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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
What criteria is used to classify vasculitides based on the size of the vessel?
Chapel Hill Criteria
26
What is polyarteritis nodosa? What are its main features?
* A medium vessel vasculitis that causes focal/sharply demarcated necrotising arteritis * It heals by fibrosis * Mainly affects the renal and mesenteric vessels
27
Which condition is polyarteritis nodosa associated with?
Hepatitis B
28
What is a characteristic feature of vasculitis?
Palpable purpuric rash
29
How is termporal arteritis diagnosed and treated?
* High ESR * Temporal artery biopsy = **definitive** **Management** - high dose prednisolone
30
What will be seen on temporal artery biopsy in temporal arteritis?
* Lymphocytic infiltration of the tunica media * Granulomatous transmural inflammation * Giant cells * Skip lesions
31
What is Kawasaki disease? What are the main clinical features?
* A medium vessel vasculitis * Conjunctivitis * Rash * Adenopathy * Strawberry tongue * Hands and feet rash * Fever \> 38 degrees for \> 7 days * Coronary artery aneurysms
32
What are the main features of granulomatosis with polyangiitis?
* **ENT** - nosebleeds, sinusitis, saddle nose * **Lungs** - haemoptysis, SOB * **Kidneys** - haematuria
33
Which antibody is associated with granulomatosis with polyangiitis?
cANCA - against proteinase 3
34
What are the main features of Churg-Strauss syndrome?
* Asthma * Eosinophilia * Vasculitis
35
Which antibody is associated with Churg-Strauss syndrome?
pANCA - against myeloperoxidase
36
How would polyarteritis nodosa present?
* May present with gut ischaemia or renal impairment * Can involve other organs but generally spares the lungs
37
What would polyarteritis nodosa look like on histology?
* Fibroid necrosis * Neutrophil infiltration
38
How would polyarteritis nodosa appear on angiography?
"string of pearls" or "rosary bead" appearance
39
What type hypersensitivity reaction is SLE an example of?
Type 3
40
What is the epidemiology of SLE?
Higher in Afro-Caribbeans F > M
41
What is the HLA association for SLE?
HLA DR3/2
42
What is the HLA association for scleroderma?
HLA DR5 and DRw8
43
What is CREST syndrome associated with in old age?
Pulmonary hypertension
44
What are the features of diffuse scleroderma?
* Tendon friction * Reynauld's phenomenon * Widespread organ involvement - especially early with heart, GI and renal disease * Associated with pulmonary fibrosis
45
What is required for a definitive diagnosis of poly/dermatomyositis?
Inflammation on muscle biopsy (abnormal EMG)
46
Which antibodies are present in poly/dermatomyositis?
Anti Jo-1 (against tRNA synthetase)
47
What is elevated in poly/dermatomyositis?
CK LDH Myoglobin
48
How would poly/dermatomyositis present?
* Difficulty doing gross motor tasks For example - getting up from a chair, climbing steps, combing hair
49
What are the cutaneous features of dermatomyositis?
* Heliotrope rash with eyelid oedema * Gottron papules - erythema over knuckles with raised scaly eruption * Systemic v-shaped rash * Facial rash
50
What is Takayasu's arteritis?
Arteritis of branches of the aortic arch, resulting in a "pulseless" presentation, claudication and cold hand
51
What is Takayasu's arteritis?
Arteritis of branches of the aortic arch, resulting in a "pulseless" presentation, claudication and cold hands
52
What other condition is GCA associated with?
Polymyalgia rheumatica
53
What are the SSx of GCA?
* Scalp tenderness * Temporal headache * Jaw claudication * Blurred vision * Non-palpable temporal pulse
54
What is Buerger's disease?
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
What would the angiogram look like in Beuger's disease?
**Corkscrew appearance** from segmental occlusive lesions
56
Which is the more common type of amyloidosis?
AL (primary)
57
What is AA amyloidosis?
Amyloid from serum amyloid A (acute phase protein) as a result of chronic inflammation or infection
58
When may AA amyloidosis be seen?
Rheumatoid arthritis IBD TB osteomyelitis Hodgkin's lymphoma Ankylosing spondylitis
59
What are the main types of amyloidosis?
* AL - most common (primary) * AA - secondary * Haemodyialysis-associated * Familial (AA)
60
What is another name for Churg-Strauss syndrome?
Eosinophilic granulomatosis with polyangiitis
61
What are some SSx of amyloidosis?
* 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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