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YEAR 5: Histopathology > 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
  • Oral ulcers
  • ANA
  • Photosensitivity
  • Bloods (low counts)
  • Renal (proteinuria)
  • Arthritis
  • Immunological (anti-dsDNA)
  • Neurological (psychiatric, seizures)
  • Malar rash
  • Discoid rash

NOTE: 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 (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
  • 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?

A

A condition characterised by excess collagen in the skin and fibrosis

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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
  • Raynaud’s phenomenon
  • Oesophageal dysmotility
  • Sclerodactyly
  • 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 and thickening of arterioles 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

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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)

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18
Q

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

A

Speckled

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19
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)

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20
Q

What is sarcoidosis of the skin called?

A

Lupus pernio

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21
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
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22
Q

What is the pathological hallmark of sarcoidosis?

A

Non-caseating granuloma

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23
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
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24
Q

Which investigations are useful in sarcoidosis?

A
  • Hypergammaglobulinaemia
  • High ACE
  • Hypercalcaemia (due to acitvation of vitamin D by macrophages)
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25
Q

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

A

Chapel Hill Criteria

26
Q

What is polyarteritis nodosa? What are its main features?

A
  • A medium vessel vasculitis that causes focal/sharply demarcated necrotising arteritis
  • It heals by fibrosis
  • Mainly affects the renal and mesenteric vessels
27
Q

Which condition is polyarteritis nodosa associated with?

A

Hepatitis B

28
Q

What is a characteristic feature of vasculitis?

A

Palpable purpuric rash

29
Q

How is termporal arteritis diagnosed and treated?

A
  • High ESR
  • Temporal artery biopsy = definitive

Management - high dose prednisolone

30
Q

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

A
  • Lymphocytic infiltration of the tunica media
  • Granulomatous transmural inflammation
  • Giant cells
  • Skip lesions
31
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
32
Q

What are the main features of granulomatosis with polyangiitis?

A
  • ENT - nosebleeds, sinusitis, saddle nose
  • Lungs - haemoptysis, SOB
  • Kidneys - haematuria
33
Q

Which antibody is associated with granulomatosis with polyangiitis?

A

cANCA - against proteinase 3

34
Q

What are the main features of Churg-Strauss syndrome?

A
  • Asthma
  • Eosinophilia
  • Vasculitis
35
Q

Which antibody is associated with Churg-Strauss syndrome?

A

pANCA - against myeloperoxidase

36
Q

How would polyarteritis nodosa present?

A
  • May present with gut ischaemia or renal impairment
  • Can involve other organs but generally spares the lungs
37
Q

What would polyarteritis nodosa look like on histology?

A
  • Fibroid necrosis
  • Neutrophil infiltration
38
Q

How would polyarteritis nodosa appear on angiography?

A

“string of pearls” or “rosary bead” appearance

39
Q

What type hypersensitivity reaction is SLE an example of?

A

Type 3

40
Q

What is the epidemiology of SLE?

A

Higher in Afro-Caribbeans
F > M

41
Q

What is the HLA association for SLE?

A

HLA DR3/2

42
Q

What is the HLA association for scleroderma?

A

HLA DR5 and DRw8

43
Q

What is CREST syndrome associated with in old age?

A

Pulmonary hypertension

44
Q

What are the features of diffuse scleroderma?

A
  • Tendon friction
  • Reynauld’s phenomenon
  • Widespread organ involvement - especially early with heart, GI and renal disease
  • Associated with pulmonary fibrosis
45
Q

What is required for a definitive diagnosis of poly/dermatomyositis?

A

Inflammation on muscle biopsy (abnormal EMG)

46
Q

Which antibodies are present in poly/dermatomyositis?

A

Anti Jo-1 (against tRNA synthetase)

47
Q

What is elevated in poly/dermatomyositis?

A

CK
LDH
Myoglobin

48
Q

How would poly/dermatomyositis present?

A
  • Difficulty doing gross motor tasks

For example - getting up from a chair, climbing steps, combing hair

49
Q

What are the cutaneous features of dermatomyositis?

A
  • Heliotrope rash with eyelid oedema
  • Gottron papules - erythema over knuckles with raised scaly eruption
  • Systemic v-shaped rash
  • Facial rash
50
Q

What is Takayasu’s arteritis?

A

Arteritis of branches of the aortic arch, resulting in a “pulseless” presentation, claudication and cold hand

51
Q

What is Takayasu’s arteritis?

A

Arteritis of branches of the aortic arch, resulting in a “pulseless” presentation, claudication and cold hands

52
Q

What other condition is GCA associated with?

A

Polymyalgia rheumatica

53
Q

What are the SSx of GCA?

A
  • Scalp tenderness
  • Temporal headache
  • Jaw claudication
  • Blurred vision
  • Non-palpable temporal pulse
54
Q

What is Buerger’s disease?

A

Inflammation of the arteries of the extremities, usually involving the tibial and radial arteries and resulting in painful ulceration of the toes, feet and fingers

Associated with young men, heavy smokers.

55
Q

What would the angiogram look like in Beuger’s disease?

A

Corkscrew appearance from segmental occlusive lesions

56
Q

Which is the more common type of amyloidosis?

A

AL (primary)

57
Q

What is AA amyloidosis?

A

Amyloid from serum amyloid A (acute phase protein) as a result of chronic inflammation or infection

58
Q

When may AA amyloidosis be seen?

A

Rheumatoid arthritis
IBD
TB osteomyelitis
Hodgkin’s lymphoma
Ankylosing spondylitis

59
Q

What are the main types of amyloidosis?

A
  • AL - most common (primary)
  • AA - secondary
  • Haemodyialysis-associated
  • Familial (AA)
60
Q

What is another name for Churg-Strauss syndrome?

A

Eosinophilic granulomatosis with polyangiitis

61
Q

What are some SSx of amyloidosis?

A
  • Nephrotic syndrome - most common presentation
  • Restrictive cardiomyopathy, heart failure, cardiomegaly
  • Macroglossia
  • Neuropathy (e.g. carpal tunnel syndrome)
  • Hepatosplenomegaly

YEAR 5: Histopathology (20 decks)

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