rheumatology Flashcards

1
Q

SLE investigations

A

Immunology
99% are ANA positive
20% are rheumatoid factor positive
anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
anti-Smith: most specific (> 99%), sensitivity (30%)
also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)

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)
anti-dsDNA titres can be used for disease monitoring (but note not present in all patients)

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2
Q

characteristic side effects of Methotrexate

A

Myelosuppression
Liver cirrhosis
Pneumonitis

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3
Q

characteristic side effects of Sulfasalazine

A

Rashes
Oligospermia
Heinz body anaemia

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4
Q

characteristic side effects of Leflunomide

A

Liver impairment
Interstitial lung disease
Hypertension

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5
Q

characteristic side effects of Hydroxychloroquine

A

Retinopathy
Corneal deposits

Accumulatesincells–appears to block TLR signalling, phagocytic functions, and DC activation however exact mode of action poorly understood

Efficacy unclear but commonly used in SLE and RA
Toxicity based on cumulative dose
– Eye: corneal (reversible) and macular retinopathy. Need baseline eye exam (peripheral and colour vision) and monitoring. Note – G6PD lysis
– Common: GI upset

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6
Q

characteristic side effects of Prednisolone

A
Cushingoid features
Osteoporosis
Impaired glucose tolerance
Hypertension
Cataracts
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7
Q

characteristic side effects of Gold

A

Proteinuria

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8
Q

characteristic side effects of Penicillamine

A

Proteinuria

Exacerbation of myasthenia gravis

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9
Q

characteristic side effects of Etanercept

A

Demyelination

Reactivation of tuberculosis

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10
Q

characteristic side effects of Infliximab and Adalimumab

A

Reactivation of tuberculosis

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11
Q

characteristic side effects of Rituximab

A

Infusion reactions are common

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12
Q

what is psoriatic arthropathy

A

Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions. Around 10% percent of patients with skin lesions develop an arthropathy with males and females being equally affected

Types*
rheumatoid-like polyarthritis: (30-40%, most common type)
asymmetrical oligoarthritis: typically affects hands and feet (20-30%)
sacroilitis
DIP joint disease (10%)
arthritis mutilans (severe deformity fingers/hand, ‘telescoping fingers’)

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13
Q

management of psoriatic arthropathy

A

Management
treat as rheumatoid arthritis
but better prognosis

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14
Q

what is rheumatoid arthritis

A

a chronic systemic inflammatory disease, characterised by a symmetricla, deforming, peripheral polyarthritis. prevalence is about 1%

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15
Q

typical presentation of RA

A

symmetrical swollen, painful, stiff small joints of the hands and feet. worse in the morning (endogenous cortisol?).

there are less common presentations however. other presentations include sudden onset, recurrent mono arthritis, persistant mono arthritis, systemic illness with initially few joint issues, or just recurrent soft tissue problems such as frozen shoulder or carpel tunnel syndrome.

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16
Q

early vs late signs in common presentations of RA

A

early - inflammation, no joint damage. swelling of joints.

late - joint damage and deformity. ulnar deviation of fingers and dorsal wrist subluxation

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17
Q

what does subluxation mean

A

A subluxation may have different meanings, depending on the medical specialty involved. It implies the presence of an incomplete or partial dislocation of a joint or organ.[a] The World Health Organization (WHO) defines both the medical subluxation and the chiropractic subluxation. It contrasts the two and states in a footnote that a medical subluxation is a “significant structural displacement, and therefore visible on static imaging studies.

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18
Q

what is a boutonniere deformity

A

Boutonniere deformity is a deformed position of the fingers or toes, in which the joint nearest the knuckle (PIP) is permanently bent toward the palm while the farthest joint (DIP) is bent back away (PIP flexion with DIP hyperextension). It is commonly caused by injury[1] or by an inflammatory condition like rheumatoid arthritis, or genetic conditions like Ehlers Danlos Syndrome.

This flexion deformity of the proximal interphalangeal joint is due to interruption of the central slip of the extensor tendon such that the lateral slips separate and the head of the proximal phalanx pops through the gap like a finger through a button hole (thus the name, from French boutonnière “button hole”). The distal joint is subsequently drawn into hyperextension because the two peripheral slips of the extensor tendon are stretched by the head of the proximal phalanx (note that the two peripheral slips are inserted into the distal phalanx, while the proximal slip is inserted into the middle phalanx). This deformity makes it difficult or impossible to extend the proximal interphalangeal joint.

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19
Q

what is a swan neck deformity

A

Swan neck deformity is a deformed position of the finger, in which the joint closest to the fingertip is permanently bent toward the palm while the nearest joint to the palm is bent away from it (DIP hyperflexion with PIP hyperextension). It is commonly caused by injury or inflammatory conditions like rheumatoid arthritis or sometimes familial (congenital, like Ehlers-Danlos syndrome[1]).

Swan neck deformity has many possible causes arising from the DIP, PIP, or even the MCP joints. In all cases, there is a stretching of the volar plate at the PIP joint to allow hyperextension, plus some damage to the attachment of the extensor tendon to the base of the distal phalanx that produces a hyperflexed mallet finger. Duck bill deformity is a similar condition affecting the thumb (which cannot have true swan neck deformity because it does not have enough joints).

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20
Q

what is a Z deformity

A

“Z-thumb” or “Z-deformity” consists of hyperextension of the interphalangeal joint, fixed flexion and subluxation of the metacarpophalangeal joint and gives a “Z” appearance to the thumb.

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21
Q

what is fealty’s syndrome

A

RA + splenomegaly + neutropenia

only occurs in about 1% of RA. splenomegaly alone is seen in about 5% of RA patients.

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22
Q

RA investigations

A

bloods:
rheumatoid factor positive in about 70% of PT. high titre associated with severe disease.

anti-CCP antibodies are 98% specific for RA.

often anaemia of chronic disease

inflam causes raised platelets, ESR and CRP.

XRAYS:
soft tissue swelling, loss of joint space, joint deformity, junta-articular osteopenia.

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23
Q

how is the disease activity of RA assessed

A

DAS28 score = 28 joint disease activity score. assesses tenderness and swelling in 28 joints, ESR and patient reported symptom severity.

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24
Q

7 components of management of RA

A
1- DMARDS
2 - biological agents
3 - steroids - useful for flairs
4 - NSAIDS - symptomatic relief only
5 - physio and occupational therapy
6 - surgery - for function and deformity
7 - managing risk factors for cardiovascular and cerebrovascular disease.
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25
overview of DMARDS in RA
disease modifying anti rheumatic drugs. 1st line for treating RA. can take 6-12 weeks for symptomatic benefit. ``` methotrexate sulfasalazine hydroxychloroquine leflunomide IM gold (rare) penicillamine azathioprine ciclosporin ```
26
overview of biological agents in RA
4 approaches 1 - TNFalpha inhibitors - infliximab, etanercept, adalimumab, etc for use in active RA after failure to respond to 2 DMARDS and with a DAS28 of over 5.1 to be given with methotrexate 2 - B cell depletion - rituximab used with methotrexate once DMARDS and TNFalpha inhibitors have failed. 3 - IL-1 and IL-6 inhibition- tocilizumab (IL-6 R blocker), with methotrexate where both a TNFalpha inhibitor and rituximab have failed. 4 - T cell function disruption - abatacept for those who fail to respond to DMARDS, TNFalpha blockers or rituximab.
27
diagnosis of RA
those with joint swelling not better explained another way should be scored ABCD, 6 or more out of 10 is diagnostic. A - joint involvement (up to 5 points) B - serology - RF and anti-CCP (up to 3 points) C - acute phase reactants - CRP and ESR (1 point max) D - duration of symptoms - over 6 weeks is one point.
28
what types of joint disease are associated with prolonged early morning inactivity stiffness
Inflammatory joint or spinal disease may be associated with prolonged early morning inactivity stiffness (> 30 minutes). Mechanical problems are usually not.
29
if symptoms develop in multiple joints over a short period what does this suggest?
Symptoms in several joints developing over a short period point towards a systemic inflammatory, rather than local mechanical process.
30
difference between hx of articular pain and primary bone pain
Articular pain is usually dependent on movement, whereas pain from primary bone pathology is usually unremitting and often worse at night.
31
Root pain is distinguished from referred spinal pain by
Root pain is distinguished from referred spinal pain by significant involvement of the hand or foot, dermatomal pattern, associated parasthesiae and electrical quality.
32
A critical aspect of any musculoskeletal pain is
A critical aspect of any musculoskeletal pain is its relationship to sleep.
33
relevance of mild fever when synovitis is present
Although mild fever can accompany many forms of synovitis it remains a key pointer to infection and/or systemic lupus.
34
if someone presents with synovitis what is an important area to ask about
A history of foreign travel or sexual contact is important in patients presenting with synovitis. (also fever to suggest infective/SLE cause)
35
Stiffness of shoulder and pelvic girdles, worse in the morning, in subjects over 50 is characteristic of, but not unique to,
Stiffness of shoulder and pelvic girdles, worse in the morning, in subjects over 50 is characteristic of, but not unique to, polymyalgia rheumatica.
36
Shoulder pain is usually referred to
9. Shoulder pain is usually referred to the outer upper arm.
37
Shoulder pain is aggravated by
Shoulder pain is aggravated by elevation (reaching up to shelves) and rotation (putting the painful arm into a coat or shirt).
38
Hip joint pain is usually referred to
Hip joint pain is usually referred to the groin and anterior thigh as far as the knee, and may be only in the knee.
39
Hip joint pain in a child or young adult requires
12. Hip joint pain in a child or young adult requires urgent investigation.
40
Back pain aggravated by coughing and sneezing suggests
Back pain aggravated by coughing and sneezing suggests a significant intervertebral disc lesion.
41
Gout usually first presents at
Gout usually first presents a t the 1st MTP joint.
42
Spontaneous swelling of a toe 1P joint suggests
Gout usually first presents a t the 1st MTP joint. Spontaneous swelling of a toe 1P joint suggests seronegative spondarthritis.
43
A patient with recent onset back pain who is systemically unwell must be
A patient with recent onset back pain who is systemically unwell must be investigated for osteomyelitis or malignancy.
44
at what sites is spinal pain unlikely to be simple and mechanical in aetiology
Spinal pain at sites other than lumbosacral or low cervical (i.e. thoracic or high lumbar) is unlikely to be a simple mechanical problem and requires further investigation.
45
Spinal pain of any sort may be more noticeable on going to bed at night, but waking with pain in the middle of the night suggests
Spinal pain of any sort may be more noticeable on going to bed at night, but waking with pain in the middle of the night suggests an intrinsic bony lesion and waking with pain in the early morning suggests spondylitis.
46
Gi symptoms to ask about in PT with back pain
18. Sphincter dysfunction must always be enquired about in cases of back pain.
47
signs of giant cell arteritis
Scalp pain and tenderness, pain on chewing and sudden loss of vision in subjects over 50, are all suggestive of giant cell arteritis.
48
Compression of the median nerve in the carpal tunnel produces
Compression of the median nerve in the carpal tunnel produces diffuse pain, subjective swelling and/or paraesthesia in the hand, worse at night, relieved by hanging or shaking the hand down. Questioning may reveal a median distribution but may not.
49
Sudden onset of diffuse swelling below the knee, following knee symptoms, suggests
Sudden onset of diffuse swelling below the knee, following knee symptoms, suggests rupture of the knee joint (often called ‘Baker’s cyst’ or Pseudo-DVT). Rupture can also occur in other joints.
50
A reliable history of Raynaud’s phenomenon requires
A reliable history of Raynaud’s phenomenon requires an account of a white phase and a level of demarcation. Raynaud’s syndrome is more likely to be associated with autoimmune rheumatic disease if it starts in adult life.
51
Joint swelling may be due to
23. Joint swelling may be due to inflammatory oedema, effusion, synovial thickening or bony outgrowth (osteophyte).
52
Inflammatory oedema leads to
Inflammatory oedema leads to diffuse periarticular puffiness, skin tightness and tenderness. (Milder oedema is probably responsible for early morning stiffness).
53
25. Effusion is usually difficult to separate from synovial thickening in joints other than the knee. why?
Effusion is usually difficult to separate from synovial thickening in joints other than the knee. It is necessary to show fluid flow or, in the knee, the positive patellar tap.
54
examinaton characteristic of Bony (osteophytic swelling)
26. Bony (osteophytic swelling) is at the joint line and is hard.
55
Chronic synovitis causes
Chronic synovitis distends the joint capsule, may be tense and firm initially, but with time tends to become spongy and may herniated into surrounding soft tissues.
56
A hot red joint usually indicates
A hot red joint usually indicates either trauma, crystal deposition or infection. It is not a feature of rheumatoid arthritis and if present suggests superinfection.
57
Both rheumatoid arthritis and gout are associated with
Both rheumatoid arthritis and gout are associated with subcutaneous lumps (nodules and tophi) over bony prominences but tophi tend to be gritty.
58
The physical signs of major articular cartilage loss
The physical signs of major articular cartilage loss are bony or grinding crepitus and, in a hinge joint, ligamentous laxity.
59
Clicking or crackling crepitus occurs in
31. Clicking or crackling crepitus occurs in normal joints and is of little significance on its own.
60
Skin Vasculitis produces
Skin Vasculitis produces non-blanching lesions, in the form of palpable purpura, livedo reticularis or frank necrosis.
61
what is gout?
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium. It is caused by chronic hyperuricaemia (uric acid > 450 µmol/l)
62
acute management of gout
Acute management NSAIDs intra-articular steroid injection colchicine* has a slower onset of action. The main side-effect is diarrhoea oral steroids may be considered if NSAIDs and colchicine are contraindicated. A dose of prednisolone 15mg/day is usually used if the patient is already taking allopurinol it should be continued
63
gout prophylaxis
Allopurinol prophylaxis - see indications below allopurinol should not be started until 2 weeks after an acute attack has settled as it may precipitate a further attack if started too early 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 Indications for allopurinol** 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 **patients with Lesch-Nyhan syndrome often take allopurinol for life Other points losartan has a specific uricosuric action and may be particularly suitable for the many patients who have coexistant hypertension calcium channel blockers also increase uric acid levels, possibly by a renal vasodilatory effect increased vitamin C intake (either supplements or through normal diet) may also decrease serum uric acid levels
64
lifestyle changes to prevent gout
Lifestyle modifications reduce 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
65
Rheumatoid arthritis: x-ray changes
Early x-ray findings loss of joint space juxta-articular osteoporosis soft-tissue swelling Late x-ray findings periarticular erosions subluxation
66
diagnosis of ankylosing spondylitis
Inflammatory markers (ESR, CRP) are typically raised although normal levels do not exclude ankylosing spondylitis. 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 Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease, later changes include: sacroilitis: 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
67
treatment of ankylosing spondylitis
The following is partly based on the 2010 EULAR guidelines (please see the link for more details): encourage regular exercise such as swimming physiotherapy NSAIDs are the first-line treatment 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 should be given to patients with persistently high disease activity despite conventional treatments' research is ongoing to see whether anti-TNF therapies such as etanercept and adalimumab should be used earlier in the course of the disease
68
Osteoarthritis: x-ray changes
``` X-ray changes of osteoarthritis decrease of joint space subchondral sclerosis subchondral cysts osteophytes forming at joint margins ```
69
what is paget's disease
Paget's disease is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity. Paget's disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients
70
features and complications of paget's disease
Clinical features - only 5% of patients are symptomatic bone pain (e.g. pelvis, lumbar spine, femur) classical, untreated features: bowing of tibia, bossing of skull raised alkaline phosphatase (ALP) - calcium* and phosphate are typically normal skull x-ray: thickened vault, osteoporosis circumscripta Indications for treatment include bone pain, skull or long bone deformity, fracture, periarticular Paget's bisphosphonate (either oral risedronate or IV zoledronate) calcitonin is less commonly used now ``` Complications deafness (cranial nerve entrapment) bone sarcoma (1% if affected for > 10 years) fractures skull thickening high-output cardiac failure ```
71
what is pseudogout
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate in the synovium Features knee, wrist and shoulders most commonly affected joint aspiration: weakly-positively birefringent rhomboid shaped crystals x-ray: chondrocalcinosis
72
management of pseudogout
Management aspiration of joint fluid, to exclude septic arthritis NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
73
what is marfan's syndrome
Marfan's syndrome is an autosomal dominant connective tissue disorder. It is caused by a defect in the fibrillin-1 gene on chromosome 15 and affects around 1 in 3,000 people. The life expectancy of patients used to be around 40-50 years. With the advent of regular echocardiography monitoring and beta-blocker/ACE-inhibitor therapy this has improved significantly over recent years. Aortic dissection and other cardiovascular problems remain the leading cause of death however.
74
features of marfan's syndrome
Features tall stature with arm span to height ratio > 1.05 high-arched palate arachnodactyly pectus excavatum pes planus scoliosis of > 20 degrees heart: dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve prolapse (75%), lungs: repeated pneumothoraces eyes: upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia dural ectasia (ballooning of the dural sac at the lumbosacral level)
75
what is Sjogren's syndrome
Sjogren's syndrome is 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) ``` Features dry eyes: keratoconjunctivitis sicca dry mouth vaginal dryness arthralgia Raynaud's, myalgia sensory polyneuropathy renal tubular acidosis (usually subclinical) ```
76
investigation of sjogren's syndrome
Investigation 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 Schirmer's test: filter paper near conjunctival sac to measure tear formation histology: focal lymphocytic infiltration also: hypergammaglobulinaemia, low C4
77
management of sjogren's syndrome
Management artificial saliva and tears pilocarpine may stimulate saliva production
78
what is spinal cord compression/when does it occur
Spinal cord compression is an oncological emergency and affects up to 5% of cancer patients. Extradural compression accounts for the majority of cases, usually due to vertebral body metastases. It is more common in patients with lung, breast and prostate cancer Features back pain - the earliest and most common symptom - may be worse on lying down and coughing lower limb weakness sensory changes: sensory loss and numbness neurological signs depend on the level of the lesion. Lesions above L1 usually result in upper motor neuron signs in the legs and a sensory level. Lesions below L1 usually cause lower motor neuron signs in the legs and perianal numbness. Tendon reflexes tend to be increased below the level of the lesion and absent at the level of the lesion
79
management of spinal cord compression
Management high-dose oral dexamethasone urgent oncological assessment for consideration of radiotherapy or surgery
80
what happens in acromioclavicular joint injury
Injury to the AC joint is relatively common and typically occurs during collision sports such as rugby following a fall on to the shoulder or a FOOSH (falls on outstretched hand). AC joint injuries are graded I to VI depending on the degree of separation. Grade I and II injuries are very common and are typically managed conservatively including resting the joint using a sling. Grade IV, V and VI are rare and require surgical intervention. The management of grade III injuries is a matter of debate and often depends on individual circumstances.
81
NICE guidelines for the management of osteoarthritis
NICE published guidelines on the management of osteoarthritis (OA) in 2014 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 NSAIDs and COX-2 inhibitors. 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
82
the role of glucosamine in osteoarthritis
What is the role of glucosamine? normal constituent of glycosaminoglycans in cartilage and synovial fluid a systematic review of several double blind RCTs of glucosamine in knee osteoarthritis reported significant short-term symptomatic benefits including significantly reduced joint space narrowing and improved pain scores more recent studies have however been mixed the 2008 NICE guidelines suggest it is not recommended a 2008 Drug and Therapeutics Bulletin review advised that whilst glucosamine provides modest pain relief in knee osteoarthritis it should not be prescribed on the NHS due to limited evidence of cost-effectiveness
83
what is fibromyalgia?
Fibromyalgia is a syndrome characterised by widespread pain throughout the body with tender points at specific anatomical sites. The cause of fibromyalgia is unknown. Epidemiology women are 10 times more likely to be affected typically presents between 30-50 years old Features chronic pain: at multiple site, sometimes 'pain all over' lethargy sleep disturbance, headaches, dizziness are common
84
diagnosis of fibromyalgia
Diagnosis is clinical and sometimes refers to the American College of Rheumatology classification criteria which lists 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
85
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 and also a BMJ review in 2014. 1 - explanation 2 - aerobic exercise: has the strongest evidence base 3 - cognitive behavioural therapy 4 - medication: pregabalin, duloxetine, amitriptyline
86
name 4 Spondyloarthropathies
ankylosing spondylitis psoriatic arthritis Reiter's syndrome (including reactive arthritis) enteropathic arthritis (associated with IBD)
87
what is Adhesive capsulitis
Adhesive capsulitis (frozen shoulder) is a common cause of shoulder pain. It is most common in middle-aged females. The aetiology of frozen shoulder is not fully understood. Features typically develop over days external rotation is affected more than internal rotation or abduction both active and passive movement are affected patients typically have a painful freezing phase, an adhesive phase and a recovery phase bilateral in up to 20% of patients the episode typically lasts between 6 months and 2 years
88
management of Adhesive capsulitis
Management no single intervention has been shown to improve outcome in the long-term treatment options include NSAIDs, physiotherapy, oral corticosteroids and intra-articular corticosteroids
89
what is temporal arteritis
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. Features typically patient > 60 years old usually rapid onset (e.g. < 1 month) headache (found in 85%) jaw claudication (65%) visual disturbances secondary to anterior ischemic optic neuropathy 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
90
investigations in temporal arteritis
Investigations 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
91
treatment of temporal arteritis
Treatment high-dose prednisolone - 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
92
what is antiphospholipid syndrome
Antiphospholipid syndrome is an acquired disorder characterised 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) 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 ``` Features venous/arterial thrombosis recurrent fetal loss livedo reticularis thrombocytopenia prolonged APTT other features: pre-eclampsia, pulmonary hypertension ``` Associations other than SLE other autoimmune disorders lymphoproliferative disorders phenothiazines (rare)
93
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 increase target INR to 3-4 arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
94
how do you assess the risk of osteoporosis
We worry about osteoporosis because of the increased risk of fragility fractures. So how do we assess which patients are at risk and need further investigation? NICE produced guidelines in 2012: Osteoporosis: assessing the risk of fragility fracture. The following is based on those guidelines. They advise that all women aged >= 65 years and all men aged >= 75 years should be assessed. Younger patients should be assessed in the presence of risk factors, such as: previous fragility fracture current use or frequent recent use of oral or systemic glucocorticoid history of falls family history of hip fracture other causes of secondary osteoporosis low body mass index (BMI) (less than 18.5 kg/m²) smoking alcohol intake of more than 14 units per week for women and more than 21 units per week for men. Methods of risk assessment NICE recommend using a clinical prediction tool such as FRAX or QFracture to assess a patients 10 year risk of developing a fracture. This is analogous to the cardiovascular risk tools such as QRISK. FRAX estimates the 10-year risk of fragility fracture valid for patients aged 40-90 years based on international data so use not limited to UK patients assesses the following factors: age, sex, weight, height, previous fracture, parental fracture, current smoking, glucocorticoids, rheumatoid arthritis, secondary osteoporosis, alcohol intake bone mineral density (BMD) is optional, but clearly improves the accuracy of the results. NICE recommend arranging a DEXA scan if FRAX (without BMD) shows an intermediate result QFracture estimates the 10-year risk of fragility fracture developed in 2009 based on UK primary care dataset can be used for patients aged 30-99 years (this is stated on the QFracture website, but other sources give a figure of 30-85 years) includes a larger group of risk factors e.g. cardiovascular disease, history of falls, chronic liver disease, rheumatoid arthritis, type 2 diabetes and tricyclic antidepressants There are some situations where NICE recommend arranging BMD assessment (i.e. a DEXA scan) rather than using one of the clinical prediction tools: before starting treatments that may have a rapid adverse effect on bone density (for example, sex hormone deprivation for treatment for breast or prostate cancer). in people aged under 40 years who have a major risk factor, such as history of multiple fragility fracture, major osteoporotic fracture, or current or recent use of high-dose oral or high-dose systemic glucocorticoids (more than 7.5 mg prednisolone or equivalent per day for 3 months or longer).
95
what is systemic sclerosis
Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females There are three patterns of disease: 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 Diffuse cutaneous systemic sclerosis scleroderma affects trunk and proximal limbs predominately associated with scl-70 antibodies hypertension, lung fibrosis and renal involvement seen poor prognosis Scleroderma (without internal organ involvement) tightening and fibrosis of skin may be manifest as plaques (morphoea) or linear Antibodies ANA positive in 90% RF positive in 30% anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis anti-centromere antibodies associated with limited cutaneous systemic sclerosis
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causes of osteoarthritis
Advancing age and female sex and significant risk factors for osteoporosis. Prevalence of osteoporosis increases from 2% at 50 years to more than 25% at 80 years in women. ``` There are many other risk factors and secondary causes of osteoporosis. We'll start by looking at the most 'important' ones - these are risk factors that are used by major risk assessment tools such as FRAX: history of glucocorticoid use rheumatoid arthritis alcohol excess history of parental hip fracture low body mass index current smoking ``` ``` Other risk factors sedentary lifestyle premature menopause Caucasians and Asians endocrine disorders: hyperthyroidism, hypogonadism (e.g. Turner's, testosterone deficiency), growth hormone deficiency, hyperparathyroidism, diabetes mellitus multiple myeloma, lymphoma gastrointestinal disorders: inflammatory bowel disease, malabsorption (e.g. Coeliac's), gastrectomy, liver disease chronic kidney disease osteogenesis imperfecta, homocystinuria ``` ``` Medications that may worsen osteoporosis (other than glucocorticoids): long term heparin therapy proton pump inhibitors glitazones aromatase inhibitors e.g. anastrozole ```
97
what is 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 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 Investigations ESR > 40 mm/hr note CK and EMG normal reduced CD8+ T cells Treatment prednisolone e.g. 15mg/od - dramatic response
98
management of osteoporosis
NICE guidelines were updated in 2008 on the secondary prevention of osteoporotic fractures in postmenopausal women. Key points include treatment is indicated following osteoporotic fragility fractures in postmenopausal women who are confirmed to have osteoporosis (a T-score of - 2.5 SD or below). In women aged 75 years or older, a DEXA scan may not be required 'if the responsible clinician considers it to be clinically inappropriate or unfeasible' vitamin D and calcium supplementation should be offered to all women unless the clinician is confident they have adequate calcium intake and are vitamin D replete alendronate is first-line around 25% of patients cannot tolerate alendronate, usually due to upper gastrointestinal problems. These patients should be offered risedronate or etidronate (see treatment criteria below) strontium ranelate and raloxifene are recommended if patients cannot tolerate bisphosphonates (see treatment criteria below)
99
what is charcot joint
Charcot joint A Charcot joint is also commonly referred to as a neuropathic joint. It describes a joint which has become badly disrupted and damaged secondary to a loss of sensation. In years gone by they were most commonly caused by neuropathy secondary to syphilis (tabes dorsalis) but are now most commonly seen in diabetics. Features Charcot joints are typically a lost less painful than would be expected given the degree of joint disruption due to the sensory neuropathy. However, 75% of patients report some degree of pain the joint is typically swollen, red and warm
100
what is pseudogout
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate in the synovium ``` Risk factors hyperparathyroidism hypothyroidism haemochromatosis acromegaly low magnesium, low phosphate Wilson's disease ``` Features knee, wrist and shoulders most commonly affected joint aspiration: weakly-positively birefringent rhomboid shaped crystals x-ray: chondrocalcinosis
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management of pseudogout
Management aspiration of joint fluid, to exclude septic arthritis NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
102
what is raynaud's
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 ``` Factors suggesting underlying connective tissue disease onset after 40 years unilateral symptoms rashes presence of autoantibodies features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages digital ulcers, calcinosis very rarely: chilblains ``` Secondary causes connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE leukaemia type I cryoglobulinaemia, cold agglutinins use of vibrating tools drugs: oral contraceptive pill, ergot cervical rib
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management of raynaud's
Management first-line: calcium channel blockers e.g. nifedipine IV prostacyclin infusions: effects may last several weeks/months
104
drugs that can cause gout
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium. It is caused by chronic hyperuricaemia (uric acid > 0.45 mmol/l) ``` Drug causes thiazides, furosemide alcohol cytotoxic agents pyrazinamide ```
105
what happens in primary hyperparathyroidism
In exams primary hyperparathyroidism is stereotypically seen in elderly females with an unquenchable thirst and an inappropriately normal or raised parathyroid hormone level. It is most commonly due to a solitary adenoma ``` Causes of primary hyperparathyroidism 80%: solitary adenoma 15%: hyperplasia 4%: multiple adenoma 1%: carcinoma ``` Features - 'bones, stones, abdominal groans and psychic moans' polydipsia, polyuria peptic ulceration/constipation/pancreatitis bone pain/fracture renal stones depression hypertension Associations hypertension multiple endocrine neoplasia: MEN I and II Investigations raised calcium, low phosphate PTH may be raised or normal technetium-MIBI subtraction scan
106
what happens in reactive arthritis
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 during the Second World War. Later studies identified patients who developed symptoms following a sexually transmitted infection (post-STI, now sometimes referred to as sexually acquired reactive arthritis, SARA). Reactive arthritis is defined as an arthritis that develops following an infection where the organism cannot be recovered from the joint. Features typically develops within 4 weeks of initial infection - symptoms generally last around 4-6 months arthritis is typically an asymmetrical oligoarthritis of lower limbs dactylitis 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) Around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease
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features of ankylosing spondylitis (the 'rule')
``` Ankylosing spondylitis features - the 'A's Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis ```
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what is Takayasu's arteritis
Takayasu's arteritis is a large vessel vasculitis. It typically causes occlusion of the aorta and questions commonly refer to an absent limb pulse. It is more common in females and Asian people Features systemic features of a vasculitis e.g. malaise, headache unequal blood pressure in the upper limbs carotid bruit intermittent claudication aortic regurgitation (around 20%) Associations renal artery stenosis Management steroids
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Rheumatoid arthritis: ocular manifestations
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye problems ``` Ocular manifestations keratoconjunctivitis sicca (most common) episcleritis (erythema) scleritis (erythema and pain) corneal ulceration keratitis ``` Iatrogenic steroid-induced cataracts chloroquine retinopathy
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management of reactive arthritis
Management symptomatic: analgesia, NSAIDS, intra-articular steroids sulfasalazine and methotrexate are sometimes used for persistent disease symptoms rarely last more than 12 months
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what is 'frozen shoulder'
Frozen shoulder (adhesive capsulitis) is an uncommon condition with global stiffening of the shoulder associated with adhesion and contraction of glerohumeral synovial membrane, which lasts for many months even with treatment.
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what vasculitis is polymyalgia rheumatica associated with
giant cell arteritis
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main risk factors for gout
The main risk factors for gout are alcohol consumption, diuretics, male gender and family history (heritable factors).
114
what eye problems are associated with ankylosing spondylitis
Ankylosing spondylitis may be associated with attacks of acute anterior uveitis which may require urgent treatment.
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what is ankylosing spondylitis
Ankylosing spondylitis is the name given to generalised enthesopathy of the spinal ligaments and sacroiliac joints.
116
what is rheumatic fever
Rheumatic fever is a post-streptococcal illness which may include arthritis, carditis, chorea and erythema marginatum. It is extremely rare in the UK.
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what is Sjögren’s syndrome
Sjögren’s syndrome is the association of sicca syndrome (dryness of the mouth and eyes) with systemic autoimmune features. Severe sicca can lead to corneal damage and blindness.
118
what is Henoch-Schönlein purpura.
Henoch–Schönlein purpura (HSP, also known as anaphylactoid purpura,[1] purpura rheumatica,[1] and Schönlein–Henoch purpura)[1] is a disease of the skin and other organs that most commonly affects children. In the skin, the disease causes palpable purpura (small hemorrhages); often with joint and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine, but this usually goes unnoticed; in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease. HSP is often preceded by an infection, such as a throat infection. HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized by deposition of immune complexes containing the antibody IgA; the exact cause for this phenomenon is unknown. It usually resolves within several weeks and requires no treatment apart from symptom control, but may relapse in a third of the cases and cause irreversible kidney damage in about one in a hundred cases. Synovitis, abdominal pain and nephritis are features of Henoch-Schönlein purpura.
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major features of SLE
The major features of systemic lupus are arthritis, fatigue, rash, photosensitivity, alopecia, glomerulonephritis, serositis, cerebral changes, cytopenias, Vasculitis, carditis, pneumonitis and antinuclear antibodies.
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what is polymyositis
Polymyositis is the term used for inflammatory muscle disease of unknown origin – usually affecting several muscles – often chiefly proximal.
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what is dermatomyositis
Dermatomyositis is a combination of myositis and a lilac or pinkish rash over the face and/or over the knuckles. It may be a non-metastatic complication of malignancy.
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who does paget's disease arise in
Paget’s disease occurs almost exclusively in the elderly and may be monostotic or polystotic. It is rarely complicated by osteosarcoma.
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what is sudeck's atrophy
Sudeck’s atrophy, reflex sympathetic dystrophy and algodystrophy are all names for painful local osteoporosis of a hand, foot or rarely knee, which is cold, hypersensitive, swollen, red or dusky.
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different types of fluid that you might aspirate from a joint?
Aspiration of clear (acellular), viscous fluid from a swollen joint indicates a mechanical rather than an inflammatory cause. Hazy fluid (moderate cell count) suggests an inflammatory cause, and opaque fluid (high cell count) the likelihood of infection.
125
evidence of gout under microscopy
The demonstration of intracellular needle shaped negatively birefringent urate crystals in leucocytes in synovial fluid using a polarising microscope is the most specific evidence for gout.
126
how do you see articular cartilage loss on an xray
77. Articular cartilage loss is best identified as joint space loss on plain radiographs. Demonstration of articular cartilage loss in the knee requires the joint to be weight-bearing when taking the radiograph.
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evidence of RA on a radiograph
Established rheumatoid arthritis is associated with peri-articular osteopaenia, juxta-articular erosion and generalised space loss on plain radiographs.
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major cause of death in systemic sclerosis
Renovascular hypertension is the major preventable cause of death in systemic sclerosis and blood pressure must be monitored regularly.
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radiograph features of paget's
The diagnostic features of Paget’s disease on radiographs are irregular patches of porosis and sclerosis, coarse trabeculation and bony enlargement.
130
what cultures are needed if you suspect joint infection
If joint infection is a possibility both aspirate culture and blood culture are necessary.
131
what are the principle indications for the replacement of a weight bearing joint
The principal indications for replacement of weight-bearing joints are night pain and poor walking tolerance inconsistent with an acceptable quality of life.
132
most common side effects of gold IM.
Gold (sodium aurothiomalate) is a slow-acting antirheumatic drug. Its commonest side- effects are mouth ulcers, rash – which may become exfoliative and fatal and membranous glomerulonephritis with proteinuria.
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treatment of septic arthritis
Treatment of septic arthritis includes systemic antibiotics (initially intravenous) once cultures are complete, lavage with large bore needle arthrocentesis or arthroscopy, pain relief by initial rest and analgesics and subsequent early mobilisation.
134
treatment of gout and pseudogout
Allopurinol, by inhibiting the enzyme xanthine oxidase, can reduce urate formation, and is a mainstay of the prophylactic treatment of gout. Acute attacks of gout and pseudogout are treated with non-steroidal anti-inflammatories or colchicine or local injection. Allopurinol is an isomer of hypoxanthine and as such is a purine analogue. It acts by inhibiting xanthine oxidase thereby blocking the oxidation of hypoxathine and xanthine. This reduces the production of uric acid. In addition, the build up of hypoxanthine and xanthine results in their conversion to adenosine and guanosine. This causes feedback inhibition of amidophosphoribosyl transferase, which is the rate-limiting enzyme of purine biosynthesis. Allopurinol therefore reduces both purine breakdown and synthesis.
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Prescribing methotrexate - considerations
methotrexate is a drug with a high potential for patient harm. It is therefore important that you are familiar with guidelines relating to its use methotrexate is taken weekly, rather than daily FBC, U&E and LFTs need to be regularly monitored. The Committee on Safety of Medicines recommend 'FBC and renal and LFTs before starting treatment and repeated weekly until therapy stabilised, thereafter patients should be monitored every 2-3 months' folic acid 5mg once weekly should be co-prescribed, taken more than 24 hours after methotrexate dose the starting dose of methotrexate is 7.5 mg weekly (source: BNF) only one strength of methotrexate tablet should be prescribed (usually 2.5 mg) avoid prescribing trimethoprim or cotrimoxazole concurrently - increases risk of marrow aplasia
136
Management of patients at risk of corticosteroid-induced osteoporosis
Management of patients at risk of corticosteroid-induced osteoporosis The RCP guidelines essentially divide patients into two groups. 1. Patients over the age of 65 years or those who've previously had a fragility fracture should be offered bone protection. 2. Patients under the age of 65 years should be offered a bone density scan, with further management dependent: T score Management Greater than 0 Reassure Between 0 and -1.5 Repeat bone density scan in 1-3 years Less than -1.5 Offer bone protection The first-line treatment is alendronate. Patients should also be calcium and vitamin D replete.
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Osteoporosis: Assessing patients following a fragility fracture
The management of patients following a fragility fracture depends on age. Patients >= 75 years of age Patients who've had a fragility fracture and are >= 75 years of age are presumed to have underlying osteoporosis and should be started on first-line therapy (an oral bisphosphonate), without the need for a DEXA scan. It should be noted that the 2014 NOGG guidelines have a different threshold, suggesting treatment is started in all women over the age of 50 years who've had a fragility fracture - 'although BMD measurement may sometimes be appropriate, particularly in younger postmenopausal women.' Patients < 75 years of age If a patient is under the age of 75 years a DEXA scan should be arranged. These results can then be entered into a FRAX assessment (along with the fact that they've had a fracture) to determine the patients ongoing fracture risk. For example, a 79-year-old woman falls over on to an outstretched hand and sustains a Colles' fracture (fracture of the distal radius). Given her age she is presumed to have osteoporosis and therefore started on oral alendronate 70mg once weekly. No DEXA scan is arranged.
138
what happens in Dactylitis
Dactylitis describes the inflammation of a digit (finger or toe). Causes include: spondyloarthritis: e.g. Psoriatic and reactive arthritis sickle-cell disease other rare causes include tuberculosis, sarcoidosis and syphilis A 'sausage-shaped' digit is a classical description of dactylitis. It would be unusual for gout to affect the middle toe, the vast majority of cases occur in the first metatarsophalangeal joint.
139
Rheumatoid arthritis: complications
A wide variety of extra-articular complications occur in patients with rheumatoid arthritis (RA): respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy osteoporosis ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus increased risk of infections depression Less common Felty's syndrome (RA + splenomegaly + low white cell count) amyloidosis
140
what are the three types of systemic sclerosis
Limited cutaneous systemic sclerosis Diffuse cutaneous systemic sclerosis Scleroderma (without internal organ involvement)
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what is CREST syndrome
a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia
142
Lupus signs and symptoms
``` SOAP BRAIN: Serositis [pleuritis, pericarditis] Oral ulcers Arthritis Photosensitivity Blood [all are low - anemia, leukopenia, thrombocytopenia] Renal [protein] ANA Immunologic [DS DNA, etc.] Neurologic [psych, seizures] ```
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Hypersensitivity reactions: Gell and Goombs nomenclature
``` ACID From I to IV: Anaphylactic type: type I Cytotoxic type: type II Immune complex disease: type III Delayed hypersensitivity (cell mediated): type IV ```
144
Sjogren syndrome: morphology
``` "Jog through the MAPLES": Sjogren is: Mouth dry Arthritis Parotid enlarged Lymphoma Eyes dry Sicca (primary) or Secondary ```
145
Raynaud's disease: causes
``` BAD CT: Blood disorders (eg polycythaemia) Arterial (eg atherosclerosis, Buerger's) Drugs (eg beta-blockers) Connective tissue disorders (rheumatoid arthritis, SLE) Traumatic (eg vibration injury) ```
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Back pain causes
DISK MASS (since near vertebral disc): Degeneration (DJD, osteoporosis, spondylosis) Infection (UTI, PID, Pott's disease, osteomyelitis, prostatitis)/ Injury, fracture or compression fracture Spondylitis (ankylosing spondyloarthropathies such as rheumatoid arthritis, Reiters, SLE) Kidney (stones, infarction, infection) Multiple myeloma/ Metastasis (from cancers of breast, kidney, lung, prostate, thyroid) Abdominal pain (referred to the back)/ Aneurysm Skin (herpes zoster)/ Strain/ Scoliosis and lordosis Slipped disk/ Spondylolisthesis
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Behcet's syndrome: diagnostic criteria
``` PROSE: Pathergy test (i/d saline injection) Recurrent genital ulceration Oral ulceration (recurrent) Skin lesions Eye lesions ```
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Osteoarthritis: x-ray signs
``` LOSS: Loss of joint space Osteopyhtes Subcondral sclerosis Subchondral cysts ```
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Arthritis: seronegative spondyloarthropathies
``` PEAR: Psoriatic arthritis Enteropathic arthritis Ankylosing sponylitis Reiter's/ Reactive ```
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Monoarthritis differential
``` GHOST: Gout Haemarthrosis Osteoarthritis Sepsis Trauma ```
151
Sulphasalazine overview
Aminosalicylate(incommonwithmesalazine) • =5-ASAlinkedtosulfapyridine. • Poorlyabsorbed,orallycleavedto2active components by colonic bacteria – 5-ASA – anti-inflammatory/immunosuppressive effects – Sulfapyridine – (purported) anti-folate activity SE: – Common: GI upset, headache – Rare: haematological dyscraisias, renal impairment, oligospermia, hypersensitivity reactions
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difference between etanercept, adalimumab and infliximab
– Infliximab, chimeric antibody of part mouse (variable region) part human antibody (Fc region) . Inhibits TNF by binding in the joint cavity, infusion with methotrexate once a week – Etanercept, recombinant human TNF fusion protein, competitively inhibits TNF binding to cell surface receptor, subcut injection twice a week – Adalimumab, recombinant human monoclonal antibody, subcut injection
153
uric acid excretion
``` UA: filtered at glomerulus. In hyperuricaemia large loads filtered, UA re-absorption increases to avoid dumping of poorly soluble urate into urinary tract (secretion impaired). Uricosuric agents (probenicid, sulfinpyrazone and high dose aspirin) block re-sorption of filtered and secreted UA in the renal tubules. Thiazide diuretics and low dose aspirin reduce renal clearance of uric acid-90%) may precipitate gout ```
154
how does colchicine work in gout
Colchine: inhibits leukocyte micro-tubular formation and migration – must be given early until good pain relief or G.I side effects (nausea, vomiting , diarrhoea, abdo pain) t1/2=30hrs clearance reduced in renal impairment.
155
iatrogenic causes of osteoporosis
Steroids • Anti-epileptics phenytoin phenobarbitone primidone valproate • Heparin- long term use
156
this antibody is most common in : antimitochondrial ab
PBC
157
this antibody is most common in : anti scl-70 (topoisomerase)
diffuse systemic sclerosis 50%
158
this antibody is most common in : anti smooth muscle
autoimmune hepatitis
159
this antibody is most common in : anti-CCP
rheumatoid arthritis
160
this antibody is most common in : anti Rho and La
sjogren's anti rho is also associated with neonatal lupus which is bad so council sexually active women.
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this antibody is most common in : antiDsDNA
SLE along with anti histone also occurs in 75% of primary sjogren's
162
this antibody is most common in : cANCA
wegener's
163
this antibody is most common in :pANCA
PSC
164
what drug can't you use with allopurinol
azathioprine as the levels will rocket up as it is broken down by xanthine oxidase.
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this antibody is most common in : anti-centromere
CREST but only found in 30% of cases. a type of limited cutaneous systemic sclerosis. crest = calcinosis, raynaud's syndrome, oesophaeal dysmotility, sclerodactyly and telangiectasia.
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cord compression by cancer met - if you think this is likely then which imaging modality do you want to use
urgent CT to find it.
167
joints most often first affected in ankylosing spondylitis
the sacroiliac
168
new drugs - where do you report side effects found in clinical use
via the yellow card system to the MHRA - medicines and healthcare products regulatory agency.
169
polyarteritis nodosum
aneurisms and thrombosis in the medium size arteries
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immediate management of an acutely hot swollen joint
an emergency 1 - in essentially all cases the first step is to aspirate the effusion. the appearance of the effusion is imminently useful. 2 - if pus is withdrawn get an orthopaedic surgeon to consider surgical washout. otherwise send it to the lab and start IV antibiotics. 3 - if the joint is prosthetic DO NOT aspirate and get an orthopod. 4 - if the effusion is difficult to aspirate get an ultrasound to help find it.
171
yellow flags for the development of chronic pain and disability
depression, time off work, extended rest and the chronic use of multiple painkillers.
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most common pathogen responsible for a septic joint
staph aureus is the commonest cause in older adults | nisseria gonorrhoeae is the most common in younger adults, most likely due to different patterns of sexual activity.
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stereotypical joints for first presentation of gout and pseudogout
``` pseudo = knee gout = 1st MTP ```
174
what is naproxen
Naproxen /nəˈprɒksən/ (INN; brand names: Aleve, Naprosyn and many others), usually sold as the sodium salt naproxen sodium, is a nonsteroidal anti-inflammatory drug (NSAID) of the propionic acid class (which puts it in the same class as ibuprofen) and is commonly used for relief of a wide variety of pain, fever, swelling and stiffness.[2][3]:665,673 It is the preferred NSAID for long-term use in people with a high risk of cardiovascular (for example, heart attacks or strokes) complications,[3]:665 due to its relatively low risk of causing such complications. Naproxen has an intermediate risk of causing stomach ulcers as compared with ibuprofen, which is low risk, and indometacin, which is high risk.
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what is indometacin
a fast acting strong NSAID = use in gout as high dose first line. second line is colchicine but CI in renal impairment .
176
when is a squared thumb seen
in bad osteoarthritis
177
when is onycholysis seen
in psoriatic arthritis.
178
in what rheum condition can serum calcium be raised
sarcoidosis
179
cauda equina syndrome
compression of cauda equine below L2 causes reduced perineal sensation
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what is pyoderma gangrenosum
unknown aetiology but thought to be neutrophil dysfunction. looks like an innocent bite at first but progresses to a wide nasty puss covered ulceration of the skin. associated with inflammatory bowel disease and haematological disorders. painful, can have a purple border, leaves scars and can be chronic.
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dermatitis herpetidiformis
often found in people with coeliac disease and imrpoves along with bowel symptoms on removal of gluten. itchy groups of blisters that appear after a burning and stinging sensation.
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what cause of anaemia is vitiligo associated with
This is vitiligo, a single organ autoimmune condition, associated with other similar conditions. Pernicious anaemia (anti-intrinsic factor) fulfills this condition. The correct answer is: pernicious anaemia
183
whats a cholesteatoma
Cholesteatoma occurs if skin from the inner side of the tympanic membrane sloughs off and mixes with ear wax that penetrates to the middle ear and fails to drain through the Eustachian tube. The accumulation of dead skin and wax causes a cyst-like mass, the cholesteatoma. If left untreated the mass can cause serious damage to the eardrum and ossicles. In serious cases, cholesteatomas can erode into the mastoid and can cause cerebral infection.
184
what is Hirschsprung's disease (
Hirschsprung's disease (HD) is a disorder of the abdomen that occurs when part or all of the large intestine or antecedent parts of the gastrointestinal tract have no ganglion cells and therefore cannot function. During normal fetal development, cells from the neural crest migrate into the large intestine (colon) to form the networks of nerves called Auerbach's plexus (myenteric plexus - between the smooth muscle layers of the gastrointestinal tract wall) and Meissner's plexus (submucosal plexus - within the submucosa of the gastrointestinal tract wall). In Hirschsprung's disease, the migration is not complete and part of the colon lacks these nerve bodies that regulate the activity of the colon. The affected segment of the colon cannot relax and pass stool through the colon, creating an obstruction.[1] In most affected people, the disorder affects the part of the colon that is nearest the anus. In rare cases, the lack of nerve bodies involves more of the colon. In five percent of cases, the entire colon is affected. Stomach and esophagus may be affected too. Hirschsprung's disease is also often called congenital aganglionic megacolon. Hirschsprung's disease occurs in about one in 5,000 of live births.[2] It is usually diagnosed in children, and affects boys more often than girls.
185
a typical description of haemochromatosis
A 50 year old woman has a five year history of pain in the middle of both feet, back, hips, and metacarpals. She has an elevated serum ferritin, with normal liver function tests and glucose concentration, full blood count, and erythrocyte sedimentation rate the serum ferritin is characteristically chronically raised.
186
se of lithium and toxicity effects
Following a number of patients receiving lithium being harmed, the National Patient Safety Agency (NPSA) produced an ‘alert’—Safer lithium therapy, in December 2009. It noted that regular blood levels (and renal/ thyroid function) were not being checked to allow reciprocal changes in dosage, and patients seemed unaware of side eff ects and symptoms of toxicity as well as many widely available interacting medications. This patient has probably suff ered an episode of gout and, although successfully self medicating with an over-the-counter non-steroidal antiinflammatory drug (NSAID), has consequently reduced the excretion of his lithium. A therapeutic lithium level can cause fi ne tremor and gastrointestinal upset, but toxic levels can cause ataxia, blurred vision, a coarse tremor, dizziness, muscle twitching, tinnitus, and polyuria, and patients need to be made aware of these.
187
how is erythema nodosum described and what virus can cause it
A 25-year-old woman has had a rash on her thighs and shins over the last week. She has no relevant past medical history and is recovering well 3 weeks after being diagnosed with glandular fever, which improved with symptomatic treatment only. The lesions are tender, smooth, shiny nodules. They are purple coloured and between 1 and 3cm in diameter. Erythema nodosum is associated in this case with Epstein–Barr virus, but in up to 50% of cases no cause is identified. It occurs as a result of infl ammation of subcutaneous fat.
188
what is Necrobiosis lipoidica
Necrobiosis lipoidica is a necrotizing skin condition usually occurring in people with diabetes. A 48-year-old woman has had ‘bruising’ over her shins for the last few weeks. She thought they were improving, but recently they changed into yellow-coloured lesions. She accidentally knocked her leg on a door a week ago and soon after she developed an ulcer over one of the yellow lesions.
189
what is Pyoderma gangrenosum
Pyoderma gangrenosum is of unknown aetiology but is thought to be due to neutrophil dysfunction. It is also associated with infl ammatory bowel disease and a number of haematological disorders. A 50-year-old woman has had some lesions on her legs for the last 2 months. Initially she thought that she had been bitten by an insect, but they did not disappear and are now much larger and more painful. The skin is now ulcerated and has developed a purple border. She takes infliximab for rheumatoid arthritis.
190
what is Dermatitis herpetiformis
Dermatitis herpetiformis is often found in combination with coeliac disease: 80% of people will see their gastrointestinal and dermatitis symptoms improve with a gluten-free diet, although sometimes dapsone is required. A 37-year-old woman has been suff ering from worsening diarrhoea and weight loss. She is increasingly tired and is becoming withdrawn from her friends and family. Over the last month, she has started to suffer from itchy groups of blisters on both her elbows and forearms, which appeared after a burning and stinging sensation.