Rheumatology Flashcards
1
Q
Polymyalgia rheumatica features
A
There is no true weakness of limb girdles in polymyalgia rheumatica on examination. Any weakness of muscles is due to myalgia (pain inhibition).
ESR elevated. CK normal.
2
Q
Imaging to support diagnosis of ankylosing spondylitis?
A
sacro-ilitis on a pelvic X-ray
well formed syndesmophytes on lumbar spine film (ossification of outer fibres of annulus fibrosus)
subchondral erosions, sclerosis
and squaring of lumbar vertebrae on X-ray
3
Q
Risk factors for pseudo gout?
A
increasing age haemochromatosis hyperparathyroidism low magnesium, low phosphate acromegaly, Wilson's disease
4
Q
Management of temporal arteritis?
A
urgent high-dose glucocorticoids should be given as soon as the diagnosis is suspected and before the temporal artery biopsy
if there is no visual loss then high-dose prednisolone is used
if there is evolving visual loss IV methylprednisolone is usually given prior to starting high-dose prednisolone
there should be a dramatic response, if not the diagnosis should be reconsidered
if visual symptoms, then same day review by opthamologist
bone protection with bisphosphonates is required as long, tapering course of steroids is required
5
Q
Lateral epicondylitis on examination
A
worse on resisted wrist extension/suppination whilst elbow extended
6
Q
How to distinguish pseudo gout from gout?
A
Crystals - Gout is caused by sodium urate crystals and pseudogout is caused by calcium pyrophosphate crystals.
X-ray - Chondrocalcinosis helps to distinguish pseudogout from gout
Joints affected - Gout most commonly affects the big toe, instep, heel, ankle, and/or knee. Pseudogout is most likely to affect the knee, wrist, and/or large knuckles of the hand (metacarpophalangeal joints). It may also involve the hip, shoulder, and/or spine
Severity, timing, duration of pain - Gout more severe than pseudo gout. Gout often strikes in middle of night, pseudo gout can strike at any time. If left untreated, gout attack symptoms will usually go away within a few days or weeks. Left untreated, an episode of pseudogout can last days, weeks, or even months
7
Q
Causes of drug-induced lupus?
A
Most common causes
procainamide
hydralazine
Less common causes
isoniazid
minocycline
phenytoin
8
Q
Antibodies in drug induced lupus?
A
ANA positive in 100%, dsDNA negative
anti-histone antibodies are found in 80-90%
anti-Ro, anti-Smith positive in around 5%
9
Q
Features of drug induced lupus?
A
arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
10
Q
Antibodies in Sjogren’s syndrome?
A
rheumatoid factor (RF) positive in nearly 50% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
anti-La (SSB) antibodies in 30% of patients with PSS
11
Q
Features of Sjogren’s syndrome?
A
dry eyes: keratoconjunctivitis sicca dry mouth vaginal dryness arthralgia Raynaud's, myalgia sensory polyneuropathy recurrent episodes of parotitis renal tubular acidosis (usually subclinical)
marked increased risk of lymphoid malignancy (40-60 fold).
12
Q
Management of Sjogren’s syndrome?
A
artificial saliva and tears
pilocarpine may stimulate saliva production
13
Q
Investigations of Sjogren’s syndrome?
A
auto-antibodies
Schirmer’s test: filter paper near conjunctival sac to measure tear formation
histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4
14
Q
What is Felty’s syndrome?
A
a condition characterized by splenomegaly and neutropenia in a patient with rheumatoid arthritis. Hypersplenism results in destruction of blood cells which classically results in neutropenia but can also cause pancytopenia
15
Q
Presentation of psoriatic arthritis?
A
seronegative spondyloarthropathies. It correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions.
males and females being equally affected
HLA-B27
Patterns: symmetric polyarthritis asymmetrical oligoarthritis: typically affects hands and feet sacroilitis DIP joint disease arthritis mutilans
Other signs
psoriatic skin lesions
periarticular disease - tenosynovitis and soft tissue inflammation resulting in:
enthesitis: inflammation at the site of tendon and ligament insertion e.g. Achilles tendonitis, plantar fascitis
tenosynovitis: typically of the flexor tendons of the hands
dactylitis: diffuse swelling of a finger or toe ‘sausage fingers’
nail changes
pitting
onycholysis
16
Q
X-ray of psoriatic arthropathy?
A
often have the unusual combination of coexistence of erosive changes and new bone formation
periostitis
‘pencil-in-cup’ appearance
17
Q
Management of psoriatic arthropathy?
A
should be managed by a rheumatologist
treatment is similar to that of rheumatoid arthritis (RA). However, the following differences are noted:
mild peripheral arthritis/mild axial disease may be treated with ‘just’ an NSAID, rather than all patients being on disease-modifying therapy as with RA
use of monoclonal antibodies such as ustekinumab (targets both IL-12 and IL-23) and secukinumab (targets IL-17)
has a better prognosis than RA
18
Q
Pattern of pyrexia in Still’s disease?
A
it typically rises in the late afternoon/early evening in a daily pattern and accompanies a worsening of joint symptoms and rash
19
Q
Malignancy + raised CK?
A
Think about polymyositis
20
Q
Features of ankylosing spondylitis? 6 A’s
A
- Apical fibrosis
- Anterior uveitis
- Aortic regurgitation
- Achilles tendonitis
- AV node block
- Amyloidosis
21
Q
Anti-phospholipid syndrome presentation
A
CLOTS: clots, livedo reticularis, obstetric complications and thrombocytopenia.
22
Q
Cause of Marfan’s syndrome?
A
caused by a mutation in a protein called fibrillin-1
23
Q
Bone pain, tenderness and proximal myopathy (→ waddling gait)?
A
Osteomalacia
24
Q
What is Behcet’s syndrome?
A
is a complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins.
Features
- classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
- thrombophlebitis and deep vein thrombosis
25
What antibody can help assist in making diagnosis of anti-phospholipid syndrome?
a positive anti-Cardiolipin antibody
26
What is dermatomyositis?
is usually an autoimmune condition, being most common in women aged 50-70. However, it can also be a paraneoplastic disease, with ovarian, breast and lung tumours being the most common underlying cancers. The possibility of underlying malignancy should be considered, especially in older patients.
27
Why are chemotherapy patients at an increased risk of gout?
from increased urate production. Cytotoxic drugs cause an increase in the breakdown of cells, releasing products that are degraded into uric acid. Hyperuricaemia is a known risk factor for gout.
28
What is osteoarthritis?
It is a chronic, degenerative disease affecting large weight bearing joints. It is clinically diagnosed, but X-ray changes can support diagnosis if needed.
Caused by 'wear and tear', so localised loss of cartilage, remodelling of adjacent bone and associated inflammation
29
What is typical OA history?
Chronic history of pain in large bearing joints (knee, hips)
Worsened by exercise, and relieved by rest
Has morning stiffness, lasting less than 30 minutes
30
Risk factors for OA?
non-modifiable = age, gender (female), previous trauma/hypermobility
modifiable = obesity
31
Management of OA
- Modifiable risk factors - lifestyle
- Medical
- Simple analgesia - paracetamol and topical NSAID (only in knee/hand)
- Oral NSAIDs (+ PPI cover), opioids, capsaicin cream and intra articular (joint) steroid injections
- Surgical
- Last resort - arthroplasty
- Post op physio and exercise, walking aid needed for 6-8 weeks
- Can impact QoL - can’t flex hip, sit in low chairs, cross legs
32
Hand signs of OA
- signs seen in hands earlier than larger joints
- painless nodes
- Bouchard in PIP
- Heberdens in DIP
- squaring of thumbs - due to fixed adduction of thumb
- x-ray changes come before Sx, and are not linked to functional dysfunction
33
What is Rheumatoid arthritis?
- Chronic inflammatory, autoimmune, synovial joint inflammation, causing intra and peri articular joint dysfunction
- Additionally has extra articular, systemic features
Common in young middle aged woman with bilateral hand pain
34
Risk factors for RA?
```
modifiable = smoking, obesity
non-modifiable = female, FHX, age
```
35
Typical features of RA?
- Swollen painful joints in bilateral hands
- Stiffness worse in morning >30 mins, better with use
- Insidious onset that gets worse
- Systemic involvement - fatigue, fevers, WL
36
How to diagnose RA?
- Initial investigations
- RF (IgM) - sensitive not specific
- Anti CCP - more specific
- x-rays:
- Loss of joint space
- Erosions
- Soft tissue swelling
- Soft bones (osteopenia)
- 2010 ACOR criteria:
- Joint involvement
- Serology
- Acute phase reactants (CRP/ESR)
- Duration of Sx (6wk)
- Scored /10, 6 = RA
37
What are poor prognostic factors for RA?
- Positive serology
- RF
- Anti CCP
- HLA DR4
- Poor function at presentation
- X-ray changes, e.g., early erosions
- Extra articular features
- Insidious onset
- development of CVD is main cause of death in these patients
38
How to assess prognosis of RA?
- DAS28 - measure of disease activity and response to Tx in RA
- HAQ
39
Hand signs in RA?
- Ulnar deviation - subluxation of MCP joint
- Boutonniere - PIP flexion with DIP extension
- Swan neck - PIP extension with DIP flexion
40
Management of RA?
1 - monotherapy with methotrexate, leflunomide or sulfalazine. mild cases treated with hydroxychloroquine
2 - dual therapy
3 - methotrexate + biologic (TNFa)
4 - methotrexate + rituximab
remission is induced with corticosteroids
41
Use of methotrexate in RA?
- is a folate antagonist - taken once a week with folate other days
- start - FBC, renal and LFT, then weekly until stabilised, then every 2-3 months
- SFx - liver and lung fibrosis, anaemia, myelosuppression, mouth ulcer (most common)
42
Use of hydroxycloroquine in RA?
- antimalarial
- can be used in pregnant women
- SFx - nightmares, skin pigmentation, macular damage (bullseye retinopathy)
- monitor - ask about visual Sx and test visual acuity annually
43
Use of sulfasalazine in RA?
- pro drug for 5-ASA, decreases neutrophil chemotaxis and suppresses proliferation of lymphocytes and cytokines
- cautions - G6PD deficiency, allergy to aspirin and sulphonamides
- Adverse effects - oliospermia, SJS, pneumonitis/lung fibrosis, myelosuppresion, Heinz body anaemia, megaloblastic anaemia, may colour tears
- safe in pregnancy and breastfeeding
44
Use of Leflunomide in RA?
- DMARD
| - SFx - HTN, rash, peripheral neuropathy
45
Use of anti TNF (etanercept, infliximab, adalimumab) in RA?
- susceptible to sepsis
| - need CXR - can reactivate TB
46
Extra-articular signs of RA?
Cardiovascular: CAD, atherosclerosis, pericarditis, rheumatoid vasculitis, Raynaud's
GI: oesophagitis, gastritis, ulcer disease, hepatotoxicity, Felty's syndrome
Skin: vascular lesions, rheumatoid nodules
Neurological: cervical myelopathy, peripheral neuropathy, mononeuritis multiplex, entrapment neuropathy
Ocular: keratoconjunctivitis sicca, episcleritis, sclerisi, scleromalacia perforans
Renal: tubulo-interstitial nephritis, amyloidosis, membranous glomerulonephritis
Pulmonary: interstitial lung disease, pleural disease (including effusion), bronchiolitis obliterates, amyloidosis, Caplan's syndrome, bronchiectasis, drug toxicity
Haematological: anaemia, thrombocytosis, Felty's syndrome
47
Most common need for revision of a hip replacement
Aseptic loosening of the joint
48
Posterior dislocation in a hip replacement?
Due to extreme hip flexion, causes painful clunk and short IR leg
49
What is ankylosing spondylitis?
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old.
50
Features of ankylosing spondylitis?
typically a young man who presents with lower back pain and stiffness of insidious onset
stiffness is usually worse in the morning and improves with exercise
the patient may experience pain at night which improves on getting up
51
Associated features of ankylosing spondylitis? (6 A's)
```
anterior uveitis
apical fibrosis
AV block
aortic regurgitation
achilles tendonitis
amyloidosis
```
52
Examination findings of ankylosing spondylitis?
reduced lateral flexion
reduced forward flexion - Schober's test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
reduced chest expansion
53
X-ray changes seen in ankylosing spondylitis?
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
'bamboo spine' (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
if no changes and high suspicion for AS - MRI, may show early signs of inflammation (bone marrow oedema)
additional spirometry -may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints.
54
Management of ankylosing spondylitis?
encourage regular exercise such as swimming
NSAIDs are the first-line treatment
physiotherapy
the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement
the 2010 EULAR guidelines suggest: 'Anti-TNF therapy (etanercept) should be given to patients with persistently high disease activity despite conventional treatments'
55
What is psoriatic arthritis?
Psoriatic arthropathy is an inflammatory arthritis associated with psoriasis and is classed as one of the seronegative spondyloarthropathies.
It correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions.
Around 10-20% of patients with skin lesions develop an arthropathy with males and females being equally affected.
56
Presentation patterns of psoriatic arthritis?
symmetric polyarthritis-
very similar to rheumatoid arthritis
30-40% of cases, most common type
asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
until recently it was thought asymmetrical oligoarthritis was the most common type
DIP joint disease (10%)
arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')
57
Presentation of psoriatic arthritis?
psoriatic skin lesions
periarticular disease -
tenosynovitis and soft tissue inflammation resulting in:
enthesitis: inflammation at the site of tendon and ligament insertion e.g. Achilles tendonitis, plantar fascitis
tenosynovitis: typically of the flexor tendons of the hands
dactylitis: diffuse swelling of a finger or toe
nail changes = pitting,
onycholysis
58
X-ray changes seen in psoriatic arthritis?
often have the unusual combination of coexistence of erosive changes and new bone formation
periostitis
'pencil-in-cup' appearance
59
Management of psoriatic arthritis?
should be managed by a rheumatologist
treatment is similar to that of rheumatoid arthritis (RA). However, the following differences are noted:
mild peripheral arthritis/mild axial disease may be treated with 'just' an NSAID, rather than all patients being on disease-modifying therapy as with RA
use of monoclonal antibodies such as ustekinumab (targets both IL-12 and IL-23) and secukinumab (targets IL-17)
has a better prognosis than RA
60
What is reactive arthritis?
HLA-B27 associated seronegative spondyloarthropathies
classic triad of urethritis, conjunctivitis and arthritis
an arthritis that develops following an infection where the organism cannot be recovered from the joint.
61
Features of reactive arthritis?
4 wks to occur, lasts 4-6 month
'can't see, pee or climb a tree' - uveitis, balanitis, arthritis
asymmetrical oligoarthritis of lower limbs
dactylitis
eye - conjunctivitis, anterior uveitis
skin - circinate balanitis (painless vesicles on coronal margin of prepuce), Keratoderma blenorrhagina
post-dystenteric - Shigella, salmonella, yersinia, campylobacter
post-STI - chlamydia
62
Management of reactive arthritis?
symptomatic: analgesia, NSAIDS, intra-articular steroids
sulfasalazine and methotrexate are sometimes used for persistent disease
symptoms rarely last more than 12 months
63
What is gout?
a form of inflammatory arthritis caused by extra uric acid in the body, causing crystals and macrophages in joints then causing swelling and pain
64
Features of gout?
episodes lasting several days when their gout flares and are often symptom-free between episodes. episodes develop maximal intensity within 12 hours.
pain, swelling, erythema
70% of first presentations affect 1st MTP joint. other common joints include: ankle, wrist, knee
65
Typical patients with gout?
Elderly, overweight, with other co-morbidities
66
Risk factors for gout?
```
Non-modifiable:
age
male
Hx of it
kidney disease
```
```
Modifiable:
weight
dietary intake - alcohol, red meats, fish
smoking
drugs - diuretics - loop and thiazides
```
67
Management of acute gout?
NSAIDs or colchicine first line
max dose of NSAID prescribed 1-2 days after Sx settled. PPI may be required
colchicine - inhibits microtubule polymerisation - slow onset, diarrhoea
oral steroids if NSAIDs and colchicine contraindicated
allopurinol continued if already taking
68
Indications for rate-lowering therapy
now for all patients after first attack of gout
```
ULT is particularly recommended if:
>= 2 attacks in 12 months
tophi
renal disease
uric acid renal stones
prophylaxis if on cytotoxics or diuretics
```
69
Prophylaxis of gout?
allopurinol is first-line - delay in starting after acute attack. Has SFx of myelosuppression with azathioprine
100mg od, with dose titrated every few weeks to aim for serum uric acid of <300 mol/l
colchicine (or NSAID if CI'd) cover when starting allopurinol - may need to continue for 6m
second-line - febuxostat
lifestyle modifications
- reduce alcohol intake
- lose weight if obese
- avoid food high in purines - liver, kidney, seafood, oily fish, yeast products
70
Findings of gout on synovial fluid analysis?
needle shaped negatively birefringent monosodium urate crystals under polarised light
71
Gout vs pseudogout?
Gout = uric acid = needle shaped negative light
Pseudo gout = calcium = rhomboid shaped positive
Pseudogout associated with:
- haemochromatosis
- high calcium - acromegaly
- Wilson's
Pseudogout on X-ray - linear calcification on knee
72
What is pseudo gout?
form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium.
associated with increasing age. if younger age have underlying risk factor: haemochromatosis, Hyperparathyroidism, low Mg, low phosphate, acromegaly, Wilsons
Features - knee, wrist and shoulders most commonly
Management - aspiration of joint fluid to exclude SA, NSAIDs or intra-articular, IM or oral steroids as for gout
73
Differentials for RA?
RA
SLE
Psoriatic arthritis
74
What is septic arthritis?
an infection in the joint (synovial) fluid and joint tissues.
It occurs more often in children than in adults.
The infection usually reaches the joints through the bloodstream. In some cases, joints may become infected due to an injection, surgery, or injury.
75
Pathophysiology of septic arthritis?
most common organism overall is Staphylococcus aureus
in young adults who are sexually active, Neisseria gonorrhoeae
most common cause is haematogenous spread - may be from distant bacterial infections (e.g., abscesses)
most common location is knee
76
Features of septic arthritis?
acute, swollen joint
restricted movement
examination findings: warm to touch/fluctuant
fever: present in the majority of patients
in children, more common in boys (2:1), joints affected are hip, knee and ankle
77
Investigations for septic arthritis?
Synovial fluid sampling (prior to Abx administration if necessary)
Blood cultures
Joint imaging
78
Management of septic arthritis?
Sepsis 6, senior help, supportive care as needed
intravenous antibiotics which cover Gram-positive cocci are indicated. The BNF currently recommends flucloxacillin or clindamycin if penicillin allergic
Abx Tx normally given for 4-6 wks, switched to oral Abx after 2 wks
needle aspiration used to decompress the joint
arthroscopic lavage may be required
79
Kocher's criteria for diagnosis of septic arthritis in children?
fever >38.5 degrees C
non-weight bearing
raised ESR
raised WCC
80
Management of anti phospholipid?
No VTE - aspirin
1 VTE - lifelong warfarin - INR 2-3
Multiple - INR 3-4
Arterial thrombosis - INR 2-3
81
Typical features of Marfan's?
Tall pt's with long arms and fingers
Hypermobility
Pectus craniartum/ excavatum
Scoliosis
High-arched palate
Pes Planus
Dilation of aortic sinuses > aortic aneurysms, aortic dissection, aortic regurgitation, MV prolapse
82
Gene mutations in Marfan's?
Autosomal dominant
Defect in protein fibrillin 1
83
Common complications in Marfan's?
Pneumothorax
MV prolapse
AV prolapse
Aortic aneurysm
84
Management of Marfan's?
Regular ECHO monitoring
Beta-blockers
ACEi
Genetic counselling
Avoid intense exercise and caffeine
85
Prognosis of Marfan's?
Used to be 50 yrs, but this has increased
Aortic dissection most common cause of death
86
Typical features of Ehlers Danlos
Joint hyper motility
Stretchy, fragile skin
87
Gene mutations of EDS
Collagen defects - mostly type III collagen
Mainly autosomal dominant
Hypermobilie variant is not AD
88
Common complications of EDS
Postural orthostatic tachycardia syndrome (POTS) - autonomic dysfunction
Joint problems - arthritis, pains, dislocation etc.
Skin problems - cuts, poor healing, bruising
AR regurgitation, MV prolapse, aortic dissection
SAH
Angioid retinal streaks
89
Management of EDS?
No cure
Manage Sx and complications
90
Prognosis of EDS?
Beighton score diagnosis (out of 9)
Palms flat, hyperextension (elbow, knee, pinky)
Thumb to forearm
91
What is systemic lupus erythematosus (SLE)?
a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body.
The signs and symptoms of SLE vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous system, and blood-forming (hematopoietic) system.
Autoimmune
Type 3 hypersensitivity reaction (immune complex deposition)
associated with HLA B8, DR2, DR3
92
Epidemiology of SLE?
F>M (9:1)
more common in Afro-Caribbean and Asian
onset 20-40 yrs
incidence has risen substantially in past 50 yrs
93
Common exam presentation of SLE?
young/middle aged female presenting with a photosensitive face rash, fatigue/malaise and possible joint pains
94
Features of SLE?
```
General features
fatigue
fever
mouth ulcers
lymphadenopathy
```
Skin
malar (butterfly) rash: spares nasolabial folds
discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas. Lesions may progress to become pigmented and hyperkeratotic before becoming atrophic
photosensitivity
Raynaud's phenomenon
livedo reticularis
non-scarring alopecia
Musculoskeletal
arthralgia
non-erosive arthritis
Cardiovascular
pericarditis: the most common cardiac manifestation
myocarditis
Respiratory
pleurisy
fibrosing alveolitis
Renal
proteinuria
glomerulonephritis (diffuse proliferative glomerulonephritis is the most common type)
Neuropsychiatric
anxiety and depression
psychosis
seizures
95
Investigations of SLE?
99% pt's are ANA positive
20% RF positive
anti-dsDNA - highly specific, but less sensitive
anti-Smith - highly specific, sensitivity (30%)
Also - anti-U1 RNP, SS-A, and SS-B
96
Monitoring of SLE?
inflammatory markers:
ESR is generally used
during active disease the CRP may be normal - a raised CRP may indicate underlying infection
complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption of complement)
anti-dsDNA titres can be used for disease monitoring (but note not present in all patients)
97
Management of SLE?
NSAIDs
sun-block
Hydroxychloroquine - the treatment of choice for SLE
If internal organ involvement e.g. renal, neuro, eye then consider prednisolone, cyclophosphamide
98
What antibodies are in drug-induced lupus?
Anti-histone antibodies
ANA positive in 100%
dsDNA negative
99
Causes of drug-induced lupus?
Most common causes
procainamide
hydralazine
Less common causes
isoniazid
minocycline
phenytoin
causes: arthralgia, myalgia, malar rash, pulmonary involvement (e.g., pleurisy)
100
What is limited cutaneous systemic sclerosis?
Raynaud's may be first sign
Scleroderma affects face and distal limbs predominately
Associated with anti-centromere antibodies
A subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia
101
What is diffuse cutaneous systemic sclerosis?
Scleroderma affects trunk and proximal limbs predominately
Associated with scl-70 antibodies
CREST + internal organ disease such as CVD, HTN< pulmonary fibrosis, glomerulonephritis
The most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
Anti-Scl 70 antibodies
Other complications include renal disease and hypertension
Poor prognosis
102
What is Sjogren's syndrome?
an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it usually develops around 10 years after the initial onset.
Sjogren's syndrome is much more common in females (ratio 9:1).
There is a marked increased risk of lymphoid malignancy (40-60 fold)
Schirmer's test - filter paper near conjunctival sac to measure tear formation
Anti-Ro and Anti-La antibodies
Hypergammaglobulinaemia, low C4
Management - artificial saliva and tears, pilocarpine to stimulate salvia production
103
What is myositis and dermatomyositis?
inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions
dermatomyositis often indicates malignancy (ovarian, breast, lung)
Gottron's papules - roughened red papules over extensor surfaces of fingers
Elevated CK
Myositis - anti-Jo 1
Dermatomyositis - anti-Jo 2
104
What is anti-phospholipid syndrome?
an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia
can be primary or secondary, commonly to SLE
antiphospholipid syndrome causes a paradoxical rise in the APTT
Lupus anticoagulant, anti-cardiolipin antibodies, beta-2 glycoprotein
105
What is polymyalgia rheumatica?
relatively common condition seen in older people characterised by muscle stiffness and raised inflammatory markers
closely related to temporal arteritis
106
Features of polymyalgia rheumatica?
Patient >60
Rapid onset (<1 month)
Aching, morning stiffness in proximal limb muscles - NOT weakness
Also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
107
Investigations for polymyalgia reumatica?
raised inflammatory markers e.g. ESR > 40 mm/hr
note creatine kinase and EMG normal
108
Treatment of polymyalgia reumatica?
prednisolone e.g. 15mg/od
patients typically respond dramatically to steroids, failure to do so should prompt consideration of an alternative diagnosis
109
What is temporal arteritis?
large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR)
Histology shows changes that characteristically 'skips' certain sections of the affected artery whilst damaging others
110
Features of temporal arteritis?
Pt's >60
Rapid onset (<1 month)
Headache
Jaw claudication
Visual disturbances - amaurosis fugal, blurring, double vision - secondary to anterior ischaemic optic neuropathy
Tender, palpable temporal artery
50% have features of PMR - aching, morning stiffness in proximal limb muscles
Lethargy, depression, low-grade fever, anorexia, night sweats
111
Investigations for temporal arteritis?
raised inflammatory markers
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
CRP may also be elevated
temporal artery biopsy
skip lesions may be present
note creatine kinase and EMG normal
112
Treatment of temporal arteritis?
urgent high-dose glucocorticoids should be given as soon as the diagnosis is suspected and before the temporal artery biopsy
- if there is no visual loss then high-dose prednisolone is used
- if there is evolving visual loss IV methylprednisolone
there should be a dramatic response with steroids, if not the diagnosis should be reconsidered
urgent ophthalmology review
bone protection with bisphosphonates is required as long, tapering course of steroids is required
low-dose aspirin is sometimes given to patients as well, although the evidence base supporting this is weak
