Rheumatology Flashcards Preview

IPE > Rheumatology > Flashcards

Flashcards in Rheumatology Deck (113):
1

Radiological features of OA

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

2

Radiological features of RA

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

3

Radiological features of Gout

 Normal joint space
 Soft tissue swelling
 Periarticular erosions

4

Red flags for back pain

 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

5

Causes of back pain

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

6

Describe what a disc prolapse is and the presenting features

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

7

Signs of disc prolapse

 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

8

Ix and Rx of Disc Prolapse

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.

9

Presenting features of spondylolisthesis

 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

10

Causes of spondylolisthesis

 Congenital malformation
 Spondylosis
 Osteoarthritis

11

Dx and Rx of spondylolisthesis

Dx
 Plain radiography

Rx
 Corset
 Nerve release
 Spinal fusion

12

Features and presentation of Spinal Stenosis

 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

13

Ix and Rx 0f Spinal Stenosis

Ix --> MRI

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

14

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

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

15

Ix for back pain

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

MRI

16

General Mx of back pain

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

17

Acute cord compression presentation

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

18

Acute Cauda Equina Compression presentation

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

19

Ix Mx of acute cord/ cauda equina compression

- bldder scan, MRI,

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

20

Causes of acute cord/ cauda equina compression

- bony met
- large disc protrusion
- myeloma
- cord/ paraspinal tumpour
- TB
- Abscess

21

Yellow flags for back pain

Attitudes
Beliefs
Compensation
Diagnosis
Emotions
Family hx

Work

22

UMN v LMN lesions

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

LMN =
- Hypotonia
- hyporeflexia
- wasting
- fasciculations
- weakness

23

Define OA

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

24

Risk factors for OA

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

25

Classification of OA

 Primary: no underlying cause
 Secondary: obesity, joint abnormality (Trauma, AVN, RA, Gout, Pagets, Perthes, SUFE), acromegaly, haemophilia

26

Symptoms of OA

 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

27

Signs and Ix in OA

 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

28

Pathophysiology of OA

 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.

29

Differentials of OA

 Septic  Crystal  Trauma

30

Features in the hx in OA

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

31

Mx of OA

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

32

Symptoms of Septic Arthritis

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

33

Causative organisms of septic arthritis

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

34

Risk factors for getting septic arthritis

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

35

Investigations for septic arthritis

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

36

Management of septic arthritis

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

37

Complications of septic arthritis

 Osteomyelitis
 Arthritis
 Ankylosis: fusion

38

Differentials of septic arthritis

Crystal arthropathy
Reactive arthritis
Trauma
Cellulitis

39

Features and presentation of RA

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

40

Diagnosis of RA

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

41

Ix for RA

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

42

Mx of RA

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

43

Differentials of RA

- reactive
- SLE/ CT disease
- Seronegative
- Polyarticualr gout
- PMR

If just in hands - Psoriatic

44

DMARDs used in RA

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

45

Action and SE of Hydroxycholoquine

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

46

Action SE, monitoring and patient advice of methotrexate

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

SE of sulphasalazine

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

48

Action, SE and pre-treatment requirementsof infliximab

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 & HBV
 Worsens HF

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

49

Pathophysiology of Gout

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

50

Presentation of gout

 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

Differentials of gout

 Septic arthritis
 Pseudogout
 Haemarthrosis

52

Causes of gout

 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

Ix for gout

 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

Acute Rx of gout

Rest and elevate joint
NSAIDS + Colchicine

CFR - steroids
Dont stop allopurinol

55

CI and SE NSAIDS

CI - warfarin, PUD, HF, CRF
SE - diarrhoea

56

Prevention of Gout

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

Action and SE of Allopurinol

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

Acute v Chronic CPPD

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

Risk factors for CPPD

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

60

Ix and Rx

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

Common features of seronegative spondyloarthropathies and the types

 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

Presentation of Ankylosing Spondylitis

 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

Extraskeletal manifestations of ankylosing spondylitis

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

Ix of Ank spond

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

Indications for referral for ank spond

- 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

Mx of ank spond

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

Psoriatic Arthritis presentation

•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

Ix of Psoriasitic arthritis

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

69

Rx of Psoriasis arthritis

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

Reactive arthritis presentation

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

Ix for reactive arthritis

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

72

Mx of reactive arthritis

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

73

Presentation and Rx of enteropathic arthritis

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

Presentation of Behcet's disease

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

75

Sjogren's pathophysiology and presentation

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

Ix adn Rx of Sjogren's

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

Pathology of Raynaud's

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

78

Presentation of Raynaud's

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

79

Rx of Raynaud's

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

80

Features of limited systemic sclerosis

 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

Features of diffuse systemic sclerosis

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

82

Ix for systemic sclerosis

 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

Mx of systemic sclerosis

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

Complications of systemic sclerosis

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

Features of Polymyositis

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

Presentation of Dermatomyositis

= 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

Extra-muscular features of Polymyositis and Dermatomyositis

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

88

Ix and Rx for Polymyositis and Dermatomyositis

 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

Differentials of Polymyositis and Dermatomyositis

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

90

Features of SLE

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

Diagnosis of Lupus

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

Monitoring disease activity in SLE

 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

Causes and Ab in drug-induced lupus

procainamide, phenytoin, hydralazine, isoniazid

Anti-histone Abs

94

Features of anti-phospholipid syndrome

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

95

Classification and Ab's in anti-phospholipid syndrome

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

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

96

Rx of anti-phospholipid syndrome

Low-dose aspirin

Warfarin if recurrent thromboses: INR 3.5

97

Mx of SLE

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

Complications of SLE

↑ risk of osteoporosis and CV disease

99

Features of Giant Cell Arteritis

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

Mx and Ix of giant cell arteritis

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

Presentation of polymyalgia rheumatica

 >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

Ix and Rx of PMR

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

Features and rx of Takayasu's arteritis

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

- Prednisolone

104

Features, symptoms and Rx of polyarteritis nodosa

Medium vessel vasculitis
 Assoc. HBV

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

Rx
 Pred + cyclophosphamide

105

What is Kawasaki's disease

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

Pathology and Features of Granulomatosis with polyangiitis

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

Ix and Rx for Granulomatosis with polyangiitis

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

Features of Churg-Strauss

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

109

Features of Microscopic polyangitis

 RPGN
 Haemoptysis
 Palpable purpura
 pANCA

- no granulomas
- give steroids and methotrexate

110

Name some ANCA -ve small vessel vasculitidies

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

Risk factors for developing fibromyalgia

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

Features of fibromyalgia

 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

Mx of fibromyalgia

- rule out organic cause

 Educate pt and impact questionnaire
 CBT and relaxation techniques
 Graded exercise programs
 Amitriptyline or pregabalin
 Paracetamol