Rheumatology Flashcards
What are the hallmarks of an inflammatory disease in regards to rheumatology?
E.g rheumatoid arthritis
Night time pain
Morning stiffness >30 mins
Pain is worse after resting (worse in morning)
Systemic symptoms (fatigue, aches, weight loss)
Acute/subacute presentation
What are the hallmarks of a non-inflammatory disease in regards to rheumatology?
E.g, osteoarthritis
Morning stiffness <30 mins
Pain worse with use
Pain worse at the end of the day
Longstanding/chronic in nature
What are the 4 types of Arthritis?
Inflammatory
Non-inflammatory (osteoarthritis)
Septic arthritis
Crystal (Gout)
What are the different types of inflammatory arthritis?
Connective tissue disease
Vasculitis
Seropositive (rheumatoid arthritis)
Seronegative
What are the 4 types of seronegative inflammatory arthritis?
Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Inflammatory bowel disease arthritis (IBD)
What is the presentation of joint involvement in rheumatoid arthritis?
Affects multiple joints (>5)
Presents symmetrically e.g, in both hands
What are the commonly affected joints in rheumatoid arthritis?
Small joints first in hands: MCP, PIP, DIP, MTP
Large joints as the disease starts to worsen:
- shoulder
- elbow
- knee
- ankle
How do the swellings in the joints differ between rheumatoid and osteoarthritis?
RA - boggy swelling (like a grape)
OA - bony swelling
What are the 3 main symptoms of rheumatoid arthritis?
Join pain - throbbing and aching
Swelling - boggy swellings in joints
Stiffness - worse after inactivity
What are the systemic symptoms seen in rheumatoid arthritis?
Fatigue Fever Sweating Loss of appetite Weight loss
What are the specific deformities seen in the hands of patients with rheumatoid arthritis, and which joints are they seen in?
Ulnar deviation - in MCP joints
Boutonnières - in PIP joints
Swan neck - in DIP joints
Also can see guttering muscle wasting over the dorsum of the hand
Describe what an ulnar deviation deformity looks like?
Swelling in the MCP joints
Causes the fingers to become displaced, they therefore tend towards the little finger
Describe what a boutonnières deformity looks like?
Swelling in the PIP joints causes:
- PIP flexion
- DIP hyperextension
Describe what a swan neck deformity looks like
Swelling in the DIP joints causes:
- PIP hyperextension
- DIP flexion
What is a bakers cyst?
Cyst that occurs within the knee joint (popliteal cysts)
Fluid filled swelling that develops at the back of the knee
Occurs when the knee joint tissue becomes swollen and inflammed - the synovial sac gets so swollen that it bulges posteriorly into the popliteal fossa
What are the risk factors for rheumatoid arthritis?
Genetics - more likely if a family member has it
Sex - more common in women, due to oestrogen
Smoking - this can trigger it
What are the susepctibility genes associated with RA?
HLA-DR1
HLA-DR4
What is the pathophysiology of RA?
Environmental triggers cause post translational modifications of proteins e.g, citrulliation
This then can cause autoimmunity - whereby immune cells recognise self cells as foreign and produce antibodies against host cells
These immune cells specifically target cells in the synovial - which is the lining of the membranes that surround the joints
What is rheumatoid factor?
RF is an autoantibody produce in RA - an antibody produced to attack healthy host tissue in joints
It is an antibody that binds to the Fc portion of IgG - it forms immune complexes that contributes to the inflammation in RA
What is Anti-CCP?
Anti-cyclic citrullinated peptide antibody
This is a type of autoantibody produced in RA
It specifically targets the altered citrullinated proteins produced in RA
This results in the formation of immune complexes which activate the inflammatory process
Why does RA cause fatigue?
The antibodies that cause the inflammation in RA can also affect the CNS as well as the joints
High inflammation levels can lead to severe fatigue
This can also lead to mood problems which causes a cycle
Why do extra-articular manifestations occur in RA?
Inflammatory cytokines leave the joint spaces and travel to different organ systems
This happens as the disease progresses
At what age does RA present?
Usually between ages 30-50 years
What are the extra-articular manifestations of RA called which are found in the skin?
Rheumatoid nodules
Commonly found in pressure points e.g elbows
What extra-articular manifestations of RA can occur in the lung?
Interstitial fibrosis
Plueral effusion
What is felty syndrome?
Triad occurring with RA: - anaemia - splenomegaly - neutropenia/leucopenia (Can lead to life threatening infections)
What is the most specific test for RA diagnosis?
Anti-CCP antibody (ANTI-CCP)
Why is the presence of rheumatoid factor (RF) itself not diagnostic of RA?
Only 66% specificity for RA
20% of patients with RA will have negative RF
May be present in 10% of normal population
Can also be present in Sjögren’s syndrome and other rheumatic conditions
What would an typical X-ray show in someone with RA?
Decreased bone density Soft tissue swelling Bony erosions Periarticular ostopenia Narrowing of joint space RA Deformities
Which patients should undergo testing for RA?
Patients with at least 1 joint with definite clinical synovitis (swelling)
Where the swelling is not better explained by another disease
What scoring system is using to determine how active a patients rheumatoid arthritis is?
DAS28
How is Rheumatoid arthritis managed?
Symptom management - analgesia (NSAIDS)
Suppression of inflammation - disease modifying anti rheumatic medications (DMARDs)
Biological response modifiers (Biologics)
Managing acute flares (steroids - short term only)
What are the common disease modifying anti rheumatic medications (DMARDs) used in Rheumatoid arthritis?
Methotrexate
Hydroxycholroquine
Sulfasalazine
What is the most common type of arthritis?
Osteoarthritis
How does osteoarthritis usually present?
Pain in joints
In elderly patients
Gradual onset - can take years to develop
Pain is worse on movement (Pain worse at the end of the day )
<30 mins of morning pain
What are the bony swellings called in osteoarthritis?
Name the common swellings found in the hand?
Osteophytes / Nodal osteoarthritis
Heberdens nodes - DIPJs
Bouchard nodes - PIPJs
What are the commonly affected joints in osteoarthritis?
Weight bearing joints - knees, feet, spine (lumbar and cervical), hips
Joints in the hands - DIPJ, PIPJ, CMCJ
What are the risk factors for developing osteoarthritis?
Age
Obesity - as excess weight puts strain on joints
Female - more common in women
Joint injury - overusing joint if it has not had time to heal following injury
Genetics - although no single gene has been found
Secondary arthritis - if patient already has RA or gout
When should you consider osteoarthritis as a secondary cause of arthritis (following RA or gout)?
If OA symptoms presents at young age (<40)
If there is atypical distribution of joints (e.g, MCP, elbows, shoulders, ankles)
What is the pathophysiology of osteoarthritis?
Condrocyte cells are responsible for maintaining a balance of breaking down and producing new cartilage in a joint
In OA, Condrocyte cells produce a higher amount of degenerative enzymes, tipping the balance in favour of cartilage loss
Loss of articular cartilage, causes friction between bones which causes inflammation in the joint
What is eburnation in osteoarthritis?
Where there is complete loss of articular cartilage so the bones rub against each other
This looks like polished ivory
What are the x-ray findings seen on a patient with osteoarthritis?
Loss of joint space
Osteophytes
Subchondral bone sclerosis
Subchondral cysts
How is osteoarthritis managed?
Lifestyle - weight loss, exercise, physiotherapy
Analgesia - NSAIDs, colchicine
Intra-articular Steroid injections - for moderate/severe pain
Surgical management - e.g joint replacement (reserved for patients who have substantial impact on quality of life)
What is gout?
Type of inflammatory arthritis (crystal arthritis)
Where monosodium urate crystals deposit into a joint
How does gout typically present?
Acute presentation - red, hot swollen joint
This is known as a “gouty attack”
Repeated attacks result in arthritis
What is the podagra presentation of gout?
Gout affecting the first metatarsal joint of big toe
Very typical presentation of gout
Patients wake up feeling like toe is on fire
Pain is severe for a few hours and then settles down
Swelling and pain can last for days/weeks
What are the risk factors for gout?
Male sex
Obesity, Diabetes
Anything that increases the amount of uric acid in the body: alcohol (as it makes you dehydrated), CKD (kidneys unable to clear uric acid), certain mediations (thiazide diuretics, aspirin), high purine diet, chemotherapy (as DNA is broken down into purines)
What is the pathophysiology of gout?
Hyperureicemia (too much uric acid in blood)
This results in formation or uric acid crystals in areas with slow blood flow e.g, joints and kidney tubules
This leads to inflammation and a gouty attack
Overtime repeated attacks destroy joints causing arthritis
Why does excess having purines lead to hyperuraecemia?
Purines are a component of DNA
When they are broken down they are broken down into uric acid
Which foods are high in purine content?
Shellfish
Anchovies
Red meat
Which medications can cause hyperuraecemia?
Thiazide diuretics
Aspirin
What is the gold standard investigations for diagnosing gout?
Synovial fluid microscopy
Allows visualisation of urate crystals
How is gout managed?
Analgesia - NSAIDs
Lifestyle - weight loss, decrease alcohol, low purine diet
Cardiovascular risk assessment - as gout increases risk
Urate lowering therapy - e.g, allopurinol
When should urate lowering therapy be used for patients with gout?
Patients who have >2 attacks of gout per year
CKD stage 2 or worse
Tophus or tophi present
If urolithiasis is present (uric stones present in bladder or urinary tract)
What levels of serum uric acid should you aim for on urate lowering therapy?
<300 micromoles/L
What is the important information to know when starting a patient on urate lowering therapy (ULT) for gout?
Should be started 2-4 weeks after acute attack has settled
During initial phase of ULT, can be an increase in attacks (due to remodelling of articular urate crystals deposits as a result of lowering concentrations)
Because of this all patients starting on ULT should be coprescirbed either colchicine or low dose NSAID
What are tophi in gout?
Permanent deposits of uric crystals which form along the bones just beneath the skin
What are patients with chronic gout more at risk of?
Cardiovascular disease
Kidney stones
Urate nephropathy
What is juvenile idiopathic arthritis (JIA)?
Swelling in joints present in children before the age of 16
Similar Pathophysiology to rheumatoid arthritis
What are the 2 most common types of juvenile idiopathic arthritis and how do you distinguish between them?
Oligoarthritis - <5 joints affected in 1st 6 months
Polyarthritis - >5 joints affected in 1st 6 months
Which type of juvenile idiopathic arthritis is associated with chronic anterior uveitis?
Oligoarthritis
What is dactylitis and which type of juvenile idiopathic arthritis is it seen in?
Dactylitis - swollen sausage like toe
Commonly seen in psoriatic arthritis
What joints does juvenile idiopathic arthritis typically occur in?
Knees
What is the prognosis for children who have juvenile idiopathic arthritis?
70% cases disappear before adulthood
Of those which disease progresses into adulthood, they may have joint damage and need knee replacements
When disease progresses into adulthood, they are more likely to develop osteoporosis
How is juvenile idiopathic arthritis managed?
Similar to that of RA Analgesia - NSAIDs Disease modifying anti-rheumatic drugs (DMARDs) e.g, methotrexate Biological therapies Steroid injections
How does septic arthritis usually present?
Acute history - hot painful joint
Systemic features present - e.g, sweats, fever, malaise
What other condition is commonly associated with rheumatoid arthritis?
Sjögren’s syndrome
How does Sjögren’s syndrome commonly present?
Dry eyes Dry mouth Fatigue Joint pain Swollen salivary glands Photosensitive skin rash
Why might a patient with rheumatoid arthritis be anaemic?
Anaemia of chronic disease
Iron deficiency anaemia due to NSAID use
Feltys syndrome - anaemia, leucopenia and enlarge spleen
Pernicious anaemia - another autoimmune disease may be present
What percentage of patients wtih rheumatoid arthritis have extra-articular manifestations?
40%
What are the important differentials for any patient presenting with multiple systemic symptoms?
Connective tissue disease Vasculitis Infection Malignancy Inflammatory arthritis
What is vasculitis?
Autoimmune disease
Causes inflammation of the blood vessels
Causing systemic symptoms across multiple body systems
What are the 3 classifications of vasculitis?
Small vessel vasculitis - inflammation in arterioles and capillaries
Medium vessel vasculitis - inflammation in arteries and arterioles
Large vessel vasculitis - inflammation in the aorta and arteries
What are the two types of large vessel vasculitis?
Giant cell arteritis
Takayasu’s arteritis
What are the 2 types of medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki’s disease
What are the 3 types of small vessel vasculitis?
Eosinophilic granulomatosis with polyangitis (Chung-Strauss disease)
Granulomatosis with polyangitis (wegeners granulomatosis)
Microscopic polyangitis
What is the pathogenesis of vasculitis?
In large and medium vessel vasculitis - Molecular mimicry (where the immune cells mistake vascular endothelial cells as foreign)
In small vessel vasculitis - indirect damage (autoimmune disease where cells attack cells near vascular endothelial cells which get indirectly damaged)
What are the important investigations to carry out in suspected vasculitis?
Urine analysis - to determine renal involvement (glomerulonephritis is a common manifestation of vasculitis)
FBC
P-ANCA and C-ANCA
Plasma viscosity - the thicker the blood, the more inflammation
Chest x ray - granulomatosis polyangitis can present with lung cavities
What is giant cell arteritis?
Type of large vessel vasculitis (arteritis) affecting branches of the carotid arteries:
- temporal artery
- ophthalmic artery
- facial artery
Which artery is most commonly affected artery in giant cell arteritis?
Temporal artery (branch of external carotid)
What are the risk factors for developing giant cell arteritis?
> 50 years of age
Female sex
How is giant cell arteritis investigated and diagnosed?
ESR - classically raised in giant cell arteritis
Biopsy (e.g temporal artery biopsy) - looking for giant cells in internal elastic lamina
How is giant cell arteritis managed?
Steroids
What is takayasu’s arteritis?
Type of large vessel vasculitis which typically affects the arteries that branch off around the aortic arch
What are the risk factors for developing takaysu’s arteritis?
Asian
Female sex
Typically <40 years
What are the specific symptoms for takayasu’s arteritis?
Weak pulse - as the arteries supplying the upper limbs are affected
Visual symptoms
Neurological symptoms
What is polyarteritis nodosa?
Medium vessel vasculitis
Caused by immune cells directly attacking vascular endothelium
How does polyarteritis nodosa look on an angiogram?
Like beads on a string
Due to vessel wall weakening and pouching out
What infection is polyarteritis nodosa associated with?
Hepatitis B infection
How does polyarteritis nodosa typically present?
Rash
Mononeuritis multiplex
Vascular hypertension
Organ infection
What is the main complication of polyarteritis nodosa?
As the vascular wall is weak it is more prone to aneurysms
Main complication is organ Ischaemia
What are ANCA?
Anti-neutrophilic cytoplasmic antibodies
These are autoimmune antibodies which the plasma cells have mistakenly produced to target the body’s own neutrophils
IgG type antibodies
What type of vasculitis would ANCA be found?
Small vessel vasculitis
What are the two types of ANCA and what do they target?
C-ANCA - targets PR3 (proteinase 3) (commonly found in cytoplasm)
P-ANCA - targets MPO (myeloperoxidase) (commonly found in peri-nuclear region)
Which type of small vessel vasculitis are c-ANCA and p-ANCA found in?
C-ANCA: granulomatosis with polyangitis
P-ANCA: microscopic polyangitis
P-ANCA can also be found in eosinophilic granulomatosis with polyangitis (EGPA) although this is less commonly found at diagnosis (30-40% of patients are p-ANCA positive)
What is Eosinopilic granulomatosis with polyangitis (Chung-Strauss syndrome)?
Type of small vessel vasculitis
Multisystem disease with increase eosinophils
Often mistaken for asthma or allergies because of this reason
What is the Lanham diagnostic criteria for eosinophilic granulomatosis with polyangitis (churg Strauss disease)?
Triad of EPGA:
Asthma (late onset)
Eosinophilia
Multi-organ involvement (pulmonary, cardiovascular, neuropathy)
How is small vessel vasculitis managed?
Induction of remission:
- steroids - prednisolone or methyprednisolone
- immunosupression - cyclophosphamide or riuximab
Then switch to maintaining remission:
- Methotrexate, azathioprine, mycophenolate mofetil (MMF)
What is the major complication of small vessel ANCA associated vasculitis?
How is this managed
Rapidly progressive glomerulonephritis
Presents with nephrotic and nephritic features
Light microscopy will show crescent formation in glomerulus
Managed with high dose steroids
What is systemic lupus erythematous (SLE)?
Multi-system autoimmune connective tissue disorder
Results in chronic systemic inflammation
What are the risk factors for developing lupus?
Female sex
Childbearing age (20-40) - due to oestrogen presence
Afro-Caribbean decent
What is thought to cause lupus?
Susceptibility genes
Environmental factors thought to trigger these e.g. sunlight, smoking, infection
What is the classic symptoms of lupus?
Fever
Inflammatory arthritis - swollen, tender joints
Butterfly (malar) facial rash
Lupus nephritis (20-25% of patients have renal involvement at diagnosis)
Photosenstivity - rash on sun exposed areas
Oral ulcers
Fatigue
Lymphadenopathy
Which antibodies are present in Lupus?
ANA (anti-nuclear antibody)
DsDNA - antibody against DNA, seen during active disease
Anti-sm (anti-smith) - antibody to Sm nuclear antigen, target ribonucleoprotein, this is specifc for lupus
Antiphospholipid antibodies - can be present but less specific for lupus
What other autoimmmune disease are patients with lupus at risk of getting?
Antiphospholipid syndrome (APLS) This is due to the presence of antiphospholipid antibodies which can be present in lupus
What are the 3 types of antiphospholipid antibodies?
Anticardiolipin
Lupus anticoagulant
Anti-B2 glycoprotein 1
How is lupus managed?
Preventing flares - avoiding triggers e.g, sunlight, stress
Managing flares:
- NSAIDs e.g, ibuprofen
- Steroids e.g, prednisolone
- DMARDs e.g, hydroxycholoquine, methotrexate, azathioprine
- Biologics e.g, ritiximab (for severe cases)
What is the common debilitating symptom of lupus and how is it managed?
Fatigue
Can be present even in remission
Managed by encouraging an active lifestyle and healthy diet
What are the antibodies present in Sjögren’s syndrome?
Ro (SSA)
La (SSB)
What is pseudogout?
Deposition of calcium dihydrate crystals in the synovium
Causing synovitis that presents very similar to gout
NOT DUE TO URIC ACID
What are the risk factors for pseudogout?
Haemochromatosis
Hyperparathyroidism
Acromegaly
Wilson’s disease
How is pseudogout managed?
Aspirate joint fluid - to exclude septic arthritis
NSAIDS or steroids for symptom management
What is ankylosing spondylitis?
Type of inflammatory arthritis
Where the spine and other joints can become inflamed
What is the gene associated with ankylosing spondylitis?
HLA-B27
What is schober’s test?
Test for ankylosing spondylitis
Assesses the amount of lumbar flexion - to see if there is a decrease in flexion
Mark is made at L5 level. One finger placed 5cm below and one finger is placed 10cm below mark
Patient then leans forward
If the increase in distance between the two fingers is less than 5cm - this suggests there is limited lumbar flexion
How does giant cell arteritis typically present?
Rapid onset
Headache
Jaw claudication
Tender, palpable temporal artery
Why should you continue steroid treatment in giant cell arteritis, even if the temporal artery biopsy is normal?
Skip lesions can occur in GCA - which can mean the biopsy may be normal
How is raynauds disease?
Symptom relief - calcium channel blocker e.g, nifedipine
If secondary Raynaud’s phenomenon is suspected, then patients should be referred to rheumatology to investigate for underlying connective tissue disease e.g, lupus
What are the cardiovascular manifestations of lupus?
Pericarditis
Myocarditis
What blood test is used as an activity marker for lupus?
Complement levels - C3 and C4 would be low during active disease (this is because formation of complexes leads to consumption of complement)
When would a TNF-inhibitor be used to treat rheumatoid arthritis?
Where there has been an inadequate response to at least two DMARDs including methotrexate
Give the name of commonly used TNF-inhibitors in RA?
Entanercept
Infliximab
Adalimumab
What are the adverse effects of methotrexate?
Mucositis - causing oral ulcers Myelosupression - if infection must go to A&E Alopecia Pneumonitis Pulmonary fibrosis Liver fibrosis
What are the poor prognostic features for RA?
Rheumatoid factor positive HLA DR4 X ray showing early erosions Extra articular features e.g, nodules Insidious onset Anti-CCP antibodies
What is Ehler’s Danlos Syndrome?
Type of connective tissue disease
Results in widespread elasticity of tissue
Patients have very flexible joints and stretchy skin
What are the cardiac complications of elder-danlos syndrome?
Aortic regurgitation
Mitral valve prolapse
Aortic dissection
What is lateral epicondylitis more commonly known as?
Tennis elbow
How does lateral epicondylitis commonly present?
What are the findings on exam?
Pain/tenderness to lateral epicondyle
Pain worse on wrist extension against resistance while elbow extended
Pain worse on supination of forearm with elbow extended
What are the common cardiorespiratory manifestations of lupus?
Pericarditis
Myocarditis
Pleurisy
Fibrosing alveolitis
What is main adverse effect of hydroxychlorioquine?
Retinopathy
Must monitor visual acuity when starting on medication
How many months prior to getting pregnant must you stop taking methotrexate?
At least 3 months
What is the difference in joint aspiration findings of gout and pseudogout?
Gout - negatively birefringent needle shaped crystals
Pseudogout - positively birefringent rhomboid shaped crystals
