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YEAR 5: HISTOPATH > 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
  • Serositis - note can cause pleuritic chest pain, abdo pain
  • Oral ulcers
  • ANA - Anti nuclear antibodies
  • Photosensitivity
  • Bloods - pancytopenia (low HB, low platelets, low WBC)
  • Renal (proteinuria)
  • Arthritis
  • Immunological (anti-dsDNA)
  • Neurological (psychiatric, seizures)
  • Malar rash
  • Discoid rash

NOTE: SOAP BRAIN MD

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3
Q

What is ANA used for

A

Anti-nuclear antibody

Used as a screening test –> high sensitivity, low specificity

If -ve –> good at ruling out SLE
If +ve –> means it could be SLE among other things

the higher the titre –> the more +ve

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4
Q

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

A
  • Anti-dsDNA -
  • Anti-smith (against ribonucleoproteins)
  • Anti-histone - seen in drug-related SLE (e.g. hydralazine)
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5
Q

How is anti-dsDNA measured?

A
  • 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

NOTE: it can also be measured with ELISA

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6
Q

Describe the appearance of skin histology in SLE.

A
  • Lymphocytic infiltration of the dermis
  • Vacuolisation (dissolution of the cells) of the basal epidermis
  • Extravasation of blood causes a rash
  • Immunofluorescence will show immune complex deposition at the epidermis-dermis junction
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7
Q

Describe the appearance of renal histology in SLE.

A

Glomerular capillaries are thickened (wire-loop capillaries) due to immune complex deposition.

NOTE: this can be visualised by immunofluorescence

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8
Q

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

A

Libman-Sacks endocarditis

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

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9
Q

What is scleroderma/systemic sclerosis?

A

A condition characterised by excess collagen in the skin and fibrosis –> tight skin

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10
Q

What is the localised form of scleroderma called?

A

Morphoea

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11
Q

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

A
  • Diffuse - involves the trunk (anti-Scl70 antibodies (aka anti-topoisomerase))
  • Limited - only affects distal to the elbows and knees (anti-centromere)
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12
Q

What are the main features of limited cutaneous systemic sclerosis?

A
  • Calcinosis - painful deposits at tip of fingertips
  • Raynaud’s phenomenon
  • Oesophageal dysmotility
  • Sclerodactyly - tight skin over fingers
  • Telangiectasia

CREST syndrome

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13
Q

What pattern of ANA immunofluorescence is seen in scleroderma?

A

Nucleolar

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14
Q

Describe the skin histology in scleroderma.

A

Increased depth and amount of collagen.

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15
Q

Describe the vascular histology in scleroderma.

A

Intimal proliferation gives an onion skin appearance.

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16
Q

What is a major consequence of vascular damage in scleroderma?

A

Renal hypertensive crisis

Need ACE-inhibitor

17
Q

What is mixed connective tissue disease?

A

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

18
Q

how does dermatomyositis and polymyositis present

A

proximal muscle weakness + tenderness - affects shoulder + pelvic girdle
can progess to respiratory muscle involvement
Can also get interstitial lung disease

dermatomyositis –> skin manifestations as well:
heliotrope rash in perioorbital
gottron’s papules

19
Q

what are the bloods in polymyositis and dermatomyositis

A

raised CK

ANA +Ve

Anti-Jo-1
Anti-Mi-2

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, high CK and skin changes (e.g. Gottron’s papules - purple rash across the knuckles)

22
Q

What is sarcoidosis of the skin called?

A

Lupus pernio

23
Q

List some features of sarcoidosis.

A
  • Arthritis
  • Lupus pernio
  • Erythema nodosum
  • Bilateral hilar lymphadenopathy
  • Pulmonary fibrosis
  • Lymphadenopathy
  • Inflammation of layers of the heart
  • Uveitis
  • Meningitis
  • Hepatitis, cirrhosis
  • Bilateral parotid enlargement
24
Q

What is the pathological hallmark of sarcoidosis?

A

Non-caseating granuloma

25
Q

What is a granuloma?

A
  • A collection of activated macrophages (aka histiocytes)
  • The macrophages are sometimes described as epithelioid macrophages
  • Some macrophages will fuse to form multinucleated giant cells - with horseshoe nucleus
26
Q

What is caseating vs non-caseating

A

caseating has necrosis inside of it

27
Q

Which investigations are useful in sarcoidosis?

A
  • Hypergammaglobulinaemia
  • High ACE
  • Hypercalcaemia (due to extra-renal production of 1-alpha hydroxylase by macrophages –> activates vitamin D)
28
Q

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

A

Chapel Hill Criteria

These are primary, can be secondary e.g. to lupus or infection

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 appearance on angiography due to multiple aneurysms
30
Q

Which condition 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 termporal arteritis diagnosed and treated?

A
  • High ESR
  • High dose prednisolone
33
Q

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

A

Lymphocytic infiltration of the tunica media

Granuloma with multinulceate giant cells

34
Q

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

A
  • A medium vessel vasculitis
  • Conjunctivitis
  • Rash
  • Adenopathy
  • Strawberry tongue
  • Hands and feet rash
  • Fever > 38 degrees for > 7 days
  • Coronary artery aneurysms
35
Q

What are the main features of granulomatosis with polyangiitis?

A
  • ENT - nosebleeds, sinusitis, saddle nose
  • Lungs - haemoptysis, SOB
  • Kidneys - haematuria
36
Q

Which antibody is associated with granulomatosis with polyangiitis?

A

cANCA - against proteinase 3

37
Q

What are the main features of eosinophilic granulomatosis with polyangitis?

A
  • Asthma
  • Eosinophilia
  • Vasculitis
38
Q

Which antibody is associated with eoisinophilic granulomatosis with polyangitis?

A

pANCA - against myeloperoxidase

YEAR 5: HISTOPATH (20 decks)

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