Flashcards in Week 224 - Rheumatoid Arthritis Deck (61)
What is the primary site of pathology in RA?
Define rheumatoid arthritis
Chronic Systemic Inflammatory Disorder of unknown trigger
Uncontrolled inflammation resulting in joint destruction.
What is Pannus formation?
Thickened, inflammed synovium. Causes damage to bone, cartilage and sometimes ligaments in and around the joint
What are the key details in the epidemiology of RA?
Race - worldwide
Female:Male > 3:1
Age - any - peak = 35-45
Smoking - clear link to incidence and increased severity
Genetics - certain influence (HLA-DR4 and PTPN22)
How can RA present?
Many different possible clinical manifestations:
- gradual onset - small peripheral joints, usually symmetrical
- acute, abrupt onset polyarthritis
- slow or acute monarticular presentation
- palindromic rheumatism
- local or systemic extra-articular features
What will typical RA histories contain to make it different to OA?
Usually small joints (MCP, proximal IP - NOT usually distal IP joint)
Worst in the morning
Improves with activity over a few hours
What is the most common progression pattern in RA patients?
75% experience ongoing joint pain with flare ups
Describe a "Swan-neck deformity"
Hyperextension of PIP joint with
Fixed flexion at DIP
Describe "Boutonnaire" deformity
Fixed flexion at PIP joint with
Hyperextension at DIP joint
What 3 signs are you likely to see on a hand x-ray in the presence of RA?
Periarticular osteopenia - areas of bone appear darker
Periarticular erosions - distal heads of metacarpals often
Reduced joint spaces
According to the ACR Classification criteria 1989 how many of the 7 criteria must be present to diagnose RA?
What causes the ulnar deviation seen in more severe progressions of RA?
Reduced integrity of the MCP joints - due to erosion for example - subluxation occurs
What combination of methods are usually required to diagnose RA?
History and examination
Bloods: ESR, CRP, Rheumatoid Factor, anti-CCP (cyclic citrullinated peptide)
Imaging: x-ray, USS/MRI - synovitis
Which of the various blood tests is more useful in the diagnosis and why?
Useful: CRP (more so than ESR) and Anti-CCP (more specific to RA than rheumatoid factor)
Arguably rheumatoid factor - present in ~80% with but also ~5% general population
What exactly is rheumatoid factor a measure of?
It detects the level of IgM antibody
Why is ESR not a very useful blood test result in the diagnosis of RA (and many other conditions!)?
Too many other factors effect levels of ESR including:
There are many extra-articular manifestations of RA. What are the constitutional manifestations alone?
Fever, fatigue and weight loss
What percentage of Rheumatoid Factor +ve patients are thought to experience rheumatoid nodules?
~20% (rare in RF -ve pts)
Can remove but often return and have little effect besides being an irritation
What pulmonary manifestations can occur with RA?
Pulmonary manifestations are frequent but often subtle
- pleural effusions
- interstitial fibrosis
- primary nodules from RF
What cardiac / CVS manifestations can occur with RA?
Vasculitis - severe RA; venous HTN and ulcers
- increased risk of IHD
- pericarditis / pericardial effusions
- coronary vasculitis
- non-specific myocarditis
- valvular disease *similar CVS disease risk to diabetics
What ocular manifestations can occur with RA?
- Scleritis - usually effects vision, painful > treat aggressively or perforation can occur
What neurological manifestations can occur with RA?
- Entrapment e.g. Carpal Tunnel
- Atlanto-axial subluxation > cervical myelopathy
- Mononeuritus multiplex
- Central NS vasculitis
What haematological manifestations can occur with RA?
- Anaemia (chronic disease - bone marrow suppression - NSAIDs and DMARDs)
- Thrombocytosis (hence increased risk of IHD)
- Rarely lymphadenopathy and Felty's syndrome
What is Amyloidosis?
A RARE condition but one to be aware of!
Result of chronic inflammation: increased serum amyloid-A protein (SAP) - every organ may be involved
What causes splinter haemorrhages and nail-fold infarcts and what condition are they most often linked with?
Caused by blocked vessels
Commonly linked to endocarditis
What occurs in pericardial tamponade?
Pericardial effusion becomes so large that the pressure squashes the ventricles to the point at which they stop functioning
What pathological manifestations do the various multi-system autoimmune connective tissue disorders tend to have in common?
- lung and renal fibrosis sometimes
There is often overlap in clinical presentation / symptoms of connective tissue disease. What do these include?
Fatigue; arthralgia/myalgia; depression; malaise; fever; weight loss; (lymphadenopathy)
What do the various common -itis (pathological) manifestations of autoimmune connective tissue disorders contribute to largely?
Increased thrombotic risk
What is Raynaud's Phenomenon?
Spasm of the blood vessels in the fingers or toes resulting in a triphasic response +/- environmental stress
Pallor > Cyanosis > Erythema
(Pallor due to vasoconstriction; cyanosis due to prolonged spasm then erythema due reactive hyperaemia)
Systemic Lupus Erythmatosis is associated with antibody formation. Which antibodies are associated with SLE?
- ANA ("anti-nuclear antibody - 100% of all SLE pts)
- ds DNA (50%, more specific than ANA)
- anti-Ro / -La (20%)
- anti-Sm (15%)
Which of the antibodies associated with SLE has been linked with neonatal SLE ?
Therefore all female pts with CTD wanting to get pregnant should be tested for anti-Ro as it crosses the placenta
List clinical manifestations of SLE
Malar/Butterfly Rash; Arthritis (non-erosive); mouth ulcers; alopecia; vasculitis > small infarcts on pads of fingers "nail fold infarcts"
Renal, neurological, haematological > serositis
What combination of factors helps to diagnose SLE?
- Clinical features
- Urine (protein and blood) & bloods
- Serology - ANA, ds DNA, anti-Ro/-La, anti-Sm
- Complements - reduced C3 and C4 - lots of complement activation so become used up due to antibody binding to them
What factors can affect the ANA antibody levels detected?
Infection; Age; Other autoimmune diseases; Malignancy; FHx
What are the treatment options for SLE?
Arthralgia - NSAIDs
Myalgia/Lethargy - hydroxychloroquine (HCQ)
Arthritis/Pleuritis/Pericarditis - prednisolone +/- HCQ / MTX
Haemolytic anaemia - Azathioprine / ???
Give 2 examples of Inflammatory Muscle Disease (a form of connective tissue disease)
What are some of the more common symptoms associated with Inflammatory Muscle Disease?
- Proximal muscle weakness (which can progress to cause complications such as aspiration and restrictive pulmonary disease)
- Heliotrope rash - on eyelids (dermato. specifically)
- Guttrons papules - skin thickening on dorsum of hands
- Cracking of skin on finger tips
What is considered the best diagnostic investigation for IMD?
In blood tests and serology what might help diagnose Inflammatory Muscle Disease?
- Elevated CK (also elevated in rhabdomyolitis)
- Troponin T - odd since cardiac specific but believed to be released from muscles when they regenerate in IMD
- Anti-Jo antibodies
Systemic Sclerosis is divided into 2 categories, what are they?
Limited Scleroderma and Diffuse Scleroderma
What is the principle common feature in both limited and diffuse scleroderma?
How is Diffuse Scleroderma diagnosed?
- If disease progresses proximal to the elbow in the arms
- Anti-scl70 antibody
How do you treat Diffuse Scleroderma?
Mycofentolate Mofeil (ltd benefit!) or Cyclophosphamides
Why must you NEVER treat diffuse scleroderma with steroids?
Can induce "renal crisis"
What was Limited Scleroderma originally known as and why?
CREST as this serves as a mnemonic for the symptoms it displays
What does CREST stand for?
Calcinosis (calcification deposits on fingers)
Telengectasia (dilation of blood vessels - blanching red spots)
What other major impact can limited scleroderma have on the fingers?
Distal phalanx reabsorption can occur
What antibody is associated with limited scleroderma?
How do you treat limited scleroderma?
There are no disease modifying medications available. Can only try to treat symptoms as they appear.
List 3 risks associated with Systemic Sclerosis (both limited and diffuse scleroderma)
- accelerated HTN
- pulmonary fibrosis
- renal crisis
A patient presents with these symptoms:
Dry eyes, dry mouth, bilateral cheek swellings anterior to each earlobe and purpura.
What is the principle differential?
What antibodies in serology would help you diagnose Sjogren's?
Anti-Ro or -La
Usually RF+ve also (90%)
What are the 5 main pharmacological treatment types involved in Rheumatoid Arthritis?
How do you manage Sjorgren's Syndrome?
Artificial tears and saliva, sugar free gum
Immunosuppressants rarely used
Which of the 5 pharmacological treatment groups prevent RA progressing?
DMARDs and Biologic agents
What are the 3 most commonly used DMARDS?
What is most important when diagnosis of RA is made with regard to treatment?
Put on more than one DMARD (combo better than 1)
"Disease Modifying Anti-Rheumatoid Drugs"
What are the side effects of DMARDs?
- bone marrow suppression
- increase susceptibility to infection
- renal and liver toxicity
What is given with Methotrexate and why?
Folic Acid - MTX is a folate pathway inhibitor