rheumatology Flashcards

(166 cards)

1
Q

what is raised in blood test of polymyositis and dermatomyositis

A

creatinine kinase

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2
Q

what is polymyositis and dermatomyositis

A

AI disorders

Polymyositis is a condition of chronic inflammation of muscles.

Dermatomyositis is a connective tissue disorder where there is chronic inflammation of the SKIN AND muscles.

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3
Q

most common cause of septic arthritis

A

staph aureus

(gram +ve diplococci in clusters)

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4
Q

Gout synovial fluid analysis

A

needle shaped
negatively birefringent monosodium urate crystals under polarised light

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5
Q

main SE of colchicine

A

diarrhoea

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6
Q

Gout mx

A

NSAIDS 1st line
(w PPI for GI protection)
or
COLCHICINE

If CI prednisolone

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7
Q

gout prevention

A

allopurinol - Inhibits Xanthine oxidase

Wait 3 wks after acute ep
once initiated, ctu during an acute attack

colchicine cover should be used when starting allopurinol
NSAIDs can be used if colchicine cannot be tolerated

2nd line = febuxostat

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8
Q

when do you get anti-dsDNA antibodies

A

SLE

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9
Q

when do you get anti-CCP antibodies

A

RA

(more specific than RF)

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10
Q

when do you get anti-GBM antibodies

(glomerular basement membrane)

A

Goodpasture’s syndrome

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11
Q

when do you get c-ANCA antibodies

A

small vessel vasculitis e.g. granulomatosis w polyangiitis (AKA Wegener’s granulomatosis)

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12
Q

when do you get p-ANCA antibodies

A

broad range of conditions inc Primary sclerosing cholangitis (PSC), autoimmune hepatitis and ulcerative colitis.

eosinophilicgranulomatosis w polyangiitis (churg stauss)

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13
Q

presentation of adult onset still’s disease (systemic onset JIA)

A

Pyrexia (often very high and of uncertain origin at first)
Arthralgia
A fine nonpruritic salmon pink rash

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14
Q

lab results in adult onset still’s disease

A

v high ferratin
elevated liver enzymes
ANA + RA are -ve

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15
Q

what is GCA/temporal arteritis

A

systemic vasculitis of the medium and large arteries. It typically presents with symptoms affecting the temporal arteries

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16
Q

what condition does GCA have a link w

A

polymyalgia rheumatica

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17
Q

who is at highest risk of GCA

A

white females over 50.

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18
Q

sx of GCA

A

HEADACHE
- unilateral
- severe
- temple + forehead

scalp tenderness

jaw claudication

blurred/double vision

-> Irreversible painless complete sight loss can occur rapidly

assoc systemic sx:
fever
fatigue
muscle aches
weight loss
peripheral oedema

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19
Q

GCA dx

A

clinical pres
raised ESR (usually 50+)
temporal artery biopsy = multinucleated giant cells
(skip lesions may be present so ctu steroids even if -ve)

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20
Q

other test results in GCA

A

Full blood count = normocytic anaemia and thrombocytosis (raised platelets)
Liver function tests =raised ALP
CRP = raised

Duplex ultrasound of the temporal artery = hypoechoic halo sign

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21
Q

mx of GCA and refs

A

STEROIDS - start b4 dx confirmed
40-60mg pred OD
review response within 48hrs
ctu high dose until sx resolved then slowly wean off

other
- aspirin
- PPI

ref to vascular surgeons for temporal artery biopsy in all suspected GCA
Rheumatology for specialist diagnosis and management
Ophthalmology review as an emergency same day appointment if they develop visual symptoms

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22
Q

pseudogout synovial fluid analysis

A

brick shaped calcium pyrophosphate crystals that are positively birefringent under polarised light

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23
Q

features of marfan’s syndrome

A
  • tall stature with arm span to height ratio > 1.05
  • high-arched palate
  • arachnodactyly (long fingers)
  • pectus excavatum
  • pes planus (flat feet)
  • scoliosis of > 20 degrees
  • heart: dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve prolapse (75%),
  • lungs: repeated pneumothoraces
  • eyes: upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia
  • dural ectasia (ballooning of the dural sac at the lumbosacral level)
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24
Q

what is marfan’s syndrome + what is its inheritance

A

autosomal dominant connective tissue disorder

caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1

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25
who typically gets ank spon
males 20-30 yrs
26
what is systemic sclerosis
autoimmune inflammatory and fibrotic connective tissue disease.
27
what types of vasculitis affect the small vessels
Henoch-Schonlein purpura EOSINIPHILIC Granulomatosis with Polyangiitis (Churg-Strauss syndrome) Microscopic polyangiitis Granulomatosis with polyangiitis
28
what types of vasculitis affect the medium sized vessels
Polyarteritis nodosa Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) Kawasaki Disease
29
what types of vasculitis affect the large vessels
Giant cell arteritis Takayasu’s arteritis
30
which antibodies are raised in Microscopic polyangiitis and Churg-Strauss syndrome (eosinophilic granulomatosis w polyangiitis)
p-ANCA
31
which antibodies are raised in Granulomatosis with polyangiitis
c-ANCA
32
what is Henoch-Schonlein Purpura (HSP) and how does it usually present
an IgA vasculitis most common in children < 10 - purpuric rash affecting the lower limbs or buttocks - joint pain - abdo pain - renal involvement - IgA nephritis often triggered by an URTI (like tonsilitis) or gastroenteritis
33
what is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
a small and medium vessel vasculitis.
34
how does Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) present
- lung and skin probs can affect other organs eg kidneys - severe asthma in late teenage years or adulthood
35
FBC in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
raised eosinophil levels
36
main features of Microscopic polyangiitis
renal failure can also affect the lungs causing shortness of breath and haemoptysis
37
what is Granulomatosis with polyangiitis (Wegner’s granulomatosis)
small vessel vasculitis
38
presentation of Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- nose bleeds - crusty nasal secretions - hearing loss - sinusitis - saddle shaped nose - cough, wheeze, haemoptysis (CXR may show consolidation) - glomerulonephritis
39
what is polyarteritis nodosa + what is it assoc w
medium vessel vasculitis hep B
40
what rash do you get in Polyarteritis Nodosa
livedo reticularis - a mottled, purplish, lace like rash.
41
what does Polyarteritis Nodosa affect + cause
medium sized vessels in locations such as the skin, gastrointestinal tract, kidneys and heart. This can cause renal impairment, strokes and myocardial infarction.
42
features of Kawasaki Disease
usually <5 yrs old Persistent high fever > 5 days Erythematous rash Bilateral conjunctivitis Erythema and desquamation (skin peeling) of palms and soles “Strawberry tongue” (red tongue with prominent papillae)
43
comp of Kawasaki Disease
coronary artery aneurysm
44
tx of Kawasaki Disease
aspirin IV immunoglobulins
45
what is Takayasu’s arteritis + what does it affect
large vessel vasculitis It mainly affects the aorta and it’s branches. It also affect the pulmonary arteries.
46
presentation of Takayasu’s arteritis
< 40 yrs w non specific sx - fever, malaise, muscle aches - arm claudication ! - syncope
47
dx of Takayasu’s arteritis
CT or MRI angiography Doppler ultrasound of the carotids can be useful in detecting carotid disease.
48
initial therapy of RA
DMARD monotherapy (methotrexate) +/- a short-course of bridging prednisolone.
49
what is paget's disease of bone
a disease of increased but uncontrolled bone turnover Excessive osteoclastic resorption followed by increased osteoblastic activity -> leads to patchy areas of high density (sclerosis) and low density (lysis) = enlarged and mishapen bones w structural probs
50
presentation of paget's disease of bone
Typically affects older adults. More common in males. Bone pain Bone deformity Fractures Hearing loss can occur if it affects the bones of the ear classical, untreated features: bowing of tibia, bossing of skull
51
bloods in paget's disease of bone
isolated raised ALP normal calcium normal phosphate
52
XR in paget's disease of bone
Bone enlargement and deformity “Osteoporosis circumscripta” = well defined OSTEOLYTIClesions that appear less dense compared with normal bone “Cotton wool appearance” of the skull = poorly defined patchy areas of increased density (sclerosis) and decreased density (lysis) “V-shaped defects” in the long bones = V shaped osteolytic bone lesions within the healthy bone
53
paget's disease of bone mx
bisphosphonates (either oral risedronate or IV zoledronate) - interfere with osteoclast activity and seem to restore normal bone metabolism NSAIDs for bone pain Calcium and vitamin D supplementation, particularly whilst on bisphosphonates Surgery is rarely required for fractures, severe deformity or arthritis
54
what are px w paget's disease of the bone more at risk of
osteosarcoma Spinal stenosis and spinal cord compression
55
features of Goodpasture's syndrome (ant-GBM disease)
pulmonary haemorrhage rapidly progressive glomerulonephritis this typically results in a rapid onset AKI nephritis → proteinuria + haematuria
56
ix for Goodpasture's syndrome (ant-GBM disease)
renal biopsy: linear IgG deposits along the basement membrane raised transfer factor secondary to pulmonary haemorrhages
57
mx of Goodpasture's syndrome (ant-GBM disease)
plasma exchange (plasmapheresis) steroids cyclophosphamide
58
what is Buerger disease (also known as thromboangiitis obliterans)
inflammatory condition that causes thrombus formation in the small and medium-sized blood vessels in the distal arterial system (affecting the hands and feet)
59
buerger disease presentation
typically affects men aged 25 – 35 and has a very strong association with smoking (consider in px w no other RFs for atherosclerosis) - painful, blue discolouration to the fingertips or tips of the toes - pain is often worse at night. - may progress to ulcers, gangrene and amputation
60
finding on angiograms in buerger disease
Corkscrew collaterals - where new collateral vessels form to bypass the affected arteries
61
buerger disease tx
stop smoking -> signif improvement IV iloprost?
62
mx septic arthritis
IV flucloxacillin Penicillin Allergy = Clindamycin
63
what is psoriatic arthropathy
inflammatory arthritis associated with psoriasis and is classed as one of the seronegative spondyloarthropathies occurs in 10-20% px w psoriasis
64
Asymmetrical oligoarthritis
affects 1-4 joints at any given time, often on only one side of the body
65
Symmetrical polyarthritis
More than four joints are affected, such as the hands, wrists and ankles
66
Spondylitis
back stiffness and pain. It involves the axial skeleton (spine and sacroiliac joints)
67
Arthritis mutilans
severe form of psoriatic arthritis. It affects the phalanges (the bones of the fingers and toes). There is osteolysis (destruction) of the bones around the joints, leading to progressive shortening of the digits. The skin folds as the digit shortens, giving an appearance described as a telescoping digit “pencil-in-cup” appearance on XR
68
diss between psoriatic arthritis + RA
Psoriatic arthritis tends to affect the distal interphalangeal (DIP) joints and axial skeleton, whereas rheumatoid arthritis tends not to affect these joints Also has dactylitis + onycholysis Psoriasis
69
presentation psoriatic arthritis
Plaques of psoriasis on the skin Nail pitting (tiny indents in the fingernails and toenails) Onycholysis (separation of the nail from the nail bed) Dactylitis (inflammation of the entire finger) Enthesitis (inflammation of the entheses, which are the points of insertion of tendons into bone)
70
XR changes in psoriatic arthritis
Periostitis (inflammation of the periosteum, causing a thickened and irregular outline of the bone) Ankylosis (fixation or fusion of the bones at the joint) Osteolysis (destruction of bone) Dactylitis (inflammation of the whole digit, seen as soft tissue swelling)
71
MX psoriatic arthritis
Non-steroidal anti-inflammatory drug (NSAIDs) Steroids DMARDs (e.g., methotrexate, leflunomide or sulfasalazine) Anti-TNF medications (etanercept, infliximab or adalimumab) Ustekinumab is a monoclonal antibody that targets interleukin 12 and 23
72
what is Ankylosing spondylitis (AS)
inflammatory condition affecting the axial skeleton (mainly the spine and sacroiliac joints), causing progressive stiffness and pain. It is also known as axial spondyloarthritis. It is part of the seronegative spondyloarthropathy group main affected joints are the sacroiliac joints and the vertebral column joints can progress to spine and sacroiliac joint fusion
73
gene link in Ankylosing spondylitis (AS)
HLA-B27 gene
74
Ankylosing spondylitis presentation
young adult male in their 20s. sx develop gradually over at least three months. Pain and stiffness in the lower back Sacroiliac pain (in the buttock region) - worse with rest + improves with movement, worsens at night + in the morning, may wake them. 30mins to improve in the morning. other sx: Chest pain related to the costovertebral and sternocostal joints Enthesitis (inflammation of the entheses, where tendons or ligaments insert into bone) Dactylitis (inflammation of the entire finger) Vertebral fractures (presenting with sudden-onset new neck or back pain) Shortness of breath relating to restricted chest wall movement)
75
conditions assoc w ankylosing spondylitis
A – Anterior uveitis A – Aortic regurgitation A – Atrioventricular block (heart block) A – Apical lung fibrosis (fibrosis of the upper lobes of the lungs) A – Anaemia of chronic disease A - Achilles tendonitis
76
what is schobers test
assesses spinal mobility Find L5, mark 10cm above 5cm below + bnd forward A length of less than 20cm indicates a restriction in lumbar movement
77
ix ankylosing spondylitis
Inflammatory markers (e.g., CRP and ESR) may rise with disease activity HLA B27 genetic testing X-ray of the spine and sacrum MRI of the spine can show bone marrow oedema early in the disease before there are any xray changes
78
XR ankylosing spondylitis
A “bamboo spine” is the typical x-ray finding in the later stages of ankylosing spondylitis, where there is fusion of the sacroiliac and spinal joints. Squaring of the vertebral bodies Subchondral sclerosis and erosions Syndesmophytes (areas of bone growth where the ligaments insert into the bone) Ossification of the ligaments, discs and joints (these structures start turning into bone) Fusion of the facet, sacroiliac and costovertebral joints SACROILIITIS
79
mx ankylosing spondylitis
rheumatology MDT Non-steroidal anti-inflammatory drugs (NSAIDs) are first-line Anti-TNF medications are second-line (e.g., adalimumab, etanercept or infliximab) Secukinumab or ixekizumab are third-line (monoclonal antibodies against interleukin-17) Upadacitinib is another third-line option (JAK inhibitor) Intra-articular steroid injections may be considered for specific joints.
80
presentation reactive arthritis
'Can't see, can't pee, can't climb a tree' within 4 wks of initial infection - sx generally last around 4-6 months around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease asymmetrical oligoarthritis of lower limbs dactylitis symptoms of urethritis conjunctivitis (seen in 10-30%) anterior uveitis circinate balanitis (painless vesicles on the coronal margin of the prepuce) keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)
81
what is reactive arthritis
Reactive arthritis is defined as an arthritis that develops following an infection where the organism cannot be recovered from the joint.
82
XR for pseudogout
chondrocalcinosis other changes similar to osteoarthritis (LOSS)
83
RFs osteoarthritis
obesity age occupation trauma F FHx
84
joints commonly affected by osteoarthrtitis
Hips Knees Distal interphalangeal (DIP) joints in the hands (furthest out) Carpometacarpal (CMC) joint at the base of the thumb Lumbar spine Cervical spine (cervical spondylosis)
85
XR changes in osteoarthritis
L – Loss of joint space O – Osteophytes (bone spurs) S – Subarticular sclerosis (increased density of the bone along the joint line) S – Subchondral cysts (fluid-filled holes in the bone) degenerative changes
86
sx osteoarthritis
joint pain + stiffness - worsen w activity + at end of day
87
general signs osteoarthritis
Bulky, bony enlargement of the joint Restricted range of motion Crepitus on movement Effusions (fluid) around the joint
88
signs of osteoarthritis in the hand
Heberden’s nodes (in the DIP joints) Bouchard’s nodes (in the PIP joints) Squaring at the base of the thumb (CMC joint) Weak grip Reduced range of motion
89
how to make dx osteoarthritis
>45, typical pain assoc w activity + no morning stiffness (/<30 mins)
90
mx osteoarthritis
non-pharm: Therapeutic exercise Weight loss if overweight to reduce load Occupational therapy pharm: Topical NSAIDs first-line for knee osteoarthritis Oral NSAIDs (WITH A PPI for gastroprotection) - best used intermittently for flares Weak opiates and paracetamol are only recommended for short-term, infrequent use Intra-articular steroid injections may temporarily improve symptoms - up to 10 weeks Joint replacement in severe cases
91
what is RA
inflam arthritis AI condition -> chronic inflam in the synovial lining of the joints, tendon sheaths and bursa symmetrical polyarteritis usually dev in middle age
92
RFs RA
F smoking obesity FHx - HLA DR4 gene assoc
93
presentation RA
pain, stiffness + swelling in joints symmetrical distal polyarthritis affecting the small joints of the hands and feet worse w rest + improve w activity worst in morning
94
what are some extra-articular manifestations of RA
Eyes: dry eyes, scleritis, UVEITIS Skin: leg ulcers, rashes Heart: PERICARDITIS Lungs: PULMONARY NODULES, pulmonary fibrosis Kidneys: amyloidosis RHEUMATOID NODULES Anaemia Hepatomegaly Muscle wasting Tendon rupture DEPRESSION
95
most common affected joints in RA
Metacarpophalangeal (MCP) joints (closest to you) Proximal interphalangeal (PIP) joints (middle) Wrist Metatarsophalangeal (MTP) joints (in the foot) Also larger joints: ankle, knee, hips + shoulders, + cervical spine
96
palpation of joints in RA
tenderness and synovial thickening, giving them a “boggy” feeling
97
what is palindromic rheumatism
self-limiting episodes of inflammatory arthritis affecting only a few joints. sx last days, then completely resolve. Joints normal in-between
98
late hand signs in RA
Z-shaped deformity to the thumb Swan neck deformity (hyperextended PIP and flexed DIP) Boutonniere deformity (hyperextended DIP and flexed PIP) Ulnar deviation of the fingers at the MCP joints
99
what is felty's syndrome
triad of rheumatoid arthritis, neutropenia and splenomegaly
100
dx RA
urgent rheumatology referral for patients with persistent synovitis (to be seen within three weeks) bloods + NSAIDs when waiting ix: Rheumatoid factor Anti-CCP antibodies Inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) X-rays of the hands and feet for bone changes Ultrasound or MRI can be used to detect synovitis (useful when clinical findings are unclear)
101
XR changes in RA
Periarticular osteopenia Loss of joint space Erosions Soft tissue swelling Subluxation
102
how to monitor success of RA tx
CRP + Disease Activity Score 28 Joints (DAS28)
103
tx RA
STEROIDS for acute exacerbations / initial pres (IM methylprednisolone) DMARDs monotherapy = METHOTREXATE weekly - must give FOLATE supplements w - need to monitor FBC + LFTs due to the risk of myelosuppression and liver cirrhosis (others: SULFASALAZINE, leflunomide, hydroxychloroquine) BIO THERAPY - if inadequate response to at least 2 DMARDs TNF-alpha blockers e.g. infliximab Anti-CD20 - rituximab risks = reactivation of TB
104
what skin changes might you see in dermatomyositis
GOTTRON lesions (SCALY erythematous patches) on the knuckles, elbows and knees HELIOTROPE RASH (a purple rash on the face and eyelids) Periorbital oedema (swelling around the eyes) Photosensitive erythematous rash on the back, shoulders and neck
105
ix dermatomyositis
muscle biopsy
106
presentation of polymyositis and dermatomyositis
gradual-onset, symmetrical, proximal muscle weakness, causing difficulties standing from a chair, climbing stairs or lifting overhead may be muscle pain (myalgia) dermatomyositis will also have skin features
107
causes of raised creatine kinase
Polymyositis and dermatomyositis Rhabdomyolysis Acute kidney injury Myocardial infarction Statins Strenuous exercise
108
what are anti-Jo-1 antibodies assoc w
polymyositis
109
mx polymyositis and dermatomyositis
steroids
110
most common infection triggers for reactive arthritis
gastroenteritis or STIs. Chlamydia may cause reactive arthritis. Gonorrhoea typically causes septic arthritis rather than reactive arthritis
111
ix reactive arthritis
exclude septic - abx till then joint aspiration - microscopy, culture and sensitivity testing for infection, and crystal examination for gout and pseudogout - there will be sterile synovial fluid with a high white blood cell count (it is an aseptic pathological process, the causative bacteria will not be there)
112
mx reactive arthritis
Treatment of the triggering infection NSAIDs Steroid injection into the affected joints Systemic steroids may be required, particularly where multiple joints are affected most resolve in 6 mths
113
what is SLE
inflammatory autoimmune connective tissue disorder. It is “systemic” because it affects multiple organs and systems. “Erythematosus” refers to the typical red malar rash across the face
114
who usually gets SLE
Women Asian, African, Caribbean and Hispanic ethnicity Young to middle-aged adults
115
course of SLE
relapsing-remitting
116
antibodies in SLE
ANA - 85% have this, also raised in other things e.g. AI hepatitis Anti-double stranded DNA (anti-dsDNA) antibodies are highly specific to SLE - half have these, levels vary w disease acitivity so gd for monitoring 30% will have +ve antiphospholipid antibodies as is assoc w antiphospholipid syndrome
117
presentation SLE
many non-specific symptoms: Fatigue Weight loss Arthralgia (joint pain) Non-erosive arthritis Myalgia (muscle pain) Fever Photosensitive malar rash Lymphadenopathy Splenomegaly Shortness of breath Pleuritic chest pain Mouth ulcers Hair loss Raynaud’s phenomenon Oedema (due to nephritis) Pericarditis
118
ix SLE
ANA Autoantibodies FBC - anaemia of chronic disease, low WCC, low platelets CRP + ESR may be raised with active inflammation C3 and C4 levels may be decreased in active disease Urinalysis and urine protein:creatinine ratio shows proteinuria in lupus nephritis Renal biopsy may be used to ix this^
119
Anti-Ro and anti-La antibodies
most associated with Sjögren’s syndrome
120
Anti-Scl-70 antibodies
most associated with diffuse cutaneous systemic sclerosis
121
Anti-centromere antibodies
most associated with limited cutaneous systemic sclerosis
122
comps SLE
CVD - chronic inflam BVs pericarditis infection anaemia pleuritis interstitial lung dis lupus nephritis neuropsychiatric SLE - optic neuritis, transverse myelitis or psychosis recurrent miscarriage VTE - assoc w antiphospholipid syndrome 2ndary to it
123
mx SLE
Suncream and sun avoidance for photosensitive malar rash. First-line options include: Hydroxychloroquine - reduce immune system activity NSAIDs Steroids (e.g., prednisolone) more resistant/ severe DMARDs (e.g., methotrexate) Biologic therapies - Rituximab (a monoclonal antibody that targets the CD20 protein on the surface of B cells) - Belimumab (a monoclonal antibody that targets B-cell activating factor)
124
what will be raised in adult onset Still's disease
serum ferratin
125
features of adult onset stills disease
arthralgia rash = salmon pink, maculopapular pyrexia - rises late afternoon + accompanies worsening of joint sx + rash lymphadenopathy RF + ANA -ve
126
mx adult onset Still's disease
NSAIDs - trial for 1 wk b4 steroids steroids - control sx if sx persist consider methotrexate, IL-1 or anti-TNF therapy
127
features of limited systemic sclerosis (CREST syndrome)
Calcinosis Raynaud's phenomenon oEsophageal dysmotility Sclerodactyly Telangiectasia
128
nail changes in iron deficiency anaemia
koilonychia - flaky, concave nails
129
what to do b4 surgery in px w RA
Anteroposterior and lateral cervical spine radiographs preoperatively to screen for atlantoaxial subluxation - px wld need c spine collar
130
what is antiphospholipid syndrome
AI disorder caused by antiphospholipid antibodies - they target the proteins that bind to the phospholipids on the cell surface -> inflammation + increasing the risk of thrombosis (blood clots). Can occur in isolation or assoc with AI conditions, particularly SLE
131
antibodies in antiphospholipid syndrome
Lupus anticoagulant Anticardiolipin antibodies Anti-beta-2 glycoprotein I antibodies
132
comps antiphospholipid syndrome
VTE (e.g., DVT + PE) Arterial thrombosis (e.g., stroke, MI and renal thrombosis) Pregnancy-related complications (e.g., recurrent miscarriage, stillbirth and pre-eclampsia) Catastrophic antiphospholipid syndrome is a rare complication with rapid thrombosis in multiple organs within a few days. This has a high mortality rate
133
rash assoc w antiphospholipid syndrome
livedo racemosa - purple lace-like (reticular) rash that gives a mottled appearance to the skin
134
mx antiphospholipid syndrome
Long-term warfarin with a target INR of 2-3 is used to prevent thrombosis. LMWH (e.g., enoxaparin) + ASPIRIN are used in pregnancy to reduce the risks. Warfarin is CI in preg.
135
what is fibromyalgia
a syndrome characterised by widespread pain throughout the body with tender points at specific anatomical sites
136
fibromyalgia features
chronic pain: at multiple site, sometimes 'pain all over' lethargy cognitive impairment: 'fibro fog' sleep disturbance, headaches, dizziness are common
137
gout RFs
Male Family history Obesity High purine diet (e.g., meat and seafood) Alcohol Diuretics Cardiovascular disease Kidney disease
138
typical joints affected by gout
The base of the big toe – the metatarsophalangeal joint (MTP joint) The base of the thumb – the carpometacarpal joint (CMC joint) Wrist
139
XR of a joint affected by gout
Maintained joint space (no loss of joint space) Lytic lesions in the bone Punched out erosions Erosions can have sclerotic borders with overhanding edges
140
SE colchicine
Abdominal symptoms and diarrhoea
141
typical presentation pseudogout
a patient over 65 years old with a rapid-onset hot, swollen, stiff and painful knee. Other commonly affected joints are the shoulders, hips and wrists.
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mx pseudogout
Symptomatic management options include: NSAIDs (e.g., naproxen) first-line (co-prescribed with a proton pump inhibitor for gastroprotection) Colchicine Intra-articular steroid injections (septic arthritis must be excluded first) Oral steroids
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Features of systemic onset JIA
Subtle salmon-pink rash High swinging fevers Enlarged lymph nodes Weight loss Joint inflammation and pain Splenomegaly Muscle pain Pleuritis and pericarditis
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how does macrophage activation syndrome (MAS) present
an acutely unwell child with: disseminated intravascular coagulation (DIC) anaemia thrombocytopenia bleeding non-blanching rash It is life threatening
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key ix finding in macrophage activation syndrome (MAS)
low ESR
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what is macrophage activation syndrome (MAS)
severe activation of the immune system with a massive inflammatory response
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how does oligoarticular JIA present
<4 joints usually single joint, the larger ones (knee/ankle) girls < 6 yrs
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what other thing do you usually get with oligoarticular JIA
anterior uveitis
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what is enthesitis-related arthritis
the paediatric version of the seronegative spondyloarthropathy group of conditions px have inflam arthritis as well as enthesitis (enthesis is the pt where the tendon of a muscle inserts into a bone) most have HLA B27 gene
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what are the seronegative spondyloarthropathy group of conditions that affect adults
ankylosing spondylitis, psoriatic arthritis, reactive arthritis and inflammatory bowel disease-related arthritis
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what scan can demonstrate enthesitis
MRI
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how do you get enthesitis
traumatic stress, such as through repetitive strain during sporting activities or an autoimmune inflammatory process
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which areas to palpate to elicit tenderness of the entheses
Interphalangeal joints in the hand Wrist Over the greater trochanter on the lateral aspect of the hip Quadriceps insertion at the anterior superior iliac spine Quadriceps and patella tendon insertion around the patella Base of achilles, at the calcaneus Metatarsal heads on the base of the foot
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what to assess px for when they have enthesitis-related arthritis
consider signs and symptoms of psoriasis (psoriatic plaques and nail pitting) + inflammatory bowel disease (intermitted diarrhoea and rectal bleeding). Will be prone to anterior uveitis - ophthalmologist for screening, even if they are asymptomatic.
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Juvenile Psoriatic Arthritis examination signs
Plaques of psoriasis on the skin Pitting of the nails (nail pitting) Onycholysis, separation of the nail from the nail bed Dactylitis, inflammation of the full finger Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone
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mx JIA
specialist in paediatric rheumatology, with a specialist MDT NSAIDs, such as ibuprofen Steroids, either oral, intramuscular or intra-artricular in oligoarthritis Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
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poor prognostic features in RA
rheumatoid factor positive anti-CCP antibodies poor functional status at presentation X-ray: early erosions (e.g. after < 2 years) extra articular features e.g. nodules HLA DR4 insidious onset
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alternative to allopurinol in gout prophylaxis
febuxostat
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what can sometimes cause dermatomyositis/polymyositis
underlying cancer -paraneoplastic syndromes (derm more common) a viral infection may be the trigger (e.g., Coxsackie virus or HIV) do a CT CAP
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causes drug induced lupus
procainamide hydralazine isoniazid minocycline phenytoin
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features drug induced lupus antibodies
arthralgia myalgia skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common ANA positive in 100%, dsDNA negative anti-histone antibodies are found in 80-90%
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score for hypermobility
Beighton score
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tx for methotrexate OD
folinic acid
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pathogenesis of visual impairment in GCA
secondary to ANTERIOR ISCHAEMIC OPTIC NEUROPATHY from inflammation of the posterior ciliary arteries that causes optic nerve infarction
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what to give if can't take bisphosphonates in osteoporosis mx
risedronate or etidronate
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monitoring whilst on hydroxychloroquine
visual acuity testing at baseline, and then every 6-12 months as it can cause retinopathy