Rheumatology

Question 1

Radiological features of OA

Answer 1

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

Question 2

Radiological features of RA

Answer 2

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

Question 3

Radiological features of Gout

Answer 3

 Normal joint space
 Soft tissue swelling
 Periarticular erosions

Question 4

Red flags for back pain

Answer 4

 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

Question 5

Causes of back pain

Answer 5

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

Question 6

Describe what a disc prolapse is and the presenting features

Answer 6

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

Question 7

Signs of disc prolapse

Answer 7

 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

Question 8

Ix and Rx of Disc Prolapse

Answer 8

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.

Question 9

Presenting features of spondylolisthesis

Answer 9

 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

Question 10

Causes of spondylolisthesis

Answer 10

 Congenital malformation
 Spondylosis
 Osteoarthritis

Question 11

Dx and Rx of spondylolisthesis

Answer 11

Dx
 Plain radiography

Rx
 Corset
 Nerve release
 Spinal fusion

Question 12

Features and presentation of Spinal Stenosis

Answer 12

 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

Question 13

Ix and Rx 0f Spinal Stenosis

Answer 13

Ix –> MRI

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

Question 14

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

Answer 14

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

Question 15

Ix for back pain

Answer 15

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

MRI

Question 16

General Mx of back pain

Answer 16

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

Question 17

Acute cord compression presentation

Answer 17

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

Question 18

Acute Cauda Equina Compression presentation

Answer 18

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

Question 19

Ix Mx of acute cord/ cauda equina compression

Answer 19

• bldder scan, MRI,

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

Question 20

Causes of acute cord/ cauda equina compression

Answer 20

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

Question 21

Yellow flags for back pain

Answer 21

Attitudes
Beliefs
Compensation
Diagnosis
Emotions
Family hx

Work

Question 22

UMN v LMN lesions

Answer 22

UMN

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

LMN =

• Hypotonia
• hyporeflexia
• wasting
• fasciculations
• weakness

Question 23

Define OA

Answer 23

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

Question 24

Risk factors for OA

Answer 24

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

Question 25

Classification of OA

Answer 25

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

Question 26

Symptoms of OA

Answer 26

 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

Question 27

Signs and Ix in OA

Answer 27

 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

Question 28

Pathophysiology of OA

Answer 28

 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.

Question 29

Differentials of OA

Answer 29

 Septic  Crystal  Trauma

Question 30

Features in the hx in OA

Answer 30

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

Question 31

Mx of OA

Answer 31

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 ```

Question 32

Symptoms of Septic Arthritis

Answer 32

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

Question 33

Causative organisms of septic arthritis

Answer 33

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

Question 34

Risk factors for getting septic arthritis

Answer 34

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

Question 35

Investigations for septic arthritis

Answer 35

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

Question 36

Management of septic arthritis

Answer 36

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

Question 37

Complications of septic arthritis

Answer 37

 Osteomyelitis  Arthritis  Ankylosis: fusion

Question 38

Differentials of septic arthritis

Answer 38

Crystal arthropathy Reactive arthritis Trauma Cellulitis

Question 39

Features and presentation of RA

Answer 39

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

Question 40

Diagnosis of RA

Answer 40

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

Question 41

Ix for RA

Answer 41

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

Question 42

Mx of RA

Answer 42

``` 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

Question 43

Differentials of RA

Answer 43

- reactive - SLE/ CT disease - Seronegative - Polyarticualr gout - PMR If just in hands - Psoriatic

Question 44

DMARDs used in RA

Answer 44

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

Question 45

Action and SE of Hydroxycholoquine

Answer 45

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

Question 46

Action SE, monitoring and patient advice of methotrexate

Answer 46

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

Question 47

SE of sulphasalazine

Answer 47

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

Question 48

Action, SE and pre-treatment requirementsof infliximab

Answer 48

``` 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

Question 49

Pathophysiology of Gout

Answer 49

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

Question 50

Presentation of gout

Answer 50

 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

Question 51

Differentials of gout

Answer 51

 Septic arthritis  Pseudogout  Haemarthrosis

Question 52

Causes of gout

Answer 52

 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

Question 53

Ix for gout

Answer 53

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

Question 54

Acute Rx of gout

Answer 54

Rest and elevate joint NSAIDS + Colchicine CFR - steroids Dont stop allopurinol

Question 55

CI and SE NSAIDS

Answer 55

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

Question 56

Prevention of Gout

Answer 56

Conservative  Lose wt.  Avoid prolonged fasts and EtOH excess Allopurinol - dont start until 3wk after attack (+NSAID/ Colchicine cover) Febuxostat (XO inhibitor) if hypersensitivity

Question 57

Action and SE of Allopurinol

Answer 57

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)

Question 58

Acute v Chronic CPPD

Answer 58

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)

Question 59

Risk factors for CPPD

Answer 59

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

Question 60

Ix and Rx for CPPD

Answer 60

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

Question 61

Common features of seronegative spondyloarthropathies and the types

Answer 61

 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 ```

Question 62

Presentation of Ankylosing Spondylitis

Answer 62

 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

Question 63

Extraskeletal manifestations of ankylosing spondylitis

Answer 63

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

Question 64

Ix of Ank spond

Answer 64

``` 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 ```

Question 65

Indications for referral for ank spond

Answer 65

- 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.

Question 66

Mx of ank spond

Answer 66

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

Question 67

Psoriatic Arthritis presentation

Answer 67

* 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

Question 68

Ix of Psoriasitic arthritis

Answer 68

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

Question 69

Rx of Psoriasis arthritis

Answer 69

``` 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

Question 70

Reactive arthritis presentation

Answer 70

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

Question 71

Ix for reactive arthritis

Answer 71

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

Question 72

Mx of reactive arthritis

Answer 72

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

Question 73

Presentation and Rx of enteropathic arthritis

Answer 73

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

Question 74

Presentation of Behcet's disease

Answer 74

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

Question 75

Sjogren's pathophysiology and presentation

Answer 75

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

Question 76

Ix adn Rx of Sjogren's

Answer 76

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

Question 77

Pathology of Raynaud's

Answer 77

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

Question 78

Presentation of Raynaud's

Answer 78

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

Question 79

Rx of Raynaud's

Answer 79

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

Question 80

Features of limited systemic sclerosis

Answer 80

```  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

Question 81

Features of diffuse systemic sclerosis

Answer 81

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

Question 82

Ix for systemic sclerosis

Answer 82

 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

Question 83

Mx of systemic sclerosis

Answer 83

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

Question 84

Complications of systemic sclerosis

Answer 84

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

Question 85

Features of Polymyositis

Answer 85

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

Question 86

Presentation of Dermatomyositis

Answer 86

= 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

Question 87

Extra-muscular features of Polymyositis and Dermatomyositis

Answer 87

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

Question 88

Ix and Rx for Polymyositis and Dermatomyositis

Answer 88

```  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

Question 89

Differentials of Polymyositis and Dermatomyositis

Answer 89

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

Question 90

Features of SLE

Answer 90

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

Question 91

Diagnosis of Lupus

Answer 91

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

Question 92

Monitoring disease activity in SLE

Answer 92

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

Question 93

Causes and Ab in drug-induced lupus

Answer 93

procainamide, phenytoin, hydralazine, isoniazid Anti-histone Abs

Question 94

Features of anti-phospholipid syndrome

Answer 94

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

Question 95

Classification and Ab's in anti-phospholipid syndrome

Answer 95

 Primary: 70%  Secondary to SLE: 30% Pathology  Anti-phospholipid Abs: anti-cardiolipin and lupus anticoagulant

Question 96

Rx of anti-phospholipid syndrome

Answer 96

Low-dose aspirin Warfarin if recurrent thromboses: INR 3.5

Question 97

Mx of SLE

Answer 97

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

Question 98

Complications of SLE

Answer 98

↑ risk of osteoporosis and CV disease

Question 99

Features of Giant Cell Arteritis

Answer 99

``` 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

Question 100

Mx and Ix of giant cell arteritis

Answer 100

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)

Question 101

Presentation of polymyalgia rheumatica

Answer 101

 >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

Question 102

Ix and Rx of PMR

Answer 102

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)

Question 103

Features and rx of Takayasu's arteritis

Answer 103

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

Question 104

Features, symptoms and Rx of polyarteritis nodosa

Answer 104

Medium vessel vasculitis  Assoc. HBV  Rash  Renal → HTN  GIT → melaena and abdo pain Rx  Pred + cyclophosphamide

Question 105

What is Kawasaki's disease

Answer 105

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

Question 106

Pathology and Features of Granulomatosis with polyangiitis

Answer 106

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 ```

Question 107

Ix and Rx for Granulomatosis with polyangiitis

Answer 107

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

Question 108

Features of Churg-Strauss

Answer 108

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

Question 109

Features of Microscopic polyangitis

Answer 109

 RPGN  Haemoptysis  Palpable purpura  pANCA - no granulomas - give steroids and methotrexate

Question 110

Name some ANCA -ve small vessel vasculitidies

Answer 110

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

Question 111

Risk factors for developing fibromyalgia

Answer 111

``` 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;

Question 112

Features of fibromyalgia

Answer 112

```  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 ```

Question 113

Mx of fibromyalgia

Answer 113

- rule out organic cause ```  Educate pt and impact questionnaire  CBT and relaxation techniques  Graded exercise programs  Amitriptyline or pregabalin  Paracetamol ```

Question 114

MOA and SE of Azathioprine

Answer 114

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