Rheumatology Flashcards
What are some causes of acute monoarthritis?
Infection - Staph Aureus/ Streptococcus
Crystal - Gout, Pseudogout
Trauma - Haemarthrosis
What are some causes of chronic monoarthritis?
Infective - TB infection
Inflammatory -
Psoriatic Arthritis
Reactive Arthritis
Foreign body
Non-inflammatory -
Osteoathritis
Trauma e.g meniscal tear
Osteonecrosis
Neuropathic e.g Charcot’s
Tumour
What are some causes of acute polyarthritis?
Inflammatory -
Rheumatoid Arthritis
Psoriatic Arthritis
Reactive Arthritis
Autoimmune - SLE, Vasculitis
Viral - HIV, Parvovirus , Chikungunya
Crystal - Uncontrolled Gout
What are some causes of chronic ( >3 months ) polyarthritis?
Inflammatory - RA, PsA, Reactive Arthritis
Autoimmune - SLE, Vasculitis
Crystal - Uncontrolled Gout
What are some causes of arthritis in the DIPJs ?
Psoriatic Arthritis - nail dystrophy on affected digit
Osteoarthritis - most common ( Heberden’s nodes )
If stiffness in the morning lasts longer than 30 minutes what is it likely to be caused by?
Inflammatory Arthritis
e.g Rheumatoid Arthritis or Psoriatic Arthritis
WHat is spinal stiffness in the early hours of the morning indicative of?
Ankylosing Spondylitis
In what condition is “ Inactivity Gelling” common in?
Osteoarthritis
What conditions do you get dry mouth and/or eyes in?
Sjogren’s syndrome
Rheumatoid Arthritis
What conditions do you get mouth ulcers in?
SLE
Reactive Arthritis
IBD
What condition do you get Iritis in?
Spondyloarthropathies
What conditions do you get Scleritis in?
Rheumatoid Arthritis
Granulomatosis with Polyangiitis ( GPA )
What conditions do you get visual disturbance in?
Giant Cell Arteritis ( Temporal Arteritis )
SLE ( retinal vasculitis )
What condition do you get Psoriatic plaques and dystrophic nails in?
Psoriatic Arthritis
What conditions do you get Raynaud’s Phenomenon?
SLE
Sjogren’s Syndrome
Scleroderma
What conditions do you get Telangiectasis, Calcinosis and Sclerodactyly in?
Scleroderma
What is Telangiectasis ?
Dilation of capillaries causing them to appear as small red or purple clusters
What is calcinosis?
Deposits of calcium in subcutaneous tissue, muscles and visceral organs
What is Sclerodactyly?
Localized thickening and tightness of the finger skin that cause a claw like appearance and loss of mobility
What deformities occur in Rheumatoid arthritis?
Swan-neck deformity
Boutonniere deformity
Rheumatoid nodules
In what condition do you get Tophi in?
Gout
What are tophi?
Deposits of monosodium urate crystals under the skin
In what conditions do you get digital ulcers?
SLE
Vasculitis
Scleroderma
In what condition do you get malae rash and photosensitivity?
SLE
In what condition do you get Erythema Nodosum?
Acute Sarcoidosis
TB
Streptococcal infections
What is Erythema Nodosum?
Tender red bumps, usually found symmetrically on the shins
In what condition do you get Livedo Reticularis?
SLE
What is Livedo Reticularis?
Netlike pattern of reddish-blue discolouration , looks like mottled skin
In what autoimmune condition do you get alopecia?
SLE
What is alopecia?
Immune system attacks hair follicles leading to bald patches
In what conditions can you get Renal Failure and Hypertension?
SLE
Vasculitis
In what condition can you get renal stones?
Gout
In what conditions can you get Microscopic haematuria +/- proetinuria?
SLE
Vasculitis
In what condition do you get vaginal/urethral discharge?
Reactive or Gonococcal Arthritis
In what condition are you more prone to miscarriages and Pre-Eclampsia ?
SLE
In what condition do patients present with headaches?
Giant Cell Arteritis
What neurological symptoms are you more likely to get with SLE?
Seizures
Headaches
Psychosis
TIA/CVA
What neurological symptoms can patients with Vasculitis have?
Seizures
Mononeuritis Multiplex
What in Mononeuritis Multiplex?
A type of peripheral neuropathy when there is damage to at least two different areas of the peripheral nervous system.
Can cause tingling, numbness, pain and paralysis.
What symptom affecting nerves can a patient with Rheumatoid Arthritis present with?
Carpal Tunnel Syndrome
What are the tests to see if a patient has Carpal Tunnel Syndrome?
Tinel’s Test
Phalen’s Test
What is Tinel’s Test?
Gently tap over the wrist just below the palm
Positive test = if patient feels a tingling , burning or numb sensation in thumb, index, middle and ring fingers
What is Phalen’s Test
Ask patient to place the backs of their hands together at 90 degree angle for 60 seconds
Positive test = tingling, burning or numb sensation in thumb, index, middle and ring fingers
What is Carpal Tunnel Syndrome?
Compression of the median nerve as it traverses through the wrist via the carpal tunnel
Symptoms are pain and paresthesia in the median nerve distribution ( thumb, index, middle and lateral half of ring finger ) Grip weakness can also develop as median nerve supplies thenar muscles
Which condition presents with peripheral neuropathy?
Polymyositis
What respiratory conditions can SLE present with?
Pleural Effusion
Pericardial Effusion
Alveolitis
Pulmonary Hypertension
Pleuritic Chest Pain
What Respiratory conditions can Rheumatoid Arthritis cause?
Pleural Effusion
Alveolitis
What respiratory conditions/symptoms can Granulomatosis with Polyangiitis causes?
Pulmonary Infiltration
Haemoptysis
Sinusitis
What respiratory condition can Scleroderma present with?
Pulmonary Hypertension
What respiratory symptom can Churg-Straus syndrome ( Eosinophilic Granulomatosis with Polyangiitis) present with?
Wheeze
What is Churg-Strauss Syndrome?
Also known as Eosinophilic Granulomatosis with Polyangiitis
Inflammatory disease of small and medium sized blood vessels. Has very high Eosinophil count
What is high on an FBC count with Churg-Strauss Sydrome?
Eosinophils
How can Antiphospholipid syndrome affect blood vessels?
Can cause recurrent thromboembolism ( DVT/PE )
What condition can cause recurrent cytopenia?
SLE
What is Cytopenia?
When one or more of your blood cells types is low ( RBCs, WBCs, Platelets )
What gastrointestinal symptoms can Scleroderma present with?
Indigestion
Constipation
What conditions can present with diarrhoea and bloody stools?
Reactive Arthritis
Ankylosing Spondylitis
Enteropathic Arthritis
What conditions can present with weight loss?
Any inflammatory condition ( RA, AS SLE, Scleroderma etc )
Neoplasia
What causes raised platelets?
Inflammation
Bleeding
What causes decreased platelets?
SLE
What causes neutrophilia?
Inflammation
Sepsis
Prednisolone usage
Infection
Stress
Vigorous exercise
What causes neutropenia?
SLE
DMARD toxicity
Post infective
Chemotherapy
What causes a decrease of lymphocytes?
SLE
DMARD usage
What condition causes raised Uric Acid?
Gout
What causes decreased Uric Acid?
Inflammation
In what condition are CK, ALT and LDH raised?
Myositis
What autoantibodies are raised in SLE?
Anti-dsDNA
Anti RO
Anti-LA
Which autoantibody rises with disease activity in SLE?
Anti-dsDNA
Which complement proteins fall in SLE flares?
C3 and C4
What autoantibodies are raised in Sjogren’s?
Anti-RO
Anti-LA
What autoantibodies are raised in Scleroderma ( Systemic Sclerosis ) ?
Anti centomere
Anti-Scl70
What autoantibodies is raised in Polymyositis?
Anti Jo-1
What are the two Anti-neutrophil cytoplasmic antibodies that are relevant? (ANCA)
C ANCA
P ANCA
What ANCA antibodies are present in Granulomatosis with Polyangiitis ?
C ANCA
Which ANCA antibody is present in patients with Microscopic Polyangiitis?
P ANCA
What gene is strongly associated with Ankylosing Spondylitis, iritis and juvenile arthritis ?
HLA-B27
What investigation do you do for SLE and vasculitis?
Urinalysis
What investigation do you do for suspected septic arthritis?
Synovial fluid anyalsis
What investigation do you do for crystal arthropathy?
Synovial fluid analysis
What would you see on polarised light microscopy in Gout?
Negatively birefringent needle shaped crystals
What would you see on polarized light microscopy in Pseudogout?
Positive birefringent rhomboid shaped crystals
What type of biopsy do you do for GCA?
Temporal artery biopsy
What type of biopsies do you do dermatomyositis?
Muscle biopsy
Skin biopsy
What type of biopsies do you do for Vasculitis?
Skin biopsy
Renal biopsy
What type of biopsy do you do for polymyositis?
Muscle biopsy
What type of biopsies do you do for SLE?
Skin biopsy
Renal biopsy
What type of biopsy do you do for Sjogren’s?
Lip/ salivary gland biopsy
What type of biopsy is uncommonly requested but useful in Vasculitis with mononeuritis multiplex neuropathy?
Sural nerve biopsy
What is Rheumatoid Arthritis ? And what antibodies are associated with it?
Autoimmune disease associated with antibodies to Fc portion of IgG ( Rheumatoid Factor ) and anti-CCP
What is the pathogenesis of RA?
Citrullination of self antigens which are then recognized by T & B cells which can then produce antibodies (RF & anti-CCP)
Stimulated macrophages and fibroblasts release TNFa
Inflammatory cascade leads to proliferation of synoviocytes , which grows over cartilage and leads to restriction on nutrients, cartilage is damaged
Activated macrophages stimulate osteoclast differentiation , causing bone damage
What is the typical history with RA?
Female
30-50
Progressive, peripheral, symmetrical polyarthrits
What is the typical pattern of RA in the hands and feet ?
Affects MCPs/PIPs/MTPs but typically spares DIPs
What are the timing features of RA?
Hx of >6 months
Morning stiffness > 30mins
What can be seen on examination of RA?
Swelling
Tenderness
Ulnar deviation
Palmar subluxation
Swan-neck deformity
Boutonniere deformity
Rheumatoid nodules
Carpal Tunnel
Where are Rheumatoid Nodules typically found?
Elbows
What investigations are done for RA?
Bloods -
Rheumatoid Factor + Anti-CCP
FBC
Raised CRP + ESR
Radiology -
USS/MRI more sensitive in early disease
X -RAY changes apparent in established disease
May need PFTs and HRCT is chest involvement suspected
What will a FBC show in RA?
Normocytic Anaemia ( Anaemia of Chronic Disease)
Why does anaemia occur in RA?
Inflammation can prevent the body from using stored iron stores to make new RBCs
What XRAY changes are seen in RA?
L - Loss of joint space
E - Erosions
S - Soft tissue swelling
S - Subluxation
What is the initial treatment for RA?
DMARD monotherapy ( e.g. Methotrexate ) , consider combination therapy
NSAIDs - symptom control with PPI cover
Can use OT/PT
What monitoring is required when a patient is on Methotrexate ( DMARDs) long term?
FBC
LFTs - can cause liver fibrosis
U&Es - can cause renal impairment
What are the effects of long-term steroid use?
Osteoporosis
Skin thinning
Hypertension
What should be used to treat RA in a flare up?
PO / IM or intra-articular Steroids ( normally Prednisolone PO )
What should be considered after combination DMARDs if still not helping?
Biologics ( anti-TNFs e.g Infliximab, Etanercept )
What are the extra-articular flare ups or Rheumatoid Arthritis? (3Cs 3As 3Ps 3Ss )
Carpal Tunnel Syndrome
Cardiovascular Risks
Cord Compression
Anaemia
Amyloidosis
Arteritis
Pericarditis
Pleural Disease (common)
Pulmonary Disease (common)
Sjogren’s Syndrome
Scleritis/Episcleritis
Splenic Enlargement
What is Giant Cell Arteritis?
Chronic Vasculitis of large and medium sized vessels that occur among individuals over 50
Commonly affects the temporal artery
Where does GCA commonly cause inflammation?
Superficial temporal artery , other branches of External carotid artery
How can GCA present ?
Anterior Ischaemic Optic Neuropathy ( Vision Loss )
Jaw claudication
Stroke
What are risk factors for GCA?
Above 50 , most above 60
Woman
Caucasian
Strong association with Polymyalgia Rheumatica
HLA-DR4 gene
What symptoms does GCA present with?
Headache ( 70% ) - usually localized, unilateral, stabbing and located over the temple
Jaw claudication upon mastication
Amaurosis Fugax
Blindness
Diplopia
Tenderness over temple area
What is Amaurosis Fugax?
Temporary loss of blood flow to one or both eyes causing transient blindness
How is the diagnosis of GCA made?
Presence of any 2 or more of the following in patients older than 50 :
-Raised ESR, CRP or PV
-New onset or localized headache
-Tenderness or decreased pulsation of temporal artery
-New visual symptoms
-Biopsy showing necrotising arteritis
How do you treat GCA?
Prednisolone 60-100mg PO per day for at least 2 weeks
If they have visual symptoms , consider 1mg methylprednisolone IV pulse therapy for 1-3 days
Then put on low dose Aspirin long term to reduce stroke risk
What is Polymyalgia Rheumatica?
A clinical syndrome categorised by pain and stiffness of shoulder, hip and neck. Typically have difficulty rising from a chair or brushing their hair.
What age people are affected by PR?
Average onset ~70 years
What are the symptoms of PR?
New onset stiffness ( neck, shoulder, hips) in elderly
Difficulty rising from chair
Night time pain
Systemic symptoms ~25%
What will be seen on examination of a patient with PR?
Deceased ROM of neck, shoulder, hips
Strength is normal
Muscle tenderness
How is diagnosis of PR made?
Typical hx and examination
Raised inflammatory markers
Association with GCA so TA biopsy if symptomatic
What is the treatment of PR?
15mg Prednisolone PO, expect dramatic response within 5 days
Then slowly taper
If a patient relapses frequently with PR what can be used as a steroid-sparing drug?
Methotrexate
What are Spondyloarthropathies?
A group of conditions that affect the spine and peripheral joints associated with the HLA-B27 gene
What are the 4 Spondyloarthropathy conditions?
Ankylosing Spondylitis
Enteropathic Arthritis
Psoriatic Arthritis
Reactive Arthritis
What clinical features are common in the Spondyloarthropathies?
Sacroiliac/ Axial disease
Inflammatory arthropathy of peripheral joints
Enthesitis (inflammation at tendon insertions)
Extra-articular features (skin/gut/eye)
What is the usual patient with Ankylosing Spondylitis?
Young male
What are the symptoms of Ankylosing Spondylitis?
Bilateral buttock pain
Chest Wall and thoracic pain
What can be seen in developed Ankylosing Spondylitis on examination?
Loss of Lumbar Lordosis
Exaggerated Thoracic Kyphosis
Reduced chest expansion
Positive Schober’s Test
What is Schober’s Test?
- Identify the location of the posterior superior iliac spine (PSIS) on each side.
- Mark the skin in the midline 5cm below the PSIS.
- Mark the skin in the midline 10cm above the PSIS.
- Ask the patient to touch their toes to assess lumbar flexion.
- Measure the distance between the two lines.
The distance between the two marks should increase from the initial 15cm to more than 20cm, if not positive test due to reduced ROM
What investigation should be done for Ankylosing Spondlylitis?
CRP - but may be normal
MRI spine and SI joints
What is the treatment for Ankylosing Spondyliti
Symptom Control - NSAIDs
Physiotherapy
Can use TNF inhibitors, IL-17 inhibitors
What is Psoriatic Arthritis?
Happens in 10% of people with Psoriasis
What are the typical exam findings with Psoriatic Arthritis?
Oligoarthritis
Dactylitits ( sausage fingers )
Can be symmetrical or mono also
Arthritis Mutans ( 5%)
What is the typical XRAY findings in Psoriatic Arthritis?
‘Pencil in cup’ appearance
What can be seen early on MRI or USS in Psoriatic Arthritis?
Central erosions
What is the treatment for Psoriatic Arthritis?
NSAIDs
DMARDs
TNF Inhibitors
IL-17 inhibitors
IL-12/IL-23 inhibitors
How does Reactive Arthritis develop?
Its sterile synovitis after a distant infection ( Salmonella/ Shigella / Campylobacter ) or following urethrits/cervicitis ( Chylamydia )
When does Reactive Arthritis present?
Few days - 2 weeks post infection
Where does Reactive Arthritis present?
Acute asymmetrical lower limb arthritis
Can’t see can’t wee, cant climb a tree ! ( Conjunctivitis/ Uveitis, Urethritis, Arthritis )
What are skin signs of Reactive Arthritis?
Circinate Balanitis ( circular lesions on penis)
Keratoderma Blenorrhagia ( Blisters on palms and soles of feet)
What other signs can be present in Reactive Arthritis?
Conjunctivitis
Uveitis
Enthesitis
What is Enthesitis?
Enthesis is where a tendon or a ligament meets bone
Enthesitis is when these places get inflamed
What investigations needed to be done for suspected Reactive Arthritis?
Serology/Microbiology
Inflammatory Markers raised
May need joint aspirate to rule out septic/crystal arthritis
What is the treatment for Reactive Arthritis?
Treat underlying infection
NSAIDs , joint injections
Most will resolve within 2 years, if they do not ( esp if they have HLA-B27) may need DMARDs
What percentage of people with IBD get Enteropathic Arthritis?
10-20%
What is the split for peripheral arthritis and axial arthritis in Enteropathic Arthritis?
2/3 get peripheral
1/3 get axial
What are two types of peripheral Enteropathic Arthritis?
Type 1 - oligoarticular, asymmetric and correlated with IBD flare ups
Type 2 - polyarticular, symmetrical, less correlated with IBD flare ups
What is the management for Enteropathic Arthritis?
DMARDs ( NSAIDs can flare IBDs )
TNF inhibitors ( treat bowel disease and the arthritis )
What are the extra-articular manifestations of Ankylosing Spondylitis?
5As
Anterior Uveitis
Aortic Incompetence
AV Block
Apical Lung Fibrosis
Amyloidosis
What are the features of inflammatory back pain as opposed to other causes?
IPAIN
Insidious onset ( no trauma )
Pain at night
Age of onset < 40
Improvement with exercise
No improvement with rest
What is Lupus?
Autoimmune disease
Inadequate T Cell suppressor activity and increased B Cell activity
Characterized by remissions and flares
What are some common signs and symptoms of SLE?
S - Serositis
O - Oral ulcers
A - Arthralgia
P - Photosensitivity
B - Blood disorders
R - Renal involvement
A - Autoantibodies ( ANA and anti ds-DNA)
I - Immunological deficit
N - Neurological manifestation (Headache)
M - Malar Rash
D - Discoid rash
Which features may suggest an active SLE flare up?
Malar rash
Fever
Feeling more tired than usual
Painful. swollen joints
What can be seen on a FBC with SLE?
Raised ESR
Raised Plasma Viscosity
Normal CRP
Anaemia
Leukopenia
What autoantibodies are found in SLE?
ANA
Anti-Ro and Anti-La
Anti-dsDNA
What autoantibody rises with disease activity?
Anti-dsDNA
What complications do Antiphospholipid antibodies in SLE increase the likelihood of ?
Pregnancy loss
Thrombosis
Which complement proteins fall with SLE disease activity?
C3 and C4
What investigations must be done SLE?
FBC ( anaemia)
Autoantibodies
Complement ( C3 and C4)
Urinalysis ( to see renal involvement )
Renal biopsy ( can be diagnostic and help prognosticate )
Skin biopsy ( can be diagnostic )
What is the lifestyle management for SLE?
Sun protection
Healthy lifestyle advice in context of CVS risk
What is the pharmacological management for SLE flares?
Prednisolone
What is the pharmacological management for SLE?
Mycophenolate Mofetil
Azathioprine
Rituximab
Hydroxychloroquine for rash and arthralgia
Which gender is SLE more common in?
Females
What is the average age of onset for SLE?
Early adulthood
Why does UV light trigger SLE?
It alters structure of the DNA in the dermis that renders it more immunogenic
Which drugs can trigger an SLE like syndrome?
Isoniazid
Minocycline
TNF inhibitors
Which other system does SLE cause a high risk in?
CVS
What is the name of the diagnostic criteria for SLE?
EULAR/ ACR classification
What is Raynaud’s Phenomenen?
It is painful and is characterized by a typical sequence of colour changes in response to a cold stimulus, also precipitated by stress.
What is Raynaud’s caused by?
Vasospasm of the digits
What do the colours of the hands in Raynaud’s indicate?
white- inadequate blood flow
blue -venous stasis
red -re-warming hyperaemia.
What is Raynaud’s Sydrome?
This term is used when Raynaud’s phenomenon is idiopathic.
Who is Raynaud’s syndrome common in?
Young women
What is the treatment for Raynaud’s Syndrome?
Keep hands warm
Avoid smoking
Which features of Raynaud’s Phenomenon make you think its a rheumatic cause?
Over age 30
Abnormal nail-fold capillaries
Puffy fingers
Photosensitive rash
What conditions is RP associated with?
Scleroderma
SLE
Dermatomyositis
Polymyositis
Sjogren’s Sydrome
What are other causes of RP?
Use of heavy vibrating tools
Cervical rib
Sticky blood ( cryoglobulinemia )
Beta blockers
What is the treatment for RP?
CCB are first line
Phosphodiesterase-5 inhibitors
Prostacylins
What are some complications of RS?
Digital Ulcers
Severe digital ischaemia
Infection
What is Vasculitis?
A large, heterogeneous group of diseases classified by the predominant size, type, and location of inflamed blood vessels
What clinical features can occur as a result of the damage to the blood vessel walls?
Thrombosis
Ischaemia
Aneurysm formation
What signs/symptoms are indicative of Vasculitis? Use a systems based approach
MSK
CNS/PNS
Head
CVS
Resp
GI
Renal
MSK - Arthralgia, Myalgia, Proximal muscle weakness, Ischaemia
CNS/PNS - Headaches, Visual Loss, Tinnitus, Stroke, Seizure, Encephalopathy, Neuropathy
Head - Oral ulcers, Epistaxis, Ulcers
CVS - Pericarditis
Resp - Cough, Chest Px, Hemoptysis, Dyspnea
GI - Abdo Px
Renal - Haematuria
What can be seen on examination in Vasculitis?
CVS / Resp-
HTN
Crackles, Pleural rubs, murmurs, arrythmias
Skin -
Palpable Purpura
Livedo Reticularis
Nodules
Digital ulcers
Gangrene
Nail bed capillary changes
What are the small vessel Vasculitides?
-Microscopic polyangiitis (MPA)
-Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome)
- IgA vasculitis
What are the medium vessel Vasculitides ?
- Polyarteritis nodosa (PAN)
- Kawasaki disease (KD)
What are the large vessel vasculitides?
- Takayasu arteritis (TAK)
- Giant cell arteritis (GCA)
What can secondary Vasculitis be caused by?
Infections
Drugs
Malignancy
Connective Tissue disorders
What can Vasculitis cause in the renal system?
Glomerulonephritis
What tests are needed for Vasculitis?
FBCs, U&Es, LFTs, CRP, PV, ESR
ANA, ANCA, RF ( to rule out)
Complement C3 + C4
Cryoglobulins
Serum and protein electrophosphoresis
CK, Blood cultures, ECG. CXR, CT, MRI, Arteriography, CT-PET to check extent of organ involvment
What screening tests also need to be done to rule out viral causes of vasculitis?
Hepatitis Screen (B&C)
HIV Screen
What is the management plan in Vasculitis?
Rule out infection, stop any offending drugs
Then commence on pharmacological management
What is the 1st line treatment for Vasculitis?
Corticosteroids
What is the second line treatment of Vasculitis?
Cytotoxic medications
Immunomodulatory
Biologic Agents (e.g Methotrexate, Rituximab)
What other condition can present as a Vasculitis like syndrome?
Atrial Myxomas
What is an Atrial Myxoma?
A tumour ( overgrowth of cells ) that can be found in the atria
How can you exclude an Atrial Myxoma ?
A normal ECHO ( no visible tumour )
What are Dermatomyositis and Polymyositis?
Rare idiopathic muscle diseases that are characterized by inflammation of striated muscle
What is the peak age of onset for DMST and PMST?
40-50
What is the usual presentation of Dermatomyositis and Polymyositis?
Insidious onset of painless, proximal muscle weakness
SOB
Rash
Raynaud’s
What is the diagnostic criteria for Dermatomyositis and Polymyositis?
-Symmetrical proximal muscle weakness
-Raised serum muscle enzyme levels
-Typical electromyographic (EMG) changes
-Biopsy evidence of myositis
-Typical rash of dermatomyositis
-PM if >= 3 of the first 4 above criteria
-DM if rash and >= 2 of the first 3 above criteria
What does the FBC appear like in Dermatomyositis and Polymyositis?
Normal usually
What do tests normally show?
Raised ALT but normal LFTs
ANA positive (80%)
Anti-Jo-1 and Anti-Mi2
Evidence on MRI
Doesn’t affect kidney
What is the treatment for Dermatomyositis and Polymyositis?
High Dose Corticosteroids for first few weeks
Long term control with Methotrexate or Azathioprine
IVIG also used
In Dermatomyositis what additional management is needed?
Sun protection
Hydroxychloroquine for the rash
Why can aspiration pneumonia happen in Dermatomyositis and Polymyositis?
Upper oesophagus is striated muscle so swallowing can be affected
Why can respiratory failure happen in Dermatomyositis and Polymyositis?
Diaphragm involvment
What do patients with Dermatomyositis have an increased risk of?
Malignancy
What type of rash is the Dermatomyositis rash?
Photosenstive - appears on sun exposed areas
What does the Dermatomyositis rash look like?
Linear plaqaues on the dorsal aspects of the hands ( Gottron’s papules )
What are some other signs of Dermatomyositis and Polymyositis?
Dilated nail-fold capilleries
Dry-cracked palms and fingers
Periorbital oedema
Heliotrope rash ( rare)
What is a heliotrope rash?
Violet rash on the eyelids
Patients with which conditions can develop Dermatomyositis and Polymyositis?
Scleroderma
SLE
What is Systemic Sclerosis ( Scleroderma) ?
Increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis.
What are the two types of Scleroderma?
Diffuse
Limited
What is Limited Scleroderma also known as?
CREST Syndrome
What are the symptom of CREST syndrome?
C- Calcinosis Cutis
R - Raynaud’s
E - Eosophageal Dysmotility
S - Sclerodactlyl
T - Telangiectasia
How does CREST syndrome usually start?
Many years of Raynaud’s before any thickening
What is Diffuse Scleroderma?
Less common
Has a sudden onset of skin involvement , proximal to elbows and knees
Which type of Scleroderma has a higher mortality rate?
Diffuse
What investigations are done for Scleroderma?
XRAY hands show Calcinosis
CXR, HRCT, PFT - pulmonary disease
ECG & ECHO - HTN, HF, Myocarditis and Arrhythmias
What autoantibodies are present in Limited Scleroderma?
ANA
Anti-centromere
What autoantibodies are present in Diffuse Scleroderma?
ANA
Scl-70
Anti RNA Polymerase III
Is there a cure for Scleroderma?
No
What is the management for Scleroderma?
Psychological support
Short courses of Prednisolone for flares
ACEi to prevent HTN crisis ( Pulmonary HTN)
Methotrexate and Myco Mof may reduce skin thickening
PPIs for GI symptoms
Calcium Antagonist/ Sildenafil/ Iloprost infusion for Raynaud’s
What is Morphea?
Localized scleroderma
What crises can take place due to Scleroderma?
Hyptensive Crisis
Renal Crisis
How does a Renal Crisis present?
Accelerated HTN
What is Sjogren’s Syndrome?
Chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland secretion ( dry eyes, dry mouth, dry vagina )
Can be primary or secondary
What are the common signs and symptoms of Sjogren’s Syndrome?
Myalgia
Arthralgia
Dry Mouth
Fatigue
Raynaud’s phenomenon Enlarged parotids
Dry eyes
What investigations are done for Sjogren’s?
Autoantibodies - Anti Ro and Anti La ( RF and Anti dsDNA can be seen )
Schimer’s test
Salivary gland biopsy maybe
What does Schimer’s test measure?
Tear Volume
What is the treatment for Sjogren’s syndrome?
Avoid dry environments
Artificial tears
Artificial saliva/ gum
Vaginal lubricants
(Sialagogues)
What conditions are the most commonly associated with Sjogren’s?
Rheumatoid
SLE
What malignancy is associated with Sjogren’s syndrome?
B Cell Lymphoma
What are patient’s with Anti-Ro antibodies at risk of in pregnancy?
Fetal loss
Complete Heart Block of fetus
Neonatal Lupus syndrome in newborn
What will a salivary gland biopsy show in Sjogren’s syndrome?
Focal lymphocyte infiltration of exocrine glands
What is Hypermobility Spectrum Disorder?
A pain syndrome where peoples joints move beyond normal limits. It is due to laxity of ligaments, capsules and tendons. It is thought the origin of the pain is from microtrauma
In which population is Hypermobility Spectrum Disorder more common in?
Women
Asian people
What are complications of Hypermobility Spectrum Disorder?
Recurrent subluxation or dislocations
What are some signs and symptoms of Hypermobility Spectrum Disorder?
Arthralgia worse after activity
Fatigue
Epicondylitis
Hyperextensible skin
Papyraceuous Scars
Marfanoid habitus
Arachnodactylyl
Myopia
Hernia
Uterine/ Rectal prolapses
What are Papyraceuous Scars?
Atrophic scars that have widened to become paper thin ( Ehlers-Danlos)
What are Marfanoid Habitus?
Constellation of signs resembling those of Marfan Syndrome
What is the management for Hypermobility Spectrum Disorder?
Paracetamol
Strengthening exercises
Splinting/Surgical interventions may be needed
What are the heritable connective tissue disorders?
Hypermobility Spectrum Disorder
Marfan Syndrome
Ehlers-Danlos Syndrome
Have considerable phenotypic overlap
What is Osteoarthritis ( OA )?
This is the commonest type of arthritis: a degenerative joint disorder in which there is progressive loss of articular cartilage accompanied by new bone formation and capsular fibrosis.
What is the pathophysiology of Osteoarthritis?
The pathogenesis of OA involves a degradation of cartilage and remodelling of bone due to an active response of chondrocytes in the articular cartilage and the inflammatory cells in the surrounding tissues.
The release of enzymes from these cells break down collagen and proteoglycans, destroying the articular cartilage. The exposure of the underlying subchondral bone results in sclerosis, followed by reactive remodelling changes that lead to the formation of osteophytes and subchondral bone cysts. The joint space is progressively lost over time.
What are the risk factors for OA?
Older age
Women
Obesity
Trauma
What are the most commonly affected joints in OA?
Hip
Knee
Spine
Weight-bearing
What is common at the knee in OA?
Inactivity gelling
What will XRAY show in OA?
J - Joint Space Narrowing
O - Osteophytes
B - Bone Cysts
S - Subarticular Sclerosis
What is the treatment options for OA?
Physiotherapy
Weight Loss
Paracetemol
NSAIDs short term
Topical NSAIDs
Intra Articular corticosteroid injections
Replacement
What nodes are found in OA?
Bouchard’s ( PIPJs)
Heberden’s (DIPJs)
Thumb
Strongly associated with progression of knee OA
Are XRAY severity and severity of symptoms associated in OA?
No - there’s a big disconnect
What is Fibromyalgia?
A common disorder of central pain processing characterized by chronic widespread pain in all 4 quadrants of the body (both sides and above and below the waist). Allodynia, a heightened and painful response to innocuous stimuli, is often present.
What is the probable tigger for Fibromyalgia?
Sleep disturbance
What is the pathogenesis of Fibromyalgia?
Hyper-activation in response to noxious stimulation, and neural activation in brain regions associated with pain perception in response to non-painful stimuli.
What are some symptoms of FM?
- joint/muscle stiffness
- profound fatigue
- unrefreshed sleep
- numbness
- headaches
- irritable bowel/bladder syndrome
- depression and anxiety
- poor concentration and memory “fibrofog”
What are risk factors for FM?
Female
40-50
May have obvious trigger e.g emotional pain
What is the treatment for FM?
Improve sleep
Increase physical activity
CBT
Low dose amitriptyline
Pregabalin
What tests should be done in FM to exclude other diagnoses?
Blood tests such as ESR, CRP, FBC, U+E, LFT, Ca, CK and TFT are recommended to help exclude other pathology
What is Osteoporosis?
A skeletal condition characterized by low bone mass, deterioration of bone tissue, and disruption of bone architecture that leads to compromised bone strength and an increased risk of fracture
What are some non modifiable risk factors for OP?
> 65
Female
White or S Asian
Family Hx
History of low trauma fracture
What are some modifiable risk factors for OP?
Low body weight >58kg
BMI > 21
Premature Menopause
Cal/Vit D Deficiency
Low physical activity
Smoking
Excess Alcohol intake
Long term Steroids
How is diagnosis make for OP?
DEXA scan of lumbar spine and hip
if over 75 no need
What is the T-score on a DEXA scan result?
T-Score is the number of standard deviations away from the mean bone density of a person of the same gender at age 25 ( peak density)
How do you interpret a DEXA scan result?
T score more than or equal to -1 = normal
T score between -1 and -2.5 = osteopenia
T score less than -2.5 = osteoporosis
What is the treatment for Osteopenia?
Weight bearing exercise
Vitamin D3 supplementation
Reduce alcohol
Smoking cessation
What is the treatment for Osteoporosis?
Vitamin D +/- calcium supplementation
plus,
1st Line - Oral bisphosphonates , I.V if not tolerated
2nd Line - Denosumab or Teriparatide
What are some secondary causes of Osteoporosis?
Coeliac disease
Eating disorder
Hyperparathyroidism
Hyperthyroidism
Multiple Myeloma
Hypogonadism ( Men )
What is Gout?
An inflammatory arthritis due to hyperuricaemia
What is the most commonly affected joint in Gout?
1st Metatarsophalangeal joint ( Big Toe)
What is the pathology of Gout?
Deposition of monosodium urate (MSU) crystals that accumulate in joints and soft tissues, result in acute and chronic arthritis, soft-tissue masses called tophi, urate nephropathy, and uric acid nephrolithiasis
How common is a second flare up after initial treatment?
A second flare occurs in ~60% of patients within 1 year and 78% within 2 years of the initial attack.
What are the non-modifiable risk factors for Gout?
> 40
Male
What are modifiable risk factors for Gout?
Increased purine uptake (meats and seafood) Alcohol intake (especially beer)
High fructose intake
Obesity
Organ transplant
Hypertension
Smoking
Diabetes mellitus
Urate-elevating medications e.g. diuretics
What is the lifestyle management for Gout?
Maintain optimal weight
Regular exercise
Diet modification (purine-rich foods)
Reduce alcohol consumption (beer and liquor)
Smoking cessation
Maintain fluid intake and avoid dehydration
What is first line treatment for acute Gout?
NSAIDs
Oral/IM Steroids
Colchicine
What is the treatment for Chronic Gout ?
Urate lowering therapy (ULT)
Allopurinol
How does Allopurinol reduce Urate levels?
Its a xanthine oxidase inhibitors so it reduces urate formation
What is the aim SUA level on term ULT?
< 360micromol/L
What investigation can be done to diagnose gout?
Synovial fluid aspirate - MSU crystals found
How quickly should therapy be commenced in a Gout flare up?
Within 24 hours - highly effective in this time period
What do the Gout crystals look like through polarised light?
Negatively birefringent
What type of crystals cause Pseudogout?
Calcium pyrophosphate crystals
What is a good way to remember the autoantibody for Limited Sclerosis?
Limited ( Central ) Sclerosis = Anti-centromere antibodies
Central = centro
What test is used to access Hypermobility?
Beighton Score
What should be given alongside Allopurinol when starting a patient on it?
NSAID or Colchicine ‘cover’
What blood test must be carried out before starting Azathioprine?
TPMT
Why must TPMT be checked before starting Azathioprine?
Thiopurine Methytransferase enzyme is responsible be metabolising thiopurine drugs . TPMT deficiency can lead to toxicity, causing bone marrow suppresion
What are the x-ray findings in Ankylosing Spondylitis?
Subchondral erosions
Sclerosis
Squaring of lumbar vertebrae
What examination should done before a patient is started on Hydroxychloroquine ?
Ophthalmologic exam
Retinopathy can be a side effect
What should one suspect if a patient has Malignancy and a raised CK?
Polymyositis
What pulmonary condition can Methotrexate cause?
Pneumonitis ( inflammation of lung parenchyma ) leads to pulmonary fibrosis
What is the advice for women trying to conceive regarding Methotrexate use?
Methotrexate should be stopped 6 months prior to trying to conceive due to the teratogenic effects
Which hand joint is commonly affected in Osteoarthritis?
The base of the thumb ( 1st MCP joint )
Why must Methotrexate and Trimethoprim not be concurrently prescribed?
Increases the risk of bone marrow suppression or fatal pancytopenia
What do patients who are about to start long term steroids and are over 65 need to also be prescribed?
Immediate bone protection
Alendronic Acid, Vitamin D and Calcium supplements
What is the key investigation for diagnosing Septic Arthritis?
Synovial fluid aspirate and analysis
What is the first line treatment for knee osteoarthritis?
Topical NSAIDs e.g Ibruprofen Gel
What is the DEXA scan Z score adjusted for?
Age
Gender
Ethnicity
What is the most important side effect to warn patients about before starting bisphosphonates?
Oesophageal problems
A DAS score of above what indicates high disease activity in Rheumatoid Arthritis?
> 5.1
What is the most common form of Rheumatoid Factor antibody?
IgM against IgG
Which condition predisposes you to Pseudogout?
Haemochromatosis
In younger patients pseudogout it typically associated with an underlying condition
What is Pseudogout?
It is deposition of calcium pyrophosphate crystals in the joint.
What is APL syndrome?
An autoimmune disorder characterised by arterial and venous thrombosis, adverse pregnancy outcomes (for mother and foetus), and raised levels of antiphospholipid antibodies
How does APL syndrome cause thrombosis?
The patient is in a hypercoagulable state
What is the initial management for suspected GCA?
40mg Oral Prednisolone - due to the chance of irreversible blindness is it important to treat GCA immediately
Dose can be weaned down over 1-2 years
How do you diagnose APL syndrome?
One or more of the following three positive blood tests are needed on two occasions, 12 weeks apart to diagnose APS:
Anticardiolipin antibodies
Anti-β2-GPI antibodies
Positive lupus anticoagulant assay
What is the management if GCA is presenting with visual symptoms?
I.V Methylprednisolone
Have an urgent same-day assessment by an ophthalmologist.
This is usually continued for three days before switching to oral prednisolone
What genetic mutation is responsible for Marfan’s?
Fibrillin 1 gene mutation
What are the symptoms of Marfan’s?
Tall and thin
Unusually long arms and legs
High-arch palate
Arachnodactyly ( long, spidery fingers )
Thoracic aortic aneurysm
Abdominal aortic aneurysm
Aortic regurgitation
Aortic root dilation
Mitral valve regurgitation/prolapse
Spontaneous Pneumothorax
Superior lens dislocation
Closed-angle glaucoma
Hypermobility
Contractures
Pectus deformities
Kyphoscoliosis
Spontaneous CSF Leaks
What nail changes are consistent with Psoriatic Arthritis?
Pitting
Onycholysis ( nail separating from nail bed)
What investigations are indicated for Septic Arthritis?
Joint aspiration - send for gram stain, culture and microscopy ( will show extremely high white cell count)
At least 2 blood cultures
Which eye condition is associated with ankylosing spondylitis?
Iritis ( Also known as anterior UVitis)
What are the symptoms on Limited Cutaneous Systemic Sclerosis ( CREST syndrome )
C - Calcinosis
R - Raynaud’s Phenomenon
E- Esophageal dysmotility
S - Sclerodactyly
T - Telangiectasia
Which antibodies is raised in Sjogren’s Syndrome?
Anti-Ro
Anti-La
How long before pregnancy should Methotrexate be stopped?
6 months
What does Swan-neck deformity appear like?
MCP flexion
PIP hyperextension
DIP hyperflexion
What is the management for Septic Arthritis?
Blood cultures x 2
Joint aspiration ( WCC > 10,000, Neutrophils < 90%, yellow fluid )
Send for Gram stain, culture, crystals, cell count
ABG - Lactate > 3mmol/L
I.V Fluids
I.V Flucloxacillin for 2 weeks and orally for 4 more weeks
May need surgical washout
What is the ‘Pencil in cup’ deformity seen in Psoriatic Arthritis?
Arthritis Migrans ( most severe psoriatic arthritis) - telescoping of affected fingers and toes
What does an X-Ray for pseudogout commonly show?
Chondrocalcinosis
What are the symptoms of Polymyositis?
Bilateral, proximal (hip and shoulder girdle) muscle weakness, developing over weeks to months
Esophageal/ pharyngeal involvement - dysphonia, dysphagia
Diaphragm - Type 2 Respiratory Failure
How does dermatopolymyositis present?
All the same features as Polymyositis
ALSO
-Heliotrope rash ( purple discolouration of the eyelid skin)
Gottron’s Papules - scaly, red papules over the knuckles and extensors
Macular erythematous rash
What will you find on investigations to confirm antiphospholipid syndrome?
Prolonged aPTT - even though it increases chance of thombosis
Lupus coagulant positive
Anti-cardiolipin antibody positive
Anti–β2-glycoprotein 1
antibodies positive
What is the treatment for hypermobility syndromes?
Referral to physiotherapy/OT
What is the clinical scoring system for hypermobility?
Beighton’s score
How often must patients on Methotrexate be monitored?
FBC ,U&ES and LFTs every 3 months to check for neutropenia
More prone to infections so look out for signs of sore throat, fever, ulcers
What is prescribed with Methotrexate?
Folic Acid 5mg, taken once weekly on a different day
What is the score used to measure disease activity in Rheumatoid Arthritis?
DAS-28 , looks at 28 joints to decide if tender or swollen
What are the benign bone tumours?
Osteoma
Osteochondroma
Giant Cell Tumour
What are the malignant bone tumours?
Osteosarcoma
Chondrosarcoma
Ewing’s Sarcoma
What are the features of Osteoma?
Benign ‘overgrowth’ of bone
Most typically occurring on the skull
Associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)
What are the features of Chondrosarcoma?
Most common benign bone tumour
More in males, usually diagnosed in patients aged < 20 years
Cartilage-capped bony projection on the external surface of a bone
What are the features of Giant Cell Tumour?
Tumour of multinucleated giant cells within a fibrous stroma
Peak incidence: 20-40 years
Occurs most frequently in the epiphyses (end) of long bones
X-ray shows a ‘double bubble’ or ‘soap bubble’ appearance
What are features of a Osteosarcoma?
Most common malignant bone tumour
Seen mainly in children and adolescents
Occurs most frequently in the metaphyseal (neck portion) region of long bones prior to epiphyseal closure, with 40% occuring in the femur, 20% in the tibia, and 10% in the humerus
X-ray shows Codman triangle (from periosteal elevation) and ‘sunburst’ pattern
Association with retinoblastoma)
Predisposed by Paget’s disease of the bone and radiotherapy
What are features of Chondrocarcinoma?
Malignant tumour of cartilage
Most commonly affects the axial skeleton (spine)
More common in middle-age
What are the features of Ewing’s Sarcoma?
Small round blue cell tumour
Seen mainly in children and adolescents
Occurs most frequently in the pelvis and long bones. Tends to cause severe pain
X-Ray shows onion skin appearance
What is Paget’s Disease of Bone?
Paget’s disease of the bone is a disease associated with brittle, abnormally shaped, weak bones. This is due to increased bone formation and bone resorption, meaning that bone turnover is very dysregulated and uncontrolled. Unlike normal bone in which the bone remodelling cycle is in balance, the increased bone turnover in Paget’s disease results in abnormal bone formation and reduced structural integrity.
What does sterile synovitis mean in Reactive Arthritis?
On joint aspiration, the infective organism cannot be recovered , there will be a negative culture
What makes the pain worse in lateral epicondylitis?
Resisted wrist extension
What is a pharmacological management option for Raynaud’s?
CCB - Nifedipine
When do you not need to do a DEXA scan to confirm Osteoporosis?
If a patient is over 75 with a fragility fracture
In this case you would check their Vit D and Ca2+ levels are normal and then commence them on bisphosphonates. If they were low you would correct them with supplementation
