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Kat's Pathology > Connective Tissue Disease, Amyloid, Sarcoid, Immune Related Multisystem Disorders > Flashcards

Connective Tissue Disease, Amyloid, Sarcoid, Immune Related Multisystem Disorders Flashcards

1
Q

Name an autoimmune condition with an organ specific with organ specific antibodies

A

Pernicious anaemia

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2
Q

Name an autoimmune condition with an organ specific without organ specific antibodies

A

Primary biliary cirrhosis

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3
Q

Name three multi-system autoimmune diseases (3)

A

Rheumatoid arthritis
Sjorgren’s syndrome
SLE

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4
Q

What systems are affected in SLE (8)

A 
Skin 
Oral ulcers
Joints 
Neurological 
Serositis
Renal 
Haematological 
Immunological
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5
Q

What is positive in SLE

A

ANA (anti-nuclear antibody)

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6
Q

What auto-antibodies are present in SLE (3)

A

Anti-dsDNA
Anti-smith (against ribonucleoproteins)
Anti-histone (drug related e.g. hydralazine)

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7
Q

Where in the skin are auto-antibodies present

A

Dermal-epidermal junction

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8
Q

What is seen in histology of the glomerulus in SLE

A

Wire loops (thickened pink glomerular capillary loops due to immune complex deposition)

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9
Q

What is scleroderma (systemic sclerosis)

A

Fibrosis and excess collagen (localised form is called morphoea in the skin)

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10
Q

What is the immunoflouresence pattern for scleroderma

A

Nucleolar pattern

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11
Q

What antibodies are present in diffuse systemic sclerosis

A

DNA topisomerase (Scl70)

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12
Q

What antibodies are present in limited cutaneous systemic sclerosis

A

Anticentremere antibody

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13
Q

What is CREST syndrome present in

A

Limited cutaneous systemic sclerosis

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14
Q

What are the feature of CREST syndrome (5)

A 
Calcinosis 
Raynauds 
Eosophageal dysmotility 
Sclerodactyly 
Telangiectasia
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15
Q

What are some clinical features of scleroderma on physical examination (5)

A

Scleroderma (tightened skin on hands and face)
Calcinosis - may be visible on ends of fingers (very painful)
Raynaud’s (blue - purple - white)
Nail fold capillary dilatation
Telangiecstasia

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16
Q

What is the pathology in the skin of scleroderma

A

Increased dermal collagen resulting in reduced skin elasticity

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17
Q

What causes oedophageal dysmotility in scleroderma

A

Collagen deposition in the submucosa causes fibrosis (leads to dysmotility)

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18
Q

What changes occur in the arteries (histologically) in scleroderma

A

Onion skin - intimal thickening of small arteries

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19
Q

What are some mixed connective tissue diseases (4)

A

SLE
Scleroderma
Polymyositis
Dermatomyositis

20
Q

What can be seen on the hands of a patient with dermatomyositis

A

Gottron’s papules

21
Q

What organ systems are involved in sarcoidosis (8)

A 
Joints 
Skin 
Lungs 
Lymphadenopathy 
Heart 
Eyes
Neuro 
Liver
22
Q

What condition is lupus pernio associated with

A

Sarcoidosis

23
Q

What condition is erythema nodosum associated with

A

Sarcoidosis

24
Q

What are the skin manifestations of sarcoidosis (2)

A

Lupus pernio

Erythema nodosum

25
Q

What are the CNS manifestations of sarcoidosis (2)

A

Meningitis

Cranial nerve lesions

26
Q

What are the eye manifestations of sarcoidosis (2)

A

Uveitis

Keratoconjunctivitis

27
Q

What are the parotid involvement in sarcoidosis

A

Bilateral enlargement

28
Q

What are the lung involvement manifestations in sarcoidosis (3)

A

BHL
Fibrosis
Lymphocytosis (CD4+ in BAL)

29
Q

What are the liver manifestations of sarcoidosis (3)

A

Hepatitis
Cholestasis
Cirrhosis

30
Q

What are the histological hallmarks of sarcoidosis (3)

A

Non-necrotizing granulomas: histocytes (epithelial cells), multinucleated giant cells of langehans (peripheral nuclei) and lymphocytes

31
Q

What are the biochemical markers of sarcoidosis (3)

A

Hypergammaglobulinaemia
Raised ACE
Hypercalcaemia (Vitamin D hydroxylation by activated macrophages)

32
Q

How is vasculitis classified (4)

A

Large vessel vasculitis
Medium vessel vasculitis
Immune complex small vessel vasculitis
ANCA- associated small vessel vasculitis

33
Q

What are the giant cells vasculitis (2)

A

Takayasu arteritis

Giant cell arteritis

34
Q

What are the medium cell vasculitis (2)

A

Polyarteritis nodosa

Kawasaki disease

35
Q

What are the immune complex small vessel vasculitis (3)

A

Cryoglobulinamic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypercomplementaemic urticarial vasculitis

36
Q

What are the ANCA-associated small vessel vasculitis (3)

A

Microscopic polyangitis
Granulamatosis with polyangitis (Wegner’s)
Eosinophilic granulamatosis with polyangitis (Churg-Strauss)

37
Q

What are the histological features of temporal arteritis (2)

A

Chronic lymphocytic inflammation in the media, giant cells.

Narrowing of the lumen

38
Q

What are the clinical features of kawasaki’s disease (6)

A 
Fever
Erythema of palms and soles, desquamation
Conjunctivitis 
Lymphadenopathy 
Coronary arteries may be affected (MI)
Otherwise disease is self-limiting
39
Q

What are the pathological features of polyarteritis nodosa (6)

A

Necrotising arteritis
Polymorphs, lymphocytes, eosinophils
Arteritis is focal and sharply demarcated
Heals by fibrosis
More often renal and mesenteric arteries
Nodular appearance on angiography (small aneurysms)

40
Q

What antibody is present in granulamatosis with polyangitis

41
Q

What does c-ANCA indicate

A

c-ANCA directed against proteinase 3

42
Q

What organ systems are affected by granulamatosis with polyangitis (3)

A

ENT
Kidneys
Lungs

43
Q

What antibody is present in eosinophilic granulamatosis with polyangitis

44
Q

What is p-ANCA directed against

A

Myeloperoxidase

45
Q

What are the manifestations of eosinophilic granulomatosis with polyangitis (3)

A

Asthma
Eosinophilia
Vasculitis

46
Q

p-ANCA

A

Eosinophilic granulomatosis with polyangitis

47
Q

c-ANCA

A

Granulomatosis with polyangitis

Kat's Pathology (105 decks)

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