for OA

A
 Age (80% > 75yrs)
 Obesity
 Joint abnormality
 Smoking
 Vit D def
 Hormone status
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25
Q

Classification of OA

A

 Primary: no underlying cause

 Secondary: obesity, joint abnormality (Trauma, AVN, RA, Gout, Pagets, Perthes, SUFE), acromegaly, haemophilia

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26
Q

Symptoms of OA

A

 Affects: knees, hips, DIPs, PIPs, thumb CMC
 Pain: worse ̄c movement, background rest/night pain,
worse @ end of day.
 Stiffness: especially after rest, lasts ~30min (e.g. AM)
+/.- crepitus
 Deformity and instability
 ↓ ROM
 Locking (loos intra-articular bodies)
+/- mild synovitis

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27
Q

Signs and Ix in OA

A

 Bouchard’s (prox), Heberden’s (dist.) nodes
 Thumb CMC squaring
 Fixed flexion deformity

  • CRP may be mildly elevated, rest normal
  • MRI - synovial thickening, effusions, oedema, periarticular lesions
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28
Q

Pathophysiology of OA

A

 Softening of articular cartilage → fraying and fissuring
of smooth surface → underlying bone exposure.
 Subchondral bone becomes sclerotic c¯ cysts.
 Proliferation and ossification of cartilage in unstressed
areas → osteophytes.
 Capsular fibrosis → stiff joints.

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29
Q

Differentials of OA

A

 Septic  Crystal  Trauma

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30
Q

Features in the hx in OA

A
 Pain severity, night pain
 Walking distance
 Analgesic requirements
 ADLs and social circumstances
 Co-morbidities
 Underlying causes: trauma, infection, congenital
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31
Q

Mx of OA

A

CONSERVATIVE
 ↓wt.
 Alter activities: ↑ rest, ↓ sport
 Physio: muscle strengthening & joint mobility
 Walking aids, supportive footwear, home mods - ↓ load
 Education
 Hot/cold packs

MEDICAL

1) Paracetamol +/- Topical NSAIDs
2) Codiene
3) NSAIDS +/- PPI protection

 Joint injection: local anaesthetic and steroids
 Capsaicin

SURGICAL
 Arthroscopic washout: esp. knee - Trim cartilage, remove foreign bodies.
 Arthroplasty
 Osteotomy: small area of bone cut out.
 Arthrodesis
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32
Q

Symptoms of Septic Arthritis

A

 Acutely inflamed tender, swollen joint
 ↓ROM
 Systemically unwell

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33
Q

Causative organisms of septic arthritis

A

 Staph aureus
 N. Gonococcus
 Streps
 Gm-ve bacilli

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34
Q

Risk factors for getting septic arthritis

A
 Joint disease (e.g. RA)
 Chronic renal fialure
 Immunosuppression (e.g. DM)
 Prosthetic joints
 IVDU
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35
Q

Investigations for septic arthritis

A
URGENT Joint aspiration
- MCS --> ↑↑ WCC mostly PMN
 Baseline obs
 -/↑ESR or CRP , ↑WCC, Blood cultures 
 X-ray (usually normal at presentation - later bone destruction
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36
Q

Management of septic arthritis

A
 IV Abx for 2 weeks - flucloxacillin then oral 4 wks 
 Consider joint washout under GA
 Splint joint
 Physiotherapy after infection resolved
NO STEROIDS
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37
Q

Complications of septic arthritis

A

 Osteomyelitis
 Arthritis
 Ankylosis: fusion

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38
Q

Differentials of septic arthritis

A

Crystal arthropathy
Reactive arthritis
Trauma
Cellulitis

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39
Q

Features and presentation of RA

A

ANTI CCP Or RF

Arthritis
- Symmetrical, polyarthritis of MCPs, PIPs of hands and feet → pain, swelling, deformity
1. Swan neck (PIPJ hyperextension) 2. Boutonniere (PIPJ prolapse)
3. Z-thumb 4. Ulnar deviation of the fingers 5. Dorsal subluxation of ulnar styloid
 Morning stiffness >1h
 Improves with exercise

Nodules - elbows also fingers, feet, heal
 Firm, non-tender, mobile or fixed  Lungs

Tenosynovitis
 De Quervain’s Tenosynovitis
 Atlanto-axial subluxation

Immune - AIHA; Vasculitis; Amyloid; Lymphadenopathy

Cardiac: pericarditis + pericardial effusion

Carpal Tunnel Syndrome

Pulmonary
 Fibrosing alveolitis (lower zones)  Pleural effusions (exudates)

Ophthalmic
 Epi-/scleritis
 2O Sjogren’s Syndrome

Raynaud’s

Felty’s Syndrome
 RA + splenomegaly + neutropenia

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40
Q

Diagnosis of RA

A

American college of rheumatlogy

A) Joint involvement

  • swelling & tenderness +/- imaging
    (1) 2-10 med large (2) 1-3 small
    (3) 4-10 small (5) >10 small

B) Serology

(2) low +ve Rf/Anti-CCP
(3) High +Ve RF/ Anto-CCP

C) Duration of synovitis
(1) > 6 weeks

D) Acute phase reactants
(1) Abnormal CSR/ ESR

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41
Q

Ix for RA

A

Bloods: FBC (anaemia of CD, ↓PMN, ↑plat), ↑ESR, ↑CRP RF +ve in 70% (false +ve in HCV, SLE, Sarcoid, Sjogrens)
 High titre assoc. ̄c severe disease, erosions and extra-articular disease
 Anti-CCP: 98% specific (Ag derived from collagen)
 ANA: +ve in 30%
 Radiography, US, MRI
- thickened synovium and widespread synovitis

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42
Q

Mx of RA

A
Conservative
 Refer to rheumatologist 
 Regular exercise
 PT
 OT: aids, splints
  annual flu jab
Medical
 DAS28: Monitor disease activity - no swollen joints, global health and inflammatory markers
 DMARDs and biologicals: use early
 Steroids: IM, PO or intra-articular for exacerbations/ until DMARD kick in 
 NSAIDs: symptom relief

 Mx CV risk: RA accelerates atherosclerosis
 Prevent osteoporosis and gastric ulcers

Surgical
 Ulna stylectomy
 Joint prosthesis
 Athroscopy/ plasty

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43
Q

Differentials of RA

A
  • reactive
  • SLE/ CT disease
  • Seronegative
  • Polyarticualr gout
  • PMR

If just in hands - Psoriatic

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44
Q

DMARDs used in RA

A

Ideally, treatment should be started within 3 months of the onset of symptoms.

1) Methotrexate + Sulfasalazine/ Hydroxycholoquine
2) If tried 2 DMARDs and ↑ DAS28 on 2 occasions 1 m apart can start Infliximab

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45
Q

Action and SE of Hydroxycholoquine

A

Reduces inflammation by inhibiting stimulation of the TLR (cellular receptors for microbial products) that induce inflammatory responses through activation of the innate immune

 retinopathy, seizures
- pre-treatment and annual eye-screen

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46
Q

Action SE, monitoring and patient advice of methotrexate

A

Inhibits the enzyme dihydrofolate reductase, essential for the synthesis of purines and pyrimidines.
+ folic acid

SE - Bone marrow suppression, hepatotoxic, pulmonary fibrosis, alopecia, headaches, GI distubrance

Monitoring - FBC, U&E, LFT,
- 1–2 wks until stabilised, then every 2–3 months

  • BE wary of signs of myelosuppression infection/ unexpected bruising/ anaemia

Stop 3m before trying to get pregnant

47
Q

SE of sulphasalazine

A

hepatotoxic, SJS, ↓ sperm count, nausea, headache/ dizzy, oral ulcers, blood disturbances/ myelosuppresion

48
Q

Action, SE and pre-treatment requirementsof infliximab

A
Anti-TNFα
	↓ inflammation – 
	↓ angiogenesis 
	↓ joint destruction 
SE
↑ risk of new malignancy if have prior malignancy.
↑ risk of skin/soft tissue infection 
 Chronic leg ulcers; recurrent chest infections; indwelling catheter 
Reactivation of TB &amp; HBV
 Worsens HF

Before start - CXR (TB), Bloods for HCV/ HIV/HBV

49
Q

Pathophysiology of Gout

A

 Deposition of monosodium urate crystals in and around joints → erosive arthritis
 May be ppted by surgery, infection, fasting or diuretic

50
Q

Presentation of gout

A

 M>F=5:1
 severe joint inflammation
- great toe MTP = Podagra
- ankle, foot, hand, wrist, elbow, knee
- Pain, hot, tender, swollen, shiny red skin
- develop rapidly (hr) and last 3-10 days
 Urate deposits in pinna and tendons = Tophi
 Renal disease: radiolucent stones and interstitial
nephritis

51
Q

Differentials of gout

A

 Septic arthritis
 Pseudogout
 Haemarthrosis

52
Q

Causes of gout

A

 Hereditary
Reduced excretion
- diuretics, NSAIDs, renal impairement
Excess production
cytotoxics, purine rich foods (beef, pork), alcohol
↑ cell turnover: lymphoma, leukaemia, psoriasis,
haemolysis, tumour lysis syndrome

53
Q

Ix for gout

A

 Polarised light microscopy
- Negatively birefringent needle-shaped crystals
 -/↑ serum urate
 X-ray changes occur late
- Punched-out erosions in juxta-articular bone & ↓ joint space

54
Q

Acute Rx of gout

A

Rest and elevate joint
NSAIDS + Colchicine

CFR - steroids
Dont stop allopurinol

55
Q

CI and SE NSAIDS

A

CI - warfarin, PUD, HF, CRF

SE - diarrhoea

56
Q

Prevention of Gout

A

Conservative
 Lose wt.
 Avoid prolonged fasts and EtOH excess

Allopurinol
- dont start until 3wk after attack (+NSAID/ Colchicine cover)

Febuxostat (XO inhibitor) if hypersensitivity

57
Q

Action and SE of Allopurinol

A

Xanthine Oxidase Inhibitors:

  • use to prevent attacks, prevent damage
  • if > 1 attacks in 12m or if on cytotoxics/ renal stones

SE: rash, fever, ↓WCC ( ̄c azathioprine)

58
Q

Acute v Chronic CPPD

A

ACUTE - Presents as acute monoarthropathy  Usually knee, wrist or hip
 Usually spontaneous and self-limiting

CHRONIC
 Destructive changes like OA
 Can present as poly-arthritis (pseudo-rheumatoid)

59
Q

Risk factors for CPPD

A
 ↑age
 OA
 DM
 Hypothyroidism
 Hyperparathyroidism
 Hereditary haemochromatosis 
 Wilson’s disease
60
Q

Ix and Rx for CPPD

A

Ix
ASPIRATION
 Polarized light microscopy
- +ve birefringent rhomboid-shaped crystals
 X-ray may show chondrocalcinosis - Soft-tissue Ca deposition
Rx
Cool pack, rest,
 Analgesia
 NSAIDs +/- colchicine
 May try steroids: PO, IM or intra-articular

61
Q

Common features of seronegative spondyloarthropathies and the types

A

 Axial arthritis and sacroiliitis
 Asymmetrical large-joint oligoarthritis or monoarthritis
 Enthesitis
 Dactylitis
 Extra-articular: iritis, psoriaform rashes, oral ulcers, AR, IBD
 HLA-B27 allele
 RF -ve

PEAR
P = Psoriatic
E = Enteric 
A = Ankylosing spondylitis
R = Reactive
62
Q

Presentation of Ankylosing Spondylitis

A

 Gradual onset back pain
 Typically man <30
 Radiates from SI joints to hips and buttocks
 Worse @ night ̄c morning stiffness
 Relieved by exercise.
 Progressive loss of all spinal movements
- Schober’s test <5cm
- ↓ thoracic expansion
 Some develop thoracic kyphosis and neck hyperextension and spinocranial ankyloses = question mark posture
 Enthesitis: Achilles tendonitis, plantar fasciitis
 Costochondritis

63
Q

Extraskeletal manifestations of ankylosing spondylitis

A

A’s
o Apical fibrosis (pulmonary)– can lead to resp failure
o AR
o Aortitis
o Ant uveitis/ acute iritis -
o AV block
o Achilles tendonitis and plantar fasciitis (enthesisitis)
o Amyloidosis
o Anterior mechanical chest pain (costochondritis – fatigue & SOB)
o Arthritis (peripheral) - Pain in non-axial joints
 Osteoporosis~ 60%(↑ risk of osteoporotic spinal fractures) – stripping of end-plates
 IBD; Cardiomyopathy; IHD; Psoriasis; dactylitis;
 General Malaise/ Fatigue; weight loss

64
Q

Ix of Ank spond

A
XRAY (or MRI/USS ealry) 
 Sacroliliitis: irregularities, sclerosis, erosions 
 Vertebra: corner erosions, squaring
syndesmophytes (bony proliferations)
 Bamboo spine: calcification of ligaments, periosteal bone formation
Blood --> 
FBC (anaemia), ↑ESR, ↑CRP , 
HLA-B27 
DEXA scan and CXR
65
Q

Indications for referral for ank spond

A
  • low back pain that started <45 y/o and lasted > 3m with:
  • 4 + or 3+ & +ve HLA-B27
    Additional criteria are:
  • low back pain that started < 35 y/o
  • waking during the second half of the night because of symptoms
  • buttock pain
  • improvement with movement
  • improvement within 48 hours of taking NSAIDs
  • a first-degree relative with spondyloarthritis
  • current or past arthritis
  • current or past enthesitis
  • current or past psoriasis.
66
Q

Mx of ank spond

A

1) Physio/ exercise - maintain posture and stability and NSAIDs (symptom relief)
2) Anti-TNF/ Local steroid injections/ Bisphosphonates
Surgical
3) Hip replacement to ↓ pain and ↑ mobility

67
Q

Psoriatic Arthritis presentation

A
  • Asymmetrical mono/oligoarthropathy – often DIPJ
  • Nail changes – pitting, onkolysis
  • Skin changes - Acneiform rashes; palmo-plantar pustulosis (rheumatoid nodules absent), psoriasis plaques (scalp, natal cleft and umbilicus)
  • Dactylitis and Enthesitis (Achilles tendonitis and plantar fasciitis)
  • Hyperuricaemia
  • Symmetrical polyarthritis (like RA - Joint stiffness, pain, swelling)
  • Arthritis mutilans - resorption of bones and the consequent collapse of soft tissue –> telescoping (shortening)
  • ¼ develop AI & vertebral changes like those in AS
68
Q

Ix of Psoriasitic arthritis

A

X-Ray
 Erosion → “pencil-in-cup” deformity
- no pero-articular ostoepenia

69
Q

Rx of Psoriasis arthritis

A
Conrtrol skin disorder
education, physio, OT
 NSAIDs
 Sulfasalazine, methotrexate, ciclosporin
 (if at serveral sites/ ↑CRP. erosions)
 Anti-TNF

CASPAR score - current/hx psoriasis, nail changes

70
Q

Reactive arthritis presentation

A

1-4wks after urethritis (chlamydia) or dysentery
(campy, salmonella, shigella)

 Asymmetrical lower limb oligoarthritis: esp. knee
 Iritis, conjunctivitis
 Keratoderma blenorrhagica: plaques on soles/palms
 Circinate balanitis: painless serpiginous penile ulceration  Enthesitis
 Mouth ulcers

reiters –> arthritis, urethritis and conjuncitivitis

71
Q

Ix for reactive arthritis

A

 ↑ESR, ↑CRP
 Stool culture if diarrhoea
 Urine chlamydia PCR and sexual health review
 culture cervix/ vagina

72
Q

Mx of reactive arthritis

A
 NSAIDs and local steroids (skin/eyes)
 Physiotherapy to maintain ROM 
 Splint
 Monitor FBC, FT
 Relapse may need sulfasalazine or methotrexate
73
Q

Presentation and Rx of enteropathic arthritis

A

Asymmetrical large joint oligoarthritis mainly affecting the lower limbs.
Seronegative Spondyloarthropathies
 Sacroiliitis may occur

Rx
 Treat the IBD
 NSAIDs or articular steroids for arthritis
 Colectomy → remission in UC

74
Q

Presentation of Behcet’s disease

A
Recurrent oral and genital ulceration
nt/post uveitis
  Erythema nodosum
 Vasculitis
 non-erosive large joint oligoarthropathy
 CN palsies
 diarrhoea, colitis
75
Q

Sjogren’s pathophysiology and presentation

A

Due to lymphocytic infiltration of exocrine gland
primary or secondary (RA/SLE)
 ↓ tear production → dry eyes - keratoconjunctivitis sicca
 ↓ salivation → xerostomia
 Bilateral parotid swelling
 Vaginal dryness → dyspareunia

 Polyarthritis;  Raynaud’s
 Bibasal pulmonary fibrosis  Vasculitis;  Myositis

76
Q

Ix adn Rx of Sjogren’s

A

Schirmer tear test
 Abs:ANA–RoandLa,RF
 Hypergammaglobulinaemia
 Parotid biopsy

Rx
 Artificial tears
 Saliva replacement solutions
 NSAIDs and hydroxychloroquine for arthralgia
 Immunosuppression for severe systemic disease

77
Q

Pathology of Raynaud’s

A

Peripheral digital ischaemia precipitated by cold or emotion.
Primary or Secondary (SLE, Syst Sclerosis, RA, Sjogrens, β-b

78
Q

Presentation of Raynaud’s

A
 Digit pain + triphasic colour change: WBC
- White (Vasoconstriciton), 
- Blue (Ischaemia)
- Crimson (Reperfusion)
 Digital ulceration and gangrene
79
Q

Rx of Raynaud’s

A
 Wear gloves
 CCBs: nifedipine
 ACEi
 IV prostacyclin
 Advice and education
- avoid cold,
80
Q

Features of limited systemic sclerosis

A
 Calcinosis
 Raynaud’s
 Esophageal and gut dysmotility → GOR
 Sclerodactyly
 Telangiectasia

Skin involvement limited to face, hands and feet
Beak nose
 Microstomia

Pulmonary HTN in 15%
- fatigue, arthralgia

81
Q

Features of diffuse systemic sclerosis

A

Organ fibrosis
 GI: GOR, aspiration, dysphagia, anal incontinence
 Lung: fibrosis and Pul HTN
 Cardiac: arrhythmias and conduction defects
 Renal: acute hypertensive crisis

82
Q

Ix for systemic sclerosis

A

 Bloods: FBC (anaemia), U+E (renal impairment)

Abs
 Centromere: limited
 Scl70 / topoisomerase: diffuse

 Urine: stix, PCR
 Imaging
- CXR: cardiomegaly, bibasal fibrosis
- Hands: calcinosis

 ECG + Echo: evidence of pulmonary HTN
Annual, ECHO and Spirometry

83
Q

Mx of systemic sclerosis

A

Advise on red flags of renal crisis and internal obstruction
 Exercise and skin lubricants: ↓ contractures
 Raynaud’s Mx

Medical
EARLY - anti-histamine and low dose corticosteroid
 Immunosuppression - IV cyclophosphamide
 Raynaud’s Mx
 Renal: BP control – ACEi
 Oesophageal: PPIs, prokinetics (metoclopramide)
 PHT: sildenafil, bosentan

84
Q

Complications of systemic sclerosis

A

Renal crisis - obliterative vasculopathy and luminal narrowing of renal arcuate arteries. Progressive reduction in renal blood flow. Aggravated by vasospasm, leads to juxtaglomerular hyperplasia and increased renin secretion –> malignnat HTN

Malignancy - lung, breast, ovarian, lympoma
Sjogrens
Depression
CTS

85
Q

Features of Polymyositis

A

Progressive symmetrical proximal muscle weakness.
 Wasting of shoulder and pelvic girdle
 Dysphagia, dysphonia, respiratory weakness
 Assoc. myalgia and arthralgia
 Commoner in females
 Often a paraneoplastic phenomenon - Lung, pancreas, ovarian, bowel

86
Q

Presentation of Dermatomyositis

A

= myositis + skin signs
 Heliotrope rash on eyelids ± oedema
 Macular rash (shawl sign +ve: over back and shoulders)  Nailfold erythema
 Gottron’s papules: knuckles, elbows, knees
 Mechanics hands: painful, rough skin cracking of finger
tips
 Retinopathy: haemorrhages and cotton wool spots
 Subcutaneous calcifications

87
Q

Extra-muscular features of Polymyositis and Dermatomyositis

A
 Fever
 Arthritis
 Bibasal pulmonary fibrosis
 Raynaud’s phenomenon
 Myocardial involvement: myocarditis, arrhythmias
88
Q

Ix and Rx for Polymyositis and Dermatomyositis

A
 Muscle enzymes: ↑CK, ↑AST, ↑ALT, ↑LDH
 Abs: Anti-Jo1 
 EMG
 Muscle biopsy
 Screen for malignancy
-  Tumour markers; CXR; Mammogram; Pelvic/abdo US; CT

Rx - Immunosupression - steroids/ Azathioprine/ methotrexate

89
Q

Differentials of Polymyositis and Dermatomyositis

A
 Inclusion body myositis
 Muscular dystrophy
 Polymyalgia rheumatica
 Endocrine / metabolic myopathy
 Drugs: steroids, statins, colchicine, fibrates
90
Q

Features of SLE

A

AutoAbs to a variety of autoantigens result in the formation and deposition of immune complexes.
 Relapsing, remitting history
 Constitutional symptoms: fatigue, wt. loss, fever, myalgia

91
Q

Diagnosis of Lupus

A

A RASH POINts MD
 Arthritis - Non-erosive, involving peripheral joints
 Renal - proteinuria and ↑BP
 ANA (+ve in 95%)
 Serositis - Pleuritis/ Pericarditis
 Haematological - AIHA; ↓WCC; ↓Plats; ↓C3&C4
 Photosensitivity and non-scarring alopecia
 Oral ulcers (and nasal)
 Immune phenomenon
- Anti-dsDNA (VERY SPECIFIC); Anti-Sm
 Neurological - Seizures, psychosis
 Malar Rash - Facial erythema sparing the nasolabial folds
 Discoid Rash - Erythema → pigmented hyperkeratotic papules → atrophic depressed lesions
- Mainly affects face and chest

92
Q

Monitoring disease activity in SLE

A
 Anti-dsDNA titres
 Complement: ↓C3, ↓C4
 ↑ESR
 Bloods: FBC, U+E, CRP, clotting (usually normal) 
 Urine: stix, PCR
 CT/MRI if other systems involved
93
Q

Causes and Ab in drug-induced lupus

A

procainamide, phenytoin, hydralazine, isoniazid

Anti-histone Abs

94
Q

Features of anti-phospholipid syndrome

A

 CLOTs: venous (e.g. DVT) and arterial (e.g. stroke)
 Coagulation defect: ↑APTT
 Livido reticularis
 Obstetric complications: recurrent 1st trimester abortion  Thrombocyotpenia

95
Q

Classification and Ab’s in anti-phospholipid syndrome

A

 Primary: 70%
 Secondary to SLE: 30%
Pathology

 Anti-phospholipid Abs: anti-cardiolipin and lupus anticoagulant

96
Q

Rx of anti-phospholipid syndrome

A

Low-dose aspirin

Warfarin if recurrent thromboses: INR 3.5

97
Q

Mx of SLE

A

Acute flare
- High-dose prednisolone + IV cyclophosphamide
Moderate - mycophenolate/ DMARD

Cutaneous Symptoms
- topical steroids and sun cream

Maintenance - skin & joint
NSAIDs and hydroxychloroquine/ ± low-dose steroids
- or azathioprine/ rituximab

Lupus Nephritis
 Proteinuria: ACEi
 Aggressive GN: immunosuppression
May need renal replacement therapy

98
Q

Complications of SLE

A

↑ risk of osteoporosis and CV disease

99
Q

Features of Giant Cell Arteritis

A
Granulomatous inflammation 
Increased CRP/ESR
Age >50 
New headache
Temporal artery and scalp tenderness

Claudication of jaw
Amaurosis fugax
Prominent temporal arteries ± pulsation
Systemic signs: fever, malaise, fatigue, weight loss

100
Q

Mx and Ix of giant cell arteritis

A

Do ESR and start pred 60mg/d PO
 ↑↑ESR and CRP
 ↑ALP
 ↓Hb (normo normo), ↑Plats

Visual symptoms - IV methylpredisolone 100mg 3d

Temporal artery biopsy w/i 2wk: but skip lesion occurrs

CK and EMG normal

  • Taper steroids gradually, guided by symptoms and ESR
     PPI and alendronate cover (~2yr course usually)
101
Q

Presentation of polymyalgia rheumatica

A

 >50yrs old
 Severe pain and stiffness in shoulders, neck and hips
- Sudden / subacute onset
- Symmetrical
- Worse in the morning: stops pt. getting out of bed
 No weakness (cf. myopathy or myositis)
 ± mild polyarthritis, tenosynovitis and CTS
 Systemic signs: fatigue, fever, wt. loss
 15% develop GCA

102
Q

Ix and Rx of PMR

A

Ix
 ↑↑ESR and CRP (+ ↑plasma viscosity)
 ↑ALP
 Normal CK
Rx
 Pred 15mg/d PO: taper according to symptoms and ESR
 PPI and alendronate cover (~2yr course usually)

103
Q

Features and rx of Takayasu’s arteritis

A
Large vessel vasculitis 
 fever, fatigue, wt. loss 
 Weak pulses in upper extremities
 Visual disturbance
 HTN
 ↑↑ESR/CRP
  • Prednisolone
104
Q

Features, symptoms and Rx of polyarteritis nodosa

A

Medium vessel vasculitis
 Assoc. HBV

 Rash
 Renal → HTN
 GIT → melaena and abdo pain

Rx
 Pred + cyclophosphamide

105
Q

What is Kawasaki’s disease

A

Childhood PAN variant

Features
 5-day fever
 Bilat non-purulent conjunctivitis
 Oral mucositis
 Cervical lymphadenopathy
 Polymorphic rash (esp. trunk)
 Extremity changes (erythema + desquamation)
 Coronary artery aneurysms

Rx –> IVIg + aspirin

106
Q

Pathology and Features of Granulomatosis with polyangiitis

A

Necrotizing granulomatous inflammation and small vessel vasculitis with a predilection for URT, LRT and Kidneys

Features 
URT
 Chronic sinusitis  Epistaxis  Saddle-nose deformity  Nasal obstruction
LRT
 Cough  Haemoptysis
 Pleuritis
Renal
 RPGN  Haematuria and proteinuria
Other
 Palpable purpura
 Ocular: conjunctivitis, keratitis, uveitis
  peripheral neuropathy
107
Q

Ix and Rx for Granulomatosis with polyangiitis

A

cANCA +ve
 Dipstick: haematuria and proteinuria
 CXR: bilat nodular and cavity infiltrates
 ↑↑ESR/CRP
 CT chest - diffuse alveolar haemorrhage
Rx –> corticosteroids and cyclophosphamide.
Maintenance - azathioprine

108
Q

Features of Churg-Strauss

A
Small vessel vasculitidies 
 Late-onset asthma
 Eosinophilia
 Granulomatous small-vessel vasculitis
-  RPGN
-  Palpable purpura 
- GIT bleeding
 pANCA +ve
109
Q

Features of Microscopic polyangitis

A

 RPGN
 Haemoptysis
 Palpable purpura
 pANCA

  • no granulomas
  • give steroids and methotrexate
110
Q

Name some ANCA -ve small vessel vasculitidies

A

HSP (IgA variatn - post URTI, purpura on buttocks, colicky abdo pain, arthragia, haematuria)

Goodpastures
 Anti-GBM Abs; RPGN; Haemoptysis
 CXR: Bilat lower zone infiltrates (haemorrhage)
- Immunosuppresion + plasmapheresis

111
Q

Risk factors for developing fibromyalgia

A
Risk Factors
 Neurosis: depression, anxiety, stress
 Dissatisfaction at work
 Overprotective family or lack of support
 Middle age
 Low income
 Divorced
 Low educational status

 Chronic fatigue syndrome; IBS;

112
Q

Features of fibromyalgia

A
 Chronic (>3) , widespread musculoskeletal pain (L,R and above and below waist) and tenderness (at 9 sites)
 Morning stiffness
 Fatigue
 Poor concentration
 Sleep disturbance
 Low mood
 headache
113
Q

Mx of fibromyalgia

A
  • rule out organic cause
 Educate pt and impact questionnaire 
 CBT and relaxation techniques 
 Graded exercise programs
 Amitriptyline or pregabalin
 Paracetamol
114
Q

MOA and SE of Azathioprine

A

Metabolised to Mercaptopurine
- Check levels of TPMT enzyme before use.

Onset of action takes at least 6 weeks.

S.E – colitis & increase risk infection in pts also receiving corticosteroids; hypersensitivity reaction; hypotension; interstitial nephritis; liver impairment; malaise; malagia; n&v&d; neutropenia; rash; rigors; thrombocytopenia

FBC weekly for first 4 weeks and tell to look out for signs of BM supression