MSK Rheumatology Flashcards
(143 cards)
1
Q
what is rheumatoid arthritis
mainstay of treatment
A
autoimmune condition that often affects the small joints (hands and feet) but can extend elsewhere and into other systems including cardiorespiratory
lifelong immunosuppression and sometimes surgery
2
Q
what are the main deformations seen in rheumatoid arthritis
A
Z-thumb
Ulnar deviations
Boutonnaires
Swan neck finger deformities
3
Q
how are T cells affected in autoimmunity
A
Regulatory T cells activity reduces
pathogenic T effector cells are upregulated
any part of the immune pathway dysregulated can cause autoimmunity
4
Q
basics of immune response
A
dendritic cell presents antigens
T-helper cells (CD4+, MHC Class 2) release inflammatory interleukins and interferon and also activate humoral response (antibody mediated)
T-killer cells (CD8+, MHC Class 1) can also play a role
These processes all cause inflammation and tissue damage
T regulatory cells dampen the inflammation
5
Q
differentiating between inflammatory and non inflammatory joint conditions
A
inflammatory such as RA tend to have morning stiffness that improves with activity
joint swelling is more indicative of inflammation with the exception of Nodal osteoarthritis and knee swelling which can be caused by trauma
non inflammatory such as OA tends to be pain more than stiffness and is exacerbated by activity
6
Q
pattern/symmetry in RA
A
tends to be symmetrical and affects groups of joints e.g. all of the metacarpophalangeal joints
7
Q
antibody tests useful in suspected connective tissue disorder
A
ANA
anti-DsDNA
8
Q
antibody tests useful in suspected small/medium vessel vasculitis
A
ANCA
9
Q
general management of inflammation in rheumatology
A
analgesia
anti-inflammatories
immunosuppression (steroids and biologics) - steroids are short term
Disease modifying drugs can also be used later (DMARDs)
10
Q
protective treatment with steroids
A
in rheumatology always give bone protection with steroids from the start - vitamin D, calcium and bisphosphonates
gastroprotection also needed
11
Q
names of DMARDs
A
azathioprine
methotrexate
hydroxychloriquine
sulfasalazine
lefunomide
12
Q
azathioprine
A
inhibits DNA replication stopping proliferating cells
test for TPMT deficiency before as can cause toxicity
co-prescribing with allopurinol can cause toxicity
main adverse effect; bone marrow suppression –> pancytopenia and immunosuppression
13
Q
methotrexate
A
dihydrofolate antagonist that targets proliferating cells
usually given once weekly and folic acid given on other days
folinic acid used as rescue therapy if OD
adverse effects; mouth ulcers, deranged LFTs, pneumonitis and bone marrow suppression
14
Q
hydroxychloriquine adverse effects
A
photo sensitivity, retinal toxicity, haemolytic anaemia and bone marrow suppression
15
Q
sulfasalazine adverse effects
A
haemolytic anaemia, azoospermia (avoid in young men), abnormal LFTs, bone marrow suppression
16
Q
leflunomide adverse effects
A
alopecia, hypertension, pneumonitis, peripheral neuropathy, hepatotoxicity and bone marrow suppression
17
Q
monoclonal antibodies in rheumatology
A
self injected or administered as infusions that can last for many weeks at a time
alternative to daily immunosuppressive medication but both are sometimes needed
only managed by specialists and need extensive screening before including blood tests, infection screens and ruling out latent TB
18
Q
what is the common target of many monoclonal antibody medications
A
Tumour Necrosis Factor (TNF) - widespread immune mediator and a central role in inflammation
19
Q
what do systemic symptoms e.g. weight loss, night sweats, reduced appetite suggest in a presentation of arthritis
A
more likely to be CTD/vaculitis/malignancy
20
Q
symptoms to ask about to determine seronegative forms of arthritis
A
GI symptoms - IBD associated?
eye symptoms - iritis
psoriasis symptoms
21
Q
what syndrome is commonly associated with rheumatoid arthritis
A
Sjrogen’s syndrome - ask about symptoms of dry eyes, dry mouth
22
Q
what does boggy swelling indicate
A
suggests synovitis which occurs in inflammatory arthritis
in OA swelling is bony
23
Q
what is the typical joint pattern in rheumatoid arthritis
A
inflammatory small joint polyarthritis affecting the hands, feet and wrists in a symmetrical distribution
in the hands tends to affect the metacarpophalangeal (MCP ) and proximal interphalangeal joints whereas in OA the distal interphalangeal joints are more likely to be affected
24
Q
swan neck deformity (RA)
A
hyperextension of the PIPJ and flexion of the DIPJ
25
boutonnière deformity (RA)
flexion of the PIPJ and hyperextension of the DIPJ
26
guttering deformity (RA)
muscle wasting seen over the dorsum of the hand
27
management of suspected RA
analgesia and urgent referral to rheumatology
needs rapid and aggressive suppression of inflammation to reduce joint damage, maintain function and quality of life and prevent disability
28
investigations in suspected RA
- FBC, liver and renal function for baselines
- inflammatory markers
- thyroid function; can present with joint pain
- immune markers; rheumatoid factor and anti-CCP antibody may suggest rheumatoid arthritis, anti-nuclear antibody may indicate connective tissue disease
- plain film x-rays
29
anti-CCP compared to RF in diagnosing RA
anti-CCP is more specific and sensitive for RA
RF can be raised in other conditions including Sjrogen's syndrome, other rheumatic conditions, malignancies and chronic infections
30
hand xray in RA
often normal in early disease
later may have periarticular osteopenia, erosions, joint space narrowing (usually uniform) and deformity
31
SoB in RA
respiratory conditions associated with RA including pulmonary fibrosis and pleural effusions can cause SoB. lung nodules also associated but wouldn't cause SoB
consider concurrent conditions too; asthma, COPD, infection
32
criteria to diagnose RA
diagnostic criteria which covers joint involvement, serology, acute phase proteins and duration of symptoms
score of 6 or more needed
33
epidemiology RA/explanation
chronic autoimmune condition - immune system attacking own body causing inflammation in joints and can also affect other parts of the body
affects around 1 in 100 women and 1 in 200 men
needs long term treatment and often aggressive treatment to aim to control symptoms and prevent joint damage and disability
33
epidemiology RA/explanation
chronic autoimmune condition - immune system attacking own body causing inflammation in joints and can also affect other parts of the body
affects around 1 in 100 women and 1 in 200 men
needs long term treatment aim to control symptoms and prevent joint damage and disability
34
how is disease activity monitored in RA
Disease Activity Score (DAS 28) provides a score out of 10 and has categories of severity
uses:
- joint tenderness score
- number of joints
- patient global assessment (VAS out of 100)
- acute phase response (ESR/CRP)
35
how to check for swelling if synovitis not obvious on examination
USS - identifies swelling and bone erosions
36
treating active newly diagnosed rheumatoid arthritis
start a DMARD such as methotrexate, sulfasalazine, or leflunomide with pt education before
steroid therapy for symptom relief
37
extra-articular features in RA
- Scleritis and episcleritis
- dry eyes and dry mouth (esp Sjrogen's)
- lymphadenopathy and splenomegaly
- vasculitis
- pericardial effusion and pleural effusion
- carpal tunnel
- palm and nail changes
- FBC changes; Normochromic normocytic anaemia; leukopenia; pancytopenia
- amyloidosis
- systemic symptoms; fatigue, weight loss, low grade fever
38
synovial changes in RA
- thickening and inflammation
- proliferative synovitis
- angiogenesis occurs --> more inflammatory cells infiltrate
- synovial fluid becomes fibrinous inflammatory exudate, containing many neutrophils
- local enzyme and mediator release can damage cartilage and cause bone erosion
39
treatment if doesn't respond to initial DMARDs in RA
biologic therapy with a monoclonal antibody drug such as Rituximab or Tocilizumab or another biologic such as Abatacept and Baracitinib
40
joints affected by OA
weight bearing joints; cervical spine, lumbar spine, hip, knees, ankles
hands; wrist, DIP joints and PIP joints
41
what drug class increases risk of gout
diuretics especially thiazide
42
examination findings in OA
effusion
painful flexion
crepitus
weakness +/- muscle wasting
joint line tenderness
deformity
bony swelling
instability
antalgic gait
43
nodes in OA
Bouchard's nodes - PIP joints
Heberdens's nodes - DIP joints
44
any investigations needed to diagnose OA?
clinical diagnosis but consider
Bloods
- to rule out inflammatory arthritis
- U+Es to get baseline for diclofenac use
Xray affected joints
45
management of OA
- conservative
- pharmacological
- surgical
Conservative;
Patient education
Weightloss
Exercise/physiotherapy
Pharmacological;
Analgesia:
- topical anti-inflammatories/capaiscin cream
- WHO Ladder – paracetamol, NSAIDs (also COX2), opiods
Intra-articular steroid injection for moderate to severe pain
Surgical (joint replacement);
only if refractory to medical management and significant impact on life
46
OA pathophysiology
Dysregulation of tissue turnove
Focal articular cartilage damage leads to
- hypertrophy of subchondral bone
- marginal osteophytes
- synovitis
- thickening of joint capsule and ligaments
47
main modifiable risk factor in OA
other risk factors
obesity
increasing age
F>M
previous joint injury
intense sport activity
occupation
alignments and muscle strength
genetic factors
48
when to suspect secondary OA (due to another cause)
suspect in presentation <40 years, atypical joint distribution,
49
causes of secondary OA
Metabolic; crystal, Wilson's, haemachromatosis, haemaglobinopathies, collagenopathies, acromegaly
Traumatic
Anatomical/congenital; slipped femoral epiphysis
epiphyseal dysplasia, congenital dislocated hip, unequal leg length, hyper mobility
neuropathic; syphilis, diabetes
inflammatory; septic or any other inflammatory A
50
steroid injections in OA
after topical anitinflammatories and analgesia
can have two injections in a joint if first worked but if severe after that consider joint replacement
51
differentials hot, painful, swollen joint
septic arthritis - must not miss
gout
psuedogout
haemarthrosis
psoriatic arthritis
reactive arthritis
52
gout risk factors
male
alcohol
high BMI
high purine intake - red meat, oily fish, marmite
other features of metabolic syndrome e.g. diabetes
53
investigations in an acutely hot, swollen joint
FBC and inflammatory markers - reactive/septic
Joint aspiration and synovial fluid analysis - gold standard for gout diagnosis
Blood cultures
Renal and Liver function
54
acute treatment of gout
fast acting NSAIDs such as Naproxen first line (coprescribe a PPI)
Colchicine if can't have NSAIDs e.g in renal impairment
55
when and what prophylactic treatment should be used in gout
urate lowering therapy such as allopurinol which is lifelong
start when attacks are recurrent (>2 in 12 months) as can damage the joint, plus consider these factors:
- tophi
- joint damage
- renal impairment or Hx of kidney stones
- diuretic therapy
- gout starting at a young age
56
when after an attack should urate lowering therapy be started
2-4 weeks after an acute attack
57
how is Allopurinol given
+ MoA, dose and S/Es
allopurinol is a xanthine oxidase inhibitor
start at 100mg daily and tirade every 2-5 weeks (start at 50mg if CKD stage 4)
max dose 900mg but lower in renal impairment
side effects:
10% of patients develop a rash
serious side effect; allopurinol hypersensitivity syndrome may occur and carries a mortality of 20-25%. more likely in south Asian or Japanese heritage - has a specific HLA-B association
58
what is second line for gout prophylaxis
febuxostat - offer if unable to take allopurinol
59
initial period of therapy with urate lowering drugs can
in first six months can increase risk of gout attacks so initially prescribe alongside an NSAID or colchicine for this first 6 months
60
screening and lifestyle advice in recurrent gout
lifestyle; weight, alcohol, red meat , compliance with treatment
screening; for CVD, hyperlipidemia, HTN, diabetes
61
what is the target urate serum level
300 micromoles/L
62
Red Flags childhood hip pain
Nocturnal pain, night sweats, weight loss - Acute Lymphoblastic Leukaemia
Holding leg abducted and fever - septic arthritis
High fever, non weight bearing - osteomyelitis of femur/pelvis
changeable history/unsual mechanism - non-accidental injury
63
where can hip pain be referred from
Abdomen, including hernia orifices and testicles, [enlarged liver spleen, mass – malignancy], the knee
64
Transient synovitis of the hip
aka irritable hip
more common in boys
typically age 4-8
acute onset limp +/- pain with reduced hip movements
systemically well
self limiting condition
often preceded by a viral URTI or gastroenteritis
65
Septic arthritis/osteomyelitis of hip
any age
associated with fever, systemic upset, acute onset pain which is non-weight bearing and extreme on movement
immediate referral to hospital
66
hip fracture presentation
any age but more likely in elderly from fall from standing height
in younger patients associated with traumatic injury
acute onset of pain and reduced movement/non-weight bearing
67
Slipped Upper Femoral Epiphysis (SUFE) presentation
more common in boys typically >10 years of age
risk factors obesity and hypothyroidism
2 possible presentations:
- acutely with sudden onset pain and non-weightbearing
- gradual onset vague pain that may be referred to the knee and a limp
68
cause of a slipped upper femoral epiphysis
proximal femoral growth plate becoming unstable and the epiphysis and diaphysis can slip
69
Developmental Dysplasia of the Hip (DDH) presentation
more common in girls
usually detected at birth but can present later with delayed developmental milestones
older children may present with gradual onset of a painless limp
70
signs of developmental dysplasia of the hip
- asymmetrical skin folds
- leg length discrepancies
- buttock flattening
- walking with the affected leg in external rotation
71
Juvenile Idiopathic Arthritis (JIA) presentation
can present at any age
different types; determine if inflammatory
note pain or limping can also be caused by ankle or knee swelling and arthritides
72
bone tumour hip pain presentation
red flags of malignancy
nocturnal bone pain which responds to NSAIDs
bone swelling may be evident
73
abdominal pathology hip pain
Testicular torsion/inguinal hernia/appendicitis
pain in the hip, non-weightbearing with or without abdominal pain, nausea, or reduced appetite
74
haematological malignancy hip pain
Red flag symptoms
typically affects younger children
Abdominal mass with splenomegaly may be present.
75
what is perthes disease
childhood condition whereblood supply to the femoral head of the hip joint is temporarily interrupted and the bone begins to die
usually in 4-10 years old
gradual onset pain
76
safety netting transient synovitis of the hip
should improve in a few days - use paracetamol and ibuprofen in the mean time
follow up in GP in a few days and if not better consider other causes
safety net parents to attend A&E if signs of sepsis ie. fever, loss of appetite, not passing urine as often
77
what is the cut off for acute vs chronic symptoms
6 weeks
78
ethnicity risk factor in lupus
African-American women are three times more likely to get lupus than white women. Lupus is also more common in Hispanic, Asian, and Native American and Alaskan Native women
79
broadly two causes of nephrotic syndrome
systemic conditions; amyloidosis, lupus, diabetes
primary renal disorders
80
what is nephrotic syndrome
heavy proteinuria >3-5g/24hr, hypoalbumineaemia, oedema,
81
acute nephritic syndrome
haematuria, proteinuria (usually <2g/24hr), hypertension, oliguria, uraemia, oedema (periorbital, sacral, pedal)
82
presentation/ associated symptoms of CTD such as Lupus
can present very non-specifically
Joint pains - inflammatory in nature
Rash
Can present with nephrotic syndrome
associated symptoms;
- Raynaud's
- hair loss
- mouth ulcers
- fatigue
- weight loss
- ask about cardiorespiratory/GI/GU symptoms
83
what is systemic lupus erythematous (SLE)
inflammatory autoimmune connective tissue disease
systemic - multiple organ systems affected
erythematous - characteristic photosensitive malar rash on the face
presents with varying and non-specific symptoms usually in young to middle age and more common in women
84
what antibodies characterise systemic lupus erythematous
anti nuclear antibodies (ANA) - 85% pts with lupus will be ANA positive but can can also be positive in other conditions
Anti-double stranded DNA (anti-dsDNA) is specific to SLE
antiphospholipid antibodies can occur secondary to SLE
85
what course does SLE tend to take
relapsing remitting course with periods of severe inflammation
death usually occurs due to infection or cardiorespriaotry involvement
86
investigations in SLE
- autoantibodies
- FBC; can see leukopenia, normocytic anaemia, autoimmune haemolytic, thrombocytopenia
- complement levels; decreased in active disease
- CRP and ESR
- urine dip and urine protein:Cr ratio
- renal biopsy can be done for lupus nephritis
- MSUS looking for casts
- LFTs (albumin)
87
diagnosing SLE
the ACR diagnostic criteria uses antibodies and a number of the other investigations/clincial features where a score of 10 or more fulfils the criteria
88
using ESR or CRP in diagnosing SLE
ESR better - raises in active SLE whereas CRP tends to stay around normal
raised CRP may suggest another infection or inflammation in present
89
what other conditions can ANA be raised in
what is the significance of a negative ANA test
many other rheumatological and autoimmune conditions including Sjrogen's and dermomyositis and 5% of the healthy population has ANA - note there are many different types of antinuclear antibodies
but if negative almost certainly rules out SLE
90
treatment for lupus nephritis
corticosteroids and immunosuppressants
91
pregnancy in SLE
pregnancy should be planned in a period of disease stability
immunosuppressants should be changed to ones safe in pregnancy
presence of particular antibodies needs to be known; Ro(SSA), La(SSB) and antiphospholipid antibodies - can cross the placenta and cause neonatal lupus
92
what can neonatal lupus cause
lupus rash, complete heart block and blood abnormalities such as cytopaenias
93
what is antiphospholipid syndrome
systemic autoimmune disease characterized by elevated antiphospholipid antibodies and an acquired thrombophilia or clotting tendency
94
what is needed to diagnose antiphospholipid syndrome
positive antiphospholipid antibodies present (lupus anticoagulant, anticardiolopin antibody, anti -β2 gylcoprotein) on two or more occasions at least 12 weeks apart
AND
vascular thrombosis OR pregnancy morbidity
95
how is the stage of lupus nephritis decided
by histology in line with WHO classification stage I-V
96
what categories of symptoms are included in the diagnostic criteria for lupus
Constitutional
Haematological
Neuropsychiatric
Mucocutaenous
Serosal (pleurisy or pericardial)
MSK
Renal
Antiphosphoplipid Abs
Complement proteins
SLE specific antibodies
97
first line treatments SLE
NSAIDs
Steroids (prednisolone)
Hydroxychloriquine (1st line for mild SLE)
SPF and sun avoidance for photosensitive rash
98
treatments SLE
NSAIDs
Steroids (prednisolone)
Hydroxychloriquine (1st line for mild SLE)
SPF and sun avoidance for photosensitive rash
if resistant to first line/more severe try other anti rheumatic drugs (methotrexate, leflunomide, azathioprine) and then if still resistant biologic therapies such as rituximab
99
what is the triad of symptoms in reactive arthritis
triad of arthritis, urethritis and conjunctivitis/uveitis
100
what is the relevance of plasma viscosity
measures the thickness of the blood which is increased in inflammatory processes
101
what antibodies are raised in vasculitis
ANCA - anti-neutrophil cytoplasmic antibody
points more to a small vessel vasculitis
102
what plain film X-ray signs may you see in
- RA
- Gout
- Pseudogout
RA - marginal erosions
Gout - juxta-articular erosions
Pseudogout - chondrocalcinosis
103
what is vasculitis
how can it be categorised
inflammation of blood vessels which presents with a multi-systemic picture including joints, lungs, skin, kidneys and nerves
small, medium and large vessel vasculitis
The size of the vessel affected can correlate with the symptoms and pathology
104
examples of large vessel vasculitis
Giant Cell Arteritis
Takasayu's arteritis
105
examples of medium vessel vasculitis
Polyarteritis nodosa
Kawasaki's disease
Eosinophilic granulomatosis with polyantitis (churg-strauss disease)
106
examples of small vessel vasculitis
microscopic polyangitis
eosinophilic granulomatosis with polyantitis (churg-strauss disease)
Granulomatosis with polyangitis (Wegener's granulomatosis)
107
two types of ANCA + their targets
p-ANCA and c-ANCA
differences are between the target of the antibody and staining produced
c-ANCA = cytoplasmic antibody
more likely to be raised in granulomatosis with polyangiitis (GPA)
Ab target is proteinase-3
p-ANCA = perinuclear
more likely to be found in microscopic polyangiitis and in eosinophilic granulomatosis with polyangiitis
Ab target is myeloperoxidase
108
key features of granulomatosis with polyangiitis
nasal crusting, cavitating lesions in the lung and a saddle-nose deformity
109
what is the diagnostic triad of eosinophilic granulomatosis with polyangiitis
asthma, eosinophilia and multi-organ involvement (Lanham criteria)
110
investigations in vasculitis
CXR
Urinalysis
111
what type of nephritis is associated with ANCA vasculitis
crescentic glomerulonephritis - aka rapidly progressive glomerulonephritis
can present with both nephrotic and nephritic features clinically
high dose immunosuppression required
112
inducing remission in a small cell vasculitis
depends on if organs threatened
if not consider methotrexate
if organs threatened glucocorticoid + cyclophosphamide or rituximab
if life-threatening same but add plasma exchange
113
maintenance therapy in a small cell vasculitis condition such as EGPA
taper Glucocorticoid
either continue rituximab if already on it
if not switch to azathioprine or methotrexate
in the long term try to taper these
114
considerations when starting a patient on high dose steroids
bone protection
gastro protection
screen for diabetes
monitor blood pressure and weight
115
multi-system presentations and complications in EGPA
Respiratory - allergic rhinitis, polyps, paranasal sinus involvement, asthma, haemoptysis, pneumonitis
Cardio - pericardial effusion, myocarditis, myocardial infarction
Skin - purpura, livedo reticularis (mottled look)
Renal - Crescentic glomerulonephritis, hypertension and renal failure
Neurological - Mononeuritis monoplex and stroke
Ophthalmology - anterior uveitis
Gastro - Mesenteric infarction and bowel perforation
116
What drug classically causes an exacerbation of asthma symptoms in someone with EGPA?
Montelukast - bear in mind if stepped up to this for asthma then suddenly gets worse
117
pathophysiology of EGPA
genetic determinants - HLA genes and IL-10
acquired determinants - allergens, infections, drugs etc
appears to be T cell mediated producing interleukins and cytokines which attract eosinophils
humoral system also has some role and produces ANCA
118
what infection is polyarteritis nodosa associated with
hepatitis B
119
vasculitis screen
FBC, U+Es, LFTs, TFTs, PV, CRP, ANA and ANCA with a chest x-ray and urine dip
120
how many joints make a
- oligoarthritis
- polyarthritis
oligo 2-5
poly >5
121
what is polymyalgia rheumatica
inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck
strong association to giant cell arteritis and often occur concurrently
122
polymyalgia rheumatica epidemiology
usually older adults
more common in women
more common in caucasians
123
core diagnostic features of polymyalgia rheumatica
clinical diagnosis with important diagnoses of exclusion
response to steroids also used to diagnose
core features present > 2 weeks:
Bilateral shoulder pain that may radiate to the elbow
Bilateral pelvic girdle pain
Worse with movement
Interferes with sleep
Stiffness for at least 45 minutes in the morning
124
treatment of polymyalgia rheumatica
steroids
if good response after 1 week prednisolone 15mg daily, then taper up
after 3-4 weeks start reducing regime
if symptoms recurring then may need to stay on steroids
125
gonococcal arthritis + treatment
most common presentation; dermatitis, polyarthritis and tenosynovitis
treatment is IV ceftriaxone
126
what joint is most commonly affected in septic arthritis
knee in adults
127
Raynaud's
exaggerated vasoconstrictive response of digital arteries in response to cold or emotional stress
primary - typically young women, bilateral
secondary - underlying disease such as CTD, malignancy + others - suggested by later onset, unilateral, autoantibodies, rash
128
Raynaud's treatment
all should be referred to secondary care
1st line; calcium channel blocker e.g. nifidipine
2nd line; IV prostacyclin infusions, effect can last several months
129
what is dermatomyositis
inflammatory disorder that causes symmetrical proximal muscle weakness and characteristic skin lesions
can be idiopathic or associated with a connective tissue disorder
130
what is polymyositis
inflammatory disorder of symmetrical proximal muscle weakness
similar to dermatomyositis but without the skin features
131
features of antiphospholipid syndrome
venous/arterial thrombosis
recurrent fetal loss
lived reticularis (mottled skin)
thrombocytopenia
prolonged APTT
132
management of antiphospholipid syndrome
primary thromboprophylaxis - low dose aspirin
secondary thromboprophylaxis - lifelong warfarin
133
systemic sclerosis / scleroderma
autoimmune inflammatory fibrotic connective tissue disease where the skin and other mucosa harden
134
patterns of disease systemic sclerosis
limited cutaneous (CREST syndrome)
Calcinosis (calcium under skin)
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Telangiectasia (small dilated vessels)
Diffuse Cutaneous - all CREST features + affects internal organs causing CVD, lung disease and kidney disease
135
autoantibodies systemic sclerosis
ANA - not specific
Anticentromere antibodies - limited SS
Anti-Scl 70 antibodies - diffuse SS and more severe disease
136
systemic sclerosis treatment
no standardised treatment
steroids and immunosuppressants can be used in diffuse or severe disease
gentle skin stretching, emollients, avoiding cold, physiotherapy and occupational therapy
can treat symptoms and complications medically
137
Still's disease
arthralgia
spiking fevers
elevated serum ferritin
lymphadenopathy
RF and ANA negative
manage with NSAIDs and steroids
138
what condition are anti-Ro antibodies associated with
Sjrogen's syndrome
139
drug induced lupus
arthralgia, myalgia and rash (usually no renal or nervous system involvement)
ANA positive
anti-histone antibody positive
anti-DsDNA negative
common causes: procainamide, hydralazine
less common causes: isionizad, phenytoin, minocycline
140
dermomyositis associated antibody
anti-Jo-1
141
what is Sjögren's syndrome
autoimmune condition that affects the exocrine glands resulting in dry mucous membranes e.g. mouth, eyes, vagina
primary - occurs in isolation
secondary - SLE or RA associated
anti-Ro and anti-La antibodies
142
the As of ankylosing spondylitis
apical fibrosis
anterior uveitis
aortic regurg
achilles tendonitis
AV node block
amyloidosis
