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Rheumatology Flashcards

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1
Q

what is SLE (systemic lupus erythematosus)

A

inflammatory autoimmune connective tissue disease
has varying and non-specific symptoms
more common in women and Asians, usually presents in young to middle aged adults but can present later in life

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2
Q

pathophysiology of SLE

A

anti-nuclear antibodies, antibodies to proteins within the person’s own cell nucleus
results in chronic inflammation, resulting in a shortened life expectancy

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3
Q

presentation of SLE

A

fatigue
weight loss
arthralgia
myalgia
fever
photosensitive malar rash, livedo reticularis, discoid rash
lymphadenopathy, splenomegaly
SOB
pleuritic chest pain
mouth ulcers
hair loss
Raynaud’s

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4
Q

autoantibodies associated with SLE

A

anti-ANA (99% of patients will have this, good rule out test, but positive in other conditions as well e.g. autoimmune hepatitis)

anti-dsDNA = specific to SLE

20% of patients have rheumatoid factor positive

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5
Q

diagnosis of SLE

A

SLICC criteria/ ACR criteria

confirming antinuclear antibodies and a certain number of clinical features suggestive of SLE

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6
Q

babies whose mothers have anti-Ro and anti-La antibodies are at an increased risk of developing what?

A

neonatal lupus

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7
Q

monitoring SLE investigations

A

ESR, (CRP during active disease may be normal)
complement levels C3 & 4 decreased
anti-dsDNA titre levels used for monitoring

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8
Q

treatment for SLE

A

anti-inflammatory medication and immunosuppression

first line:
NSAIDs
Steroids e.g. prednisolone
Hydroxychloroquine (first line for mild SLE)
suncream and sun avoidance

more resistant/severe lupus:
methotrexate
azathioprine
leflunomide

then biological therapies e.g. rituximab, belimumab

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9
Q

what is spared regarding the malar rash in SLE

A

nasolabial folds

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10
Q

risk factors for osteomalacia

A

darker skin
reduced sun exposure
chronic kidney disease
malabsorption disorders e.g. IBD
drug induced e.g. anticonvulsants
inherited

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11
Q

pathophysiology of osteomalacia

A

vitamin D = a hormone created from cholesterol by the skin in response to UV radiation

inadequate vitamin D results in lack of calcium and phosphate in the blood as it is not absorbed from the intestines and kidneys
low calcium causes a secondary hyperparathyroidism

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12
Q

complications of SLE

A

** all due to chronic inflammation

CVD - hypertension, coronary artery disease resulting in leading cause of death
Infection
Anaemia of chronic disease = chronic normocytic anaemia
Pericarditis
Pleuritis
Interstitial lung disease = pulmonary fibrosis
Lupus nephritis
Neuropsychiatric SLE = optic neuritis, transverse myelitis, psychosis
Recurrent miscarriage

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13
Q

what is osteomalacia

A

defective bone mineralisation causing soft bones secondary to low vitamin D levels

if this occurs in growing children it is termed rickets

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14
Q

what organ is responsible for metabolising vitamin D to its active form

A

kidneys

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15
Q

presentation of osteomalacia

A

fatigue
bone pain
bone/muscle tenderness
muscle weakness
fractures e.g. especially femoral neck
proximal myopathy = waddling gait

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16
Q

investigations for osteomalacia

A

vitamin D deficiency (<25nmol/L)
low calcium, low phosphate
high ALP
secondary hyperparathyroidism

x-rays may show osteopenia (more radiolucent bones)

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17
Q

management of osteomalacia

A

supplementary vitamin D (colecalciferol)
** a loading dose often needed initially

calcium supplementation if dietary calcium inadequate

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18
Q

what is polymyalgia rheumatica?

A

inflammatory condition causing pain and stiffness in the shoulders, pelvic girdle, and neck
strong association with giant cell arteritis

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19
Q

demographics associated with polymyalgia rheumatica

A

older age >50 yrs
more common in women, Caucasians

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20
Q

features of polymyalgia rheumatica

A

should be present for at least 2 weeks: usually rapid onset

bilateral shoulder pain that may radiate to the elbow
bilateral pelvic girdle pain
worse with movement
interferes with sleep
stiffness for at least 45 mins in the morning
** weakness is not a symptom

can be associated with fever, night sweats, low mood, anorexia, fatigue

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21
Q

investigations for polymyalgia rheumatica

A

inflammatory markers e.g. CRP, ESR are raised, but diagnosis mainly based on clinical presentation and response to steroids

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22
Q

treatment for polymyalgia rheumatica

A

started on 15mg of prednisolone
if 3-4 weeks has given a good response, then start a reducing regime, aim is to get patient off steroids

long term steroids advice: Don’t STOP
don’t stop taking steroids after 3 weeks as will have become steroid dependant and don’t want an adrenal crisis

S = sick day rule, increase steroid dose if become unwell
T = treatment card to alert others
O = osteoporosis, think about prophylaxis with biphosphonates, calcium, vitamin D
P = PPI

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23
Q

what is antiphospholipid syndrome

A

acquired disorder resulting in a hyper-coagulable state where they have a predisposition to venous and arterial thromboses

associated with recurrent miscarriages and thrombocytopenia
can occur on its own or secondary to an autoimmune condition e.g. SLE

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24
Q

autoantibodies associated with antiphospholipid syndrome

A

lupus anticoagulant

anticardiolipin antibodies

anti-beta-2-glycoprotein I antibodies

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25
features of antiphospholipid syndrome
VTE = DVT, PE Arterial thromboses = stroke, MI, renal thrombosis pregnancy complications = recurrent miscarriage, stillbirth, pre-eclampsia livedo reticularis thrombocytopenia prolonged APTT Libmann-Sacks endocarditis = non-bacterial endocarditis with growths on the valves, particularly mitral valve
26
management of antiphospholipid syndrome
primary thromboprophylaxis = low dose aspirin secondary = initial venous and arterial events, lifelong warfarin with INR of 2-3 recurrent venous = warfarin INR 3-4, consider adding aspirin if pregnant = low molecular weight heparin e.g. enoxaparin, and aspirin
27
What is psoriatic arthritis
Form of inflammatory arthritis, associated with psoriasis Occurs in 10-20% of patients Part of the seronegative spondyloarthropathy
28
What is rheumatoid arthritis
An autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths, and bursa Form of inflammatory arthritis Symmetrical polyarthritis
29
Demographics of RA
More common in women Develops in middle age, but can present at any age FHx increases the risk
30
Patterns of affected joints in PA
Symmetrical poly arthritis = hands, wrists, ankles, and DIP joints MCP joints are less effected, unlike in RA Asymmetrical pauciarthritis = affecting mainly the digits and feet (when the arthritis only affects a few joints) Spondylitic pattern = often seen in men, causing back stiffness, sacroilitis, Atlanta-axial joint involvement Other areas can be affected = spine, Achilles’ tendon, plantar fascia
31
Nail signs and other associated signs of PA
Pitting of nails Psoriatic plaques Onycholysis Dactylitis Enthesitis (point of insertion of tendons onto bones) Eye disease = conjunctivitis, anterior uveitis Aortitis Amyloidosis
32
What is the varying severity of PA
mild stiffening or soreness Or the joint can be completely destroyed in arthritis mutilans
33
What screening tool is recommended for PA
PEST Psoriatic epidemiological screening tool
34
What is arthritis mutilans
Most severe form of psoriatic arthritis occurring in the phalanxes There is osteolysis of the bones around the joint in the digits, leading to progressive shortening of the digit and the skin folds ‘Telescopic finger’
35
Changes seen on X-ray in psoriatic arthritis
Dactylitis (soft tissue swelling) Osteolysis Periostitis Pencil in cup appearance
36
Management of PA
NSAIDs for pain DMARDs e.g methotrexate (treats skin plaques as well), leflunomide, sulfasalazine Anti-TNF e.g. etanercept, infliximab, adalimumab Last line = ustekinumab, a monoclonal antibody targeting interleukin 12 and 23
37
What is the seronegative spondyloarthropathy group?
No RF autoantibody present Group of conditions associated with the HLAB27 gene Other conditions in this group include psoriatic arthritis and reactive arthritis
38
What is ankylosing spondylitis
An inflammation condition that causes pain and stiffness of the spine Tends to be a young male in their last teens or 20s Affects the vertebral column and sacroiliac joints
39
What can ankylosing spondylitis look like on X-ray?
The condition can progress to fusion of the spine and sacroiliac joints, where it can lead to bamboo spine on X-ray
40
Features of ankylosis spondylitis
Lower back pain and stiffness Sacroiliac pain in the buttock region ** present insidiously, gradually over 3 months Worse with rest and improves with movement, interferes with sleep at night Takes around 30 minutes to overcome the stiffness in the morning
41
Other associations of ankylosing spondylitis
Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendinitis AV block Amyloidosis Also associated with systemic symptoms such as weight loss and fatigue Chest pain = due to inflamed Costovertebral and Costosternal joints Enthesitis
42
Clinical examination findings in ankylosing spondylitis
Reduced lateral flexion Reduced chest expansion Schobers test: Find L5 vertebrae and mark a point 10cm above and 5cm below Get patient to bend forward as much as they can, if the space between the points is less than 20cm they have lumbar restriction
43
Investigations for ankylosing spondylitis
Inflammatory markers e.g. ESR, CRP X-ray = Squaring of the vertebral bodies Ossification of the ligaments, discs, and joints = single central radiodense line, dagger sign Syndesmophytes = bony growths Fusion of joints, sacroilitis presents as sclerosis of joint lines CXR - apical fibrosis Spirometry may show a restrictive defect
44
Management of ankylosing spondylitis
Encourage regular exercise e.g. swimming Physiotherapy Avoid smoking NSAIDs for pain Steroids can be used during flares to control symptoms Anti-TNF e.g. etanercept, ** persistently high disease activity despite conventional treatment
45
Autoantibodies involved in RA
Rheumatoid factor: targets Fc of the IgG, causes activation of the immune system against immunoglobulins, causing systemic inflammation anti-CCP = more sensitive and specific, often predate development of RA
46
Presentation of RA
Symmetrical distal polyarthropathy Pain, swelling, stiffness of joints Complain of this in the small joints of the hands and feet, wrist, ankle, MCP, PIP Can present with larger affected joints e.g. knee, shoulder, elbow Insidious onset Stiffness worse in the mornings, takes longer than 30 mins to go away Worse after rest, improves with activity Systemic symptoms = fatigue, weight loss, flu like illness, muscle aches and pains
47
What is palindromic rheumatism?
self limiting short episodes of inflammatory arthritis, causing joint pain, swelling, and stiffness typically affecting only a few joints Lasts only 1-2 days and then resolves, having positive antibodies may indicate it will progress to full RA
48
Signs of RA in the hands
Boggy feeling of joints Z shaped deformity of thumb Swan neck deformity Boutonnières deformity Ulnar deviation at MCP
49
What causes Boutonnières deformity?
Due to a tear in the central slip of the extensor components of the fingers. when the patient tries to straighten their finger, the lateral tendons that go around the PIP (called the flexor digitorum superficialis tendons) pull on the distal phalynx without any other supporting structure, causing the DIPs to extend and the PIP to flex.
50
poor prognosis in RA patients
Autoantibodies positive Poor functional status at presentation Quick onset and younger X-ray = early erosions < 2 years Extra articulate manifestations e.g. nodules HLA DR4 gene
51
Extra articular manifestations of RA
Pulmonary fibrosis Bronchiolitis obliterans Anaemia of chronic disease CVD carpal tunnel syndrome Episcleritis and scleritis Felty’s syndrome (splenomegaly, RA, neutropenia) Secondary Sjögren’s syndrome
52
RA ocular manifestations
keratoconjunctivitis sicca (Dry eyes - most common) Episcleritis (erythema) Scleritis (erythema and pain)
53
RA pulmonary manifestations
Pulmonary fibrosis Pulmonary nodules Bronchiolitis obliterans Caplans syndrome (nodules with occupational coal dust exposure) Methotrexate pneumonitis
54
Investigations for RA
Autoantibodies ESR, CRP Baseline bloods = FBC, U&Es, LFTs X-ray of hands and feet USS to detect synovitis if unsure
55
Diagnosis & monitoring of RA
ACR score > 6 Includes joints involved (smaller joints score higher), serology, inflammatory markers, duration of symptoms (>6 weeks) DAS28 score: monitoring Assessment of 28 joints, points are given for swollen joints, tender joints, inflammatory markers
56
Management of RA
DMARD monotherapy + bridging course of prednisolone ** steroids can be used at first presentation and flares of disease NSAIDs for pain, as well as PPI MDT = physio, OT, psychology, podiatry Mono therapy DMARD Then 2 used in combination Then methotrexate + biological therapy, usually TNF inhibitor 4th line = methotrexate + rituximab Surgery regarding joint deformity and functionality
57
What are the DMARDs used in RA
Methotrexate, leflunomide, sulfasalazine hydroxychloroquine (considered in mild disease, mildest DMARD) Sulfasalazine & hydroxychloroquine considered safe in pregnancy
58
Why do pregnant women tend to have an improvement in symptoms in RA?
Higher natural production of steroid hormones
59
Biological therapies used in RA
Anti-TNF = adalimumab, etanercept, infliximab Anti-CD20 = rituximab
60
Side effects of RA treatment
Anti - TNF = immunosuppression, so can cause serious infections. Can also cause reactivation of dormant infections e.g TB, hep B Methotrexate = bone marrow suppression and leukopenia, liver toxicity, teratogenic Leflumomide = hypertension, peripheral neuropathy (** take pyrimidine) teratogenic Sulfasalazine = male infertility (reduces sperm count) Hydroxychloroquine = nightmares, reduced visual acuity Rituximab = vulnerability to severe infections, thrombocytopenia, night sweats
61
Regime of taking methotrexate
Once a week orally or IV, 5mg of folic acid to be taken on a different day
62
Examples of small vessel vasculitis
Henoch-Schonlein purpura Eosinophilic granulomatosis with polyangitis (Churg-Strauss Syndrome) Microscopic polyangitis Granulomatosis with polyangitis (Wegener’s granulomatosis)
63
Examples of medium sized vessel vasculitis
Kawasaki disease Polyarteritis nodosa Eosinophilic granulomatosis with polyangitis
64
Examples of vasculitis affecting large vessels
Giant cell arteritis Takayasu’s arteritis
65
General presentation of vasculitis
Purpura (non blanching spots) Arthralgia and myalgia Peripheral neuropathy Renal impairment GI disturbance e.g. diarrhoea, abdominal pain, bleeding Anterior uveitis and scleritis Hypertension Systemic: Fatigue Fever Weight loss Anorexia Anaemia Each type can have specific features
66
Investigations for vasculitis
Inflammatory markers e.g. ESR, CRP ANCA p-ANCA = microscopic polyangitis, Churg-Strauss syndrome C-ANCA = wegener’s granulomatosis
67
Antibodies associated with ANCA
PANCA = MPO CANCA = PR3
68
What is Henoch-Schonlein purpura
IgA mediated vasculitis Inflammation occurs due to IgA deposits in the blood vessels of affected organs e.g. skin, kidneys, GI tract Most common in children under age of 10
69
Cause of henoch schonlein purpura
Often triggered by an upper airway infection e.g. tonsillitis or gastroenteritis
70
Presentation of henoch schonlein purpura
Purpura (100%) - inflammation and leaking of blood from small blood vessels **affects lower limbs/buttocks in children, extensor surfaces of arms and legs Joint pain Abdominal pain Renal involvement (causes IgA nephritis in 50% of patients e.g. haematuria)
71
Management of henoch schonlein purpura
Supportive e.g. simple analgesia, rest, and proper hydration Self limiting condition BP and urinalysis should be monitored to detect progressive renal involvement 1/3 of patients have a relapse within 6 months
72
What is eosinophilic granulomatosis with polyangiitis
Small and medium sized vasculitis Commonly called Churg-Strauss syndrome Most associated with lung and skin problems, can affect other organs such as kidneys
73
Presentation of eosinophilic granulomatosis with polyangiitis
Often presents with severe asthma in late teenage years/adulthood Paranasal sinusitis Mononeuritis multiplex Characteristic finding is elevated eosinophils
74
what may precipitate eosinophilic granulomatosis with polyangiitis
leukotriene receptor antagonists
75
what is microscopic polyangiitis
small vessel vasculitis main feature is renal failure can also affect the lungs causing dyspnoea & haemoptysis
76
what is granulomatosis with polyangiitis (Wegener's granulomatosis )
small vessel vasculitis affecting the respiratory tract & kidneys (autoimmune condition) upper respiratory tract = nose bleeds, nasal crusting, sinusitis **saddle shaped nose due to a perforated nasal septum lower respiratory tract = dyspnoea, haemoptysis, cough glomerulonephritis also: vasculitic rash, eye involvement (proptosis), cranial nerve lesions
77
investigations for granulomatosis with polyangiitis
pANCA CXR = wide variety of presentations, may present as consolidation being mistaken as pneumonia, or cavitating lesions renal biopsy = epithelial crescents in Bowman's capsule
78
management of GPA
steroids cyclophosphamide plasma exchange median survival time = 8-9 years
79
what is polyarteritis nodosa
medium vessel vasculitis associated with hepatitis B infection, but can have no clear cause or be associated with hepatitis C or HIV more common in middle aged men necrotising inflammation leading to aneurysm formation
80
features of polyarteritis nodosa
fever, malaise, arthralgia weight loss hypertension mononeuritis multiplex testicular pain livedo reticularis** hematuria, renal failure
81
complications of polyarteritis nodosa
renal impairment, strokes, and MIs
82
investigations for polyarteritis nodosa
pANCA hepatitis B serology angiogram - shows beading associated with aneurysms
83
what is kawasaki disease
large vessel vasculitis mainly seen in children, typically under 5 years old no clear cause
84
features of kawasaki disease
persistent fever >5 days erythematous rash bilateral conjunctivitis strawberry tongue erythema and desquamation of palms & soles cervical lymphadenopathy bright red, cracked lips
85
management of kawasaki disease
high dose aspirin IV immunoglobulins echocardiogram
86
complication of kawasaki disease
coronary artery aneurysm
87
what is takayasu's arteritis
large vessel vasculitis mainly affects the aorta, its branches, and the pulmonary arteries causing occlusion more common in younger females (10-40) and Asians
88
features of takayasu's arteritis
systemic features of vasculitis e.g. headache, malaise, muscle aches, fever absent/weak peripheral pulses unequal blood pressure in upper limbs carotid bruit & tenderness upper/lower limb claudication on exertion aortic regurgitation (20%)
89
association of takayasu's arteritis
renal artery stenosis
90
investigations for takayasu's arteritis
CT/MRI angiography Doppler USS of carotids
91
management of TA
steroids
92
general management of vasculitis
steroids/immunosuppressants e.g. oral(prednisolone) IV (hydrocortisone) nasal sprays inhaled for lung involvement e.g. eosinophilic granulomatosis with polyangitis immunosuppressants e.g. methotrexate cyclophosphamide azathioprine rituximab & other monoclonal antibodies

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