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_Sem 4. Immune System NOTES > S3 L2 Clinicial approach to autoimmunity > Flashcards

S3 L2 Clinicial approach to autoimmunity Flashcards

1
Q

What are autoimmune rheumatic diseases (ARDs)?

A

Heterogenous group of diseases
Immune tolerance breakdown → self antigens and proteins, recognises own body as foreign, develops antibodies that attach to individual and cause disease
Production of pathogenic antibodies
Multisystemic features → affects more than one system

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2
Q

Why are autoantibodies important?

A
  • Aid diagnosis
  • Associated with specific clinical features
  • Disease prognosis
  • To stratify therapy - see how aggressive the disease is, guide to management +ve serology or -ve serology
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3
Q

What are examples of autoimmune rheumatic disease?

A

Rheumatoid arthritis
Systemic lupus erythematosus
Systemic sclerosis

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4
Q

What is the epidemiology of systemic lupus erythematosus?

A

F:M → 9:1
Prevalence → 24/100,000
Race → Afro-Caribbean > south asians > caucasians
Genetic factors are important - monozygotic twins (if one has it the other more likely to have it)
Environmental factors - UV light mediated (cytokine and humoral mediated factors from skin causing systemic features), Smoking

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5
Q

When taking a history of someone with suspected autoimmune rheumatic disease (ARD) what features would you look for and ask about?

A 
Current symptoms 
- Pain - SQITARS/SOCRATES
- Stiffness - whole body stiffness, worse in the mornings, eases throughout the day - suggests presence of inflammatory cytokines (different to mechanical stiffness)
- Swelling - warm swelling 
- Pattern of joint involvement - small joints/ non weight bearing joints normally affected first (wrists, hands, ankles)
Evolution 
- Acute or chronic?
- Associated events 
- Response to treatment 
- Family history 
Involvement
- Skin, eyes or lungs 
- Malaise, weight loss, fevers, night sweats 
Impact on patients lifestyle
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6
Q

What are the constitutional symptoms?

A 
Symptoms that are systemic and affect the whole body 
- Fever 
- Fatigue 
- Weight loss 
- Night sweats
- Poor appetite 
Cancer has to be excluded
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7
Q

What is the glove and sweater approach?

A

Systematic approach to areas to look at when trying to determine whether someone has ARD
Gloves - hands
Sweater - arms and trunk

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8
Q

What are you looking for when examining the glove area?

A
  • Raynauds (hyper exaggerated response to cold, stress etc) → colour of hand? pale/yellow (vasospasms of digits) → cyanotic digits → red (return of circulation) - (triphasic pattern of raynauds)
  • Joint pain and swelling
  • Hand rash
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9
Q

What are you looking for when examining the sweater area?

A
  • Proximal muscle weakness → myalgia (difficulty washing hair- inflammation of muscles causes more weakness than pain)
  • Hair loss → bald patches, hair on pillow on waking
  • Eye and mouth dryness → red eyes, difficulty looking at bright lights, uveitis
  • Nose bleeds → vasculitis
  • Mouth ulcers → sjogrens
  • Pleuritic chest pain
  • Pericardial pain
  • Truncal rash/ photosensitivity
  • Limb weakness - nerve involvement
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10
Q

What other investigations would you do for ARD?

A

Bloods

  • FBC
  • U&E and creatinine
  • Liver enzymes
  • C-reactive protein (lupus- CRP normal, infection raised)
  • Plasma viscosity and ESR
  • Autoantibodies / lupus associated bloods
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11
Q

What would you expect to see on examination of a patient with Lupus?

A

Swollen joints
Facial rash - mallor rash
Hair loss (significant loss)
Bloods- low WCC, antinuclear antibodies, anti-Smith antibodies

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12
Q

What is the treatment for SLE?

A

Patient education- life style modification, use of sunscreen (SPF= 50 at least)
Start DMARDs: Hydroxychloroquine, Azathioprine, Mycophenolate
Use of steroids: Prednisolone, methylprenisolone
In severe cases: IV cyclophopamides

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13
Q

What is a good mnemonic for Lupus?

A 
A RASH POINts Medical Diagnosis
A- ANA positive
R- Renal abnormalities 
A- Arthralgia/arthritis 
S- Serositis
H- Haematological abnormalities 
P- Photosensitivity 
O- Oral ulcers 
I- Immunological abnormalities 
N- Neurological abnormalities 
M- Malar rash/ D- Discoid rash 
4/11 criteria = definite lupus
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14
Q

What is the epidemiology of rheumatoid arthritis?

A

F:M → 3:1
Prevalence → 1%
No race predilection
Genetic factors/ environmental factors → genetically predisposed

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15
Q

What are the 3 things to look out for in a history of someone with suspected Rheumatoid arthritis?

A

S, S, S
Stiffness → early morning joint stiffness lasting over 30 mins
Swelling → persistent swelling of one joint or more, especially hand joints
Squeezing → squeezing the joints is painful in inflammatory arthritis

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16
Q

What would you expect to see on examination of someone with rheumatoid arthritis?

A

Swelling in hands

Swelling in feet

17
Q

What other investigations are carried out for rheumatoid arthritis?

A

Blood test - confirm diagnosis and tell you about prognosis
- Full blood count
- U&Es, and creatinine
- Liver enzymes
- CRP
- Plasma viscosity and ESR (erythrocyte sedimentary ratio)
- Autoantibodies → ANA, rheumatoid factor Ab, anti-CCP Ab, anti Ro and La antibodies
X-ray and/or ultrasounds

18
Q

What is the classification criteria for RA?

A

Points given for joint distribution, serology, symptom duration, acute phase reactants
>/= 6 point definite RA
- More points given for joint involvement than serology results

19
Q

How is RA treated?

A

Start DMARDs early: Methotrexate hydroxychloroquine, sulfasalazine, leflunomide
Use of steroids: prednisolone, methylprednisolone
Combination therapy is usual

20
Q

What is the role of a rheumatologist?

A
  • Prompt diagnosis of patients with suspected ARDs/ non- ARDs
  • Oversee use of DMARDs in these patients
  • Holistic/long term management of patient with ARDs
21
Q

What is RA?

A
  • Autoimmune disease with autoantibodies to the Fc portion of immunoglobulin G (rheumatoid factor) and citrullinated cyclic peptides
  • Persistent synovitis causing chronic symmetrical polyarthritis with systemic inflammation
  • Can develop non articular problems affecting all parts of the body
22
Q

How does RA present?

A

Approx 70% cases progressive, symmetrical, peripheral polyarthritis evolving over a period of a few weeks or months in patients 30-50yrs

23
Q

How do the symptoms of RA occur?

A
  • Primarily a synovial disease and synovitis occurs when chemoattractants produced in the joint recruit circulating inflammatory cells
  • Over production of tumour necrosis factor TNF- alpha leads to synovitis and joint destruction
  • Interaction of macrophages and T and B lymphocytes drives this over production
  • TNF alpha stimulates the over production of IL-6 as well as other cytokines
24
Q

How do some of the targeted biological therapies work?

A

Blockade of TNF-alpha and IL-6 has produced marked improvements in synovitis and systemic malaise
Indicating the pivotal role of these cytokines in chronic synovitis

25
Q

What is the D-dimer test?

A

Level of D-dimer can be used to assess the level of clotting

26
Q

How does the D-dimer test work?

A
  • Blood clotting (coagulation) → stop bleeding from a damaged blood vessel → maintain a constant blood volume- hemostasis
  • Coagulation → formation of a clot, thrombus, consisting of a plug of platelets and meshwork of the protein fibrin
  • Clots are temporary patches that must be removed once wound repair has begun
  • Fibrin clot cleaved by the protein plasmin to fibrin degradation products
  • D-dimer is a fibrin degradation product - two cross-linked D domains released by the action of plasmin
27
Q

What is needed for the D-dimer test?

A

Blood based assay that uses antibodies to the d-dimer protein to measure the presence of level of circulating d-dimer

28
Q

What are elevated levels of D-dimer associated with?

A
  • Elevated fibrin or clot somewhere in the body
  • Can occur for a variety of reasons - acute PE, DVT, malignancy, pregnancy, chronic inflammatory conditions, recent surgery, infection, stroke, myocardial infarction, advance age
29
Q

What is a negative test useful for?

A

Ruling out PE or other significant clot

_Sem 4. Immune System NOTES (7 decks)

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