Histopathlogy 5: Connective Tissue Disorders Flashcards

1
Q

List 3 auto-antibodies seen in SLE ?

A
  • Anti-dsDNA
  • Anti-Smith (most specific but not very sensitive - only 30% with SLE have)
  • Anti-histone (drug related)

NB ANA (Anti-nuclear antibody) is just a screening test which tells you that the patient has antibodies that bind to nuclear antigens (it does NOT tell you which antigens it binds to)

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2
Q

What type of hypersensitivity reaction is seen in SLE ?

A

Type 3 hypersensitivity reaction

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3
Q

What is the HLA association with SLE ?

A

HLA DR3

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4
Q

Name the heart condition that can be caused by SLE ?

A
  • Libman-sack endocarditis
  • This is a NON-INFECTIVE form of endocarditis (inflammatory form of endocarditis)
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5
Q

List the signs and symptoms of SLE using the mnemonic SOAP BRAIN MD

A

Serositis (inflam of serous tissue - surround organs)
Oral ulcers
Arthritis
Photosensitivity

Blood disorders- AIHA, ITP
Renal involvement - Type 2 RPGN
ANA +ve
Immune- anti-dsDNA, anti-smith
Neuro symptoms

Malar rash
Discoid rash

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6
Q

Which auto antibodies are seen in Diffuse scleroderma ?

A

Anti-scl70 - this is the antibody to DNA topoisomerase

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7
Q

Which autoantibodies are seen in limited scleroderma (CREST) ?

A

Anti-centromere (ANCA)

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8
Q

Which HLA is associated with scleroderma ?

A

HLA DR5

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9
Q

List the signs and symptoms of limited scleroderma (CREST) ?

A
  • Calcinosis
  • Raynauds phenomenon
  • Esophageal dysmotility
  • Sclerodactily
  • Telangiectasia

No involvement of the trunk

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10
Q

Aetiology of scleroderma effect in skin

A
  • EXCESS deposition of collagen
  • resulting in reduced skin elasticity
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11
Q

Which autoantibodies are seen in Polymyositis and dermatomyositis ?

A

anti- Jo1

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12
Q

Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. No rash is seen.

Most likely diagnosis?

A

Polymyositis

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13
Q

Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. A heliotrope rash is seen on the eyelids. There are Gottron papules on the knuckles

Most likely diagnosis?

A

Dermatomyositis

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14
Q

Japanese lady with absence of pulse and Claudication (pain in muscles).

The Rheumatologist says that its a large vessel arthritis.

Most likely diagnosis ?

A

Takayasu’s arteritis

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15
Q

Elderly man presents with a temporal headache, jaw claudication and scalp tenderness.

Most likely diagnosis?

A

Temporal arteritis

(Large vessel vasculitis)

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16
Q

A man presents with abdo pain and signs of renal impairment. Angiogram shows “rosary beads” because of multiple aneurysms.

Most likely diagnosis?
Associated with which disease?

A
Polyartheritis nodosa (medium vessel arteritis)
Hepatitis B
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17
Q

A heavy smoker with ulceration of the toes and fingers has an angiogram which shows a “corkscrew appearance”.

Most likely diagnosis?

A

Buerger’s disease

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18
Q

List the triad of features seen in Polyangitis with granulomatosis ?

A

Saddle nose
Pulmonary haemorrhage
Glomerulonephritis (Crescentic)

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19
Q

Which ANCA is associated with Granulomatosis with polyangiitis (Wegener’s Granulomatosis) ?

A

C-ANCA

directed against proteinase 3

20
Q

List 3 characteristic features of Eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome) ?

A
  • Asthma
  • Eosinophilia
  • P-ANCA vasculitis

NB this is small vessel disease

21
Q

List 3 features of Microscopic polyangitis?

A

Pulmonary haemorrhage (haemoptysis)
Glomerulonephritis (Pauci immune RPGN)
P-ANCA small vessel vasculitis

22
Q

Which type of amyloidosis is seen in multiple myeloma ?

A

AL amyloidosis (primary)

With free light chains, monoclonal Ig and Bence jones proteins in the urine

23
Q

List some causes of AA amyloidosis? (4)

A
  • Rheumatoid arthritis
  • Chronic TB infection
  • IBD
  • Hodgkin’s lymphoma
24
Q

Name one familial amyloidosis ?

A

Familial Mediterranean fever

25
Q

Congo red staining shows ……………. under polarised light if there is Amyloidosis

A

apple green birefringence

26
Q

An Afro Caribbean patient presents with SOB and cough. Examination shows erythema nodosum, lymphadenopathy and hepatosplenomegaly. X-ray shows bilateral hilar lymphadenopathy
Histology shows: non-caseating granulomas

Most likely diagnosis?

A

Sarcoidosis

27
Q

Which electrolyte is often elevated in Sarcoidosis?

A

Ca2+

28
Q
A
29
Q

How to measure anti-dsDNA in SLE

A
  • incubate the patient’s serum with Crithidia Luciliae (protozoan)
  • This organism has a big mitochondrion with dsDNA (kinetoplast)
  • Also measure with ELISA
30
Q

Skin histology for SLE

A
  • lymphocytic infiltration (black dots)
  • vacuolisation (dissolution of the cells)
  • extravasation of the RBCs
31
Q

How do glomerulus capillaries look in SLE?

A
  • thickened (wire-loop capillaries)
  • capillaries are thick due to immune complex deposition in the basement membrane (capillaries are surrounded by basement membranes)
32
Q

Limited vs diffuse Systemic Sclerosis (scleroderma) in terms of where on the body affected

A
  • Diffuse= involves the truncal skin
  • Limited= does NOT involve the trunk (only affects distal to the elbows and knees)
33
Q

immunofluorescence finding in scleroderma

A

nucleolar pattern

34
Q

Effect of trichome staining on muscle and collagen

A
  • Red staining- muscle
  • Blue staining- collagen

NB can use this in scleroderma (see XS collagen)

35
Q

Histological finding in scleroderma

A

onion skin appearance - due to intimal layer of artery proliferating and obliterating the lumen

36
Q

Why do ANA test?

A

Screen for mixed connective tissue disease

Then need to follow-up to look for specific antibodies

37
Q

Sign on knuckle in dermatomyositis

A

Gottron’s papules (erythematous purple rash over the knuckles)

38
Q

Sites affected in sarcoidosis

A
  • Joints (arthralgia)
  • Skin (e.g. lupus pernio, erythema nodosum)
  • Lungs (bilateral hilar lymphadenopathy, fibrosis, lymphocytosis (increased CD4+ cells in the bronchoalveolar lavage)
  • Lymphadenopathy
  • Heart (any layer: pericardium, myocardium, endocardium)
  • Eyes (uveitis, keratoconjunctivitis)
  • Neuro (meningitis, cranial nerve lesions)
  • Liver (e.g. hepatitis, cirrhosis, cholestasis)
  • Parotids (bilateral enlargement)

pathological hallmark of Sarcoidosis is a non-caseating granulomatous inflammation

39
Q

Enzyme and electolyte raised in sarcoidosis

A
  • Raised serum ACE levels (due to abnormality in the lungs where ACE is made)
  • HyperCa
40
Q

2 visible features of vasculitis

A
  • palpable purpuric rash
  • Nail fold infarcts
41
Q

Definitive Dx of temporal arteritis

A
  • temporal artery biopsy
  • show narrowing of the lumen and lymphocytic infiltration of the tunica media
42
Q

Tx of Giant Cell Arteritis (Temporal Arteritis)

A

high dose prednisolone

43
Q

Example of Medium Vessel vasculitis

A

Polyarteritis Nodosa

  • Necrotising arteritis
  • most commonly affecting the renal vessels or mesenteric vessels
  • May present with gut ischaemia or renal impairment
  • Creates a rosary beads appearance on the angiogram because of the presence of multiple aneurysms
44
Q

rosary beads appearance on the angiogram due to which vasculitis?

A

Polyarteritis Nodosa

45
Q

Polyarteritis Nodosa associated with which infection?

A

Hep B

46
Q

Example of Small Vessel vasculitis

A

Granulomatosis with polyangiitis (Wegener’s Granulomatosis)

  • ENT- nosebleeds, sinusitis, saddle nose
  • Lungs- haemoptysis, SOB
  • Kidneys- haematuria
47
Q

p-ANCA seen in which small vessel vasculitis?

A

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)

3 hallmarks:

  • Asthma
  • Eosinophilia
  • Vasculitis