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Endocrine / Chemical Pathology > Histo: Connective tissue disease, amyloid, sarcoid, Immune related multisystem disorders > Flashcards

Histo: Connective tissue disease, amyloid, sarcoid, Immune related multisystem disorders Flashcards

1
Q

Give an example of an autoimmune disease that is:

  1. Organ-specific with a specific antigen
  2. Organ-specific without a specific antigen
  3. Multisystem disease
A
  1. Organ-specific with a specific antigen: pernicious anaemia
  2. Organ-specific without a specific antigen: primary biliary cirrhosis
  3. Multisystem disease: rheumatoid arthritis, Sjogren syndrome, SLE
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2
Q

What are the main features of SLE?

A

SOAP

  • Serositis
  • Oral ulcers
  • ANA
  • Photosensitivity

Brain

  • Bloods (low counts)
  • Renal (proteinuria)
  • Arthritis
  • Immunological (anti-dsDNA)
  • Neurological (psychiatric, seizures)

MD

  • Malar rash
  • Discoid rash

SOAP BRAIN MD

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3
Q

What units are used for ANA levels?

A

It is a titre - the highest dilution at which you can see the fluorescence (e.g. 1:1000 is a higher level than 1:10)

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4
Q

List three autoantibodies found in SLE. Which is most specific?

A
  • Anti-dsDNA
  • Anti-Smith (Sm, against ribonucleoproteins) - most specific but low sensitivity
  • Anti-histone - drug-related (e.g. hydralazine)
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5
Q

How is anti-dsDNA measured?

A

ELISA

Crithidia luciliae

  • Incubate the patient’s serum with Crithidia Luciliae (protozoan)
  • It has a big organism with dsDNA (kinetoplast) so if the patient has anti-dsDNA antibodies it will bind to the dsDNA in the kinetoplast
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6
Q

What can be seen on blood film in SLE?

A

LE (lupus erythromatosus) cell - neutrophil or macrocyte that has engulfed nuclear material of another cell

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7
Q

Describe the appearance of skin histology in SLE.
What would immunofluorescence show?

A
  • Lymphocytic infiltration of the dermis
  • Vacuolisation (dissolution of the cells) of the basal epidermis
  • Extravasation of blood causes a rash

NOTE: immunofluorescence will show immune complex deposition at the epidermis-dermis junction

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8
Q

Describe the appearance of renal histology in SLE.

A

Thickened pink glomerular capillaries (wire-loop capillaries) due to immune complex deposition.

NOTE: immune complex deposition can be visualised by immunofluorescence

This is what a normal glomerulus looks like

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9
Q

What is the name of non-infective endocarditis associated with SLE?

A

Libman-Sacks endocarditis

NOTE: the vegetation is made up of fibrin, lymphocytes, neutrophils, histocytes

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10
Q

What is scleroderma?

A

An autoimmune condition characterised by excess collagen deposition and fibrosis

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11
Q

What is the localised skin-only form of scleroderma called?

A

Morphoea (sclerosis of skin only)

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12
Q

What are the two types of scleroderma?
Name the antibodies that they are associated with.

A

Diffuse

  • Involves the trunk and proximal limbs
  • Anti-Scl70 antibodies (anti-topoisomerase)

Limited (CREST syndrome)

  • Only affects skin distal to the elbows and knees
  • Anti-centromere antibodies
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13
Q

What are the main features of limited cutaneous systemic sclerosis (CREST syndrome)?

A
  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
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14
Q

What pattern of ANA immunofluorescence is seen in scleroderma?

A

Nucleolar

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15
Q

Describe the skin histology in scleroderma.

A

Increased depth and amount of collagen (results in reduced skin elasticity)

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16
Q

How does scleroderma cause gastric and oesophageal dysmotility?

A

Collagen deposition (fibrosis) with the submucosa - affects muscle contraction

17
Q

Describe the vascular histology in scleroderma.

A

Intimal proliferation gives an onion skin appearance.

18
Q

What is a major consequence of vascular damage in scleroderma?

A

Renal hypertensive crisis

19
Q

What is mixed connective tissue disease?

A

A condition characterised by the overlap of several connective tissue diseases including SLE, scleroderma, polymyositis and dermatomyositis

20
Q

What ANA immunofluorescence pattern is seen in mixed connective tissue disease?

A

Speckled

21
Q

What is dermatomyositis?

A

A condition characterised by proximal muscle pain and weakness and skin changes

22
Q

Which marker would be raised in the blood in dermatomyositis?

A

Creatine kinase

23
Q

What signs would be seen on the skin in dermatomyositis?

A
  • Gottren’s papules
  • Heliotrope rash
  • Facial rash
  • Systemic v-shaped rash
24
Q

What is sarcoidosis of the skin called?

A

Lupus pernio

25
Q

List some features of sarcoidosis.

A
26
Q

What is the pathological hallmark of sarcoidosis?

A

Non-caseating granuloma

Langhans giant cell, epitheloid macrophages, lymphocytes

27
Q

What are some biochemical changes seen the blood in sarcoidosis?

A
  • Hypergammaglobulinaemia
  • Raised ACE
  • Hypercalcaemia (due to 1-alpha hydroxylase expression by granuloma macrophages)
28
Q

What criteria is used to classify vasculitides based on the size of the vessel?

A

Chapel Hill Criteria

29
Q

What is polyarteritis nodosa? What are its main features?

A
  • A necrotising arteritis which is focal and sharply demarcated
  • It heals by fibrosis and mainly affects the renal and mesenteric vessels
  • May present with gut ischaemia or renal impairment
  • It produces a rosary beads (nodular) appearance on angiography due to multiple aneurysms
30
Q

Which infection is polyarteritis nodosa associated with?

A

Hepatitis B

31
Q

What is a characteristic feature of vasculitis?

A

Palpable purpuric rash

32
Q

How is temporal arteritis diagnosed and treated?

A

Diagnosis

  • Clinical
  • High ESR
  • Temporal artery biospy

Treatment

  • High dose prednisolone
33
Q

What will be seen on temporal artery biopsy in temporal arteritis?

A

Lymphocytic infiltration of the tunica media

34
Q

What is Kawasaki disease? What are the main clinical features?

A

Medium vessel vasculitis

Fever lasting longer that 5 days accompanied by:

  • Conjunctivitis
  • Rash
  • Adenopathy
  • Strawberry tongue
  • Hand and feet skin desquamation
35
Q

What is a complication of Kawasaki’s?

A

Coronary artery aneurysm (screen using echocardiogram)

36
Q

What are the main features of granulomatosis with polyangiitis (Wegner’s)?

A
  • ENT - nosebleeds, sinusitis, saddle nose
  • Lungs - haemoptysis, SoB
  • Kidneys - haematuria
37
Q

Which antibody is associated with granulomatosis with polyangiitis?

A

cANCA - directed against proteinase 3

38
Q

What are the main features of eosinophilic granulomatosis with polyangitis (Churg-Strauss)?

A
  • Asthma (1st stage)
  • Eosinophilia (2nd stage)
  • Vasculitis (3rd stage)
39
Q

Which antibody is associated with Churg-Strauss syndrome?

A

pANCA - directed against myeloperoxidase

Endocrine / Chemical Pathology (20 decks)

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