Rheumatology Flashcards
1
Q
What is enthesitis? What can it be present in?
A
inflammation in area where tendon inserts into bone
seronegative spondyloarthropathy
2
Q
name 3 clinical features of spondyloarthopathy:
A
- enthesitis
- positive family history
- uveitis
- asymettric arthritis
- postive response to NSAIDs
3
Q
What are the posture changes with ankylosing spondylitis, what is the first line imaging? And 2nd line
A
- kyphosis
- loss lumbar lordosis
- compensatory extension of cervical spine
- question mark posture
- X-ray first line, then CT
4
Q
Extra-axial clinical features of Ankylosing spondylitis?
A
- uveitis (acute anterior)
- cardiac involvement (A. regurg., AV block, LBBB)
- pulmonary fibrosis (mainly apical)
5
Q
The modified new york criteria is used for ank spond. diagnosis, name 2+ of the criteria:
A
- low back pain of 3months+ duration
- limitation of lumbar spinal motion in sagittal and frontal planes
- decreased chest expansion (<5cm)
- sacroiliitis
6
Q
What is a Romanus lesion in the context of ank spond.?
What is the name for a lesion from calcification of the spinal ligaments?
A
- early erosive spinal disease (looks like bite taken out of vertebra)
- syndesmophytes
7
Q
Name 4 spondyloarthropathies that are associated to 4 HLA B27 :
A
- ank spond
- reactive arthritis
- juvenile spondyloarthropathy
- enteropathic spondyloarthritis (joint involvement in IBD)
- psoriatic spondyloarthritis
- ant. uveitis
- aortic incompetence with heart block
8
Q
How much of the general healthy population are HLAB27 postitve (to bear in mind)
A
8%
-also not all pts with spondyloarthropathies will be +
9
Q
Name 3 treatments for ank spond
A
- physio home exercises
- NSAIDS
- sulfasalazine, methotrexate
- biologics e.g. anti TNF, anti IL-17, IL-12. 23
10
Q
psoriatic arthritis affects people at what age? Are they likely to have skin disease at onset?
A
- age of onset 30-50yrs
- 2/3rs have skin disease first (scalp and nail disease are important to look for as well as rash)
11
Q
What the rash keratoderma blenorrhagicum seen in? What is the triad in this condition?
A
-rash over soles of feet
-in reactive arthritis
(arthritis, conjunctivitis, urethritis)
12
Q
Name some possible arthritis patterns in psoriatic arthritis:
A
- DIP involvement with dactylitis and nail dystrophy
- arthritis mutilans (progressive bone erosion and collapse of soft tissues)
- symmetrical polyarticular
- spondyloarthritis
- asymmetric oligoarthritis (larger joints)
13
Q
Name 3 medications used in the treatment of psoriatic arthritis:
A
- analgesics. NSAIDs
- sulfasalazine
- methotrexate
- azathioprine
- cyclosporin
- biologics, anti-TNF, anti IL-17/IL-12,23 agents
14
Q
Name 3 aetiologies of inflammatory arthritis:
A
- autoimmune
- crystal
- pathogen (Septic)
15
Q
Name 3 changes in the synovium (tissue lining joints in inflammatory arthritis:
A
- hyperaemia of blood vessels
- angiogenesis
- synovial hypertrophy
- inflammatory cells infiltrate with release of cytokines (t b cells, mo)
- formation of a “pannus” -invading eroding synovium into bone and cartilage damaging joints
- vascular villi
16
Q
RA is an autoimmune inflammatory arthritis, the immune dysregulation leads to synovial ___ & ___ cell infiltration and excessive cytokine release e.g. __ _ __, ______ activation and release of ___ leads to destruction of bone and cartilage
A
- vascularity & hypertrophy
- TNFa. IL-6, IL-7
- activation of osteoclasts (breaks down bone) and MMP release damages cartilage
17
Q
In a history characteristic of inflammatory arthritis, the stiffness/joint pain is
- worse with ___
- e.g. difficulty ..
- better with ___ e.g.
- joint ___ e.g.
- pain med __
A
- worse with rest
e. g. early morning stiffness >30mins, sleep disturbance, difficult getting out chair - better w activity (e.g. ok once i get going)
- joint swelling e.g. rings tight
- NSAIDs work well
18
Q
In an exam characteristic of inflammatory arthritis, the findings include:
A
- tender joints
- synovial swelling
- synovial effusion
- bone e.g osteophytes
- characteristic patterns/distribution
- tophi, nodules, rashes..
19
Q
RA may be positive for __ and ___
A
RF and anti-CCP
20
Q
Name 2 conditions in which ANA is often positive
A
lupus
sjogren’s
scleroderma
21
Q
Suggest some investigations in a pt presenting with suspected inflammatory arthritis:
A
- FBC, CRP, ESR (inflamm check), +/- ferritin, alk phos
- LFT and U&Es (to check what meds can be given)
- autoantibodies (RF, anti-CCP, ANA, if ANA positive do subset based on ddx e.g. ds-DNA-lupus, anti-ro anti-la sjogren’s)
- US (synovial swelling confirmation and increased vascularity on dopplers)
- X-rays to look for erosions
- aspiration of effusion for mc&S
22
Q
Autoimmune causes of arthritis: name 3
A
- RA
- Psoriatic arthritis
- reactive arthritis
- CT disease: SLE/Sjogren’s
23
Q
Rheumatoid arthritis RFs/triggers
A
- HLA-subtypes (Ask family hx of autoimmune conditions)
- smokers
- chronic periodontal disease (Porforimonis gingivalis causative organism-leads to higher levels of citrilination)
- specific antibodies: RF, anti-CCP
24
Q
RA affects which joints/distribution?
A
- peripheral (DIP not affected)
- tends to be symmetrical
- mainly affected: wrists, MCP, PIP, MTP, shoulder and knees
- ask re neck symptoms as risk of atlanto-axial subluxation
25
Extra-axial manifestations of rheumatoid arthritis:
- tendon sheaths
- nodules
- eye: conjunctivitis, scleritis, dryness (+/- secondary Sjogren's)
- rare: felty's syndrome: neutropenia + splenomegaly
26
what are poor prognostic factors in rheumatoid arthrits:
- large no joints affected
- RF strong positive
- anti-CPP +
- smoker
- early erosions
- high disability score
27
RA inflammation chronically impacts what system? Leading to high mortality from what? Therefore control of traditional risk factors should be to what range of targets?
- cardiovascular
- 3x increase in heart attack/stroke risk (x50 increase risk in lupus!)
- control to "diabetic targets" e.g. BP <130/80, LDL <2.6
28
RA treatment comprises of fast acting ____ acutely and starting 2 agents of DMARS that take 12+ weeks to kick in, examples include:
Name 3 biologics
- cortiosteroids (IM)
- methotrexate, sulphasalazine, hydrozychloriquine, lefunomide (can cause HT and eye disease)
- monitor blood tests regularly
- biologics: anti-TNF, anti IL-6, B cell and T cell inhibitors and JAK stat inhibitors
29
Name a medication of the following classes:
- Anti TNF agents
- Anti IL-6
- Anti B cell
- T cell co-stimulation inhibitor
TNF: adalimumab, etanercept, certolizumab, golimumab, inflixumab
- anti IL-6: tocilizumab
- anti B cell: rituximab
- T cell inhibitor: abatacept
30
what 3 fts alert you to refer a pt with suspected RA urgently for specialist rx?
- small joints of hands/feet affected
- more than 1 joint affected
- delay of >3months between symptom onset and seeking medical advice
31
Sjogren's is an autoimmune connective tissue disorder, where does it primarily affect? What does sicca symptoms entail?
- exocrine glands: salivary, lacrimal, bronchial, vaginal, pancreatic, colonic
- sicca=dry eyes, dry mouth
32
Sjogren's is an autoimmune connective tissue disorder, what symptoms can it present with excluding gland related?
- fatigue
- inflammatory arthlalgia
- tubulointerstitial nephritis leading to renal tubular acidosis
- sensorimotor neuropathy
- hypergammaglobulinaemia - raised ESR (normal CRP) do serum electrophoresis
33
Sjogren's is an autoimmune connective tissue disorder, it is associated with an increased risk of what cancer? Where does this often happen?
- lymphome (B cell non-hodgkins)
- parotid and submandibular gland most common
- so test immunoglobulins annually and investigate gland lumps
34
Sjogren's is an autoimmune connective tissue disorder, if ANA is positive, what would you test for next if suspecting this dx? If these are present in pregnant women and the Igs cross the placenta what is the risk to fetus?
- anti Ro
- anti La
- complete heart block to fetus
35
raised ESR with normal CRP ddx: give 3
- sjogren's
- lupus
- myeloma
36
name some treatment used for sjogren's
- artificial saliva
- lubricant eye drops
- hydroxychloroquine for arthralgia
37
acute knee swelling in 75yo male, give 3 ddx:
- acute OA
- gout
- pseudogout
- septic arthritis
38
acute knee swelling ddx:
| -acute OA, gout, pseudogout, septic arthritis, what investigations would be done?
- aspirate for gram stain and culture
| - send for cytology analysis to visualise urate crystal or calcium pyrophosphate crystals (pseudogout)
39
what are the 3 principles of management of an acute monoarthritis (emergency):
- prompt diagnosis, early aspiration (more chance to identify pathogen)
- adequate drainage of joint (if builds up can affect function then mobility risk of muscle loss and poor rehab)
- immediate antimicrobials if septic arthritis suspected (e.g. fever, raised WCC and CRP) follow guidelines
40
Effects of NSAIDs in gut:
- more prostoglandin release
- these affect the gastric mucosa epithelium
- gastric erosions and effects blood vessels in gut
- so prescribe NSAIDs with PPI
41
Name 3 non- cox-2 selective NSAIDS
- ibroprofen
- naproxen
- diclofenac
- indomethacin
42
Name a cox-2 selective NSAID (has less gastric SEs but not recommended 1st line)
- celecoxib
| - meloxicam
43
give 2 environmental risks for OA:
- obesity
- biomechanics: e.g. previous injury, meniscectomy
- overuse
- post-traumatic OA
44
Heberden's nodes affect the ___ joints
| Bouchard's nodes affect the ___
- heberden -DIPS - bony swelling and sclerosis of bone
| - bouchards PIPs
45
Early OA new rx for early fibrillation and chondral lesions, if lesion is small enough pt can be reffered for ACI, what is this and the process?
Autologous chondrocyte implantation
- cells harvested form pts peripheral blood and grown in culture, form chondrocytes, injected back into the joint to encourage regeneration of the cartilage, repairs the small chondral defect to avoid it getting worse
- esp good post-traumatic OA in young pts where the whole disease process hasn't set into the whole cartilage
46
- cytology from joint aspiration showing rhomboid shaped crystals
- x-ray with white line of linear calcification = chondrocalcinosis is a result of deposition of __ __ __ in joint space
- dx is likely to be __
- on polarisation how would they appear?
- calcium pyrophosphate crystals
- pseudo gout
- weak birefringence
47
What can chondrocalcinosis be a marker of? (can be idiopathic, 2ndry to arthopathy or 2dry to ___ ___ such as: _
- metabolic disorders
e. g. hypothyroid, primary hyperPTH, DM, Wilson's, gout, heamatochromatosis
- therefore may be worth screening for these
48
Colchicine is used to treat acute gout, it is a ___ ___ inhibitor, this inhibits ___ and the shift of ___ ___ into the joint
side effects?
- micro tubule inhibitor (inhibits phagocytosis and the shift of inflammatory cells into the joint)
- abdo pain/cramps and diarrhoea
49
for chronic management of gout, indicated in pts with recurrent attacks defined as > __ / yr of ___ attacks, treatment is __ or __ that lowers the serum ___ below target
->2-3/yr
-symptomatic attacks
allopurinol or febuxostat
-lowers serum urate
50
What shape are gout crystals (mono-sodium urate)? on polarised light effect?
- needle shaped
| - negative birefringence
51
10% of gout is primary due to ___, 90% is primary due to ___ .
There is an inherited cause in young males, where gout is caused by overproduction and is associated with ___ __
- 10%-overproduction
- 90% -undersecretion of urate
-hyperuricaemic nephropathy
52
Give some secondary causes of gout
- overproduction: (2)
- undersection: (2)
- overproduction: haem disease, psoriasis, trauma, alcohol, cytotoxics, warfarin
- undersecretion: renal failure, alcohol, aspirin, diuretics, laxative, L-dopa
53
Lifestyle treatment of gout
- limit purines in diet (rich red meat)
- weight loss
- avoid cider and beers and alcohol
54
what type of drug is allopurinal
xanthine oxidase inhibitor
55
osteoporosis is asymptomatic between fractures with the exception of which fractures that can cause long-term problems?
- scaphoid
- forearm
- through joint --> secondary OA
- vertebral fractures
56
Give 3 reasons osteoporosis is underdiagnosed?
-asymptomatic
-blood tests are normal
-long latent period
( since age 35+ after peak bone mass where bone is lost each yr, and women lose additional bone every 5yrs after menopause-years under the radar)
57
A low trauma fracture in a pt >50yrs, you should consider a dx of ____ but it is important also to exclude ____ and ___
- osteoporosis
- exclude bony mets
- exclude myeloma (lytic lesion in bone that then collapses)
58
-T score on DEXA compares to
-average peak bone mass of a healthy individual
59
-Z score on DEXA compares to
-average bone mass of a person of same age and sex
60
DEXA measures BMD which is only one compartment of bone strength, suggest 3 other aspects that are important in contributing to bone strength: therefore what tool should be used to take these risks into account?
- mineralisation
- bone turnover
- geometry
- trabecular architecture
- cortical thickness and porosity
- use FRAX risk assessment
61
How do bisphosphonates need to be taken in order to work?
on an empty stomach
62
In some pts with osteoporosis you must exclude malignancy and look for secondary causes, suggest 3 investigations to look into this:
- PTH
- TFT
- LTF
- Testosterone
- Coeliac
63
In pts with osteoporosis, it is essential to do 3 tests, which ones?
- vitamin D levels
- bone profile
- U&Es
64
Treatment of severe vit D defiiciency:
| -high dose loading to replenish stores ____ units for __wks then maintenance therapy __ x__ daily
- 60,000 units weekly for 5 weeks
| - maintenance : adcal D3 x2daily
65
If pt is on alendronate but is failing rx (BMD still poor/fracture despite rx) and low vit D, you should treat the vit D deficiency and could start a new rx e.g. a RANK-L inhibitor like ____ which is _monthly ___. With this you must prescribe ___
- Denosumab 6 monthly injections
| - co-prescribe CALCIUM (adcal d3) as they reduce osteoclastic bone resorption, calcium moves from blood to skeleton
66
Review osteoporosis every 5yrs in case you can stop rx for a "bisphosphonate drug holiday". what is reviewed and when may you choose to continue rx?
- annual vit D. calcium, U&Es....
| - unlikely to stop if previous vertebral or hip fracture
67
All pts on steroids should be prescribed ____ (also consider ____ and ____)
CALCIUM e.g. adcal (steroids cause a negative calcium balance)
-bisphosphonates, PPIs
68
Patchy sclerosis and lytic areas throughout the hemi-pelvis on an X-ray, is likely to be ___ affecting the ___ ___ skeleton
-Paget's disease
-proximal axial skeleton
(common incidental finding on X-ray)
69
If Paget's is symptomatic, what may pt experience?
| NB: ALP is raised (bone turnover), and NM bone scan shows high uptake in the areas of Paget's
- bone pain at rest
- long bone deformity
- pathological fractures
- secondary OA
- neuro from bone expansion: Headache, nerve compression, lumbar and skull --> deafness
70
Treatment for active (symptomatic) Paget's:
| following which bone pain should resolve, and ALP normalise
-short course of bisphosphonates
| PO risedronate/IV zoledronate
71
Gout is caused by what? Epidemiology and pathophys?
- caused by hyperuricaemia and intra-articular sodium urate crystals
- x5 more common in men, usually adult onset, often a family history present
- overproduction of uric acid or renal under secretion --> hyperuricaemia. Urate is derived from the breakdown of purines in DNA.
72
Hyperuricaemia and sodium urate crystal deposition can result in 4 clinical syndromes, name 2:
- acute sodium urate synovitis (acute gout)
- chronic interval gout
- chronic polyarticular gout
- chronic tophaceous gout
- urate renal stone formation
73
gout is caused by: -overproduction of uric acid or renal under secretion --> hyperuricaemia.
(Urate is derived from the breakdown of purines in DNA.)
Give 3 causes of each the overproduction and under secretion that may lead to gout:
- overproduction: increased de novo purine synthesis due to HGPRT deficiency or PPS overactivity, increased turnover of purines, myeloproliferative disorders e.g. polycythemia vera, lymphoproliferative disorders, carcinoma, severe psoriasis
- impaired excretion: CKD, drug therapy, HT, lead toxicity, hyperparathyroidism, hypothyroidism, increased lactic acid secretion, glycogen storage disease type 1
74
How does acute gout typically present?
- in middle aged male, with sudden onset, severe pain, swelling and redness of the metatarsophalangeal joint of the big toe
- signs of inflamm may extend beyond the joint giving the impression of cellulitis
- attack may be precipitated by dietary or alcoholic excess by dehydration or starting a diuretic
75
how does chronic tophaceous gout present?
- large, smooth white deposits (tophi)
| - in the skin and around joints, particularly on the ear, fingers and on the Achilles tendon
76
Although gout is usually diagnosed clinically and by swift response to NSAIDs/colchicine, what other ix ( and their results ) can support the diagnosis?
- joint fluid microscopy (long, needle-shaped crystals, negatively birefringent under polarised light)
- serum uric acid (usually high), can be normal in acute attack
- check serum urea and creatinine for renal impairment
77
How are acute attacks of gout managed?
- NSAIDs e.g. diclofenac or cox inhibitors, high dose initially (48hrs) then reduced doses for another week
- if NSAIDs not tolerated, colchicine given but narrow TI, toxic in OD
- steroids: IM or intra-articular depot methylprednisolone
78
Chronic management/advice for pts with gout?
| -any meds to avoid/stop?
- prevent further attacks by lowering serum uric acid
- if obese, lose weight
- diet that reduces total cholesterol and calorie intake and avoids purine rich foods
- reduce alcohol consumption
- stop thiazides and salicylates
79
What med would you consider for pts with gout in whom are having frequent attacks >2/year despite dietary changes/with gouty tophi/renal impairment, what is important about the time of starting this medication?
- allopurinol (xanthine oxidase inhibitor)
- do not start within 1 month post acute attack, give NSAIDs/colchicine for 4 weeks before and after as otherwise allopurinol may trigger an acute attack
80
Pseudogout (pyrophosphate arthropathy) is caused by what? what leads to acute synovitis? Epidemiological difference to gout?
- deposition of pyrophosphate dihydrate in articular cartilage and periarticular tissue --> chondrocalcinosis appearance radiologically
- shedding of crystals into a joint produces acute synovitis that resembles acute gout, except it's more common in elderly women and affects knee/wrist mostly.
81
Pseudogout (pyrophosphate arthropathy) in young people may be associated with other medical conditions such as: __...?
- haemochromatosis
- hyperparathyroidism
- Wilson's disease
- alkaptonuria (excess homogentisic acid polymerises to produce a dark product deposited in cartilage and other tissues)
82
Pseudogout (pyrophosphate arthropathy) is investigated how? and the findings?
- joint fluid microscopy (demonstrates small brick-shaped pyrophosphate crystals which are positively birefringent under polarised light)
- presence of chondrocalcinosis on radiography is suggestive
- blood count may show high WCC
83
How is Pseudogout (pyrophosphate arthropathy) managed?
- joint aspiration with NSAIDs/colchicine
| - injections of local corticosteroids can help after septic arthritis has been excluded on joint fluid microscopy
84
Septic arthritis is an emergency with risk of what?
- common causative organism is?
- RFs for it include what?
- risk of irreversible joint damage, long term disability, death
- results from staph aureus commonly, gram - common in elderly/immunosuppressed
- RFs: prosthetic joints, pre-existing joint disease, recent intra-articular steroid injections and Diabetes
85
Clinical features of Septic arthritis:
- hot, painful, swollen joint that's developed acutely
- may be fever/evidence of infection elsewhere
- in elderly/immunosuppressed can be masked, so high index of suspicion
- prosthetic infection can be early (<3months) or delayed
86
Acute monoarthritis is Septic arthritis until proven otherwise, what is the emergency pathway taken in the management?
- joint what?
- bloods what?
- swab of what?
- joint aspiration and synovial fluid analysis (appearance (straw coloured=normal), WCC, gram stain and culture, polarised light microscopy for crystals
- bloods: FBC, ESR, CRP, blood cultures
- swab of urethra, cervix & anorectum (if gonococcal infection possible)
87
How to treat acute (non-gonococcal) bacterial arthritis (Septic arthritis):
- initially what abx IV and what abx ___ acid orally?
- if immunosuppressed add which abx?
- how long should abx be continued and by what route?
- any other management relating to joint and pain?
- initial rx pending sensitivities = flucloxacillin 1-2g / 6hrly IV (erythro/clinda-mycin if penicillin allergic)
- oral fusidic acid 500mg 8hrly
- add gentamicin in immunosuppressed pts to cover gram -
- modify rx on sensitivities but continue rx for 6 weeks (first 2 weeks IV, then 4 weeks orally)
- adequate joint drainage by needle aspiration, arthroscopy or open drainage (refer prosthetic infection to orthopaedics)
- immobilise joint in acute stages, mobilise early to avoid contractures
- NSAIDs for pain relief
88
Gonococcal arthritis is most common cause of Septic arthritis in previously fit, young, adults. What involvement is common? where are cultures often positive even if joint culture isn't? What is the rx?
- skin involvement (maculopapular pustules)
- culture from genital tract, note blood cultures also often +
- rx with penicillin, ciprofloxacin or doxycycline for 2 weeks and joint rest
89
Osteomyelitis can be due to local infection or where? Most common causative organism? Symptoms?
- metastatic haematogenous spread (e.g. from a boil)
- staphylococcus commonest (Haemophilus influenza and Salmonella (esp sickle cell pts) also common
- sx: fever, local pain, erythema and sinus formation in chronic osteomyelitis
90
Diagnosis of osteomyelitis is by what ix? Usual treatment is with ___ and ____ acid for at least how long? initial route?
- CT, MRI or bone scan.
- Blood cultures, bone biopsy, cultures can be done to identify organism + sensitivities
- flucloxacillin and fusidic acid, initially IV, continue 4-6weeks.
91
-systemic sclerosis is a multisystem disease with involvement of skin and Raynaud's occurring early. 3x more in females, usually presents ~30-50yrs. Aetiology is complex and not fully known but what is the gist and the end result?
- vascular damage -> increased vasc. permeability and activation of endothelial cells --> upregulation of adhesion molecules, cell adhesion and migration through the leaky endothelium and into the ECM
- these interactions stimulate cytokine, GF and CT cell production esp. fibroblasts
- result is uncontrolled and irreversible proliferation of CT, and thickening of vascular walls, narrowing of lumen
92
- systemic sclerosis is a multisystem disease, 70% of cases are LcSSc, 30% are DcSSc
- state 5 features of the more common LcSSc:
- (clue: what does CREST syndrome fts entail?)
Limited Cutaneous
- Raynaud's 1st feature
- thickened skin bound to underlying structures and fingers taper = 'sclerodactyly'
- characteristic facial appearance, with 'beaking' of nose, radial furrowing of lips and limitation of mouth opening 'microstomia'
- digital ulcers
- telangiectasia
- palpable subcutaneous nodules of calcium deposition in fingers 'calcinosis'
- CREST syndrome: calcinosis, raynauds, esophageal involvement, sclerodactyly, telangiectasia
93
- systemic sclerosis is a multisystem disease, 70% of cases are LcSSc, 30% are DcSSc
- state what organ involvement may include in the DcSSc:
Diffuse Cutaneous:
- skin changes develop more rapidly and are more widespread vs limited cutaneous
- early involvement of other organs:
- GI: dilation and atony in the oesophagus (heartburn and dysphagia), small intestine (bacterial, overgrowth and malabsorption) and colon (pseudo-obstruction)
- Renal involvement: acute and CKD, acute HT crisis is a complication
- Lung disease: fibrosis and pulmonary vascular disease resulting in pulmonary HT
- Myocardial fibrosis leads to arrythmias and conduction disturbances
94
Suggest 4 investigations for diagnosing Systemic Sclerosis:
| -specifically what will be + in limited cutaneous and what + in diffuse?
- blood count: normochromic, normocytic anaemia, (+/-raised ESR)
- urea and creatinine rise with renal disease
- serum autoantibodies (ANA often +). limited: anti-centromere abs. diffuse: scl-70 and anti-RNA polymerase I and III abs
- radiology: hand X-ray may show calcium deposits around fingers, +/-erosion/reabsorption of tufts of the distal phalanges
- Barium swallow: impaired motility
- High resolution CT can show fibrotic lung involvement
95
Systemic sclerosis is a multisystem disease, 70% of cases are LcSSc, 30% are DcSSc
-basis for management depends on organ involvement.. so what med helps with kidneys? What with pulmonary HT? and what for pulmonary fibrosis?
- symptomatic rx
- ACEi treats HT and prevents further kidney damage
- vasodilators, o2 and warfarin treat pulmonary HT
- Pulmonary fibrosis rx with cyclophosphamide or azathioprine + low dose oral prednisolone
96
PM (Polymyositis) is a rare muscle disorder of unknown aetiology, what happens? what is it called if the skin is involved? More common in who?
- there is inflammation and necrosis of skeletal muscle fibres
- dermatomyositis
- more common in women
97
PM (Polymyositis) is a rare muscle disorder where you get inflammation and necrosis of skeletal muscle fibres.
What pattern of muscle change is seen? Which muscles predominantly affected? Leading to difficulty with what kind of activities? Pharyngeal muscle involvement can lead to what sx?
- symmetrical progressive muscle weakness and wasting affecting the proximal muscles of the shoulder and pelvic girdle
- difficulty e.g. squatting, going upstairs, rising from a chair and raising arms above head
- pharyngeal, laryngeal and resp muscle involvement can -> dysphagia, dysphonia and resp failure
- NB: arthralgia is another clinical feature
98
What characteristic skin changes may be present in dermatomyositis?
-e.g. discolouration where? Scaly plaques where referred to as what? Finger pathology? and there is an increased risk of what?
- heliotrope (purple) discolouration of the eyelids and scaly erythematous plaques over knuckles (Gottron's papules)
- Raynaud's
- increased incidence of underlying malignancy
99
Suggest 3 investigations in the diagnosis of myositis or dermatomyositis:
- muscle biopsy for definitive diagnosis (shows inflammatory cell infiltration and necrosis of muscle cells)
- elevated serum muscle enzymes (CK, amniotransferases, aldolase)
- positive anti-JO antibodies
- EMG shows characteristic changes
- MRI can show areas of muscle inflammation
100
What is the treatment of choice in myositis/dermatomyositis:
| -if there is a disease relapse what other form of medication is given?
- oral prednisolone 0.5-1.0mg/kg for at least 1 month then gradually tapered down
- if there is disease relapse on tapering immunosuppression e.g. azathioprine, methotrexate, ciclosporin can be given
101
Sjogren's syndrome is characterised by:
- immunologically mediated destruction of what
- especially affects who?
- of epithelial exocrine glands, esp. lacrimal and salivary glands
- esp/ affects middle-aged women
102
Give 5 clinical features of Sjogren's syndrome:
clue: joints? hands? kidneys? lungs? vessels? increased which malignancy?
- dry eyes 'keratoconjunctivitis sicca',
- dry mouth 'xerostomia',
- arthritis,
- Raynaud's,
- renal tubular defects --> diabetes insipidus, renal tubular acidosis
- pulmonary fibrosis
- vasculitis
- increased incidence of non-Hodgkin's B cell lymphoma
103
suggest 2 diseases Sjogren's syndrome may occur in conjunction with?
-may occur with rheumatoid arthritis or SLE
104
Suggest 3 investigations in the diagnosis of Sjogren's Syndrome:
- Serum antibodies: ANA (80% +), anti-Ro (~70%+) and rheumatoid factor
- Labial gland biopsy shows lymphocyte infiltration and destruction of acinar tissue
- Positive Schirmer test: filter paper on inside lower eyelid, wetting <10mm in 5mins = +
