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Rheumatology Flashcards

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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
What is the management for granulomatosis with polyangiitis?
Steroids Cyclophosphamide Plasma exchange
26
What is the acute management of gout?
NSAIDs Intra articular steroid injections Colchicine Oral steroids
27
What are features of temporal arteritis?
``` 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 ```
28
What are some side effects of methotrexate?
Myelosuppression Liver cirrhosis Pneumonitis
29
What are some side effects of sulfasalazine?
Rashes Oligospermia Heinz body anaemia Interstitial lung disease
30
What are some side effects of leflunomide?
Liver impairment Interstitial lung disease HTN
31
What are some side effects of hydroxychloroquine?
Retinopathy | Corneal deposits
32
What are some side effects of prednisolone treatment?
``` Cushingoid features Osteoporosis Impaired glucose tolerance HTN Cataracts ```
33
What is a side effects of gold?
Proteinuria
34
What are some side effects of penicillamine?
Proteinuria | Exacerbation of myasthenia gravis
35
What are some side effects of etanercept?
Demyelination | Reactivation of TB
36
What are some side effects of azathioprine?
``` Black tarry stools Bleeding gums Blood in urine Fever Sores/ulcers in lips or mouth ```
37
What are X-ray findings of osteoarthritis?
Loss of joint space Osteophytes Subchondral sclerosis Subchondral cysts
38
What are X-ray findings of rheumatoid arthritis?
Loss of joint space Erosions Soft tissue swelling Soft bones - osteopenia
39
What is schobers test?
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
40
Which respiratory problems may present in patients with rheumatoid arthritis?
``` Pulmonary fibrosis Pleural effusion Pulmonary nodules Bronchiolitis obliterans Methotrexate pneumonitis Pleurisy Caplans syndrome Infection secondary to immunosuppression ```
41
Which antibodies are associated with systemic sclerosis?
ANA RF Anti scl 70 Anti centromere
42
What tests can confirm antiphospholipid syndrome?
Lupus anticoagulant | Anti cardiolipin antibodies
43
What clotting abnormality might be detected in antiphospholipid syndrome?
Prolonged APTT that doesn't correct when mixed with normal plasma
44
What are similarities and differences between granulomatosis with polyangiitis and churg Strauss syndrome?
Wegeners: renal failure, epistaxis, haemoptysis, cANCA Both: vasculitis, sinusitis, dyspnoea Churg-Strauss: asthma, pANCA, eosinophilia
45
Which systems are commonly involved in polyarteritis nodosa?
``` Skin Joints Peripheral nerves GI tract Kidney ```
46
What type of glomerulonephritis occurs in henoch schonlein purpura?
Membranoproliferative
47
What is lupus pernio?
Macular popular violaceous rash over nose cheeks and ears due to granulomatous infiltration of the skin usually due to sarcoidosis
48
What is lofgrens syndrome?
Erythema nodosum Polyarthritis Bilateral hilar lymphadenopathy
49
Why can sarcoidosis be associated with diabetes insipidus?
Sarcoid tissue may infiltrate endocrine glands | If it infiltrates the hypothalamus and posterior pituitary it can lead to cranial DI
50
What are the most common causes of erythema nodosum in the U.K?
``` Sarcoidosis Tuberculosis OCP Strep infections EBV IBD Haematological malignancies ```
51
What are some complications of sarcoidosis?
Eyes: Anterior uveitis, conjunctival deposits, dry eyes, choroidoretinitis, retinal haemorrhages Heart: Cardiomyopathy, conduction system disease, cardiac failure Diabetes insipidus Skin: lupus pernio, erythema nodosum
52
Which types of clots do people with antiphospholipid syndrome get?
``` MI Recurrent miscarriage DVT Stroke Pulmonary hypertension due to thromboembolic disease ```
53
What is Takayasus arteritis?
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
54
What is Buergers disease?
Thromboangiitis obliterans Inflammation and thrombosis of small and medium arteries and veins of hands and feet Strongly associated with smoking
55
What are some facial features of systemic sclerosis?
Facial telangiectasia Small mouth Beaking of the nose
56
What is the management for raynauds?
Calcium channel blockers eg nifedipine | IV prostacyclin infusions
57
What is lofgrens syndrome?
``` Acute form of sarcoidosis Bilateral hilar lymphadenopathy Erythema nodosum Fever Polyarthralgia ```
58
What is Mikulicz syndrome?
Form of sarcoidosis with enlargement of parotid and lacrimal glands Can also be due to TB or lymphoma
59
What is Heerfordts syndrome?
``` Uveoparotid fever Parotid enlargement Fever Uveitis Secondary to sarcoidosis ```
60
When should allopurinol be started after an attack of gout?
Start if 2 or more episodes in a 12 month period
61
What level of uric acid is diagnostic for gout?
>450 micromol/l
62
What are skin features of dermatomyositis?
``` Photosensitive Macular rash over back and shoulders Heliotrope rash in periorbital region Gottrons papules Nail fold capillary dilatation ```
63
What are non skin features of dermatomyositis?
``` Proximal muscle weakeness/tenderness Raynauds Respiratory muscle weakness Interstitial lung disease - fibrosing alveolitis or organising pneumonia Dysphagia Dysphonia ```
64
What are characteristics of behchets syndrome?
``` Recurrent oral and genital ulcers Uveitis Seronegative arthritis Central nervous system symptoms Fever Thrombophlebitis Erythema nodosum Abdominal symptoms Vasculitis ```
65
What is adult onset stills disease?
Persistent high spiking fevers Joint pain Salmon coloured bumpy rash
66
What is stills disease?
Systemic onset juvenile idiopathic arthritis | Type of JIA with extra articular manifestations like fever, rash, splenomegaly, lymphadenopathy
67
What are the major criteria for diagnosis of adult onset stills disease?
High fever lasting one week or longer Joint pain lasting two weeks or longer Rash Abnormal white cell count and other blood problems
68
What are minor criteria for the diagnosis of adult onset stills disease?
Sore throat Swelling of lymph nodes or spleen Liver problems Absence of rheumatoid arthritis
69
Which scoring system is used to assess disease activity in RA?
DAS score
70
Which scoring system is used to assess disease activity in ankylosing spondylitis?
BAS DAI
71
What conditions can underly dermatomyositis?
Malignancy: lymphoma, breast, lung, ovarian, colon RA IBD
72
What are radiological features of rheumatoid arthritis?
``` Joint space narrowing Periarticular osteoporosis Erosions Periarticular soft tissue swelling Joint destruction Subluxation ```
73
What are possible causes for anaemia in a patient with RA?
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
What is the name of the joint deforming arthropathy found in SLE which can resemble RA?
Jaccouds arthropathy
75
What is the name given to the genital rash in reactive arthritis?
Circinate balanitis
76
What is the name of the skin rash in reactive arthritis?
Keratoderma blennorrhagica
77
What are the 5 types of psoriatic arthropathy?
``` Symmetric Asymmetric DIP predominant Spondylitis Arthritis mutilans ```
78
Which disease is associated with pencil in cup deformity on X-ray?
Psoriatic arthritis
79
What is the tetrad of symptoms in henoch schonlein purpura?
Purpuric rash Abdominal pain Arthritis/arthralgia Glomerulonephritis
80
How is a diagnosis of Ankylosing spondylitis made?
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
What is shiny corner sign?
Enthesitis in spine - ankylosing spondylitis
82
What is the gold standard treatment for ANCA vasculitis?
Three pulses of IV steroids | Pulsed IV cyclophosphamide
83
In what proportion of primary biliary cirrhosis does sicca syndrome occur?
70%
84
Which antibody is associated with limited systemic sclerosis?
Anti centromere
85
Which antibody is associated with diffuse systemic sclerosis?
Anti Scl70
86
What is the management of a patient on long term steroids who's dexa scan shows osteopenia?
Vitamin D Calcium Oral bisphosphonate Repeat scan in 1-3 years
87
What are causes of pyoderma gangrenosum?
``` Idiopathic IBD RA SLE Myeloproliferative disorders Lymphoma Myeloid leukaemia Monoclonal gammopathy PBC ```
88
Which antibody is present in drug induced lupus?
Anti histone antibodies
89
Which drugs cause drug induced lupus?
Procainamide | Hydralazine
90
What are the 3 stages of churg strauss?
Stage 1: allergy, asthma, allergic rhinitis Stage 2: eosinophilia Stage 3: vasculitis
91
What are skin manifestations of SLE?
Photosensitive butterfly rash Discoid lupus Alopecia Livedo reticularis
92
What are the American college of rheumatology criteria for diagnosis of SLE?
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
What are oslers nodes? Which conditions is it associated with?
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
What X-ray findings are seen early and late in RA?
Early: loss of joint space, juxta articular osteoporosis Late: periarticular erosions, subluxation
95
Which autoantibody is associated with PBC?
Antimitochondrial antibody
96
What is usually the earliest symptom of PBC?
Pruritus which can precede jaundice and hepatomgaly by several years
97
What are patients with PBC at risk of?
Hypercholesterolaemia Oesophageal varices Malabsorption (fat soluble vitamins ADEK) Osteomalacia
98
What is primary antiphospholipid syndrome characterised by?
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
Which autoantibodies are raised in CREST syndrome?
Anticentromere antibodies
100
What are featues of CREST syndrome?
``` Calcinosis Raynauds Oesophageal dysmotility Sclerodactyly Telangiectasia ```
101
What are rheumatoid factor antibodies?
IgM autoantibodies directed against IgG
102
What are the criteria for diagnosis of rheumatoid arthritis?
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
With which ANCA is MPA associated?
pANCA with MPO specificity
104
With which ANCA is GPA (wegeners granulomatosis) associated?
cANCA with PR3 specificity
105
Which autoimmune conditions are associated with vitiligo?
``` Hashimoto’s thyroiditis Graves’ disease Addison’s disease Diabetes mellitus Alopecia areata Pernicious anaemia IBD Psoriasis Autoimmune polyglandular syndrome ```
106
Which ENA antibody can be used to test for dermatomyositis?
Anti Jo
107
Which ENA can be used to test for SLE?
Anti smooth muscle
108
What type of antibody is rheumatoid factor?
IgM against IgG
109
Who should be screened for antiphospholipid antibodies?
``` 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
Which antiphospholipid antibodies can be checked?
Lupus anticoagulant Anticardiolipin (aCL) antibody Anti-b2-glycoprotein I antibody
111
What are some causes of a raised CK?
``` 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
What investigations should be done for PMR?
``` 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
What is management for PMR?
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
What are the most common sites for gouty tophi?
``` Fingers Helix/ anti-helix of ears Toes Olecranon bursae Olecranon ```
115
What type of crystals are found in gout?
monosodium urate
116
What investigations are useful for a diagnosis of gout?
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
What medication is used for acute gout?
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
What is the chronic management of gout?
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
What is second line for chronic gout if allopurinol is not tolerated or contraindicated?
Febuxostat
120
What is the mechanism of action of allopurinol?
Purine analogue | Inhibitor of xanthine oxidase so reduces production of uric acid
121
What can cause an increase in uric acid levels?
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
What are rheumatological causes of secondary raynauds?
``` 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
What are haematological causes of secondary raynauds?
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
What features make primary raynauds more likely than secondary?
``` 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
What are key symptoms and signs of connective tissue disease?
``` Alopecia Arthralgia Arthritis Breathlessness Carpal tunnel syndrome Indigestion/GORD Mouth ulcers Photosensitivity Rashes Severe digital ischemia Weight loss ```
126
What investigations should be done for raynauds?
FBC Inflammatory markers ANA and ENA and dsDNA binding Antibody If unilateral Raynauds: CXR – cervical rib, MRI if suspect thoracic outflow obstruction
127
What is the management of raynauds?
Conservative management: Keep warm peripheries, STOP SMOKING | Medical management: Vasodilators, Vasoconstrictor inhibitors, Calcium channel blockers - Nifedipine
128
What signs may be present in a patient with OA?
``` 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
What is the management of OA?
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
What pain control can be used in OA?
``` Paracetamol Topical NSAIDs Paracetamol/codeine NSAIDs: beware use in elderly, consider co-prescribing PPI, short-term use, lowest effective dose only TENs machine ```
131
What is joint hypermobility syndrome?
Musculoskeletal symptoms in presence of generalised joint | hypermobility not attributable to a systemic rheumatologic disease
132
What is the Beighton hypermobility score?
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
What is the modified Brighton criteria for hypermobility?
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
What are signs of connective tissue disease/joint hypermobility syndrome?
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
What is chronic fatigue syndrome?
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
What are causes of back pain?
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
What are causes of vasculitis?
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
What is the chapel hill consensus conference classification for vasculitis?
Infection Non-infective: Large vessel, Medium vessel, Small vessel, Variable vessel, Single organ, Systemic disease-associated, Probably associated
139
What are examples of infective vasculitis?
Rickettsial vasculitis Syphilitic aortitis Aspergillus arteritis
140
What are examples of different non infective vasculitis?
``` 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
What are signs of small vessel vasculitis?
``` Palpable purpura 1-3 mm (may join to form plaques/ulcer) Tiny papules Splinter haemorrhages Urticaria Vesicles Livedo reticularis (rare) ```
142
What are features of medium vessel vasculitis?
``` Ulcers Digital infarcts Nodules Livedo reticularis Papulo-necrotic lesions Hypertension: damage to renal vessels ```
143
What are features of large vessel vasculitis?
End-organ ischaemia (TIA/CVE) Hypertension Aneurysms Dissection/ haemorrhage or rupture
144
What is alopecia areata?
Autoimmune condition Localised well demarcated patches of hair loss At edge of hair loss might be small broken exclamation mark hairs
145
What is the prognosis in alopecia areata? What is the management?
``` 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
What is antiphospholipid syndrome?
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
Why does antiphospholipid syndrome cause rise in APTT despite being a prothrombotic condition)?
Ex vivo reaction of lupus anticoagulant autoantibodies with phospholipids involved in coagulation cascade
148
What is the management of anti phospholipid syndrome?
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
Which drugs are used as an induction agent to rapidly gain control of disease in ANCA vasculitis? What is the mechanism?
Cyclophosphamide: causes DNA crosslinking and so apoptosis of rapidly dividing cells including lymphocytes Rituximab: anti CD20 monoclonal antibody, causes B cell depletion
150
Which drugs are used as maintenance agents for ANCA vasculitis?
Azathioprine | Mycophenolate
151
What is the classic triad of symptoms in behcets syndrome?
Oral ulcers Genital ulcers Anterior uveitis
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What are features of behcets syndrome?
``` Oral ulcers Genital ulcers Anterior uveitis Thrombophlebitis Arthritis Aseptic meningitis Abdo pain, diarrhoea, colitis Erythema nodosum DVT ```
153
What is feltys syndrome?
Rheumatoid arthritis Splenomegaly Granulocytopenia
154
What is polyarteritis nodosa?
Medium vessel vasculitis Necrotising inflammation leading to aneurysm formation More common in middle aged men Associated with hep B
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What are features of polyarteritis nodosa?
``` Fever Malaise Arthralgia Weight loss HTN Mononeuritis multiplex Testicular pain Livedo reticularis Haematuria ANCA Hep B ```
156
What are extra articular manifestations of RA?
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
What is Auspitz sign?
If you pick off a psoriatic plaque, capillary bleed under it
158
What are the different types of psoriatic arthritis?
``` Mono/oligo (less than 4 joints) Spondylitis Asymmetrical polyarthritis (including DIP) Arthritis mutilans Rheumatoid like ```
159
Which antibodies are associated with dermatomyositis?
Anti Jo1 Anti Mi2 RhF ANA
160
What are causes of raynauds?
``` SLE RA Dermatomyositis Ehlers Danlos Beta blockers Polycythemia rubra Vera MGUS Hypothyroidism ```
161
What are examples of different types of vasculitis?
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
What are causes of mononeuritis multiplex?
``` Vasculitis HIV/AIDS RA Diabetes Sarcoidosis Polyarteritis nodosa Leprosy Carcinomatosis ```

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