Rheumatology Flashcards
1
Q
What is rheumatoid arthritis?
A
Symmetrical inflammatory arthritis affecting mainly the peripheral synovial joints including both articular and extra-articular structures.
Can lead to systemic involvement of lung, kidney and cardiovascular.
2
Q
What is the gender ratio for rheumatoid arthritis?
A
Females:males = 3:1.
3
Q
What is the term given for rheumatoid arthritis in children?
A
Juvenile idiopathic arthritis.
4
Q
What is the genetic basis of rheumatoid arthritis?
A
Genetics: HLA-DR4 mediated.
Environmental trigger triggers genetic predisposition leading to autoimmune cascade.
5
Q
What main structure is targeted in rheumatoid arthritis?
A
Synovium and tenosynovium.
6
Q
What is the main difference between osteoarthritis and rheumatoid arthritis?
A
Osteoarthritis: non-inflammatory, cartilage affected.
Rheumatoid arthritis: inflammatory, synovium affected.
7
Q
What is pannus?
A
RA causes an inflammatory response to synovium that leads to the formation of a hypervascular abnormal tissue called rheumatoid pannus if left untreated.
8
Q
What is the pathogenesis of rheumatoid arthritis
A
APCs present self-antigen to T cells which stimulate B cells and macrophages.
Macrophages release pro-inflammatory cytokines.
B cells produce rheumatoid factor and other inflammatory cytokines.
Net result is increased osteoclast stimulation leading to inflammation and joint destruction.
9
Q
What time length is the therapeutic window for rheumatoid arthritis?
A
3 months.
Early rheumatoid is defined as less than 2 years since symptom onset.
10
Q
How can rheumatoid arthritis be diagnosed?
A
History/clinical exam.
Routine blood testing for anaemia of chronic disease (normochromic/normocytic anaemia), raised platelets, FBC (MCV normal, Hb low).
Inflammatory markers (CRP, ESR/plasma viscosity) - raised.
Autoantibodies.
X-rays of hands, feet and chest.
11
Q
What are the clinical features of rheumatoid arthritis?
A
Prolonged morning stiffness lasting longer than an hour.
Involvement of small joints of the hands and feet.
Symmetric distribution.
Positive compression tests of MCP and MTP joints.
12
Q
What are the clinical presentations of rheumatoid arthritis?
A
PIP, MCP, wrist, MTP synovitis.
Monoarthritis.
Tenosynovitis.
Trigger finger.
Carpal tunnel syndrome.
Polymyalgia rheumatica.
Palindromic rheumatism.
Systemic symptoms.
Poor grip strength.
13
Q
Which autoantibodies are associated with rheumatoid arthritis? How specific/sensitive are they?
A
Rheumatoid factor (produced by B cells) - sensitivity 50-80%; specificity 70-80%.
Anti-cyclic citrullinated peptide (CCP) - sensitivity 60-70%; specificity 90-99%.
14
Q
What clinical findings of rheumatoid arthritis can be seen on plain xrays?
A
Soft tissue swelling.
Periarticular osteopenia.
Erosions.
There may be absence of findings in early disease.
15
Q
What clinical findings of rheumatoid arthritis can be seen by ultrasound?
A
Increased sensitivity for synovitis in early disease.
Can detect up to 7 times more MCP joint erosions than plain xray in early RA.
16
Q
What clinical findings of rheumatoid arthritis can be seen by MRI?
A
Bone marrow oedema on MRI associated with inflammatory joint disease.
Integrity of tendons can be assessed.
Can distinguish synovitis from effusions.
Can detect erosions earlier.
Can be used to monitor disease activity..
17
Q
What is the management of rheumatoid arthritis?
A
Early recognition and diagnosis.
Early treatment by DMARDs for all patients with RA.
Importance of tight control with target of remission or low disease activity.
Use of NSAIDs and steroids only as adjuncts.
Steroids serve as a bridge between diagnosing the patient and starting the immunosuppressants as they can take up to 8 weeks to work.
Patient education.
18
Q
Why is regular blood monitoring needed for patients on DMARDs?
A
They cause bone marrow suppression.
Can lead to infection, liver function derangement, pneumonitis (if on methotrexate).
19
Q
When would a patient with rheumatoid arthritis be put on a biologic agent?
A
Failure to respond to 2 DMARDs including Methotrexate and DAS 28 greater than 5.1 on two occasions 4 weeks apart.
20
Q
When would you use steroids as a treatment for rheumatoid arthritis?
A
As a bridging therapy and for flares only.
21
Q
What is osteoarthritis?
A
Articular cartilage thinning or loss (loss of joint space seen on xray).
22
Q
What are the risk factors for cartilage loss in osteoarthritis?
A
Age.
Female versus male sex.
Obesity.
Previous injury (occupation, sports activities).
Muscle weakness.
Proprioceptive deficits.
Genetic elements.
Acromegaly.
Joint inflammation.
Crystal deposition in cartilage.
23
Q
What is the pathogenesis of osteoarthritis?
A
Cartilage breaks down by initially becoming thinner and then crack occur on the surface.
Gaps in the cartilage expand until they reach the bone.
Synovial fluid leaks into the cracks causing further damage and can lead to cysts in the bone and other deformities.
24
Q
What types of osteoarthritis are there?
A
Idiopathic.
Secondary (previous injury, calcium crystal deposition disease, rheumatoid arthritis, etc.).
25
What joints are normally affected by osteoarthritis?
26
What is the clinical presentation of osteoarthritis?
Pain (worse on activity, relieved by rest; mechanical pain).
Stiffness (usually morning stiffness lasting less than 30 mins and can occur after periods of inactivity).
27
What signs are found on examination of osteoarthritis?
Crepitus.
Joint swelling - bony enlargements due to osteophytes.
Joint tenderness.
Joint effusion - in late cases.
28
What are Heberden's nodes?
Hard bony swellings that can develop in the DIP joints.
29
What are Bouchard's nodes?
Hard bony swellings of the PIP joints.
30
How is osteoarthritis diagnosed?
Clinical (history/exam) and radiographs.
31
What radiographic findings suggest osteoarthritis?
Loss of joint space.
Subchondral sclerosis.
Subchondral cysts.
Osteophytes.
32
What is the non-pharmacologic management of osteoarthritis?
Physiotherapy - muscle strengthening, proprioceptive.
Weight loss.
Exercise.
Trainers.
Walking stick.
Insoles.
33
What is the pharmacologic management of osteoarthritis?
Analgesia – paracetamol, compound analgesics, topical anagesia.
NSAIDs - topical/systemic, may give additional symptomatic relief, consider risk/benefit ratio.
Pain modulators – tricyclics (amitriptyline), anti-convulsants (Gabapentin).
Intra-articular – Steroids, (Hyaluronic acid).
34
What is the surgical management of osteoarthritis?
Arthroscopic washout, loose body, soft tissue trimming.
Joint replacement.
35
What is gout?
Inflammation in the joint triggered by uric acid crystals.
36
What can cause hyperuricaemia from increased urate production?
Inherited enzyme defects.
Myeloproliferative/Lymphoproliferative disorders.
Psoriasis.
Haemolytic disorders.
Alcohol (beer, spirits).
High dietary purine intake (red meat, seafood, corn syrup).
37
What can cause hyperuricaemia from reduced urate excretion?
Chronic renal impairment.
Volume depletion e.g. heart failure.
Hypothyroidism.
Diuretics.
Cytotoxics, e.g. cyclosporin.
38
What is the gender ration of gout?
UK = male:female = 4:1.
USA = male:female = 9:1.
39
What is the classical clinical presentation of acute gout?
Monoarthropathy of the 1st MTP\>ankle\>knee.
40
How long will it take for gout to settle without treatment?
About 10 days.
41
How long will it take for gout to settle with treatment?
About 3 days.
42
What is chronic tophaceous gout?
Chronic joint inflammation often diuretic associated.
High serum uric acid.
Tophi present.
May get acute attacks.
43
What investigations will suggest a diagnosis of gout?
Raised inflammatory markers.
Serum uric acid raised (may be normal during acute attack).
Synovial fluid polarising microscopy.
Renal impairment (may be cause or effect).
Xrays (acute attack will be normal).
44
What will uric acid crystals appear as under polarising microscopy?
Needle-shaped, negatively birefringent crystals.
45
What is the acute treatment for gout?
NSAIDs.
Colchicine.
Steroids.
46
What is the prophylaxis treatment for gout?
Allopurinol.
Febuxostat.
*Start 2-4 weeks after an acute attack.*
47
When would you give someone prophylaxis treatment for gout?
If a patient has had more than 2 attacks in a year.
48
What are the types of calcium pyrophosphate deposition disease?
Calcium pyrophosphate crystals (pseudogout).
Calcium hydroxyapatite crystals (Milwaukee shoulder).
49
What joints are affected by calcium pyrophosphate deposition disease?
It affects the fibrocartilage in the knees, wrists and ankles.
50
What would calcium pyrophosphate crystals look like under polarising microscopy?
Envelope-shaped, mildly positively birefringent.
51
What is the treatment of pseudogout?
NSAIDs.
Colchicine.
Steroids.
Rehydration.
52
Why does hydroxyapatite crystal deposition cause acute and rapid deterioration?
It causes release of collagenases, serine proteinases and IL-1.
53
What is the treatment for calcium hydroxyapatite deposition?
NSAIDs.
Intra-articular steroid injection.
Physiotherapy.
Partial/total arthroplasty.
54
What is soft tissue rheumatism?
General term used to describe pain that is caused by inflammation/damage to ligaments, tendons, muscles or nerves near a joint rather than either the bone or cartilage.
## Footnote
*Pain should be confined to a specific site e.g. shoulder, wrist, etc.*
55
What structure is affected by soft tissue rheumatism of the neck?
Muscular - usually self-limiting.
56
What structure is affected by soft tissue rheumatism of the shoulder?
Commonest area for soft tissue pain which could be from:
* Adhesive Capsulitis.
* Rotator cuff tendinosis.
* Calcific tendonitis.
* Impingement.
* Partial rotator cuff tears.
* Full rotator cuff tears.
57
What soft tissue rheumatisms can occur in the elbow?
Medial and lateral epicondylitis - cubital tunnel syndrome.
58
What soft tissue rheumatisms can occur in the wrist?
De-Quervains tenosynovitis - carpal tunnel syndrome.
59
What soft tissue rheumatisms can occur in the pelvis?
Trochanteric.
Iliopsoas.
Ischiogluteal.
Bursitis and stress enthesopathies.
60
What soft tissue rheumatisms can occur in the foot?
Plantar fasciitis.
61
What investigations can aid diagnosis of soft tissue rheumatism?
Tests usually unnecessary.
X-ray (shows calcific tendonitis).
MRI if it fails to settle.
Identify precipitating factors.
62
What treatment is used for soft tissue rheumatism?
Pain control.
Rest and ice compressions.
Physiotherapy.
Steroid injections.
Surgery.
63
What are the features of joint hypermobility?
Presents with arthralgia.
Premature osteoarthritis.
Investigations normal.
64
60 year old patient who is experiencing knee pain for 5 years seen by GP who organised xray. Can it differentiate osteoarthritis from rheumatoid arthritis?
Yes.
65
Is hyperuricaemia a prerequisite for gout?
Yes.
66
50 year old lady who is otherwise fit and well, presents with pain in hip when walking for 1 year. Examinations show relatively normal range of movement in the hip. What is the best treatment option?
NSAIDs and physiotherapy.
67
75 year old lady presented with acute swelling of the knee and therefore, unable to walk. O/E, patient is pyrexial and looking slightly unwell. Knee is markedly swollen, skin over the joint is red and joint is hot to touch. Range of movement is markedly reduced. What is the most likely diagnosis?
Septic arthritis.
68
What are connective tissue diseases?
Spontaneous overactivity of the immune system that produces specific auto-antibodies.
69
What is SLE?
A systemic autoimmune disease that can affect any part of the body, where the immune system attacks the body's cells and tissue damage.
Antibody-immune complexes precipitate and cause a further immune response.
70
What is the gender ratio of lupus?
Females:males = 9:1.
71
What are the risk factors for SLE?
Increased oestrogen exposure (early menarche, on oestrogen containing contraceptives and HRT).
Smoking.
Genetic predisposition.
72
SLE is more common in which ethnic populations?
African-American, African-Caribbean, Asian.
73
What is the pathogenesis of SLE?
Loss of immune regulation.
Increased and defective apoptosis.
Necrotic cells release nuclear material which act as auto-antigens.
Speed of clearance of dead cells is reduced allowing internal cellular components to be exposed to immune system for longer.
Autoimmunity results from exposure to nuclear and intracellular auto-antigens.
B and T cells stimulated.
Auto-antibodies produced.
74
How does renal disease occur in SLE?
Deposition of immune complexes (nuclear antigens and anti-nuclear antib odies) in mesangium.
Activate complement which attracts leukocytes -\> release cytokines.
Cytokine release perpetuates inflammation -\> necrosis and scarring.
75
What pathogenesis drives SLE disease?
Immune complex formation.
76
What renal investigations are done if you suspect renal involvement of SLE?
Urinalysis.
Renal biopsy.
77
What are features of anti-phospholipid syndrome?
Venous and arterial thrombosis.
Recurrent miscarriage.
Livido reticularis.
Association with other autoimmune conditions especially SLE..
Thrombocytopenia.
Prolonged APTT.
78
What test is highly sensitive to SLE but not specific?
Anti-nuclear antibody titre.
79
Which autoantibody is most specific to SLE?
Anti-dsDNA.
80
What autoantibody correlates with disease activity in SLE?
Anti-dsDNA antibody.
81
What autoantibody is associated with congenital heart block and neonatal LE when it crosses the placenta during pregnancy?
Anti-Ro antibody.
82
What autoantibodies are associated with anti-phospholipid syndrome?
Anti-cardiolipin antibody.
Lupus anticoagulant.
Anti-beta 2 glycoprotein.
83
84
What other investigations may need to be carried out in someone suspected of organ involvement in SLE?
CXR.
Pulmonary function tests.
CT chest.
Urine protein quantification.
Renal biopsy.
Echocardiogram.
Nerve conduction studies.
MRI brain.
85
What result of SLE would make you most suspicious of a disease flare?
Fall in C4 level.
86
How would disease activity of SLE be monitored?
Thorough clinical assessment.
Anti-dsDNA level positively correlates with activity.
C3/C4 levels negatively correlate with activity.
Urine examination including protein, cells and casts.
Full blood count.
Blood biochemistry.
87
What drug treatment should be given for SLE?
NSAIDs and simple analgesia.
Hydroxychloroquine - all SLE patients should have this.
Steroids - for flares only.
Immunosuppressives - moderate/severe disease.
Biologics.
88
What is the most appropriate first investigation for someone with SLE?
Urinalysis.
89
What treatment must all patients with SLE be on?
Hydroxychloroquine.
90
What is Raynaud's?
Vasospasm in response to cold weather.
91
What are the phases of Raynaud's?
**Ischaemia** - blood flow capillaries impeded; hands must blanche.
**Cyanosis** - capillaries and venules dilate in response to ischaemia and are filled with deoxygenated blood.
**Rubor** - capillaries are still dilated but are now carrying oxygenated blood.
92
What is primary Raynaud's?
Seen in teenagers with no underlying autoimmune disease; benign.
93
What is secondary Raynaud's?
Older age group, underlying autoimmune disease.
94
What is the treatment for Raynaud's?
Keep warm.
Vasodilators - calcium channel blockers, PDE5 inhibitors such as sildenafil.
Treatment for digital ulcers - prostacyclin analogues (Iloprost), endothelin receptor antagonists (Bosentan), botox injections.
95
What feature of systemic sclerosis do 90% of patients present with?
Raynaud's.
96
What is diffuse cutaneous systemic sclerosis?
Skin involvement on extremities above and below the elbows and knees (plus face and trunk).
97
What is limited cutaneous systemic sclerosis?
Skin involvement on extremities and only below elbows and knees (plus face).
98
What is the pathogenesis of systemic sclerosis?
Changes in blood vessels.
Chronic inflammation causing vasculopathy, autoimmunity and tissue fibrosis.
99
What is the acronym for the limited symptoms of systemic sclerosis?
**CREST**:
**C**alcinosis.
**R**aynaud's phenomenon.
o**E**sophageal dysfunction.
**S**clerodactyly.
**T**elangiectasia.
100
What is calcinosis?
Calcium deposits in the skin.
101
What is sclerodactyly?
Thickening and tightening of the skin on the fingers and hands.
102
What is telangiectasia?
Dilation of capillaries causing red marks on the skin surface.
103
What are the structural changes in the glomerular in a systemic sclerosis renal crisis?
They are due to fibrosis and not inflammation.
104
What is the management for systemic sclerosis?
Yearly ECHO and pulmonary function tests.
Treat Raynaud's and digital ulcers.
Treat reflux with PPI +/- ranitidine +/- Gaviscon.
If pulmonary fibrosis - immunosuppression.
If rapidly progressive skin involvement - immunosuppression.
If pulmonary hypertension - prostacyclin analogues, endothelin receptor antagonists, PDE5 inhibitors.
Tight control of BP - ACE inhibitors.
105
What is Sjogren's syndrome?
Characterised by lymphocytic infiltration of the exocrine glands (tear ducts, salivary glands, skin dryness, vaginal dryness).
106
What are the features of Sjogren's syndrome?
Blepharitis (edges of eyelids become inflamed).
Salivary gland inflammation.
Tooth decay.
Lymphoma (at high risk).
Dry cough.
Multisystem involvement.
107
What autoantibodies are associated with Sjogren's?
Anti-nuclear antibody.
Anti-Ro, anti-La.
108
If you suspect someone has Sjogren's syndrome and their antibodies are negative, what would you do next?
Salivary gland ultrasound and biopsy.
109
What is the treatment for Sjogren's syndrome?
Artificial tears, salivary supplements and vaginal lubricants.
Good dental hygiene - strong fluoride toothpaste.
Hydroxychloroquine for fatigue and arthralgia.
Immunosuppression for organ involvement.
110
What is spondyloarthropathy?
Family of inflammatory arthritides characterized by involvement of both the spine and joints, principally in genetically predisposed (HLA B27 positive) individuals.
111
What conditions is HLA B27 associated with?
Ankylosing spondylitis.
Reactive arthritis.
Crohn's disease.
Uveitis.
Psoriasis.
112
What are the subgroups of spondyloarthropathies?
Ankylosing spondylitis.
Psoriatic arthritis.
Reactive arthritis.
Enteropathic arthritis.
113
What's the difference in characteristics between mechanical and inflammatory pain?
Mechanical - worsened by activity, typically worst at end of day, better with rest.
Inflammatory - worse with rest, better with activity, significant early morning stiffness (\>30 minutes).
114
What are the shared rheumatological features of the spondyloarthropathies?
Sacroiliac and spinal involvement.
Enthesitis.
Inflammatory arthritis (oligoarticular, asymmetric, predominantly lower limb).
Dactylitis.
115
What is enthesis?
Site of insertion of a tendon, ligament or articular capsule into bone.
116
What is enthesitis?
Inflammation at the insertion of tendons into bones e.g. Achilles tendinitis, plantar fasciitis.
117
What is dactylitis?
Inflammation of the entire digit.
118
What are the shared extra-articular features of spondyloarthropathies?
Ocular inflammation - anterior uveitis, conjunctivitis.
Mucocutaneous lesions.
Rare aortic incompetence or heart block.
No rheumatoid nodules.
119
What is ankylosing spondylitis?
Chronic systemic inflammatory disorder that primarily affects the spine.
Hallmark = sacroiliac joint involvement.
120
What are the clinical features of ankylosing spondylitits?
Back pain (neck, thoracic and lumbar).
Enthesitis.
Peripheral arthritis (shoulders, hips) - rare.
Anterior uveitis.
Cardiovascular involvement (aortic valve/root).
Pulmonary involvement (fibrosis upper lobes).
Asymptomatic enteric mucosal inflammation.
Neurological involvement.
Amyloidosis.
121
How is ankylosing spondylitis diagnosed?
History.
Examination (tragus/occiput to wall, chest expansion, modified Schober test).
Bloods (inflammatory markers, HLA B27).
X-rays (sacroiliitis, syndesmophytes, bamboo spine).
122
What is the difference between syndesmophytes and osteophytes?
Syndesmophytes tend to link with one another whereas osteophytes tend to poke out.
123
What early sign of ankylosing spondylitis can be seen on MRI?
Bone marrow oedema.
124
What is the treatment for ankylosing spondylitis?
Physiotherapy.
Occupational therapy.
NSAIDs.
DMARDs.
Anti-TNF treatment.
Anti-IL-17 (Secukinumab) - newest licensed treatment.
125
What is psoriatic arthritis?
Inflammatory arthritis associated with psoriasis.
*10-15% patients can have psoriatic arthritis without psoriasis.*
RF negative and no rheumatoid nodules.
126
What are the clinical features of psoriatic arthritis?
Inflammatory arthritis (5 subgroups/presentations).
Sacroiliitis (often asymmetric and may be associated with ankylosing spondylitis).
Nail involvement e.g. pitting, onycholysis.
Dactylitis.
Enthesitis (Achilles tendonitis, plantar fasciitis).
Eye disease.
127
What are the clinical subgroups of psoriatic arthritis?
Confined to distal interphalangeal joints (DIP) hands/feet.
Symmetric polyarthritis (similar to RA).
Spondylitis (spine involvement) with or without peripheral joint involvement.
Asymmetric oligoarthritis with dactylitis.
Arthritis mutilans.
128
How is psoriatic arthritis diagnosed?
History/exam.
Usually FH of psoriasis).
Blood (inflammatory markers - raised; negative rheumatoid factor).
Xrays - marginal erosions and whiskering; pencil in cup deformity; osteolysis; enthesitis.
129
What is the treatment for psoriatic arthritis?
NSAIDs.
Corticosteroids.
DMARDs.
Anti-TNF in severe disease unresponsive to DMARDs.
Secukinumab.
*Physiotherapy, occupational therapy, orthotics, chiropodist.*
130
What is reactive arthritis?
Infection-induced systemic illness characterised primarily by an inflammatory synovitis from which viable microorganisms can' be cultured.
131
If microorganisms can be cultured from an arthritic joint, what is the name for this arthritis?
Septic arthritis.
132
What is Reiter's syndrome?
A form of reactive arthritis that involves urethritis, conjunctivitis/uveitis/iritis, arthritis.
133
What are the clinical features of reactive arthritis?
General symptoms - fever, fatigue, malaise.
Asymmetrical monoarthritis oligoarthritis.
Enthesitis.
Mucocutaneous lesions (keratoderma blenorrhagica, circinate balanitis, painless oral ulcers, hyperkeratotic nails.
Ocular lesions - uni/bilateral conjunctivitis, iritis.
Visceral manifestations - mild renal disease, carditis.
134
How is reactive arthritis diagnosed?
History/exam.
Bloods - inflammatory markers, FBC, U&Es, HLA B27 (rarely necessary).
Cultures - blood, urine, stool.
Joint fluid analysis - rule out infection.
Xray of affected joints.
Ophthalmology review.
135
What is the treatment for reactive arthritis?
90% of cases resolve spontaneously within 6 months.
Treat with NSAIDs, corticosteroids if needed, antibiotics for underlying infection.
If resistant/chronic treat with DMARDs.
Physiotherapy/occupational therapy.
136
What is enteropathic arthritis?
Associated with inflammatory bowel disease e.g. Crohn's, ulcerative colitis.
9-20% of patients with IBD will develop this arthritis.
137
What is the clinical presentation of enteropathic arthritis?
Arthritis in several joints, especially the knees, ankles, elbows and wrists.
Sometimes also in the spine, hips or shoulders.
*20% of Crohn's patients will have sacroiliitis.*
138
What are the clinical symptoms of enteropathic arthritis?
Loose, watery stool with mucous and blood.
Weight loss, low-grade fever.
Uveitis.
Pyoderma gangrenosum.
Enthesitis (Achilles tendonitis, plantar fasciitis, lateral epicondylitis).
Apthous ulcers.
139
How is enteropathic arthritis diagnosed?
Upper and lower GI endoscopy with biopsy showing ulceration/colitis.
Joint aspirate - no organisms or crystals.
Raised inflammatory markers - CRP, PV.
Xray/MRI showing sacroiliitis.
USS showing synovitis/tenosynovitis.
140
What is the treatment for enteropathic arthritis?
Treat the IBD in order to control the arthritis.
NSAIDs are not a good idea as they exacerbate IBD.
Normal analgesia.
Steroids.
DMARDs.
Anti-TNF if required.
141
What are the medical treatments for spondyloarthropathies?
NSAIDs.
Corticosteroids/joint injections.
Topical steroid eye drops.
DMARDs (methotrexate, sulfasalazine, lefunomide).
Anti-TNF in severe disease unresponsive to NSAIDs and methotrexate (infliximab, etanercept, adalimumab, golimumab, certolizumab).
Secukinumab (anti-IL17) only for psoriatic arthritis and ankylosing spondylitis.
142
What is vasculitis?
Inflammation of blood vessels, often ischaemia, necrosis and organ inflammation.
143
What is primary vasculitis?
Results from an inflammatory response that targets the vessel walls and has no known cause.
Sometimes this is autoimmune.
144
What is secondary vasculitis?
May be triggered by an infection, drugs, toxins or may occur as part of another inflammatory disorder or cancer.
145
What is the pathogenesis of vasculitis?
Dendritic cells activated and release inflammatory cytokines -\> activate T cells and vascular inflammation.
Activated T cells promote inflammation, granuloma formation and macrophage activation and differentiation.
Activated macrophages produce mediators that cause progressive vascular inflammation, endothelial damage, disruption of the internal elastic lamina and hyperplasia of the intima.
146
What is the main pathogenesis of large vessel vasculitis?
Granulomatous infiltration of the walls of the large vessels.
147
What are the presenting features of large vessel vasculitis?
Bruit (commonly the carotid artery).
Blood pressure difference of extremities.
Claudication.
148
What is a main consequence of temporal (giant cell) arteritis?
Risk of blindness due to ischaemia of the optic nerve.
149
What investigations aid diagnosis of temporal arteritis?
ESR, plasma viscosity and CRP are raised.
Temporal artery biopsy (remember that skip lesions occur so the biopsy may be negative).
MR angiogram or PET CT.
150
What is the management of giant cell arteritis?
40-60mg prednisolone (start patient on steroids before biopsy result comes back).
Steroid-sparing agents (e.g. methotrexate) may be considered.
151
What is the pathology of granulomatosis with polyangiitis (Wegener's granulomatosis)?
Granulomatous inflammation of the respiratory tract, small and medium vessels.
Necrotising glomerulonephritis is common.
152
What is the pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
Eosinophilic granulomatous inflammation of the respiratory tract, small and medium vessels.
Associated with asthma.
153
What is the pathology of microscopic polyangiitis?
Necrotising vasculitis with few immune deposits.
Necrotising glomerulonephritis is very common.
154
What are the ENT features of granulomatosis with polyangiitis?
Sinusitis.
Nasal crusting.
Epistaxis.
Mouth ulcers.
Sensorineural deafness.
Otitis media and deafness.
"Saddle nose" due to cartilage ischaemia.
155
What are the respiratory features of granulomatosis with polyangiitis?
Pulmonary infiltrates.
Cough.
Haemoptysis.
Diffuse alveolar haemorrhage.
Cavitating nodules on CXR.
156
What are the cutaneous features of granulomatosis with polyangiitis?
Palpable purpura.
Cutaneous ulcers.
157
What are the renal features of granulomatosis with polyangiitis?
Necrotising glomerulonephritis.
158
What are the nervous system features of granulomatosis with polyangiitis?
Mononeuritis multiplex.
Sensorimotor polyneuropathy.
Cranial nerve palsies.
159
What are the ocular features of granulomatosis with polyangiitis?
Conjunctivitis.
Episcleritis.
Uveitis.
Optic nerve vasculitis.
Retinal artery occlusion.
Proptosis.
160
pANCA is found in which small/medium vessel vasculitis?
Eosinophilic granulomatosis with polyangiitis.
Microscopic polyangiitis.
161
cANCA is found in which small/medium vessel vasculitis?
Granulomatosis with polyangiitis.
162
What is Henoch-Schonlein purpura?
An acute IgA-mediated disorder.
Generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints and, rarely, the lungs and CNS.
~75% cases occur in children aged 2-11.
163
What is associated with onset of Henoch-Schonlein?
More than 75% of patients have had a preceding URTI, pharyngeal infection or GI infection.
Most common is group A streptococcus.
Preceding illness usually predates HSP by 1-3 weeks.
164
What is the presentation of Henoch-Schonlein purpura?
Purpuric rash typically over the buttocks and lower limbs.
Colicky abdominal pain.
Bloody diarrhoea.
Joint pain +/- swelling.
Renal involvement (~50% of cases).
165
What is the management of Henoch-Schonlein?
Usually self-limiting.
Symptoms tend to resolve within 8 weeks.
Relapses may occur for months to years.
Essential to perform urinalysis to screen for renal involvement.
166
What are the major causes of myopathy?
Inflammatory.
Endocrine disorders.
Electrolyte disorders.
Metabolic myopathies.
Drugs and toxins.
Infections.
Rhabdomyolysis.
167
What is the histology of polymyositis and dermatomyositis?
Muscle fibre necrosis.
Degeneration.
Regeneration.
Inflammatory cell infiltrate.
168
What are the clinical features of polymyositis and dermatomyositis?
Muscle weakness of insidious onset, worsening over months - usually symmetrical, proximal muscles.
Often have specific problems e.g. difficulty brushing hair, climbing stairs.
Myalgia in 25-30% - usually mild.
169
What are the features of dermatomyositis?
Cutaneous disease.
Gottron's sign.
Heliotrope rash.
Shawl sign.
Lung involvement - interstitial disease, respiratory muscle weakness.
Oesophageal - dysphagia.
Cardiac - myocarditis.
Other - fever, weight loss, Raynaud's phenomenon, non-erosive polyarthritis.
170
What tests would you ask the patient to do if you suspected polymyositis or dermatomyositis?
Confrontational testing - direct testing of power.
Isotonic testing - 30 seconds sit to stand test.
171
What investigations would you do to diagnose polymyositis or dermatomyositis?
Blood tests - muscle enzymes (creatine kinase), inflammatory markers, electrolytes, calcium, PTH, TSH.
Auto-antibodies - ANA, anti-Jo-1.
Electromyography - increased fibrillations, abnormal motor potentials, complex repetitive discharges.
Muscle biopsy (definitive test) - perivascular inflammation and muscle necrosis.
MRI - muscle inflammation, oedema, fibrosis and calcification.
172
What is the treatment for polymyositis and dermatomyositis?
Glucocorticoids.
Azathioprine.
Methotrexate.
Ciclosporin.
IV Ig.
Rituximab.
173
What is the most definitive test for polymyositis?
Muscle biopsy.
174
What drug may cause a presentation similar to myositis?
Atorvastatin.
175
What are the clinical manifestations of polymyalgia rheumatica?
Ache in shoulder and hip girdle.
Morning stiffness.
Usually symmetrical.
Fatigue, anorexia, weight loss and fever may occur.
Reduced movement of shoulders, neck and hips.
Muscle strength is normal.
176
What are the clinical manifestations of fibromyalgia?
Diffuse and chronic pain - neck, shoulders, lower back, chest wall.
Varies in intensity.
Symptoms worse with exertions, fatigue and stress.
Sensation of swelling.
Fatigue and poor, unrefreshing sleep.
Pins and needles/tingles, headaches, depression, abdominal pain (IBS), poor concentration and memory.
177
What are the clinical findings of fibromyalgia?
Excessive tenderness on palpation of soft tissues.
Need 11/18 tender points for diagnosis.
No other abnormality of musculoskeletal system.
178
What is the treatment for fibromyalgia?
Patient education.
Graded exercise programme.
Cognitive behavioural therapy.
Complementary medicine e.g. acupuncture.
Anti-depressants e.g. tricyclics, SSRIs.
Analgesia.
Gabapentin and pregabalin.
179
A 63 year old man presents with onset of pain and stiffness around his shoulders and hips. Ongoing for around 3 weeks. He now needs help to get out of bed in the morning. Inflammatory markers raised, creatine kinase normal. What is the most likely diagnosis and how should he be managed?
Polymyalgia rheumatica.
Prednisolone 15mg reducing course over 18 months.
180
A 64 year old man has been noticing a heavy feeling in his arms for about 6 months. He is finding it increasingly tiring climbing the stairs. Inflammatory markers raised. Creatine kinase raised. EMG suggests myositic change in the proximal muscles. Biopsy shows changes characteristic of dermatomyositis. What should the treatment be?
Prednisolone 40mg.
181
What are the main side effects of NSAIDs?
Peptic ulceration.
Renal impairment.
Increased cardiovascular event risk - on long-term use.
Exacerbation of asthma.
182
In a patient with newly diagnosed rheumatoid arthritis, what is the best first line treatment?
Methotrexate.
183
Ideally, when should DMARD therapy be started in rheumatoid arthritis?
Within 3 months of symptoms starting.
184
Which DMARD is safe in pregnancy?
Sulfasalazine.
185
Which are more effective DMARDs or biologics?
**Biologics**, however, they are not available for everyone, are more expensive than DMARDs don't work for everyone and have many side effects.
186
What are the side effects of anti-TNF therapy?
Increased risk of infection, skin cancer, re-activation of latent tuberculosis and exacerbation of heart failure.
187
What can be used to treat an acute flare of gout?
NSAIDs (e.g. naproxen).
Steroids (e.g. oral prednisalone, intramuscular or intra-articular steroid).
Colchicine.
188
What are the main side effects of steroids?
They can cause bone density loss.
They may contribute to the development of diabetes.
They make you fat.
