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Phase 3 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are features of polymyalgia rheumatica?

A 
Weight loss
Proximal muscle stiffness and tenderness
Headaches 
Scalp tenderness 
Raised ESR
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2
Q

Why can false negatives occur with temporal biopsy in temporal arteritis?

A

The disease may patchily affect the artery so the sample may not contain inflammation

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3
Q

Why is it important to recognise and treat temporal arteritis early?

A

Reduce morbidity

Prevent blindness due to involvement of the optic arteries with retinal ischaemia

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4
Q

A 7 year old girl presents with ankle and knee pain and a skin rash. She has been well apart from a cold two weeks previously. She had a full term normal delivery with no neonatal problems. Her immunisations are up to date. There is no family or social history of note. On examination she looks well. The temperature is 37.8 with RR15, HR 80 and BP 100/70. She has a scanty non blanching rash over the shins. Her abdomen is soft and non tender. There is swelling, redness, tenderness and deceased movement of the right knee and left ankle. What is the likely diagnosis? What tests should be done now?

A

Henoch Schonlein purpura

Urine dipstick to look for renal involvement

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5
Q

A 76 year old lady presents with severe headache and pain on combing her hair. She has also noticed recent episodes of visual loss which last only a few seconds. During the past weeks she has been feeling more tired and has found it difficult to get up in the mornings due to stiff and aching joints. What is the immediate management?

A

Prednisolone 40-60mg daily

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6
Q

What are presenting complaints of Wegeners granulomatosis?

A 
Severe haemorrhagic rhinorrhoea
Paranasal sinusitis
Nasal mucosal ulceration
Serous or purulent otitis media
Hearing loss
Cough 
Haemoptysis 
Pleuritis 
Haematuria
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7
Q

What renal changes do you often see in Wegeners granulomatosis?

A

Glomerulonephritis with proteinuria, haematuria and red cell casts

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8
Q

What blood results might you see in Wegeners granulomatosis?

A

Raised ESR
Leukocytosis
Anaemia
ANCA

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9
Q

What are the Duckett Jones criteria for acute rheumatic fever?

A 
Major: 
Migratory polyarthritis
Pancarditis
Chorea
Erythema marginatum
Nodules
Minor:
Fever
Arthralgia
Raised inflammatory indices
Prolonged PR interval 
Diagnosis: recent strep infection, two major or one major and two minor criteria
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10
Q

What is splenomegaly associated with RA called?

A

Feltys syndrome

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11
Q

What are associated complications with RA?

A

Vasculitis
Pericarditis
Normochromic normocytic anaemia
Instability of Atlanto-axial joint

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12
Q

A 24 year old woman from western India presents with symptoms of lethargy and dizziness worse on turning her head. On examination her blood pressure is 176/128. Her pulses are impalpable at all peripheral sites. Auscultation of her chest reveals a systolic heart murmur. What is the diagnosis?

A

Takayasus arteritis

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13
Q

What conditions are associated as part of the autoimmune polyendocrine syndrome?

A 
Pernicious anaemia
Type 1 diabetes
Addison's disease
Vitiligo 
Sjogrens syndrome
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14
Q

What are features of SLE?

A 
Fatigue
Fevers
Mouth ulcers
Lymphadenopathy 
Malar rash 
Photosensitivity
Raynauds
Livedo reticularis
Arthralgia
Myocarditis
Pleurisy
Fibrosing alveolitis 
Glomerulonephritis
Anxiety and depression
Psychosis
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15
Q

What is the prevalence of ankylosing spondylitis?

A

1 in 2000

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16
Q

What are some complications of ankylosing spondylitis?

A 
Chest pain/carditis
Aortic regurgitation
Cardiac conduction abnormalities
Decrease in thoracic excursion
Periositis of calcaneum and ischial tuberosities
Amyloidosis
Iritis
Lung fibrosis
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17
Q

What immunology tests may be positive in SLE?

A 
ANA 99%
RF 20% 
Anti ds DNA 
Anti Smith 
Anti Ro 
Anti La
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18
Q

How can SLE be regularly monitored?

A

ESR
Complement levels low during active disease
Anti ds DNA titres

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19
Q

What is Feltys syndrome?

A

Splenomegaly and neutropenia in patient with RA

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20
Q

What are complications of RA?

A 
Resp: pulmonary fibrosis, pleural effusion, nodules, methotrexate pneumonitis
Ocular: keratoconjunctivitis sicca, episcleritis, scleritis, corneal ulceration, keratitis, steroid induced cataracts, chloroquine retinopathy 
Osteoporosis 
Ischaemic heart disease
Increased risk of infections
Depression 
Feltys syndrome
Amyloidosis
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21
Q

A 48 year old male presents with an 8 week history of epistaxis and nasal stuffiness. On examination there is evidence of nasal crusting. A chest X-ray demonstrates multiple cavitary lesions, what is the diagnosis and what is the most appropriate antibody test?

A

Granulomatosis with polyangiitis (wegeners)

ANCA

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22
Q

What are features of granulomatosis with polyangiitis?

A

Upper respiratory tract: epistaxis, sinusitis, nasal crusting
Lower respiratory tract: dyspnoea, haemoptysis
Rapidly progressing glomerulonephritis
Saddle shaped nose deformity
Vasculitic rash
Proptosis
Cranial nerve lesions

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23
Q

What are features of antiphospholipid syndrome?

A 
Recurrent miscarriages 
Strokes
Thrombocytopenia 
DVT/PE
MI
Rash: livedo reticularis 
Chronic headaches, migraines
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24
Q

What investigations should be done for granulomatosis with polyangiitis?

A

cANCA positive in 90%
Chest X-ray
Renal biopsy: epithelial crescents in bowmans capsule

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25
Q

What is the management for granulomatosis with polyangiitis?

A

Steroids
Cyclophosphamide
Plasma exchange

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26
Q

What is the acute management of gout?

A

NSAIDs
Intra articular steroid injections
Colchicine
Oral steroids

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27
Q

What are features of temporal arteritis?

A 
Rapid onset <1 month
Headache
Jaw claudication
Visual disturbance secondary to anterior ischaemic optic neuropathy 
Tender palpable temporal artery
Features of PMR: aching, morning stiffness 
Lethargy
Depression
Low grade fever
Anorexia 
Night sweats
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28
Q

What are some side effects of methotrexate?

A

Myelosuppression
Liver cirrhosis
Pneumonitis

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29
Q

What are some side effects of sulfasalazine?

A

Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease

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30
Q

What are some side effects of leflunomide?

A

Liver impairment
Interstitial lung disease
HTN

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31
Q

What are some side effects of hydroxychloroquine?

A

Retinopathy

Corneal deposits

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32
Q

What are some side effects of prednisolone treatment?

A 
Cushingoid features
Osteoporosis 
Impaired glucose tolerance
HTN
Cataracts
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33
Q

What is a side effects of gold?

A

Proteinuria

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34
Q

What are some side effects of penicillamine?

A

Proteinuria

Exacerbation of myasthenia gravis

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35
Q

What are some side effects of etanercept?

A

Demyelination

Reactivation of TB

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36
Q

What are some side effects of azathioprine?

A 
Black tarry stools
Bleeding gums
Blood in urine 
Fever 
Sores/ulcers in lips or mouth
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37
Q

What are X-ray findings of osteoarthritis?

A

Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts

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38
Q

What are X-ray findings of rheumatoid arthritis?

A

Loss of joint space
Erosions
Soft tissue swelling
Soft bones - osteopenia

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39
Q

What is schobers test?

A

Line drawn 10 cm above and 5cm below back dimples. Distance between 2 lines should increase by more than 5cm when patients bends as far forwards as possible

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40
Q

Which respiratory problems may present in patients with rheumatoid arthritis?

A 
Pulmonary fibrosis 
Pleural effusion 
Pulmonary nodules
Bronchiolitis obliterans
Methotrexate pneumonitis 
Pleurisy 
Caplans syndrome 
Infection secondary to immunosuppression
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41
Q

Which antibodies are associated with systemic sclerosis?

A

ANA
RF
Anti scl 70
Anti centromere

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42
Q

What tests can confirm antiphospholipid syndrome?

A

Lupus anticoagulant

Anti cardiolipin antibodies

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43
Q

What clotting abnormality might be detected in antiphospholipid syndrome?

A

Prolonged APTT that doesn’t correct when mixed with normal plasma

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44
Q

What are similarities and differences between granulomatosis with polyangiitis and churg Strauss syndrome?

A

Wegeners: renal failure, epistaxis, haemoptysis, cANCA
Both: vasculitis, sinusitis, dyspnoea
Churg-Strauss: asthma, pANCA, eosinophilia

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45
Q

Which systems are commonly involved in polyarteritis nodosa?

A 
Skin
Joints
Peripheral nerves
GI tract 
Kidney
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46
Q

What type of glomerulonephritis occurs in henoch schonlein purpura?

A

Membranoproliferative

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47
Q

What is lupus pernio?

A

Macular popular violaceous rash over nose cheeks and ears due to granulomatous infiltration of the skin usually due to sarcoidosis

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48
Q

What is lofgrens syndrome?

A

Erythema nodosum
Polyarthritis
Bilateral hilar lymphadenopathy

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49
Q

Why can sarcoidosis be associated with diabetes insipidus?

A

Sarcoid tissue may infiltrate endocrine glands

If it infiltrates the hypothalamus and posterior pituitary it can lead to cranial DI

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50
Q

What are the most common causes of erythema nodosum in the U.K?

A 
Sarcoidosis 
Tuberculosis 
OCP 
Strep infections
EBV 
IBD
Haematological malignancies
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51
Q

What are some complications of sarcoidosis?

A

Eyes: Anterior uveitis, conjunctival deposits, dry eyes, choroidoretinitis, retinal haemorrhages
Heart: Cardiomyopathy, conduction system disease, cardiac failure
Diabetes insipidus
Skin: lupus pernio, erythema nodosum

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52
Q

Which types of clots do people with antiphospholipid syndrome get?

A 
MI
Recurrent miscarriage
DVT
Stroke
Pulmonary hypertension due to thromboembolic disease
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53
Q

What is Takayasus arteritis?

A

Large vessel granulomatous vasculitis with intimal fibrosis and vascular narrowing
Young or middle age women of Asian descent
Mainly affects aorta, branches of and the pulmonary arteries
Inflammatory phase: malaise, fever, night sweats, joint pain
Fainting due to subclavian steak syndrome or carotid sinus sensitivity
Pulseless phase: vascular insufficiency, arm or leg claudication, renal artery stenosis and HTN

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54
Q

What is Buergers disease?

A

Thromboangiitis obliterans
Inflammation and thrombosis of small and medium arteries and veins of hands and feet
Strongly associated with smoking

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55
Q

What are some facial features of systemic sclerosis?

A

Facial telangiectasia
Small mouth
Beaking of the nose

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56
Q

What is the management for raynauds?

A

Calcium channel blockers eg nifedipine

IV prostacyclin infusions

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57
Q

What is lofgrens syndrome?

A 
Acute form of sarcoidosis 
Bilateral hilar lymphadenopathy 
Erythema nodosum
Fever
Polyarthralgia
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58
Q

What is Mikulicz syndrome?

A

Form of sarcoidosis with enlargement of parotid and lacrimal glands
Can also be due to TB or lymphoma

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59
Q

What is Heerfordts syndrome?

A 
Uveoparotid fever 
Parotid enlargement 
Fever 
Uveitis 
Secondary to sarcoidosis
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60
Q

When should allopurinol be started after an attack of gout?

A

Start if 2 or more episodes in a 12 month period

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61
Q

What level of uric acid is diagnostic for gout?

A

> 450 micromol/l

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62
Q

What are skin features of dermatomyositis?

A 
Photosensitive
Macular rash over back and shoulders
Heliotrope rash in periorbital region
Gottrons papules
Nail fold capillary dilatation
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63
Q

What are non skin features of dermatomyositis?

A 
Proximal muscle weakeness/tenderness
Raynauds
Respiratory muscle weakness
Interstitial lung disease - fibrosing alveolitis or organising pneumonia 
Dysphagia 
Dysphonia
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64
Q

What are characteristics of behchets syndrome?

A 
Recurrent oral and genital ulcers
Uveitis
Seronegative arthritis 
Central nervous system symptoms
Fever
Thrombophlebitis 
Erythema nodosum
Abdominal symptoms
Vasculitis
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65
Q

What is adult onset stills disease?

A

Persistent high spiking fevers
Joint pain
Salmon coloured bumpy rash

66
Q

What is stills disease?

A

Systemic onset juvenile idiopathic arthritis

Type of JIA with extra articular manifestations like fever, rash, splenomegaly, lymphadenopathy

67
Q

What are the major criteria for diagnosis of adult onset stills disease?

A

High fever lasting one week or longer
Joint pain lasting two weeks or longer
Rash
Abnormal white cell count and other blood problems

68
Q

What are minor criteria for the diagnosis of adult onset stills disease?

A

Sore throat
Swelling of lymph nodes or spleen
Liver problems
Absence of rheumatoid arthritis

69
Q

Which scoring system is used to assess disease activity in RA?

A

DAS score

70
Q

Which scoring system is used to assess disease activity in ankylosing spondylitis?

A

BAS DAI

71
Q

What conditions can underly dermatomyositis?

A

Malignancy: lymphoma, breast, lung, ovarian, colon
RA
IBD

72
Q

What are radiological features of rheumatoid arthritis?

A 
Joint space narrowing
Periarticular osteoporosis 
Erosions
Periarticular soft tissue swelling 
Joint destruction 
Subluxation
73
Q

What are possible causes for anaemia in a patient with RA?

A

Anaemia of chronic disease
GI blood loss secondary to nsaid use
Bone marrow suppression due to gold, phenylbutazone, indomethacin, penicillamine
Megaloblastic anaemia due to folic acid deficiency or pernicious anaemia
Feltys syndrome

74
Q

What is the name of the joint deforming arthropathy found in SLE which can resemble RA?

A

Jaccouds arthropathy

75
Q

What is the name given to the genital rash in reactive arthritis?

A

Circinate balanitis

76
Q

What is the name of the skin rash in reactive arthritis?

A

Keratoderma blennorrhagica

77
Q

What are the 5 types of psoriatic arthropathy?

A 
Symmetric
Asymmetric 
DIP predominant
Spondylitis
Arthritis mutilans
78
Q

Which disease is associated with pencil in cup deformity on X-ray?

A

Psoriatic arthritis

79
Q

What is the tetrad of symptoms in henoch schonlein purpura?

A

Purpuric rash
Abdominal pain
Arthritis/arthralgia
Glomerulonephritis

80
Q

How is a diagnosis of Ankylosing spondylitis made?

A

Clinical criteria: low back pain for more than 3 months improved by exercise but not relieved by rest
Limitation of lumbar spine motion in sagittal and frontal planes
Limitation of chest expansion relative to normal values for age and sex
Radiological: sacroiliitis on X-ray
Definite if radiological criteria plus at least one clinical criteria
Probable if three clinical criteria alone or if radiological criteria but no clinical

81
Q

What is shiny corner sign?

A

Enthesitis in spine - ankylosing spondylitis

82
Q

What is the gold standard treatment for ANCA vasculitis?

A

Three pulses of IV steroids

Pulsed IV cyclophosphamide

83
Q

In what proportion of primary biliary cirrhosis does sicca syndrome occur?

A

70%

84
Q

Which antibody is associated with limited systemic sclerosis?

A

Anti centromere

85
Q

Which antibody is associated with diffuse systemic sclerosis?

A

Anti Scl70

86
Q

What is the management of a patient on long term steroids who’s dexa scan shows osteopenia?

A

Vitamin D
Calcium
Oral bisphosphonate
Repeat scan in 1-3 years

87
Q

What are causes of pyoderma gangrenosum?

A 
Idiopathic
IBD
RA
SLE
Myeloproliferative disorders
Lymphoma
Myeloid leukaemia
Monoclonal gammopathy 
PBC
88
Q

Which antibody is present in drug induced lupus?

A

Anti histone antibodies

89
Q

Which drugs cause drug induced lupus?

A

Procainamide

Hydralazine

90
Q

What are the 3 stages of churg strauss?

A

Stage 1: allergy, asthma, allergic rhinitis
Stage 2: eosinophilia
Stage 3: vasculitis

91
Q

What are skin manifestations of SLE?

A

Photosensitive butterfly rash
Discoid lupus
Alopecia
Livedo reticularis

92
Q

What are the American college of rheumatology criteria for diagnosis of SLE?

A

Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis: non erosive, 2 or more joints
Serositis: pleuritis or pericarditis
Renal disorder: proteinuria or casts
Neurological disorder: fits or psychosis
Haematological disorder: anaemia, lymphopenia, leucopenia, thrombocytopenia
Immunological disorder: anti dsDNA or anti SM antibodies
ANA positive

93
Q

What are oslers nodes? Which conditions is it associated with?

A

Tender purple/red raised lesions with a pale centre which occur as a result of immune complex deposition
Occur in association with endocarditis, SLE, gonorrhoea, typhoid, haemolytic anaemia

94
Q

What X-ray findings are seen early and late in RA?

A

Early: loss of joint space, juxta articular osteoporosis
Late: periarticular erosions, subluxation

95
Q

Which autoantibody is associated with PBC?

A

Antimitochondrial antibody

96
Q

What is usually the earliest symptom of PBC?

A

Pruritus which can precede jaundice and hepatomgaly by several years

97
Q

What are patients with PBC at risk of?

A

Hypercholesterolaemia
Oesophageal varices
Malabsorption (fat soluble vitamins ADEK)
Osteomalacia

98
Q

What is primary antiphospholipid syndrome characterised by?

A

Thrombosis (arterial and venous)
Recurrent (more than 2) miscarriages
In absence of SLE
Other features: chorea, autoimmune thrombocytopenia, livedo reticularis, thrombotic stroke, heart valve abnormalities

99
Q

Which autoantibodies are raised in CREST syndrome?

A

Anticentromere antibodies

100
Q

What are featues of CREST syndrome?

A 
Calcinosis
Raynauds
Oesophageal dysmotility
Sclerodactyly
Telangiectasia
101
Q

What are rheumatoid factor antibodies?

A

IgM autoantibodies directed against IgG

102
Q

What are the criteria for diagnosis of rheumatoid arthritis?

A

Joint distribution: 1 large joint - 0 points, 2-10 large joints - 1 point, 1-3 small joints - 2 points, 4-10 small joints- 3 points, >10 joints- 5 points
Serology: Negative RF and negative anti-CCP antibodies - 0 points, Low positive RF or anti-CCP antibodies (less than 3 x normal) - 2 points, High positive RF or anti-CCP antibodies (>3 x upper normal limit) - 3 points
Symptom duration: <6 weeks - 0 points, 6 weeks or more - 1 point
Acute-phase reactants: Normal CRP and ESR - 0 points, Abnormal CRP or ESR - 1 point
6 or more points is RA

103
Q

With which ANCA is MPA associated?

A

pANCA with MPO specificity

104
Q

With which ANCA is GPA (wegeners granulomatosis) associated?

A

cANCA with PR3 specificity

105
Q

Which autoimmune conditions are associated with vitiligo?

A 
Hashimoto’s thyroiditis
Graves’ disease
Addison’s disease
Diabetes mellitus 
Alopecia areata 
Pernicious anaemia
IBD
Psoriasis 
Autoimmune polyglandular syndrome
106
Q

Which ENA antibody can be used to test for dermatomyositis?

A

Anti Jo

107
Q

Which ENA can be used to test for SLE?

A

Anti smooth muscle

108
Q

What type of antibody is rheumatoid factor?

A

IgM against IgG

109
Q

Who should be screened for antiphospholipid antibodies?

A 
Young adults (50 years old or less) with ischaemic stroke 
Women with recurrent pregnancy loss (3 or more pregnancy losses) before 10 weeks of gestation
110
Q

Which antiphospholipid antibodies can be checked?

A

Lupus anticoagulant
Anticardiolipin (aCL) antibody
Anti-b2-glycoprotein I antibody

111
Q

What are some causes of a raised CK?

A 
Neuromuscular disorders
Myopathies
Muscular dystrophies eg duchennes
Rhabdomyolysis 
Myositis
Acute myocardial infarction
After strenuous exercise
Intramuscular injection
Renal disease
Drugs: Statins, Neuroleptic malignant syndrome: Antipsychotic drugs  and Dopaminergic drugs esp if abrupt dose reduction
112
Q

What investigations should be done for PMR?

A 
FBC
CRP/ESR
CK
TFTs
Igs and EP strip
Early morning Urinary light chains
Assess for malignancy
Exclude rheumatoid with RF and anti-CCP antibodies
113
Q

What is management for PMR?

A

Prednisolone 15mg – 20 mg daily for 2-4 weeks
Followed by tapering dose: Reduce by 2.5 mg every 2–4 weeks until the patient is at 10 mg daily, Once on 10mg daily, reduce by 1mg monthly
Warn patients to expect a temporary increase in symptoms for about a week after each dose reduction
Prophylaxis if prednisolone >7.5mg od, or high fracture risk. Don’t forget calcium and vitamin D
Gastroprotection should be considered, particularly in older patients
Advice on physical activity, weight bearing activity is important

114
Q

What are the most common sites for gouty tophi?

A 
Fingers
Helix/ anti-helix of ears
Toes
Olecranon bursae
Olecranon
115
Q

What type of crystals are found in gout?

A

monosodium urate

116
Q

What investigations are useful for a diagnosis of gout?

A

Raised serum urate > 420umol/l
Synovial fluid with negatively birefringent crytals when viewed under polarised light
Measure serum urate at 6 weeks post-attack

117
Q

What medication is used for acute gout?

A

NSAIDs
Colchicine (inhibits microtubule polymerisation by binding to tubulin, blocks neutrophil mediated inflam responses)
Prednisolone
Canakinumab (recombinant monoclonal antibody, inhibitor of proinflammatory cytokine IL-1. Licensed for use in patients whose condition has not responded to treatment with NSAIDs or colchicine, or who are intolerant of them. Can be used for symptomatic treatment of frequent gouty arthritis attacks (at least three in previous 12m)

118
Q

What is the chronic management of gout?

A

Lifestyle measures: Weight loss, Diet, Exercise, Alcohol reduction, Increase water intake
Medication: Start allopurinol after two or more attacks of gout within a year or after first attack in people at higher risk- with one or more tophi, X-ray features of gouty arthritis, renal impairment, known uric acid stones, on long-term diuretic medication
Start allopurinol 1–2 weeks after inflammation has settled and titrate dose every few weeks until serum uric acid level below 300 micromol/L
When starting allopurinol, co-prescribe low dose NSAID or low-dose colchicine for at least 1 month to prevent acute attacks
Prescribe NSAIDs for up to 6 weeks and consider need for
gastroprotective medication. Prescribe colchicine for up to 6 months
If NSAIDs and colchicine are contraindicated, consider low-dose oral
prednisolone once a day for 4 to 12 weeks

119
Q

What is second line for chronic gout if allopurinol is not tolerated or contraindicated?

A

Febuxostat

120
Q

What is the mechanism of action of allopurinol?

A

Purine analogue

Inhibitor of xanthine oxidase so reduces production of uric acid

121
Q

What can cause an increase in uric acid levels?

A

Dietary intake - meats, seafood, beans, yeast
Haematological causes - Chronic Haemolytic anaemia, CML
Alcohol
Genetic
Reduced renal excretion: Drugs - loop and thiazide diuretics, aspirin, Kidney disease, Diabetic ketoacidosis, Starvation, Genetic

122
Q

What are rheumatological causes of secondary raynauds?

A 
Systemic sclerosis (90% of patients have Raynaud’s phenomenon)
Mixed connective tissue disease 
Systemic lupus erythematosus 
Dermatomyositis or polymyositis 
Rheumatoid arthritis 
Sjögren’s syndrome
Vasculitis
123
Q

What are haematological causes of secondary raynauds?

A

Polycythaemia ruba vera
Leukaemia
Thrombocytosis
Cold agglutinin disease (Mycoplasma infections)
Paraproteinaemias
Protein C deficiency, protein S deficiency, antithrombin III deficiency
Presence of the factor V Leiden mutation
Hepatitis B and C (associated with cryoglobulinaemia)

124
Q

What features make primary raynauds more likely than secondary?

A 
Younger age (usually under 30 yrs old)
Female
Genetic component (30% have an affected first degree relative)
No symptoms/signs of underlying disease
No tissue necrosis or gangrene
Normal nail fold capillaries
125
Q

What are key symptoms and signs of connective tissue disease?

A 
Alopecia
Arthralgia
Arthritis
Breathlessness
Carpal tunnel syndrome
Indigestion/GORD
Mouth ulcers
Photosensitivity
Rashes
Severe digital ischemia
Weight loss
126
Q

What investigations should be done for raynauds?

A

FBC
Inflammatory markers
ANA and ENA and dsDNA binding Antibody
If unilateral Raynauds: CXR – cervical rib, MRI if suspect thoracic outflow obstruction

127
Q

What is the management of raynauds?

A

Conservative management: Keep warm peripheries, STOP SMOKING

Medical management: Vasodilators, Vasoconstrictor inhibitors, Calcium channel blockers - Nifedipine

128
Q

What signs may be present in a patient with OA?

A 
Tenderness to palpation of joint
Bony thickening
Small effusions
Crepitus
Deformity and restricted movement of joint
Muscle wasting
Generalised or localised arthritis
Heberdens and Bouchards nodes in hands
129
Q

What is the management of OA?

A

Weight reduction
Exercise to improve specific muscle strength
Exercise to improve aerobic fitness
Pain Control
Social support
Aids and appliances: insoles, walking sticks
Intra-articular corticosteroid injection if pain severe
Surgery: prosthetic joint replacement, consider referral for joint surgery for people with OA who experience joint symptoms (pain, stiffness and reduced function) that have a substantial impact on their quality of life and are refractory to non-surgical treatment

130
Q

What pain control can be used in OA?

A 
Paracetamol
Topical NSAIDs  
Paracetamol/codeine
NSAIDs: beware use in elderly, consider co-prescribing PPI, short-term use, lowest effective dose only
TENs machine
131
Q

What is joint hypermobility syndrome?

A

Musculoskeletal symptoms in presence of generalised joint

hypermobility not attributable to a systemic rheumatologic disease

132
Q

What is the Beighton hypermobility score?

A

Passively extend the fifth MCP joint to > 90° (1+1)
Oppose thumb to the ant aspect of ipsilateral forearm (1+1)
Hyperextend elbow to > 10° (1+ 1)
Hyperextend knee to > 10° (1 + 1)
Place hands flat on floor without bending knees (1)
Score 5 or more for adults, 6 for children

133
Q

What is the modified Brighton criteria for hypermobility?

A

Main criteria: Four or more points of Beighton criteria, Joint pain for longer than 3 months in at least 4 joints
Minor criteria: One to 3-points of Beighton criteria, Joint pain in one, 2 or 3 joints or back pain or dorsal root pain for more than 3 months, Dislocation or subluxation of more than one joint, or one joint more than once, Three or more periarticular lesions (tennis elbow, tenosynovitis, bursitis), marfanoid habitus (tall, thin, arachnodactyly), skin abnormalities: striations, hyperextensibility, thin skin, Ocular signs myopia or Antimongoloid type, Varicosities or hernias or uterine or rectal prolapse

134
Q

What are signs of connective tissue disease/joint hypermobility syndrome?

A

Skin: striae
Cardiovascular: thoracic aortic dilatation/rupture/dissection, aortic regurgitation, mitral valve prolapse, mitral regurgitation, abdominal aortic aneurysm, cardiac dysrhythmia
Lungs: pleural rupture causing pneumothorax
Eyes: lens dislocation, closed-angle glaucoma, high myopia
Skeleton: arachnodactyly, hypermobility, arthralgia, joint instability, finger contractures, pectus excavatum, kyphoscoliosis
Nervous system: dural ectasia hernias presenting with low back
pain and symptoms akin to cauda equina syndrome or chronic
postural headache due to CSF leakage
Facial characteristics: maxillary/mandibular retrognathia, long
face, high, arched palate, enophthalmos, downslanting palpebral fissures and malar hypoplasia

135
Q

What is chronic fatigue syndrome?

A

Disabling fatigue of at least six months with one or more of the following symptoms:
Difficulty with sleeping, insomnia, hypersomnia, unrefreshing sleep, disturbed sleep-wake cycle
Muscle/ joint pain, multi-site and without evidence of inflammation
Headaches
Painful lymph nodes without pathological enlargement
Sore throat
Cognitive dysfunction, difficulty thinking, inability to concentrate, impairment of short-term memory, difficulties with word-finding, planning/organising thoughts and information processing
Physical or mental exertion makes symptoms worse
General malaise or flu-like symptoms
Dizziness and/or nausea
Palpitations in the absence of identified cardiac pathology

136
Q

What are causes of back pain?

A

Mechanical back pain: muscle strain, ligament, disc herniation, compression fracture, lumbar spinal stenosis, osteoarthritis, spondylolisthesis
Infections: discitis, epidural abscess, osteomyelitis
Primary Tumours (spinal cord or vertebrae)
Metastatic Vertebral Tumours
Ankylosing spondylitis and other inflammatory disorders

137
Q

What are causes of vasculitis?

A

Idiopathic (45-55%)
Infection (15-20%): HSP, septic vasculitis, upper respiratory tract flares of Wegener’s granulomatosis, polyarteritis nodosa
Inflammatory disease (15-20%): SLE, RA, Crohn’s disease, UC
Drug-induced (10-15%): sulfonamides, beta-lactams, quinolones, NSAIDs,oral contraceptives, thiazides, anti-influenza vaccine
Chemicals such as insecticides and petroleum products
Neoplastic (<5%): paraproteinaemia or lymphoproliferative disorder

138
Q

What is the chapel hill consensus conference classification for vasculitis?

A

Infection
Non-infective: Large vessel, Medium vessel, Small vessel, Variable vessel, Single organ, Systemic disease-associated, Probably associated

139
Q

What are examples of infective vasculitis?

A

Rickettsial vasculitis
Syphilitic aortitis
Aspergillus arteritis

140
Q

What are examples of different non infective vasculitis?

A 
Large vessel: GCA
Medium vessel: Kawasaki disease
Small vessel: immune complex
Variable vessel: Behçet's disease
Single organ: isolated aortitis
Systemic disease-associated: rheumatoid
Probably associated: hepatitis B, hepatitis C
141
Q

What are signs of small vessel vasculitis?

A 
Palpable purpura 1-3 mm (may join to form plaques/ulcer)
Tiny papules
Splinter haemorrhages
Urticaria
Vesicles
Livedo reticularis (rare)
142
Q

What are features of medium vessel vasculitis?

A 
Ulcers
Digital infarcts
Nodules
Livedo reticularis
Papulo-necrotic lesions
Hypertension: damage to renal vessels
143
Q

What are features of large vessel vasculitis?

A

End-organ ischaemia (TIA/CVE)
Hypertension
Aneurysms
Dissection/ haemorrhage or rupture

144
Q

What is alopecia areata?

A

Autoimmune condition
Localised well demarcated patches of hair loss
At edge of hair loss might be small broken exclamation mark hairs

145
Q

What is the prognosis in alopecia areata? What is the management?

A 
Hair will regrow in 50% patients by 1 year and 80-90% eventually 
Topical or intralesional steroids 
Topical minoxidil 
Phototherapy 
Dithranol 
Contact immunotherapy 
Wigs
146
Q

What is antiphospholipid syndrome?

A

Acquired disorder characterised by predisposition to venous and arterial thromboses, recurrent foetal loss and thrombocytopenia
Can be primary or secondary to other conditions eg SLE, lymphoproliferative disorders

147
Q

Why does antiphospholipid syndrome cause rise in APTT despite being a prothrombotic condition)?

A

Ex vivo reaction of lupus anticoagulant autoantibodies with phospholipids involved in coagulation cascade

148
Q

What is the management of anti phospholipid syndrome?

A

Initial VTE: warfarin, target INR 2-3 for 6 months
Recurrent VTE: lifelong warfarin. If VTE whilst already on warfarin then target INR 3-4
Arterial thrombosis: lifelong warfarin, target INR 2-3

149
Q

Which drugs are used as an induction agent to rapidly gain control of disease in ANCA vasculitis? What is the mechanism?

A

Cyclophosphamide: causes DNA crosslinking and so apoptosis of rapidly dividing cells including lymphocytes
Rituximab: anti CD20 monoclonal antibody, causes B cell depletion

150
Q

Which drugs are used as maintenance agents for ANCA vasculitis?

A

Azathioprine

Mycophenolate

151
Q

What is the classic triad of symptoms in behcets syndrome?

A

Oral ulcers
Genital ulcers
Anterior uveitis

152
Q

What are features of behcets syndrome?

A 
Oral ulcers
Genital ulcers
Anterior uveitis
Thrombophlebitis
Arthritis
Aseptic meningitis
Abdo pain, diarrhoea, colitis
Erythema nodosum
DVT
153
Q

What is feltys syndrome?

A

Rheumatoid arthritis
Splenomegaly
Granulocytopenia

154
Q

What is polyarteritis nodosa?

A

Medium vessel vasculitis
Necrotising inflammation leading to aneurysm formation
More common in middle aged men
Associated with hep B

155
Q

What are features of polyarteritis nodosa?

A 
Fever
Malaise
Arthralgia
Weight loss
HTN
Mononeuritis multiplex
Testicular pain
Livedo reticularis 
Haematuria
ANCA
Hep B
156
Q

What are extra articular manifestations of RA?

A

Pulmonary: effusion, fibrosis, bronchiectasis, caplans nodules
Eyes: secondary sjogrens, scleritis
Haem: anaemia, feltys syndrome
Neuro: carpal tunnel, Atlanto axial subluxation
Other: amyloidosis, nephrotic syndrome, pericarditis

157
Q

What is Auspitz sign?

A

If you pick off a psoriatic plaque, capillary bleed under it

158
Q

What are the different types of psoriatic arthritis?

A 
Mono/oligo (less than 4 joints)
Spondylitis
Asymmetrical polyarthritis (including DIP) 
Arthritis mutilans
Rheumatoid like
159
Q

Which antibodies are associated with dermatomyositis?

A

Anti Jo1
Anti Mi2
RhF
ANA

160
Q

What are causes of raynauds?

A 
SLE 
RA
Dermatomyositis
Ehlers Danlos 
Beta blockers
Polycythemia rubra Vera
MGUS
Hypothyroidism
161
Q

What are examples of different types of vasculitis?

A

Small vessel ANCA positive: microscopic polyangiitis, churg Strauss, wegeners
Small vessel ANCA negative: HSP, cryoglobulinaemia, goodpastures
Medium vessel: polyarteritis nodosa, Kawasaki disease
Large: takayasus arteritis, temporal arteritis

162
Q

What are causes of mononeuritis multiplex?

A 
Vasculitis
HIV/AIDS
RA
Diabetes
Sarcoidosis 
Polyarteritis nodosa
Leprosy 
Carcinomatosis

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