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PATH FINAL > 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 (recurrent inflammation of pleura/pericardial sac - recurrent pleuritic chest pain)
  • Oral ulcers
  • ANA
  • Photosensitivity
  • Bloods (pancytopenia: MAHA, ITP, leucopenia)
  • 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-nuclear antibodies (ANA):
* Anti-dsDNA
* Anti-smith (against ribonucleoproteins) - most specific but low sensitivity
* Anti-histone - drug-related (e.g. hydralazine)

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5
Q

Which autoantibody is present and specific to drug-related SLE

A

anti-histone antibody

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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 RBCs into upper dermis causes the typical rash seen.
  • 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

Patient with SLE can present with endocarditis (e.g. new onset murmur, stroke, embolic event but not grow anything on cultures - 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 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 II))
  • Limited - only affects distal to the elbows and knees (anti-centromere)
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11
Q

What are the main features of limited cutaneous systemic sclerosis?

A
  • Calcinosis (painful deposits of Ca in tips of fingers)
  • Raynaud’s phenomenon
  • Esophageal dysmotility (difficulty eating)
  • Sclerodactyly (tight skin in fingers - hard to stretch)
  • Telangiectasia

CREST syndrome

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12
Q

What are the main features of diffuse scleroderma

A
  • Skin changes (tightness) can occur anywhere (including truncal involvement!).
  • Tendon friction
  • Raynauld’s phenomenon
  • Widespread organ involvement: early heart, GI and renal disease. Associated with pulmonary fibrosis.
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13
Q

What pattern of ANA immunofluorescence is seen in scleroderma?

A

Systemic = Nucleolar
CREST = Centromere pattern

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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

Tunica Intima proliferation and collagen deposition 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

17
Q

What is mixed connective tissue disease?

What antibody is associated?

A

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

Antibody: Anti-RNP (ribonucleoprotein)

18
Q

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

A

Speckled

19
Q

What is dermatomyositis and polymyositis?

Which antibodies is associated?

A

A condition characterised by proximal muscle pain and weakness and inflammation on muscle biopsy.
If associated with skin changes = dermatomyositis.

Antibody = Anti-Jo 1

20
Q

What is commonly associated with dermatomyositis and polymyositis

A

MALIGNANCY

Dermatomyositis = ovarian, pancreatic, NHL

Polymyositis = lung, bladder, NHL

21
Q

Classical blood results seen in dermatomyositis / polymyositis

A

High CK
High myoglobin
High LDH

22
Q

Clinical feature of polymyositis and dermatomyositis

A

Both have proximal muscle weakness –> difficulty performing gross motor tasks (e.g. getting up from a chair, climbind steps, combing hair).

Dermatomyositis has additional associated skin changes:
- grotton papules (erythema of knuckles w raised scaly eruption)
- Heliotrope rash with eyelid oedema
- Systemic v shaped rash

23
Q

What is sarcoidosis of the skin called?

A

Lupus pernio

24
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
25
Q

What is the pathological hallmark of sarcoidosis?

A

Non-caseating granuloma

26
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
27
Q

Which investigations are useful in sarcoidosis?

A
  • Hypergammaglobulinaemia
  • High ACE
  • Hypercalcaemia (due to ectopic 1-alpha hydroxylation of vitamin D by macrophages)
  • CXR = Bilateral hilar lymphadenopathy
28
Q

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

A

Chapel Hill Criteria

29
Q

List the main diseases that full under:
- Large vessel vasculitis
- Medium vessel vasculitis
- Small vessel vasculitis

A

Large vessel vasculitis
- Temporal arteritis (Giant cell arteritis)
- Takayasu arteritis

Medium Vessel vasculitis
- Polyarteritis nodosa
- Kawasaki disease

Small vessel vasculitis
- Granulomatosis with polyangiitis
- Eosinophilic granulomatosis with polyangitis

30
Q

What is a characteristic feature of vasculitis?

A

Palpable non-blanching petechiael / purpuric rash

31
Q

Temporal Arteritis recap:
- Main symptoms
- Diagnosis (investigations + histology)
- Treatment

A

Main symptoms
- Scalp tenderness, temporal headache, jaw claudication, blurred vision
- Elderly (>50)

Diagnosis
- High ESR
- Temoral artery biopsy = granulomatous transmural inflammation with giant cells and narrowed lumen

Treatment:
* High dose prednisolone

32
Q

What is polyarteritis nodosa? What are its main features?

A
  • A necrotising medium sized 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
33
Q

Which condition is polyarteritis nodosa associated with?

A

Hepatitis B

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

REMEMBER THIS TRIAD!

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 polyangitis (Churg-Strauss syndrome)?

A
  • Asthma
  • Eosinophilia
  • Vasculitis
38
Q

Which antibody is associated with Churg-Strauss syndrome?

A

pANCA - against myeloperoxidase

PATH FINAL (36 decks)

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