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PM Rheumatology > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are the main ANCA associated vasculitis conditions?

A
  • granulomatosis with polyangiitis
  • eosinophilic granulomatosis with polyangiitis
  • microscopic polyangiitis
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2
Q

Common features of ANCA associated vasculitis:

A
  • renal impairment: immune complex GN (haematuria, proteinuria, increased creatinine)
  • respiratory: dyspnoea, haemoptysis
  • systemic: lethargy, weight loss, fatigue
  • vasculitic rash
  • sinusitis
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3
Q

cANCA (target, association, monitoring)

A
  • seine proteinase 3
  • mostly granulomatosis with polyangiitis
  • some microscopic polyangiitis
  • some correlation with disease activity
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4
Q

pANCA (target, association, monitoring)

A
  • myeloperoxidase
  • mostly eosinophilic granulomatosis with polyangiitis
  • some microscopic polyangiitis
  • also in UC, primary sclerosis cholangitis, anti GBM, Crohn’s
  • no correlation with disease activity
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5
Q

Typical presentation of ankylosing spondylitis:

A
  • HLA-B27 associated spondyloarthropathy
  • more common in males 3:1 20-30yo
  • present with lower back pain and stiffness, worse in morning, at night, improves with exercise
  • reduced lateral flexion, forward flexion and chest expansion
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6
Q

What are the 8 A’s of ankylosing spondylitis:

A
  • apical fibrosis
  • anterior uveitis
  • achilles tendonitis
  • aortic regurgitation
  • amyloidosis
  • peripheral arthritis
  • cauda equina
  • AV node block
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7
Q

Investigations and signs of ankylosing spondylitis:

A

-increased CRP and ESR
-HLA B27 positive
x-ray sacroiliac joints:
-sacroiliitis: subchondral erosion, sclerosis
-squaring of lumbar vertebrae
-bamboo spine (late, rare)
-syndesmophytes
-apical fibrosis on CXR
spirometry: restriction

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8
Q

Management of ankylosing spondylitis:

A
  • exercise
  • physio
  • NSAIDs
  • DMARD if peripheral joint involvement
  • anti-TNF for persistent disease (etanercept, adalimumab)
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9
Q

What is antiphospholipid syndrome and what are the typical features?

A
  • acquired disorder of arterial and venous thromboses, recurrent foetal loss and thrombocytopaenia
  • paradoxical increase in APTT
  • other symptoms: livedo reticularis, pulmonary hypertension, pre-eclampsia
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10
Q

What are the associations of antiphospholipid syndrome and the management?

A
  • associated: SLE, autoimmune, lymphoproliferative disorders, phenothiazines
  • primary prophylaxis: aspirin
  • secondary prophylaxis: lifelong warfarin (target INR 2-3 for initial and arterial, 3-4 if occurred whilst taking aspirin and warfarin)
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11
Q

What is antisynthetase and the features:

A
  • autoantibodies against aminoacyl-tRNA synthetase e.g. Jo-1
  • myositis
  • interstitial lung disease
  • dry and cracked skin of hands
  • Raynaud’s
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12
Q

What is Behcet’s, epidemiology and features?

A

-multisystem disorder of autoimmune mediated inflammation of arteries and veins
-typically eastern mediterranean, men, young, HLA B51 positive, 30% FHx
Features:
-triad: genital ulcers, oral ulcers, anterior uveitis
-thrombophlebitis and deep vein thrombosis
-aseptic meningitis
-arthritis
-GI: colitis, abdominal pain, diarrhoea
-erythema nodosum

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13
Q

Calcium, phosphate, ALP and PTH levels in osteoporosis, osteopetrosis, Paget’s, CKD and osteomalacia:

A
  • everything normal in osteoporosis and osteopetrosis
  • Paget’s: everything normal except ALP increased
  • CKD: calcium decreased, everything else increased
  • Osteomalacia: calcium and phosphate decreased, everything else increased
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14
Q

What qualifies as CFS?

A

4 months of disabling fatigue affecting mental and physical function more than 50% of the time
more common in females

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15
Q

What can dactylitis be caused by?

A
  • spondyloarthopathies e.g. psoriatic and reactive arthritis
  • sickle cell
  • rare: TB, sarcoidosis, syphilis
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16
Q

What is dermatomyositis?

A
  • inflammatory disorder causing symmetrical, proximal muscle weakness and skin lesions
  • idiopathic or associated with CTD or underlying malignancy
  • polymyositis is variant with less skin manifestation
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17
Q

What are the features of dermatomyositis?

A

skin:

  • photosensitivity
  • macular rash over back and shoulder
  • heliotrope rash in periorbital region
  • mechanic’s hands
  • Gottron’s papules
  • nailfold capillary dilatation
  • proximal muscle weakness and tenderness
  • Raynaud’s
  • respiratory muscle weakness
  • interstitial lung disease
  • dysphagia, dysphonia
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18
Q

What are the investigations of dermatomyositis?

A
  • 80% ANA +ve

- 30% Ab to aminoacyl-tRNA synthetase (Jo-1, SRD, anti-Mi-2)

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19
Q

What are the features of drug induced lupus?

A
  • arthralgia
  • myalgia
  • malar rash
  • pleurisy
  • ANA +ve
  • dsDNA -ve
  • anti-histone Ab +ve
  • some anti-Ro, anti-Smith
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20
Q

Causes of drug induced lupus?

A

common: procainamide, hydralazine
rare: isoniazid, minocycline, phenytoin

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21
Q

What is EDS and what are the typical features?

A
  • autosomal dominant CTD affecting type III collagen
  • joint hypermobility
  • elastic, fragile skin
  • easy bruising
  • aortic regurgitation, aortic dissection, mitral valve prolapse
  • SAH
  • angioid retinal streaks
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22
Q

How can you treat fibromyalgia?

A
  • pregabalin
  • duloxetine
  • amitriptyline
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23
Q

Presentation of gout:

A
  • form of inflammatory arthritis
  • microcrystal synovitis - deposition of monosodium urate monohydrate in synovium
  • chronic hyperuricaemia (uric acid >0.45mmol/L)
  • episodes last several days with no symptoms in between
  • pain, swelling, erythema
  • most present 1st MTP joint
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24
Q

Radiological features of gout:

A
  • joint effusion early sign
  • well defined erosion with sclerotic margins in juxta-articular distribution
  • relative joint space preservation until late
  • eccentric erosions
  • no periarticular osteopaenia unlike RA
  • soft tissue tophi
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25
Q

Predisposing factors of gout:

A 
Decreased uric acid excretion:
-drugs: diuretics, aspirin
-CKD
-lead toxicity
Increased uric acid production:
-myeloproliferative/lymphoproliferative disorder
-cytotoxic drugs
-severe psoriasis
Lesch-Nyhan syndrome
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26
Q

What is Lesch Nyhan syndrome?

A
  • HGPRTase deficiency
  • x-linked recessive (only boys)
  • features: gout, renal failure, neuro deficits, learning difficulties, self-mutilation
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27
Q

Management of gout:

A

Acute:

  • NSAIDs or colchicine
  • max dose NSAIDs 1-2 days after symptoms settled
  • gastroprotection - PPI
  • colchicine slower onset (ADR: diarrhoea)
  • if CONTRA, oral steroids (prednisolone 15mg/day)
  • intra articular steroid injection
  • continue allopurinol
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28
Q

Indications for urate lowering therapy in gout:

A
  • > 2 attacks in 12 months
  • tophi
  • renal disease
  • uric acid renal stones
  • prophylaxis if on cytotoxic or diuretics
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29
Q

Use of urate lowering therapy in gout:

A

-allopurinol
-don’t start during attack
-initial 100mg od
-aim serum uric acid <300micromol/L
-lower initial dose if reduced eGFR
-2nd line: febuxostate (also xanthine oxidase inhibitors)
refractory cases:
-uricase - catalyses urate to allantoin
-pegloticase - rapid control hyperuricaemia

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30
Q

Lifestyle modifications for gout:

A
  • reduce alcohol and avoid in acute attack
  • lose weight
  • avoid food high in purines: kidney, liver, seafood, oily fish, yeast products
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31
Q

How does greater trochanteric pain syndrome present?

A
  • due to repeated movement of fibroelastic iliotibial band
  • pain and tenderness over lateral thigh
  • most common women 50-70yo
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32
Q

How does neuralgia paraesthetica present?

A
  • compression of lateral cutaneous nerve of thigh

- burning sensation over anterolateral thigh

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33
Q

How does transient idiopathic osteoporosis present?

A
  • uncommon condition sometimes in 3rd trimester
  • groin pain associated with limited range of movement in hip
  • unable to weight bear
  • ESR may be elevated
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34
Q

Which chromosome encodes the HLA genes?

A

chromosome 6

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35
Q

What is HLA A3 associated with?

A

haemachromatosis

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36
Q

What is HLA B51 associated with?

A

Behcet’s

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37
Q

What is HLA-B27 associated with?

A
  • ankylosing spondylitis
  • Reiter’s syndrome
  • acute anterior uveitis
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38
Q

What is HLA DQ2/8 associated with?

A

coeliac

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39
Q

What is HLA DR2 associated with?

A
  • narcolepsy

- goodpasture’s

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40
Q

What is HLA DR3 associated with?

A
  • dermatitis herpetiformis
  • Sjogren’s
  • primary biliary cirrhosis
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41
Q

What is HLA DR4 associated with?

A
  • T1DM

- RA

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42
Q

Type I hypersensitivity reaction:

A
  • anaphylactic
  • antigen reacts with IgE on mast cells
  • anaphylaxis, atopy
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43
Q

Type II hypersensitivity reaction: (7)

A
  • cell bound
  • IgG or IgM binds to antigen on cell surface
  • autoimmune haemolytic anaemia, ITP, good pasture’s, pernicious anaemia, acute haemolytic transfusion reactions, rheumatic fever, pemphigus vulgaris
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44
Q

Type III hypersensitivity reaction: (4)

A
  • immune complex
  • free antigen and antibody combine
  • serum sickness, SLE, post-streptococcal GN, extrinsic allergic alveolitis (acute phase)
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45
Q

Type IV hypersensitivity reaction: (7)

A
  • delayed hypersensitivity
  • T cell mediated
  • tuberculosis, GVHD, allergic contact dermatitis, scabies, extrinsic allergic alveolitis (chronic phase), MS, Guillain Barre
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46
Q

Type V hypersensitivity reaction:

A
  • Grave’s

- Myasthenia Gravis

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47
Q

What is Langerhans cell histiocytosis?

A
  • abnormal proliferation of histiocytes
  • typically child with bony lesions
  • bone pain (especially skull or femur)
  • cutaneous nodules
  • recurrent otitis media/mastoiditis
  • tennis racket shaped Birbeck granules on electromicroscopy
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48
Q

What is lateral epicondylitis?

A
  • typically after unaccustomed activity in 45-55yo affecting dominant arm
  • worse on wrist extension against resistance with elbow extended or supination of forearm with elbow extended
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49
Q

What is Marfan’s syndrome incl features and therapy?

A
  • autosomal dominant CTD
  • defect FBN1 gene on chromosome 15 (codes for fibrillin1)
  • tall stature with arm span to height >1.05
  • high arched palate
  • arachnodactyly
  • pectus excavatum
  • pes planus
  • scoliosis >20 degrees
  • heart: dilation aortic sinuses leading to aneurysms, dissection, regurgitation, mitral valve prolapse
  • lungs: repeated pneumothoraces
  • eyes: upwards lens dislocation, blue sclera, myopia
  • dural ectasia
  • therapy: regular echocardiography monitoring and beta blockers/ACEi
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50
Q

What is McArdle’s disease?

A
  • autosomal recessive type II glycogen storage disease
  • myophosphorylase deficiency
  • causes decreased muscle glycogenolysis
  • myalgia and stiffness after exercise, cramps, myoglobinuria, decreased lactate levels during exercise
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51
Q

Causes of myopathies:

A
  • polymyositis
  • Duchenne/Becker muscular dystrophy, myotonic dystrophy
  • endocrine: Cushing’s, thyrotoxicosis
  • alcohol
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52
Q

Osteoarhritis vs Rheumatoid differences:

A

Osteoarthritis:
-mechanical wear and tear
-localised loss of cartilage, remodelling of adjacent bone, associated inflammation
-mostly in elderly
-large weight bearing joints, carpometacarpal joints, DIP, PIP joints
-pain following use, improves with rest, unilateral, no systemic
Rheumatoid arthritis:
-autoimmune
-more in women
-all ages
-MCP, PIP joints
-morning stiffness, improves with use, bilateral, systemic upset

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53
Q

X-ray findings in osteoarhtirtis and rheumatoid arthritis:

A 
Osteoarthritis:
-loss of joint space
-subchondral sclerosis
-subchondral cyst
-osteophytes forming at joint margins
Rheumatoid:
-loss of joint space
-juxta-articular
-osteoporosis
-periarticualr erosions
-subluxation
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54
Q

Management of osteoarthritis:

A
  • weight loss, muscle strengthening
  • paracetamol and topical NSAIDs
  • second line: oral NSAIDs/COX-2 inhibitors (+PPI), opioids, capsaicin cream, intraarticular corticosteroids
  • joint replacement
  • glucosamine: part of glycosaminoglycans in cartilage and synovial fluid
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55
Q

Osteogenesis imperfecta, features and investigations:

A
  • brittle bone disease
  • type I collagen - decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
  • autosomal dominant
  • presents in childhood
  • family history following minor trauma
  • blue sclera
  • deafness secondary to otosclerosis
  • dental imperfections
  • adjusted calcium, phosphate, PTH and ALP normal
56
Q

Osteomalacia features and treatment:

A
  • normal bone tissue but decreased mineral content
  • rickets if whilst growing: knock-knee, bow leg, features of hypocalcaemia
  • osteomalacia if after epiphysis fusion: bone pain, fractures, muscle tenderness, proximal myopathy
  • Tx: calcium and vit D tablets
57
Q

Investigations of osteomalacia:

A
  • reduced 25 (OH) vit D
  • increased ALP
  • reduced calcium and phosphate
  • x-ray: children have cupped, ragged metaphysical surfaces, adults have translucent bands
58
Q

What is osteoporosis?

A
  • loss of bone mass
  • bone m mineral density <2.5 SD below young adult mean density
  • increased risk of fragility fractures
59
Q

What are the risk factors for osteoporosis?

A
  • corticosteroids
  • smoking
  • alcohol
  • low BMI
  • FHx
  • RA
  • sedentary
  • premature menopause
  • caucasians and asians
  • endocrine
  • multiple myeloma, lymphoma
  • GI disorders: IBD, malabsorption, gastrectomy, liver disease
  • CKD
  • osteogenesis imperfecta, homocystinuria
  • other meds: SSRIs, anti epileptics, PPI, glitazones, long term heparin therapy, aromatase inhibitors e.g. anastrozole
60
Q

DEXA scan values:

A

T score (based on bone mass of young reference population)
->1.0 normal
- -1.0 to -2.5 osteopenia
-

61
Q

Management of osteoporosis:

A
  • if osteoporotic fragility fracture in postmenopausal women (DEXA not needed >=75yo)
  • vitamin D and calcium supplementation
  • alendronate 1st line
  • if not tolerated, risedronate or etidronate
  • if not tolerate, strontium ranelate and raloxifene
62
Q

Bisphosphonates:

A
  • post menopausal and glucocorticoid induced osteoporosis
  • reduces risk of both vertebral and non vertebral fractures
  • alendronate and risderonate superior to etidronate
  • ibandronate once monthly oral bisphosphonate
63
Q

Raloxifene:

A
  • selective oestrogen receptor modulator
  • prevent bone loss and reduce risk of vertebral fractures
  • increase bone density in spine and proximal femur
  • may worsen menopausal symptoms
  • increased risk of thromboembolic events
  • decreases risk of breast cancer
64
Q

Strontium Ranelate

A
  • dual action bone agent
  • increased new bone by osteoblasts and decreased resorption by osteoclasts
  • increased risk of CV events and thromboembolic
  • may cause skin reaction e.g. Stevens Johnson syndrome
65
Q

Denosumab:

A
  • single subcutaneous injection every 6 months

- monoclonal Ab that inhibits osteoclast maturation

66
Q

Teriparatide

A
  • recombinant PTH

- very effective at increased bone mineral density

67
Q

HRT

A
  • decreased incidence vertebral fractures and non-vertebral fractures
  • increased risk CVD and breast cancer
68
Q

How do you assess patients for osteoporosis after a fracture?

A 
>=75yo
-assumed to have underlying osteoporosis
-start on 1st line bisphosphonates
-no scan needed
<75yo
-DEXA
-FRAX assessment
69
Q

How do you manage glucocorticoid induced osteoporosis:

A
  • increased risk if equivalent of prednisolone 7.5mg/day for 3 or more mo
  • start bone protection
  • patients >65yo or fragility fracture offer bone protection
  • <65yo offer bone density scan and further management
  • 1st line alendronate
  • should be calcium and vitamin D replete
70
Q

Paget’s disease of bone, features:

A
  • increased and uncontrolled bone turnover
  • disorder of osteoclasts
  • predisposing: age, male, northern latitude, family history
  • typically older male with bone pain and isolate increase ALP
  • bowing of tibia, bossing of skull, thickened vault, osteoporosis circumscripta
  • calcium and phosphate normal
  • markers bone turnover: PINP, CTx, NTx, urinary hydroxyproline
  • treatment if: bone pain, skull or long bone deformity, fractures, periarticular Paget’s - bisphosphonates, calcitonin
71
Q

Paget’s disease complications:

A
  • deafness (cranial nerve entrapment)
  • bone sarcoma
  • fractures
  • skull thickening
  • high output cardiac failure
72
Q

Polyarteritis nodosa and features:

A
  • vasculitis affecting medium-sized arteries with necrotising inflammation - aneurysm
  • more common middle aged men and hep B associated
  • fever, malaise, arthralgia, weight loss, HTN, mono neuritis multiplex, sensorimotor polyneuropathy, testicular pain, lived reticular, haematuria, renal failure, pANCA, hep B positive
73
Q

What causes poly arthritis?

A
  • RA
  • SLE
  • seronegative spondyloarthropathies
  • HSP
  • sarcoidosis
  • TB
  • pseudogout
  • viral infection
74
Q

Polymyalgia rheumatica, features, investigations, treatment:

A

-closely related to temporal arteritis
Features
->60yo
-rapid onset <1 month
-aching, morning stiffness in proximal limb muscles (not weakness)
-mild polyarthralgia, lethargy, depression, low grade fever, anorexia, night sweats
-investigations: raised inflammatory markers, creatinine kinase, EGM normal
-treat with prednisolone e.g. 15mg/od

75
Q

Polymyositis:

A
  • inflammatory disorder causing symmetrical proximal muscle weakness
  • T cell mediate cytotoxic process against muscle fibres
  • idiopathic or associated with CTD
  • associated with malignancy
  • Raynauds, respiratory muscle weakness, ILD, dysphagia, dysphonia
  • investigations: increased creatinine kinase, other muscle enzymes, EMG, muscle biopsy, anti-synthetase Ab (anti-Jo-1)
76
Q

What will joint aspiration show in gout?

A

needle shaped negatively charged birefringent crystals under polarised light

77
Q

Confirming diagnosis of anti-phospholipid?

A

anti-cardiolipin Ab

78
Q

Features of pseudo gout:

A
  • strongly associated with increasing age
  • if younger: haemachromatosis, hyperparathyroidism, hypomagnasaemia and hypophosphataemia, acromegaly, Wilson’s disease
  • knee, wrist and shoulder most common
  • joint aspiration: weakly positive birefringent rhomboid shaped crystals
  • x-ray: chondrocalcinosis (e.g. knee - linear calcifications of meniscus and articular cartilage)
79
Q

Management of pseudo gout:

A
  • aspiration of joint, to exclude septic arthritis

- NSAIDs or intra-articular, intramuscular or oral steroids as for gout

80
Q

Psoriatic arthropathy:

A

-correlates poorly with cutaneous psoriasis and often precedes development of skin lesions

types:

  • rheumatoid like polyarthritis
  • assymmetrical oligoarthritis (typically hands and feet) - pencil in cup appearance
  • sacroilitis
  • DIP joint disease
  • arthritis mutilans
  • managed by rheumatologist
  • treat as RA but better prognosis
81
Q

What is Raynaud’s phenomenon?

A
  • exaggerated vasoconstrictive response of digital arteries and cutaneous arteriole to cold or emotional stress
  • primary: disease
  • secondary: phenomenon
82
Q

Secondary causes of Raynaud’s phenomenon:

A
  • CTD - RA, scleroderma, RA, SLE
  • leukaemia
  • type I cryoglobulinaemia, cold agglutins
  • use of vibrating tools
  • drugs: COCP, ergot
  • cervical rib
83
Q

Factors suggesting CTD with Raynaud’s phenomenon:

A
  • onset after 40yo
  • unilateral symptoms
  • rashes
  • presence autoAb
  • digital ulcers, calcinosis
  • rarely chilblains
84
Q

Management of Raynaud’s phenomenon:

A
  • secondary care
  • calcium channel blockers e.g. nifedipine
  • IV prostacyclin infusions (epoprostenol)
85
Q

Reactive arthritis:

A
  • HLA B27 seronegative spondyloarthropathies
  • following an infection: can’t see, can’t pee, can’t climb up a tree
  • post STI: chlamydia trachomatis
  • post dysenteric: shigella, salmonella typhimurium and enteritidis, yersinia enterocolitica, campylobacter
  • also known as Reiter’s syndrome
86
Q

Features of reactive arthritis:

A
  • within 4 weeks of initial infection
  • symptoms 4-6 months
  • typically asymmetrical oligoarthritis of lower limb
  • dactylitis
  • urethritis
  • eye: conjunctivitis, anterior uveitis
  • skin: circinate balanitis, keratoderma blenorrhagica
87
Q

Management of reactive arthritis:

A
  • symptomatic: analgesia, NSAIDs, intra-articular steroids
  • sulfasalazine and methotrexate for persistent
  • symptoms rarely more than 12 months
88
Q

Septic arthritis:

A
  • most commonly staph aureus
  • typically young, sexually active adults - neisseria gonorrhoea (disseminated gonococcal infection)
  • most haematogenous spread
  • knee in adults
  • acute, swollen joint, restricted movement, warm, fever
89
Q

Investigations for septic arthritis:

A
  • synovial fluid sampling obligatory before Abx - urgent orthopaedic review
  • blood cultures
  • joint imaging
90
Q

Management of septic arthritis:

A
  • IV Abx for gram +ve cocci
  • flucloxacillin or clarithromycin
  • needle aspiration to decompress
  • arthroscopic lavage may be required
91
Q

Seronegative spondyloarthropathy:

A
  • associated with HLA-B27
  • rheumatoid factor negative
  • peripheral arthritis, usually asymmetrical
  • sacroiliitis
  • extra articular manifestations: uveitis, pulmonary fibrosis, amyloidosis, aortic regurgitation
    e. g. ankylosing spondylitis, psoriatic arthritis, Reiter’s syndrome, enteropathic arthritis
92
Q

Sjogren’s disease overview (associations and increased risk off…):

A
  • autoimmune disorder affecting exocrine glands
  • dry mucosal surfaces
  • primary or secondary to RA or other CTD
  • female 9:1
  • increased risk of lymphoma
93
Q

Sjogren’s features:

A
  • dry eyes: keratoconjunctivitis sicca
  • dry mouth
  • vaginal dryness
  • arthralgia
  • Raynaud’s, myalgia
  • sensory polyneuropathy
  • recurrent episodes of parotitis
  • RTA
94
Q

Investigations for Sjogren’s:

A
  • rheumatoid factor positive 50%
  • ANA 70%
  • anti Ro Ab 70%
  • anti-La Ab 30%
  • Schirmer’s test - measure tear formation
  • histology: focal lymphocyte infiltration
  • hypergammaglobulinaemia, low C4
95
Q

Management of Sjogren’s:

A
  • artificial saliva and tears

- pilocarpine - saliva production

96
Q

Still’s disease features:

A
  • arthralgia
  • increased serum ferritin
  • salmon-pink maculopapular rash
  • pyrexia in late afternoon in daily pattern with worsening symptoms and rash
  • lymphadenopathy
  • rheumatoid and ANA negative
  • Yamaguchi criteria sensitivity 93.5%
97
Q

Management of Still’s disease:

A
  • NSAIDs (1st line, trialled week before steroids)
  • steroids (won’t improve prognosis)
  • methotrexate, IL1 and anti-TNF if persistent
98
Q

Overview of SLE:

A
  • more common in females 9:1
  • Afro-caribbean and asian
  • onset 20-40yo
  • autoimmune type III hypersensitivity
  • HLA B8, DR2, DR3
  • immune complex deposition affecting skin, joints, kidneys and brain
99
Q

Features of SLE:

A
  • general: fatigue, fever, mouth ulcers, lymphadenopathy
  • skin: malar rash (not nasolabial), discoid rash, photosensitivity, Raynaud’s, livedo reticularis, non-scarring alopecia
  • MSK: arthralgia, non-erosive arthritis
  • CV: pericarditis, myocarditis
  • respiratory: pleurisy, fibrosing alveolitis
  • renal: proteinuria, GN
  • neurospych: anxiety and depression, psychosis, anxiety
100
Q

Investigations for SLE:

A

-99% ANA positive (useful to rule out)
-20% RF positive
-anti-dsDNA most sensitive test
-anti Smith, anti U1 RNP, SSA (anti-Ro) and SSB (anti-La)
monitoring:
-inflammatory markers
-decreased C3, C4
-anti-dsDNA for monitoring

101
Q

Types of systemic sclerosis:

A
  • limited cutaneous
  • diffuse cutaneous
  • scleroderma
102
Q

Limited cutaneous systemic sclerosis:

A
  • Raynaud’s may be 1st sign
  • associated with anti-centromere Ab
  • scleroderma affects face and distal limbs
  • CREST: calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangiectasia
103
Q

Diffuse cutaneous systemic sclerosis:

A
  • scleroderma affects trunk and proximal limbs
  • associated with scl-70 Ab
  • respiratory involvement most common death
  • other complications: renal disease and HTN
  • poor prognosis
104
Q

Scleroderma:

A
  • positive anti-centromere
  • without internal organ involvement
  • tightening and fibrosis of skin
  • manifest as plaques or linear
105
Q

Antibodies in systemic sclerosis:

A
  • ANA 90% positive
  • RF 30% positive
  • anti-scl-70 Ab associated with diffuse cutaneous
  • anti-centromere Ab associated with limited cutaneous
106
Q

Temporal arteritis features:

A
  • large vessel vasculitis
  • skip lesions
  • typically >60yo
  • rapid onset <1mo
  • headache
  • jaw claudication
  • visual disturbances - check vision, secondary to anterior ischaemic optic neuropathy
  • tender palpable temporal artery
  • lethargy, depression, low fever, anorexia, night sweats
107
Q

Investigations for temporal arteritis:

A
  • inflammatory markers increased
  • temporal artery biopsy (skip lesions)
  • creatinine kinase and EMG normal
108
Q

Treatment of temporal arteritis:

A
  • high dose prednisolone (dramatic response)

- urgent ophthalmology review same day, irreversible

109
Q

Azathioprine (MOA, ADR, interaction, pregnancy)

A
  • active component: mercaptopurine (inhibits purine synthesis)
  • thiopurine methyltransferase (TPMT) test for people prone to azathioprine toxicity
  • ADR: bone marrow depression, n&v, pancreatitis, increased risk non-melanoma skin cancer
  • significant interaction with allopurinol (decrease dose of azathioprine)
  • safe in pregnancy
110
Q

Bisphosphonates (MOA, uses, ADR, how to take)

A
  • analogues of pyrophosphate (decreases demineralisation and inhibits osteoclasts by reducing recruitment and increasing apoptosis)
  • uses: osteoporosis, hypercalcaemia, Paget’s disease, pain from bone metastases
  • ADR: oesophagi’s, ulcers (especially aldendronate), jaw osteonecrosis, increased risk atypical stress fracture at proximal femoral shaft (alendronate), cue phase response (fever, myalgia, arthralgia), hypocalcaemia
  • correct hypocalcaemia and vit D before
  • take 30 mins before breakfast with water and sit up for 30 mins
111
Q

What are the conditions for stopping bisphosphonates at 5 years?

A
  • <75yo
  • femoral neck T-score >-2.5
  • low risk according to FRAX/NOGG
112
Q

Hydroxychloroquine (MOA, ADR, pregnancy)

A
  • management of RA and systemic/discoid lupus erythematosus
  • similar to chloroquine to treat malaria
  • ADR: bull’s eye retinopathy (may cause severe and permanent loss)
  • fine in pregnancy
113
Q

Interferon alpha

A
  • produced by leucocytes
  • antiviral action
  • used in hep B, C, Kaposi’s sarcoma, metastatic RCC, hairy cell leukaemia
  • ADR: flu-like symptoms and depression
114
Q

Interferon beta

A
  • produced by fibroblasts
  • antiviral action
  • reduced frequency of exacerbations in patients with relapsing-remitting MS
115
Q

Interferon gamma

A
  • predominantly NKC, also T helper cells
  • weaker antiviral action, more in immunomodulation esp. macrophage activation
  • useful chronic granulomatous disease and osteoporosis
116
Q

Methotrexate (MOA, indications, ADR, pregnancy)

A
  • antimetabolite inhibits dihydrofolate reductase (used for purine and pyrimidine synthesis)
  • used for inflammatory arthritis, psoriasis, some chemotherapy acute lymphoblastic leukaemia
  • ADR: mucositis, myelosuppression, pneumonitis, pulmonary fibrosis, liver fibrosis
  • avoid in pregnancy for 6 months after treatment stopped
  • men use contraception 6 months after
117
Q

How is methotrexate prescribed?

A
  • take weekly until stable
  • monitor every 2-3 months
  • folic acid 5mg once weekly, take >24 hours after methotrexate
  • start at 7.5mg weekly
  • only one dose strength
118
Q

Interactions of methotrexate:

A
  • avoid trimethoprim or co-trimoxazole (increased risk of marrow aplasia)
  • increased dose of aspirin - methotrexate toxicity due to reduced excretion
  • methotrexate toxicity treatment is folinic acid
119
Q

Penicillamine (use and ADR)

A
  • used for RA
  • decreased IL-1 synthesis and decreased maturation of newly synthesised collagen
  • ADR: rashes, disturbance of test, proteinuria
120
Q

Sulfasalazine (MOA, cautions, ADR, pregnancy)

A
  • pro drug for 5-ASA
  • decreased neutrophil chemotaxis and decreased proliferation lymphocytes and pro-inflammatory cytokines
  • cautions: G6PD deficiency, allergy to aspirin or sulphonamide
  • ADR: oligospermia, Steven-Johnson syndrome, pneumonitis/lung fibrosis, myelosuppression, Henz body anaemia, megaloblastic anaemia, may colour tears (stained contact lenses)
  • safe in pregnancy and breast feeding unlike other DMARDs
121
Q

Supraspinatus

A
  • abducts arm before deltoid

- most commonly injured

122
Q

Infraspinatus

A

-rotates arm laterally

123
Q

Teres minor

A
  • adducts

- rotates arm laterally

124
Q

Subscapularis

A
  • adducts

- rotates arm laterally

125
Q

Rotator cuff muscles:

A
  • supraspinatus
  • infraspinatus
  • teres minor
  • subscapularis
126
Q

IgG (frequency, shape etc.)

A
  • 75%
  • monomer
  • enhance phagocytosis of bacteria and viruses
  • fixes complement and passes to foetal circulation
  • most abundant isotope in blood serum
127
Q

IgA (frequency, shape etc.)

A
  • 15%
  • monomer/dimer
  • IgA is predominant immunoglobulin found in breast milk
  • also found in secretions of digestive, respiratory, urogenital tracts
  • provides localised protection on mucous membranes
  • most commonly produced in body but lower serum conc that IgG
128
Q

IgM (frequency, shape etc.)

A
  • 10%
  • pentamer
  • first secreted in response to infection
  • fixes complement but does not pass into foetal
129
Q

IgD (frequency, shape etc.)

A
  • 1%
  • monomer
  • role in immune system largely unknown
  • involved in activation of B cells
130
Q

IgE (frequency, shape etc.)

A
  • mediates type I hypersensitivity reactions
  • binds to Fc receptors found on man cells and basophils
  • provides immunity to parasites such as helminths
  • least abundant isotope in serum
131
Q

Typical finding in arthrocentesis of reactive arthritis:

A
  • cloudy yellow colour

- no crystals

132
Q

Arthrocentesis in RA:

A
  • high WBC
  • turbic yellow
  • no crystals
133
Q

Septic arthritis arthrocentesis:

A

same as RA but systemic symptoms and precipitate by trauma

134
Q

Most common organism septic arthritis in children:

A

strep pyogenes

135
Q

Most common organism septic arthritis after joint replacement:

A

staph epidermidis

PM Rheumatology (3 decks)

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