Autoimmune rheumatic diseases Flashcards

1
Q

What is most rheumatic disease?

A

most rheumatic disease is multisystematic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is inflammation of the:

1) joints?
2) lungs?
3) vasculature
4) skin
5) nervous s
6) eyes
7) kidneys
8) muscles?

A

joints = synovitis

lungs = alveolities / and end stage pulmonary fibrosis, reduced gas exchange and resp failure

veins and arteries = vasculitis inflammation of vessel wall, thrombosis due to impaired flow

Skin = rashes

nervous system = neuropathies or central nervous sytem and cerebral damage

eyes = retinitis

kidneys = nephritis

muscles = myostitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the cause of most rheumatic disease?

A

most rheumatic disease is autoimmune

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the components of autoimmune reaction in rheumatic disease and how are they evidenced?

A
  • Autoreactive T cells (can be found at the site of inflammation)
  • Autoreactive B/ plasma cells –> autoantibodies (found within blood plasma and at site of inflammation)
  • Antibodies detectable at sites of tissue damage
  • immunosuppresion treats the disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are some examples of organ specific autoimmune diseases?

A

Brain –> multiple sclerosis

thyroid –> hashimotos thyroiditis, primary myxedema, thyrotoxicosis

muscle –> myasthenia gravis against nAchR

stomach –> pernicious anaemia

Adrenal –> addison’s disease

pancreas –> insulin dependent diabetes mellitus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are antinuclear antibodies?

When is an ANA test requested?

A

ANA test identifies the presence of antinuclear antibodies (ANA) in blood.

ANA’s are antibodies directed against components within the cell’s nucleus.

ANA test is requested in cases of suspected autoimmune disease, ANA antibodies is associated with several autoimmune diseases but is most commonly seen in systemic lupus erythematosus (SLE).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

When are anti-DNA antibodies most commonly seen?

A

Anti DNA antibodies most commonly seen in SLE.

Because the ANA test result may be positive in a number of these other diseases, SLE can be tricky to diagnose correctly and additional tests may be used to help. For example, tests to look for antibodies to ENA and dsDNA are used to help interpret the ANA results. All of the results are used in conjunction with patient symptoms and clinical history to find a diagnosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are ENA’s?

When is this test requested?

what can it help distinguish between?

A

ENA is an antigen directed against one or more proteins within the cell’s nucleus. These proteins are known as extractable as they can be removed from the cell nuclei with saline.

ENA test often requested following a postive ANA test with pts with symptoms of autoimmune disorder.

The 4-test ENA panel is used to help diagnose mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), and Sjögren syndrome. The 6-test ENA panel can also help identify scleroderma (systemic sclerosis) and polymyositis/dermatomyositis (inflammatory myopathies).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What antibodies may be found in SLE?

A

Anti DNA

Anti - Sm

others may be found but these are most common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What anti nuclear antibodies and ENA’s may be found in systemic sclerosis?

A

Anti-scl70

Anti centromere

both common in systemic sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What anti nuclear antibodies and ENA’s may be found in sjrogrens syndrome?

A

Anti-Ro

Anti -La

both common in sjorgren’s syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What anti nuclear antibodies and ENA’s may be found in Anti Jo -1 syndrome?

A

Anti- JO-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is the significance of a positive ANA assessed?

A

Positive ANA’s are common in healthy people

therefore the significance of a positive ANA needs to be interpreted alongside the pts symptoms and signs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are 4 common symptoms in patients with autoimmune rheumatic diseases?

A

1) Secondary Raynaud’s phenomenon (note primary raynauds phenomenon is common and 10% of healthy women have it).
2) Joint pain
3) Rashes
4) Fatigue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the three phases of Raynaud’s phenomenon?

A

After arterial spasm –> Fingers tends to go white, then blue, then hyper red

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the difference between primary and secondary raynauds?

A

Primary raynaud’s comes on earlier in life, occurs in 10% of healthy women.

Secondary rayauds comes on later in life and could be a sign there is underlying autoimmune disease

17
Q

What are some examples of multisystem rheumatic diseases?

A

Rheumatoid arthritis (RA)

Systemic Lupus erythematosus (SLE)

Primary Sjogren’s syndrome

Systemic sclerosis

Anklosing spondylitis (AS)

Primary vasculitis e.g. granulomatous polyangitis (GPA)

18
Q

What are the typical diagnostic features of SLE?

A
  • commonly affects women in reproductive years
  • presence of antinuclear antibodies
  • Rash:
    • malar / butterfly rash
    • photosensitive rash
    • discoid rash (forms disc shape in areas exposed to sunlight)
  • common sx:
    • fatigue, fever,
    • weight loss
    • oral ulcers
    • alopecia
    • arthritis
    • fibromyalgia (musculoskeletal pain)
    • raynaud’s phenomenon
    • abdo pain plus vomiting, diarrhoea
    • lymphadenopathy
    • venous/arterial thrombosis
    • signs of nephrosis
    • HTN
    • chest pain/ SOB
19
Q

Sjogren syndrome (SS):

What is it? What types are there?

A

Sjogren syndrome = systemic autoimmune disorder characterised by presence of dry eyes, dry mouth, as consequence of lymphocytic infiliration into lacrimal and salivary glands.

Two types:

Primary Sjogren syndrome –> occurs alone

secondary sjrogrens syndrome –> occurs with other autoimmune disease, commonly lupus/RA/ systemic sclerosis

20
Q

What is the prevalence of sjrogrens in UK women?

What is damaged?

How does it develop?

when does it present?

who does it affect more?

A

prevalance up to 0.4% of adult women in UK

autoimmune damage to secretory glands –> salivary, lacrimal, other mucosa within airways/ gut/ genitourinary tract

Takes years to develop

most commonly seen in 50-60 yrs age group

females > males

21
Q

What are the symptoms of sjogren’s?

A

Sicca symptoms:

Dry eyes, xerostomia, dry vagina, cough

Increase dental caries

inflammatory arthritis

Rashes –> can look similar to SLE

rare –> pulmonary fibrosis, renal involvement (nephritis) and peripheral neuropathies

22
Q

What are some examination findings of sjogrens syndrome?

A

Reduced tear flow – >Schirmer test (measure how wet strip is within 5 minutes)

dry oral mucosa, dental decay

oral candidiasis

salivary gland swelling

Plus skin involvement –> palpable purpura, often vasculitic rash. Or annular rash (named subacute cutaneous lupus)

23
Q

What investigations are done in sjogren’s syndrome?

A

1) FBC –> look for anaemia of chronic disease (low Hb due to inflammatory response reducing blood cell production), sometimes low WBC’s and platelets
2) Raised immunoglobulins due to B cell activation, high IgG
3) High ESR e.g. ESR 80 mm/hr (as a result of high IgG, high viscosity of RBC’s)
4) ANA positive
5) Anti Ro/La positive
6) salivary gland biopsy –> intense blue staining, picking up lymphocytes, called lymphocytic sialadenitis, particular focus score is diagnostic

24
Q

How do we treat sjogren’s syndrome?

A

Sicca sx: –> humidified environement, wear glasses, punctal plugs, tear/saliva substitutes, chew sugar free gum, saliva stimulants e.g. pilocarpine

Oral candiasis with topical antifungal nystatin

dental care –> avoid sweet foods, fluoride toothpaste, regular dental check ups

inflammatory arthritis –> immunosuppresants methotrexate

Rashes –> hydroxychloroquine

25
Q

What secondary diseases can patients with sjogrens syndrome develop?

A

1) Autoimmune thyroid disease –> hypothyroid
2) coeliac disease –> autoimmune disease of small bowel
3) primary biliary cirrhosis –> autoimmune damage of biliary tree

26
Q

What are some of the complications of sjogren’s syndrome?

A
  • incidence of lymphoma in SS = 40x higher (lymphoma within salivary glands or lymph glands in the gut, present with malabsorption)
  • inflammatory lung disease
  • vasculitis / peripheral neuropathies