Rheumatology Flashcards
1
Q
when are anti-Ro antigens found?
A
Anti-Ro: Sjogren’s syndrome, SLE, congenital heart block
2
Q
when are anti-la antigens found?
A
Anti-La: Sjogren’s syndrome
3
Q
when are anti-jo 1 antigens found?
A
Anti-Jo 1: polymyositis
4
Q
when are Anti-SCL-70 found?
A
Anti-SCL-70: diffuse cutaneous systemic
sclerosis
5
Q
when are Anti-centromere found?
A
Anti-centromere: limited cutaneous systemic sclerosis
6
Q
what is gout caused by?
-why does this happen?
A
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium. It is caused by chronic hyperuricemia (uric acid > 450 μmol/l) mostly due to ↓ renal execretion of UA (90%)
7
Q
what causes exist for decreased excretion of uric acid?
A
Drugs - diuretics
CKD
Lead toxicity
8
Q
what causes exist for increased production of uric acid?
A
Myeloproliferative/lymphoproliferative disorder
Cytotoxic drugs
Severe psoriasis
9
Q
what is Lesch-Nyhan syndrome?
-what is this caused by?
-how is this inherited?
-what are the clinical features?
A
• Hypoxanthine-guanine phosphoribosyl transferase deficiency
• Inheritance = X-linked recessive
• Features: gout, renal failure, learning difficulties,
head-banging
10
Q
Tophaceous gout
-what is this assoc. with?
-how do affected joints appear?
-what is this due to?
-what is seen on X-Ray?
A
• Associated with renal impairment and prolonged diuretics use.
• Affected joints are hot swollen and knobby appearance.
• Due to deposition of Na+ urate in skin and joint.
• X-ray: punched out bony cyst
11
Q
what are drug causes of gout?
A
• Thiazides, furosemide
• Alcohol
• Cytotoxic agents
• Pyrazinamide
12
Q
what is the acute management of gout?
A
• NSAIDs
• Intra-articular steroid injection
• Colchicine has a slower onset of action. (main side-effect is diarrhea and ↑ INR with warfarin)
• If the patient is already taking allopurinol it should be continued
• Rasburicase: is a recombinant version of a urate oxidase enzyme given in acute setting, it allows
allopurinol to be commenced without worsening of symptoms. Only used when other Rx can not be given
13
Q
when is colchicine helpful in gout?
A
Colchicine is useful in patients with renal impairment who develop gout as NSAIDs are relatively contraindicated. BNF advises to ↓ the dose by up to 50% if creatinine clearance is less than 50 ml/min and to avoid if creatinine clearance is less than 10 ml/min.
14
Q
when should prednisolone not be used in gout?
A
diabetic patients
15
Q
when should allopurinol prophylaxis be started in gout?
-what is the initial dose?
-what is the aim level of uric acid?
-what should be used as ‘cover’?
A
Allopurinol prophylaxis
• Allopurinol should not be started until 2 weeks after an acute attack has settled.
• Initial dose of 100 mg od, with the dose titrated every few weeks to aim for a serum uric acid of
< 300 μmol/l
• NSAID or colchicine cover should be used when starting allopurinol
16
Q
when should allopurinol be considered?
A
• Recurrent attacks - the British Society for Rheumatology recommend ‘In uncomplicated gout uric acid lowering drug therapy should be started if a second attack, or further attacks occur within 1 year’
• Tophi
• Renal disease
• Uric acid renal stones
• Prophylaxis if on cytotoxics or diuretics
17
Q
what are lifestyle modifications for gout?
A
Lifestyle modifications
• ↓ alcohol intake and avoid during an acute attack
• Lose weight if obese
• Avoid food high in purines e.g. Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast
products
18
Q
what is pseudogout?
A
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate in the synovium
19
Q
what are the features of pseudogout?
A
Features
• Knee, wrist and shoulders most commonly affected
• X-ray: chondrocalcinosis (linear calcification of the articular cartilage)
• Joint aspiration: weakly-positively birefringent rhomboid shaped crystals
20
Q
what are risk factors for pseudogout?
A
Risk factors
• Hyperparathyroidism
• Hypothyroidism
• Hemochromatosis
• Acromegaly
• ↓ magnesium, ↓ phosphate
• Wilson’s disease
21
Q
what investigations are indicated in pseudogout?
A
Investigations:
• Transferrin saturation (may indicate hemochromatosis, a recognised cause of pseudogout)
• Aspiration of joint fluid, to exclude septic arthritis and show weakly-positively birefringent brick shaped crystals
22
Q
what is the treatment for pseudogout?
A
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
23
Q
what is the peak onset of rheumatoid arthritis?
-what is the female:male ratio?
-how prevalent is this in the UK
what HLA is this assoc with?
A
Epidemiology
• Peak onset = 30-50 years, although occurs in all age groups
• ♀:♂ ratio = 3:1
• Prevalence in UK = 1%
• Some ethnic differences e.g. High in native Americans
• Associated with HLA-DR4 (especially felty’s syndrome)
24
Q
what criteria is used for the diagnosis of rheumatoid arthritis?
A
American College of Rheumatology criteria
• Requires 4 of the following 7 criteria
• Sensitivity = 92%, specificity = 89%
1. Morning stiffness > 1 hr (for at least 6 weeks)
2. Soft-tissue swelling of 3 or more joints (for at least 6 weeks)
3. Swelling of PIP , MCP or wrist joints (for at least 6 weeks)
4. Symmetrical arthritis
5. Subcutaneous nodules
6. Rheumatoid factor positive
7. Radiographic evidence of erosions or periarticular osteopenia
25
what antibody should patients who have suspected rheumatoid arthritis and are rheumatoid factor negative be tested for?
Anti-cyclic citrullinated peptide antibody may be detectable up to 10 years before the development of rheumatoid arthritis. It may therefore play a key role in the future of rheumatoid arthritis, allowing early detection of patients suitable for aggressive anti-TNF therapy. It has sensitivity similar to rheumatoid factor (70-80%, see below) with a much higher specificity of 90-95%.
NICE recommends that patients with suspected rheumatoid arthritis who are rheumatoid factor negative should be tested for anti-CCP antibodies.
26
what are early x-ray findings in rheumatoid arthritis?
Early x-ray findings
• Loss of joint space (seen in both RA and osteoarthritis)
• Juxta-articular osteoporosis
• Soft-tissue swelling
27
what are late x-ray findings in rheumatoid arthritis?
Late x-ray findings
• Periarticular erosions (osteopenia and osteoporosis)
• Subluxation
28
what is key in the pathophysiology of rheumatoid arthritis?
Rheumatoid arthritis - TNF is key in pathophysiology
29
what are poor prognostic features of rheumatoid arthritis?
Poor prognostic features
• Rheumatoid factor positive
• Poor functional status at presentation
• HLA DR4
• X-ray: early erosions (in < 2 years)
• Extra articular features e.g. Nodules
• ♀sex
• Insidious onset
• Anti-CCP antibodies
30
what respiratory complications exist in rheumatoid arthritis?
• Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans,
methotrexate pneumonitis, pleurisy
31
what ocular complications exist in rheumatoid arthritis?
Ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration,
keratitis, steroid-induced cataracts, chloroquine retinopathy
32
what endocrine complication exists in RA?
• Osteoporosis
33
what cardiac complications exist in RA?
ISCHEMIC heart disease: RA carries a similar risk to T2DM
34
what systemic complications exist in RA?
Increased risk of infections
35
what psych. complications exist in RA?
• Depression
36
what syndromes are assoc with RA?
• Felty's syndrome (RA + splenomegaly + low white cell count)
• Amyloidosis
37
what organism predisposes patients to RA?
Proteus mirabilis is a (G-ve rod), causes UTI → predisposes susceptible patients to RA
38
what is the general treatment combination for RA?
In 2018 NICE updated their rheumatoid arthritis guidelines. They now recommend DMARD monotherapy +/- a short-course of bridging prednisolone. In the past dual DMARD therapy was advocated as the initial step.
Flares
flares of RA are often managed with corticosteroids - oral or intramuscular
39
Methotrexate:
-what needs to be monitored?
-what side effect does this cause?
Methotrexate is the most widely used DMARD. Monitoring FBC & LFTs is essential due to the risk of myelosuppression and liver cirrhosis. Other important side-effects include pneumonitis
40
which 2 DMARDs can be used in pregnancy?
• Sulfasalazine
• Hydroxychloroquine
Azathiopurine can be used if sulfasalazine and hydroxychloroquine are not controlling
41
what RA therapy is absolutely contraindicated in pregnancy?
Methotrexate and NSAIDs are absolutely contraindicated
42
when are TNF-inhibitors indicated in RA?
The current indication for a TNF-inhibitor is an inadequate response to at least two DMARDs including methotrexate
43
anti-TNF: Etanercept
-administration
-adverse effect
Etanercept: subcutaneous administration
can cause demyelination, risks include reactivation of tuberculosis
44
anti-TNF: Infliximab
-administration
-adverse effect
Infliximab: intravenous administration, risks include reactivation of
tuberculosis
45
anti-TNF: adalimumab
-administration
Adalimumab: subcutaneous administration
46
when should anti-TNF meds be stopped prior to surgery?
Stop 2-4 weeks before any major surgery
47
what is rituximab:
-how is this given?
-what reaction is common?
• Anti-CD20 monoclonal antibody, results in B-cell depletion
• Two 1g intravenous infusions are given two weeks apart
• Infusion reactions are common
48
what is abatacept?
-how is this given?
• Fusion protein that modulates a key signal required for activation of T lymphocytes
• Leads to ↓ T-cell proliferation and cytokine production
• Given as an infusion
49
• Pulmonary fibrosis
• Pleural effusion
• Pulmonary nodules
• Bronchiolitis obliterans
• Complications of drug therapy e.g. Methotrexate pneumonitis
• Pleurisy
• Caplan's syndrome - massive fibrotic nodules with occupational coal dust exposure
• Infection (possibly atypical) secondary to immunosuppression
are all complications of
Rheumatoid arthritis
50
what is adult still's disease?
Adult Still's Disease: is a form of rheumatoid arthritis typically affects 16-35 year olds
51
what are clinical features of adult still's disease?
-what blood tests are raised?
-RF? ANA?
• Arthralgia
• Fever (noticeable at afternoon and evening)
• Elevated serum ferritin
• Rash: salmon-pink, maculopapular, pruritic
• Pyrexia
• Lymphadenopathy
• RF and ANA negative (but ANA 25% positive). ↑ ESR and CRP
• Leukocytosis and thrombocytosis
52
what is the management of adult still's disease?
Management:
• NSAIDs
• Steroids
• Methotrexate
53
what is mandatory in all patients with a hot swollen joint?
Joint aspiration is mandatory in all patients with a hot, swollen joint to rule out septic arthritis. If
this was excluded then intra-articular or system steroid therapy may be considered
54
what is the most common organisms overall and in young adults who are sexually adults that causes septic joint?
• Most common organism overall is Staphylococcus aureus
• In young adults who are sexually active Neisseria gonorrhea should also be considered
• WBC > 50 x 109/L or > 75% of baseline – neutrophils
55
what is the management for septic joint?
• Synovial fluid should be obtained before starting treatment
• Intravenous antibiotics which cover gram-positive cocci are indicated. The BNF currently
recommends flucloxacillin + fusidic acid or clindamycin if penicillin allergic
• Antibiotic treatment is normally be given for several weeks (BNF states 6-12 weeks)
• Needle aspiration should be used to decompress the joint
• Surgical drainage may be needed if frequent needle aspiration is required
56
what is the most common site of hand osteoarthritis?
trapeziometacarpal joint (base of thumb) is the most common site of hand osteoarthritis
57
what is the management of osteoarthritis?
-conservative/non-pharmacological
-pharmacalogical 1st line and 2nd line
`
• All patients should be offered help with weight loss, given advice about local muscle
strengthening exercises and general aerobic fitness
• Paracetamol and topical NSAIDs are first-line analgesics. Topical NSAIDs are indicated only
for OA of the knee or hand
• Second-line treatment is oral NSAIDs/cox-2 inhibitors, opioids, capsaicin cream and intra-
articular corticosteroids. A proton pump inhibitor should be co-prescribed with either drug.
These drugs should be avoided if the patient takes aspirin
• Non-pharmacological treatment options include supports and braces, tens and shock absorbing
insoles or shoes
• If conservative methods fail then refer for consideration of joint replacement
58
what is reactive arthritis/reiter's syndrome?
Reactive Arthritis is one of the HLA-B27 associated seronegative spondyloarthropathies. It encompasses Reiter's syndrome, a term which described a classic triad of urethritis, conjunctivitis and arthritis following a dysenteric illness
The American College of Rheumatology now define reactive arthritis as an episode of peripheral arthritis lasting for greater than 1 month associated with urethritis/cervicitis or diarrhea
59
what are the clinical features of reiter's syndrome?
• Typically develops within 4 weeks of initial infection - symptoms generally last around 4-6
months
• Arthritis is typically an asymmetrical oligoarthritis of lower limbs
• May present as monoarthritis e.g. Knee
• Symptoms of urethritis
• Eye: conjunctivitis (seen in 50%), anterior uveitis
• Skin: circinate balanitis (painless vesicles on the coronal margin of the prepuce), keratoderma
blenorrhagica (waxy yellow/brown papules on palms and soles)
60
what percentage of patients with reactive arthritis have recurrent episodes? what percentage develop chronic disease?
Around 25% (15-50%) have recurrent episodes whilst 10% (15-30%) develop chronic disease.
61
what percentage of HLA B27 positive patients will develop ankylosing spondylitis?
Ankylosing spondylitis develop in upto 50% of HLA B27 +ve patients
62
what two forms of reactive arthritis exist?
• Post-STI form much more common in men (e.g. 10:1)
• Post-dysenteric form equal sex incidence with better prognosis
63
what organisms are responsible for post-dysenteric reactive arthritis?
Organisms often responsible for post-dysenteric form
• Shigella flexneri
• Salmonella typhimurium
• Salmonella enteritidis
• Yersinia enterocolitica
• Campylobacter
64
what organism is responsible for post-STI reactive arthritis?
Organisms often responsible for post-STI form
• Chlamydia trachomatis
65
what is the 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
66
what is palindromic arthritis?
Rare type of recurrent inflammatory arthritis that causes sudden inflammation in one or several joints (pain, swelling and erythema) followed by complete recovery with no permanent damage
67
what does palindromic arthrtis affect? how long does this last?
• Affects articular or periarticular areas, any joint could be affected but mostly large joints
• Lasts < 72 hours before recovering completely
68
what age of patient can be affected by palindromic arthritis?
♂=♀ - age 20-50 years.
69
what may palindromic arthritis progress to? what antibodies are present?
• May progress to RA: incidence of RA may ↑ when RF is present
• Anti-CCP and antikeratin antibodies (AKA) are present in a high proportion of patients
70
what is recent-onset arthritis?
Recent-Onset Arthritis can be due to parvovirus-induced arthritis
71
what is a risk factor for recent-onset arthritis?
• Exposure to children with recent febrile illness is known risk
72
who does recent-onset arthritis affect?
Affects ♀>♂ - mostly women with contact to children (at home or at work)
73
what joints are affected in recent-onset arthritis?
Small hands joints, wrists, elbows, hips, knees, and feet are each affected in > 50% of cases.
74
what antibody is strong evidence of parvovirus being a cause of recent-onset arthritis?
Detection of IgM antibody, produced only in early disease, is a marker of recent infection and
therefore strong evidence in favor of parvovirus being the cause of recent-onset arthritis.
75
what HLA is ankylosing spondylitis assoc. with?
-who does this present in?
-how is this inherited?
-is HLA-? of use in making diagnosis?
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathies. It typically presents in ♂s (sex ratio 5:1) aged 20-30 years old. It has polygenic inheritance.
HLA-B27 is of little use in making the diagnosis as it is positive in:
• 90% of patients with ankylosing spondylitis
• 10% of normal patients
76
what are the clinical features of ankylosing spondylitis?
• Typically a young man who presents with lower back pain and stiffness
• Stiffness is usually worse in morning and improves with activity
• The patient may experience pain at night which improves on getting up
• Peripheral arthritis (25%, more common if ♀)
77
what are the 'A's of ankylosing spondylitis?
• Apical fibrosis (CXR)
• Anterior uveitis
• Aortic regurgitation
• Achilles tendonitis
• A V node block
• Amyloidosis
• And cauda equina syndrome
78
what is seen on examination in 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
↓ Lateral flexion of the lumbar spine is one of the earliest signs of ankylosing spondylitis. There tends to be a loss of lumbar lordosis and an accentuated thoracic kyphosis in patients with ankylosing spondylitis
79
what is the most useful investigation for diagnosis and monitoring of ank spond?
X-ray of the sacro-iliac joints is the most useful investigation for diagnosis and monitoring, but changes may not be seen for many years after the onset of symptoms
80
what is seen on x-rays sacroiliac joints and spine in ank spond?
X-rays are often normal early in disease, later changes include:
• Sacroilitis: subchondral erosions, sclerosis
• Squaring of lumbar vertebrae
• 'Bamboo spine' (late & uncommon)
81
what is seen in CXR in ank spond?
- CXR: apical fibrosis
82
what may be seen on spirometry in a patient with ank spond?
Spirometry may show a restrictive defect due to a combination of pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints
83
what is the management of ankylosing spondylitis?
• NSAIDs
• Physiotherapy
• Sulphasalazine may be useful if there is peripheral joint involvement - doesn't improve spinal
mobility
• TNF-α blockers such as etanercept and adalimumab are increasingly used. This approach for
severe ankylosing spondylitis was supported by NICE in 2008
84
what are common features of seronegative spondyloarthropathies?
• Associated with HLA-B27
• Rheumatoid factor negative - hence 'seronegative'
• Peripheral arthritis, usually asymmetrical
• Sacroilitis
• Enthesopathy: e.g. Achilles tendonitis, plantar fasciitis
• Extra-articular manifestations: uveitis, pulmonary fibrosis (upper zone), amyloidosis, aortic
regurgitation
85
list four seronegative spondyloarthropathies
• Ankylosing spondylitis
• Psoriatic arthritis
• Reiter's syndrome (including reactive arthritis)
• Enteropathic arthritis (associated with IBD)
86
what is pseudoxanthoma elasticum?
Pseudoxanthoma Elasticum is an inherited condition (usually autosomal recessive) characterized by an abnormality in elastic fibers
87
what are the features of pseudoxanthoma elasticum?
Features
• Retinal angioid streaks
• 'Plucked chicken skin' appearance - small yellow papules on the neck, antecubital fossa and
axillae
• Cardiac: mitral valve prolapse, ↑ risk of ischemic heart disease
• Gastrointestinal hemorrhage
88
low levels of ? have been shown to be assoc with increased risk of developing SLE
Low levels of C4a and C4b have been shown to be associated with ↑ risk of developing systemic lupus erythematous
89
SLE:
-females or males?
-age of onset?
• Much more common in ♀s (F:M =9:1)
• More common in Afro-Caribbeans and Asian communities
• Onset is usually 20-40 years
• Incidence has risen substantially
during the past 50 years (3 fold using American College of Rheumatology criteria)
90
what is the pathophysiology of SLE?
-what HLA is assoc.?
Pathophysiology
• Autoimmune disease
• Associated with HLA B8, DR2,
DR3
• Thought to be caused by immune system dysregulation leading to immune complex formation
• Immune complex deposition can affect any organ; skin, joints, kidneys and brain most
commonly affected
91
SLE serology:
-what is 99% sensitive?
-what is highly specific?
-what is most specific?
-what is positive in drug induced lupus?
-what is a predisposing factor?
• ANA positive (99% SENSITIVE)
• 20% are rheumatoid factor positive
• Anti-dsDNA: HIGHLY SPECIFIC (> 99%), but less sensitive (70%)
• Anti-Smith: MOST SPECIFIC (> 99%), sensitivity (30%)
• Antihistone positive in drug indiced lupus
• Congenital ↓ C4 is a predisposing factor for SLE
92
what is used in disease monitoring of SLE?
Monitoring
• ESR: during active disease the CRP is characteristically 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) ↓ C4 is early marker for disease activity
• Anti-DsDNA titers: used for disease monitoring disease activity (but not present in all patients)
93
does SLE become better or worse during pregnancy?
SLE and Pregnancy: Unlike many autoimmune diseases systemic lupus erythematous (SLE) often becomes worse during pregnancy and the puerperium
94
what is the risk of SLE in pregnancy?
• Risk of maternal autoantibodies crossing placenta
• Leads to condition termed neonatal lupus erythematous
• Neonatal complications include congenital heart block, it is strongly associated with anti-Ro
(SSA) antibodies
95
what is drug induced lupus?
Drug-induced Lupus not all the typical features of systemic lupus erythematosus are seen, with renal and nervous system involvement being unusual. It usually resolves on stopping the drug
96
what are the features of drug induced lupus?
-what is the serology found?
Features
• Arthralgia
• Myalgia
• Skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
• ANA positive in 100%, dsDNA negative
• Anti-Ro, anti-Smith positive in around 5%
97
Causes of ?
• Anti-epileptics: phenytoin
• Chlorpromazine
• Hydralazine
• Isoniazid
• Minocycline
• Procainamide
drug induced lupus
98
what is discoid lupus?
-what is this characterised by and what is the aetiology?
Discoid Lupus Erythematous is a benign disorder generally seen in younger ♀s. It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases). Discoid lupus erythematous is characterized by follicular keratin plugs and is thought to be autoimmune in etiology
99
what are the features of discoid lupus?
• Erythematous, raised rash, sometimes scaly
• May be photosensitive
• More common on face, neck, ears and scalp
• Lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation
100
what is the management of discoid lupus?
Management
• Topical steroid cream
• Oral antimalarials may be used second-line e.g. Hydroxychloroquine
• Avoid sun exposure
101
what kind of vasculitis is temporal arteritis?
-what does histology show?
Temporal Arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR). Histology shows changes which characteristically 'skips' certain sections of affected artery whilst damaging others
102
what are the clinical features of temporal arteritis?
• Typically patient > 60 years old
• Usually rapid onset (e.g. < 1 month)
• Headache (found in 85%)
• Jaw claudication (65%)
• Tender, palpable temporal artery
• Features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
• Also lethargy, depression, low-grade fever, anorexia, night sweats
• Associated with sudden blindness due to the involvement of anterior ischemic optic neuropathy
103
what are the investigations for temporal arteritis?
• 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 CK and EMG normal
• ↓ CD8+ T cells
104
what is the treatment for temporal arteritis?
• Should be started immediately with high dose steroids (e.g. prednisolone 1mg/kg/day) to ↓ the chance of visual loss. there should be a dramatic response, if not the diagnosis should be reconsidered
• Urgent ophthalmology review. Patients with visual symptoms should be seen the same-day by an ophthalmologist. Visual damage is often irreversible
105
what is takayasu disease?
Takayasu disease (TD): is a continuous or patchy granulomatous inflammatory process involving macrophages, lymphocytes, and multinucleated giant cells which cause progressive occlusive disease of the aorta and its branches.
106
is Takayasu arteritis common? male or female? mean age onset?
• Very rare in the Western world with an annual incidence of between 2 and 3 per million.
• Approximately 80% of patients are women, and the mean age of onset is 30 years.
107
what is the presentation of takayasu arteritis?
-neurological features
-vascular features
-cardiac features
-renal manifestation
Presentation may be with constitutional symptoms such as fever, malaise, and weight loss;
neurological symptoms such as transient ischemic attacks; or vascular symptoms such as
claudication.
• Cardiac features include angina, heart failure, and aortic regurgitation.
• Renal manifestations may include mesangial proliferative glomerulonephritis.
108
what is the management of takayasu arteritis?
• Corticosteroids with the addition of steroid sparing second agents such as methotrexate or
azathioprine are the mainstay of therapy.
• With good care, 15-year survival rates approach 90%.
109
what is polyarteritis nodosa?
-who is this more common in?
-what infection is assoc?
Polyarteritis Nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis B infection
110
what are the features of polyarteritis nodosa?
-what antibodies are found?
• Fever, malaise, arthralgia
• Hypertension
• Mononeuritis multiplex, sensorimotor polyneuropathy
• Hematuria, renal failure
• Testicular pain
• Abdominal pain (e.g. From mesenteric ischemia)
• Perinuclear-antineutrophil cytoplasmic antibodies (pANCA) are found in around 20% of
patients with 'classic' PAN
111
what is the diagnostic criteria for polyarteritis nodosa?
Diagnostic Criteria: 3 of the 10 following signs known as the 1990 ACR (American College of Rheumatology):
1. Weight loss ≥ to 4.5 kg.
2. Livedo reticularis
3. Testicular pain or tenderness. (Occasionally, a site biopsied for diagnosis).
4. Muscle pain, weakness, or leg tenderness.
5. Nerve disease (either single or multiple).
6. Diastolic BP > 90mmHg
7. ↑ Kidney function tests (BUN and Creatinine)
8. Hepatitis B positive (surface antigen or antibody).
9. Abnormal arteriogram (angiogram)
10. Biopsy of small/medium size artery (typically inflamed arteries).
112
The combination of pulmonary and renal involvement combined with a history of chronic sinusitis points towards a diagnosis of ?
Granulomatosis with polyangiitis (Wegener's granulomatosis)
113
what is granulomatosis with polyangitis?
is an autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.
114
what are the features of granulomatosis with polyangitis?
• Upper respiratory tract: epistaxis, sinusitis, nasal crusting
• Lower respiratory tract: dyspnea, hemoptysis
• Glomerulonephritis ('pauci-immune', 80% of patients)
• Saddle-shape nose deformity
• Also: vasculitic rash, eye involvement (e.g. Proptosis), cranial
nerve lesions
115
what investigations are used in granulomatosis with polyangitis?
Investigations
• cANCA positive in > 90%, pANCA positive in 25%
• Chest x-ray: wide variety of presentations, including cavitating lesions
116
what is the management of granulomatosis with polyangitis?
• Steroids • Plasma exchange
• Cyclophosphamide (90% response) • Median survival = 8-9 years
117
Asthma + eosinophilia + nerve lesion = ?
Churg-Strauss Syndrome
118
What is churg strauss?
Churg-Strauss Syndrome is an ANCA associated small-medium vessel vasculitis
119
what are the features of churg strauss syndrome?
Features
• Asthma and sometimes pulmonary esinophilic infiltrate
• Blood eosinophilia (e.g. > 10%)
• Paranasal sinusitis
• Mononeuritis multiplex
• pANCA positive in 60% - Anti myeloperoxidase antibody
120
what may precipitate churg-strauss syndrome?
Leukotriene receptor antagonists may precipitate the disease
121
What is henoch schonlein purpura?
-what mediates disease?
-what is the clinical presentation?
Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Buerger's disease). HSP is usually seen in children following an infection.
122
what is henoch-schonlein purpura characterised by?
Characterized by (Features):
• Palpable purpuric rash (with localized edema) over buttocks, extensor surfaces of arms and legs
• Abdominal pain, non-bloody diarrhea.
• Polyarthritis
• Features of IgA nephropathy may occur e.g. Hematuria, renal failure
123
what is thrombangiitis obliterans?
Thrombangiitis Obliterans (Buerger’s disease) is a disease of small and medium- sized arteries and veins resulting in inflammation and ulceration
124
what is the presentation of thrombangiitis obliterans?
• No excessive atheroma
• Does not involve the coronary arteries like atherosclerosis.
• Patients present with symptoms of arterial ischemia.
• Migratory phlebitis in the superficial vein is present in 40% of cases.
• The disease progresses proximally, resulting in gangrene of the digits.
125
who does thombangiitis obliterans occur in?
• Occurs mainly in cigarette smokers; it has not been documented in non-smokers.
126
what is the diagnosis of thrombangiitis obliterans?
Diagnosis is usually clinical. Arteriogram is also of benefit and will show occlusion of distal
arteries of the hands and feet.
127
what is the treatment of thrombangiitis obliterans?
• Treatment is supportive and patients should stop smoking.
128
what is behcets syndrome?
Behcet's Syndrome is a complex multisystem disorder associated with presumed autoimmune mediated inflammation of the arteries and veins. The precise etiology has yet to be elucidated however.
129
Epidemiology behcets:
-where in the world?
-men or women?
-age distribution?
-serology?
• More common in the eastern Mediterranean (e.g. Turkey)
• More common in men (complicated gender distribution which varies according to country.
Overall, Behcet's is considered to be more common and more severe in men)
• Tends to affect young adults (e.g. 20 - 40 years old)
• Associated with HLA B51 and MICA6 allele
• C. 30% of patients have a positive family history
130
what is the classic triad of symptoms in behcets?
-what other features exist?
Features
The classic triad of symptoms is oral ulcers, genital ulcers and anterior uveitis
• Thrombophlebitis
• Arthritis
• Neurological involvement (e.g. Aseptic
meningitis)
• GI: abdo pain, diarrhea, colitis
• Erythema nodosum, DVT
Other ocular problems seen include retinal vasculitis, iridocyclitis and chorioretinitis
131
what is the diagnosis of behcets?
• No definitive test
• Diagnosis based on clinical findings
• Positive pathergy test is suggestive (puncture site following needle prick becomes inflamed
with small pustule forming)
132
what is the management of behcets?
Mangement:
• Steroids
• Azathioprine
133
? syndrome: (paradoxically) prolonged APTT + low platelets
Antiphospholipid syndrome: (paradoxically) prolonged APTT + low platelets
134
what is antiphospholipid syndrome?
Antiphospholipid Syndrome (Hughes syndrome) is an acquired disorder characterized by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or secondary to other conditions, most commonly systemic lupus erythematosus (SLE)
135
what coagulation test is prolonged in anti-phopholipid syndrome?
A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade.
136
what are the features of antiphospholipid syndrome?
Features
• Venous/arterial thrombosis
• Recurrent fetal loss
• Livedo reticularis
• Thrombocytopenia
• Prolonged APTT
• Other features: pre-eclampsia, pulmonary hypertension
137
what are other assoc. with antiphospholipid syndrome other than SLE
• Other autoimmune disorders
• Lymphoproliferative disorders
• Phenothiazines (rare)
138
what is the general management of antiphospholipid syndrome?
Management - based on BCSH guidelines
• Initial venous thromboembolic events: evidence currently supports use of warfarin with a target
INR of 2-3 for 6 months
• Recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin
then ↑ target INR to 3-4
• Arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
139
what is the management of antiphospholipid syndrome in pregnancy?
Management
• Low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing
• Low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually
discontinued at 34 weeks gestation
• These interventions ↑ the live birth rate seven-fold
140
what is the pathophysiology of polymyalgia rheumatica?
Pathophysiology
• Overlaps with temporal arteritis
• Histology shows vasculitis with giant cells, characteristically 'skips' certain sections of affected
artery whilst damaging others
• Muscle bed arteries affected most in polymyalgia rheumatica
141
what are the features of PMR?
Features
• Typically patient > 60 years old
• Usually rapid onset (e.g. < 1 month)
• Aching, morning stiffness in proximal limb muscles (not weakness)
• Also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
142
what are the investigations of PMR?
Investigations
• ESR > 40 mm/hr
• Note CK and EMG normal
• ↓ CD8+ T cells
143
what is the treatment of PMR?
Treatment
• Prednisolone e.g. 15mg/od - dramatic response
144
Anti-ribonuclear protein (anti-RNP) = ?
Anti-ribonuclear protein (anti-RNP) = mixed connective tissue disease
Features of SLE, systemic sclerosis and polymyositis
145
what is cryoglobulinaemia?
Cryoglobulinemia: immunoglobulins which undergo reversible precipitation at 4°C, dissolve when warmed to 37°C. One third of cases are idiopathic
146
what are the three types of cryoglobulinaemia?
• Type I (25%): monoclonal
• Type II (25%): mixed monoclonal and polyclonal: usually with RF
• Type III (50%): polyclonal: usually with RF
147
Type 1 cryoglobulinaemia:
-what antibodies
-what conditions are assoc
Type I
• Monoclonal - IgG or IgM
• Associations: multiple myeloma, Waldenström macroglobulinemia
148
Type 2 cryoglobulinaemia:
-monoclonal or polyclonal?
-assoc with what conditions?
Type II
• Mixed monoclonal and polyclonal: usually with RF
• Associations: hepatitis C, RA, Sjogren's, lymphoma
149
Type 3 cryoglobulinaemia:
-monoclonal or polyclonal?
-what is this assoc. with?
-
Type III
• Polyclonal: usually with RF
• Associations: RA, Sjogren's Symptoms (if present in high concentrations)
150
what are the symptoms of cryoglobulinaemia?
• Raynaud's only seen in type I
• Cutaneous: vascular purpura, distal ulceration, ulceration
• Arthralgia
• Renal involvement (diffuse mesangiocapillary glomerulonephritis)
151
what are the tests for cryoglobulinaemia?
Tests
• Low complement (esp. C4)
• High ESR
152
what is the treatment of cryoglobulinaemia?
Treatment
• Immunosuppression • Plasmapheresis
153
Raynauds phenomena:
-what are the two distinctions?
-how does this typically present?
Raynaud's Phenomena may be primary (Raynaud's disease) or secondary (Raynaud's phenomenon). Raynaud's disease typically presents in young women (e.g. 30 years old) with symmetrical attacks
154
what features suggest underlying connective tissue disease with raynauds phenomena?
• Onset after 40 years
• Unilateral symptoms
• Rashes
• Presence of autoantibodies
• Digital ulcers, calcinosis
• Very rarely: chilblains (pernio) are itchy, painful
purple swellings which occur on the fingers and
toes after exposure to the cold. They are
occasionally associated with underlying connective tissue disease but this is rare
• Recurrent miscarriages: This indicates SLE or antiphospholipid syndrome.
155
what are secondary causes of raynauds phenomena?
• Connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE with bilateral symptoms
• Leukemia
• Type I cryoglobulinemia, cold agglutinins
• Use of vibrating tools
• Drugs: oral contraceptive pill, ergot
• Cervical rib
156
what is the management of raynauds phenomena?
Management
• Calcium channel blockers
• IV prostacyclin infusions
157
what is morphea?
Morphea is a form of localized scleroderma that may be circumscribed or generalized.
158
describe the lesions in morphea?
• In circumscribed morphea, there may be just one or two lesions with no generalized spread.
• Changes often begin with small, violaceous, or erythematous skin lesions, which enlarge and
progress to firm hidebound skin with a variable degree of hypo- or hyperpigmentation.
• Lesions eventually settle into a waxy, white appearance with subsequent atrophy.
• Lesions vary in diameter between 1 and 10 cm.
159
how long does morphea last?
• Condition generally resolves within 3-5 years, although sometimes a patch may persist for over
25 years.
160
are autoantibodies found in morphea?
Auto-antibodies such as anti-nuclear antibody (ANA) are only rarely positive in localized forms
of scleroderma, as against systemic subtypes where a positive ANA is one of the hallmarks of the disease.
161
in psoriatic arthropathy what percentage of patient who have skin lesions will develop arthropathy?
Psoriatic Arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions. Around 10% of patients with skin lesions develop an arthropathy with ♂s and ♀s being equally affected
162
what are the different types of psoriatic arthropathy?
• Rheumatoid-like polyarthritis: (30-40%, most common type)
• Asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
• Sacroilitis
• DIP joint disease = arthropathy (10%)
• Arthritis mutilans (severe deformity fingers/hand, 'telescoping fingers')
163
what is the management of psoriatic arthropathy?
Management
• Treat as rheumatoid arthritis, but better prognosis
164
what are the different causes of dactylitis?
Causes include:
• Spondyloarthritis: e.g. Psoriatic and reactive arthritis
• Sickle-cell disease
• Other rare causes include tuberculosis, sarcoid and syphilis
165
what is systemic sclerosis characterised by?
Systemic Sclerosis is a condition of unknown etiology characterized by hardened, sclerotic skin and other connective tissues. It is four times more common in ♀s
166
what are the three different patterns of systemic sclerosis?
1. Limited cutaneous systemic sclerosis:
2. Diffuse cutaneous systemic sclerosis:
3. Scleroderma (without internal organ involvement):
167
Limited cutaneous systemic sclerosis:
-what may be the first sign?
-where does scleroderma affect?
-what antibodies are assoc?
-what is CREST syndrome
• Raynaud's may be first sign
• Scleroderma affects face and distal limbs predominately
• Associated with anti-centromere antibodies
• CREST syndrome is a subtype of limited cutaneous systemic sclerosis: Calcinosis, Raynaud's
phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia. In CREST MALABSORPTION can develop secondary to bacterial overgrowth of the sclerosed small intestine. Unfortunately pulmonary hypertension is one of the more common late complications seen in such patients.
168
Diffuse cutaneous sclerosis:
-where does scleroderma affect?
-what antibodies are assoc?
-what clinical features are seen?
• Scleroderma affects trunk and proximal limbs predominately
• Associated with SCL-70 antibodies
• Hypertension, lung fibrosis and renal involvement seen
• Poor prognosis
169
scleroderma:
-what is this?
-how may this manifest?
• Tightening and fibrosis of skin
• May be manifest as plaques (morphoea) or linear
170
what percentage of patients with systemic sclerosis have positive ANA/RF?
• ANA positive in 90%
• RF positive in 30%
171
what is the management of systemic sclerosis?
• Topical treatment for skin changes do not alter the disease course, but may improve pain and ulceration.
o NSAIDs
o Limited benefit from steroids
o Episodes of Raynaud's sometimes respond to nifedipine or other calcium channel
blockers. Dual endothelin-receptor antagonist (bosentan) may be beneficial.
o Severe digital ulceration may respond to prostacyclin analogue iloprost
o The skin tightness may be treated systemically with methotrexate and ciclosporin
• Scleroderma renal crisis: benefit fro ACE-I to control BP and delay progression to CRF
• Active alveolitis is often treated with pulses of cyclophosphamide, often together with a small
dose of steroids. The benefit of this intervention is modest
172
what is dermatomyositis?
• Inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions
• May be idiopathic or associated with connective tissue disorders or underlying malignancy (found in 20-25% - more if old patient)
• Polymyositis is a variant of the disease where skin manifestations are not prominent
173
describe the skin features in dermatomyositis?
• Photosensitive
• Macular rash over back and shoulder
• Heliotrope rash over cheek
• Gottron's papules - roughened red papules over extensor
surfaces of fingers
• Nail fold capillary dilatation
174
describe the non-skin features in dermatomyositis?
• Proximal muscle weakness +/- tenderness
• Raynaud's
• Respiratory muscle weakness
• Interstitial lung disease: e.g. Fibrosing alveolitis or organizing pneumonia
• Dysphagia, dysphonia
175
what investigations are indicated for dermatomyositis?
• ↑CK
• EMG
• Muscle biopsy
• Anti-jo-1 antibodies are not commonly seen in dermatomyositis - they are more common in
polymyositis where they are seen in a pattern of disease associated with lung involvement,
raynaud's and fever
• ANA positive in 60%
• anti-Mi-2 antibodies are highly specific for dermatomyositis, but are only seen in around 25% of patients
• Screen for malignancy by U/S abdomen + pelvis-(♀)/+PSA(♂) – CT chest might be needed.
176
what is the management of dermatomyositis?
Management:
• Prednisolone
177
How is familial mediterranean fever inherited?
at what age does this occur? who is this more common in?
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder which typically presents by the second decade (90% have their first attacks before the age of 18 years). It is more common in people of Turkish, Armenian and Arabic descent
178
what are the features of familial mediterranean fever?
Features - attacks typically last 1-3 days
• Pyrexia (on-off)
• Constipation/Diarrhea may occur with or after the fever
• Abdominal pain (due to peritonitis) – many times appendectomy scar is seen due to multiple
admissions due to abdominal pain
• Pleurisy
• Pericarditis
• Arthritis
• Nephrotic syndrome due to renal amyloidosis (that might even need transplantation)
• Erysipeloid rash on lower limbs
179
what is the management of familial mediterranean fever?
Management
• Attacks are self-limiting, and require analgesia
• Colchicine may ↓ attack frequency.
180
what is relapsing polychondritis?
Relapsing Polychondritis (RP) is an inflammatory condition that involves cartilaginous structures, predominantly those of the pinna, nasal septum and larynx.
181
Does relapsing polychondritis affect men or women? at what age?
♂=♀ - Can occur at any age, but mostly in the fifth decade (40s of age).
182
what are the symptoms of relapsing polychondritis?
General symptoms include intermittent fever and weight loss, but other more specific symptoms
include sudden onset of ear pain with an inability to sleep on the affected side, diminished hearing, monoarthritis or polyarthritis, back pain, myalgias, mild epistaxis, saddle-shaped nose, redness of the eyes indicative of conjunctivitis, episcleritis and/or scleritis, hoarseness of the voice and recurrent respiratory infections.
183
what is the diagnosis of relapsing polychondritis?
No specific diagnostic laboratory findings in patients with RP but the non-specific indicators of inflammation (ESR, CRP) are often elevated.
184
what is the management of relapsing polychondritis?
• No specific therapy exists for RP but the goal of treatment is to control the patient symptoms and to preserve the integrity of the affected cartilaginous structures.
• The mainstay of treatment is systemic corticosteroids. It is important in these patients to investigate for the presence of other concurrent autoimmune diseases
185
what is sjogrens?
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
186
how may sjogrens syndrome occur?
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.
187
does sjogrens affect males or females more? what does this increase the risk of?
Sjogren's syndrome is much more common in ♀s (ratio 9:1). There is a marked ↑ risk of lymphoid malignancy (40-60 folds)
188
what are the features of sjogrens?
• Dry eyes: keratoconjunctivitis sicca
• Dry mouth
• Vaginal dryness
• Arthralgia
• Raynaud's, myalgia
• Sensory polyneuropathy
• Renal tubular acidosis (usually
subclinical)
189
what serology is used in sjogren syndrome?
• Rheumatoid factor (RF) positive in nearly 100% 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
Also: hypergammaglobulinemia, low C4
190
what test is used in sjogrens?
Schirmer's test: filter paper near conjunctival sac to measure tear formation
191
what is seen on histology in sjogrens?
Histology: focal lymphocytic infiltration → (marked ↑ risk of lymphoid malignancy)
192
what is the management of sjogrens?
Management
• Artificial saliva and tears
• Pilocarpine may stimulate saliva production
193
avascular necrosis of femoral head:
what is this?
Avascular necrosis (AVN) may be defined as death of bone tissue secondary to loss of the blood supply. This leads to bone destruction and loss of joint function. It most commonly affects the epiphysis of long bones such as the femur.
194
avascular necrosis of femoral head:
what is this caused by?
Causes
long-term steroid use
chemotherapy
alcohol excess
trauma
195
what are the features of AVN femoral head?
Features
initially asymptomatic
pain in the affected joint
196
what is the investigation of choice for AVN?
Investigation
plain x-ray findings may be normal initially. Osteopenia and microfractures may be seen early on. Collapse of the articular surface may result in the crescent sign
MRI is the investigation of choice. It is more sensitive than radionuclide bone scanning
197
what is the management of AVN femoral head?
Management
joint replacement may be necessary
198
what is the most common target of cANCA
cANCA
most common target serine proteinase 3 (PR3)
199
what is the most common target of pANCA
pANCA
most common target is myeloperoxidase (MPO)
200
what is mccardles disease?
-how is this inherited
-what is it caused by and what is the pathophysiology?
Overview
autosomal recessive type V glycogen storage disease
caused by myophosphorylase deficiency
this causes decreased muscle glycogenolysis
201
what are the features of mccardles disease?
Features
muscle pain and stiffness following exercise
muscle cramps
myoglobinuria
low lactate levels during exercise
202
Leflunomide:
-what are the rules in pregnancy?
pregnancy - the BNF advises: 'Effective contraception essential during treatment and for at least 2 years after treatment in women and at least 3 months after treatment in men (plasma concentration monitoring required'
caution should also be exercised with pre-existing lung and liver disease
203
what are the adverse effects of leflunomide?
Adverse effects
gastrointestinal, especially diarrhoea
hypertension
weight loss/anorexia
peripheral neuropathy
myelosuppression
pneumonitis
204
what is required for monitoring in leflunomide?
Monitoring
FBC/LFT and blood pressure
205
how is leflunomide stopped?
Stopping
leflunomide has a very long wash-out period of up to a year which requires co-administration of cholestyramine
206
iliopsoas absess:
what are the primary causes?
Primary
Haematogenous spread of bacteria
Staphylococcus aureus: most common
207
what are secondary causes of iliopsoas abscess?
Secondary
Crohn's (commonest cause in this category)
Diverticulitis, colorectal cancer
UTI, GU cancers
Vertebral osteomyelitis
Femoral catheter, lithotripsy
Endocarditis
intravenous drug use
208
what are the symptoms of iliopsoas abscess?
Clinical features
Fever
Back/flank pain
Limp
Weight loss
209
what is found on examination of iliopsoas abscess?
Clinical examination
Patient in the supine position with the knee flexed and the hip mildly externally rotated
Specific tests to diagnose iliopsoas inflammation:
Place hand proximal to the patient's ipsilateral knee and ask patient to lift thigh against your hand. This will cause pain due to contraction of the psoas muscle.
Lie the patient on the normal side and hyperextend the affected hip. This should elicit pain as the psoas muscle is stretched.
210
what is the investigation of choice in iliopsoas abscess?
Investigation
CT abdomen is the investigation of choice
211
what is the management of iliopsoas abscess?
Management
Antibiotics
Percutaneous drainage is the initial approach and successful in around 90% of cases
Surgery is indicated if:
1. Failure of percutaneous drainage
2. Presence of an another intra-abdominal pathology which requires surgery
212
what is uhthoffs phenomenon?
Uhthoff ’s phenomenon where neurological symptoms are exacerbated by increases in body temperature is typically associated with multiple sclerosis
Important for meLess important
213
What doe patients with sjogrens syndrome have an increased risk of?
Patients with Sjogren's syndrome have an ↑ risk of lymphoid malignancies
214
what is the most common tumour of the anterior mediastinum?
Thymomas are the most common tumor of the anterior mediastinum
215
what are thymomas associated with?
Associated with
• Myasthenia gravis (30-40% of patients with thymoma)
• Red Cell Aplasia
• Dermatomyositis
• Also : SLE, SIADH
216
how do thymomas cause death?
Causes of death
• Compression of airway
• Cardiac tamponade
217
Fibromyalgia:
-who does this affect?
• Women are 10 times more likely to be affected
• Typically presents between 30-50 years old
218
Fibromyalgia
-what are the features?
• Pain: at multiple site, sometimes 'pain all over'
• Lethargy
• Sleep disturbance, headaches, dizziness are common
219
what is the diagnosis of fibromyalgia?
Diagnosis is clinical and sometimes refers to the American College of Rheumatology classification criteria which list 9 pairs of tender points on the body. If a patient is tender in at least 11 of these 18 points it makes a diagnosis of fibromyalgia more likely
220
what is the management of fibromyalgia?
The management of fibromyalgia is often difficult and needs to be tailored to the individual patient. A psychosocial and multidisciplinary approach is helpful. Unfortunately there is currently a paucity of evidence and guidelines to guide practice. The following is partly based on consensus guidelines from the European League against Rheumatism (EULAR) published in 2007.
• Explanation
• Exercise programme
• Cognitive behavioural therapy
• Anti-depressants: amitriptyline
