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a path 3 > HISTO: Immune related multisystem disorders – Connective tissue disease, amyloid, sarcoid > Flashcards

HISTO: Immune related multisystem disorders – Connective tissue disease, amyloid, sarcoid Flashcards

1
Q

Give an example of an autoimmune disease which is:

  1. organ specific with organ specific Ag
  2. ogran specific without organ specific Ag
  3. multisystem disease
A
  1. organ specific with organ specific Ag - pernicious anaemia
  2. ogran specific without organ specific Ag - primary biliary cirrhosis
  3. multisystem disease - RhA, Sjogren’s syndrome, SLE
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2
Q

What are the features of SLE?

A
  • Skin - malar rash, discoid lupus with atrophic centre
  • Oral ulcers
  • Joints
  • Neurological
  • Serositis
  • Renal - glomerulonephritis
  • Haematological - pancytopenia
  • Immunological - autoantibodies
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3
Q

What antibodies are commonly found in SLE? Which one is common in drug-related SLE? Which is most specific?

A

ANA - antinuclear antibodies; titre will be given - this is the highest dilution at which the antibodies are still present

Anti-dsDNA

Anti-smith - against ribonucleoproteins; very specific so you know it’s SLE if present, but not very sensitive

Anti-histone - drug related e.g. hydralazine taken for HTN

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4
Q

What investigations are used for anti-dsDNA detection in SLE?

A

Crithidia luciliae (shown)- incubate patient’s serum with this protozoa which has a big mitochondrion with double stranded DNA (kinetoplast). If the patient has anti-dsDNA antibodies then they will bind the DNA.

ELISA

Other:

LE cells - old test which looks at neutrophils which have taken up denatured nuceli

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5
Q

What is seen on skin histology in SLE?

A
  • Lymphocytic infiltration of dermis
  • Vacuolisation (dissolution of the cells) of basal epidermis
  • Extravasation of RBCs causes the rash

Immunofluorescence (antibody to IgG) will show immune complex deposition at the epidermis-dermis junction

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6
Q

Where does immune complex deposition occur in the skin in SLE?

A

Epidermis-dermis junction as shown using immunofluorescence (antibody to IgG)

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7
Q

What does renal histology show in SLE? (normal glomerulus shown below)

A
  • Glomerular capillaries thick (aka “wire-loop” appearance) - due to deposition of immune complexes in BM

Immunofluorescence - can show immune complex deposition (electron microscopy will also show dark areas of immune complex deposition)

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8
Q

What is Libman-Sacks?

A
  • SLE related-endocarditis
  • non-infective
  • patients may present with emboli, HF and murmurs
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9
Q

What are the vegetations composed of in Libman-Sacks?

A

Vegetations = lymphocytes, neutrophils, fibrin strands etc.

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10
Q

What is scleroderma? What is the localised form called?

A

AKA systemic sclerosis = tight skin due to fibrosis and excess collagen (the localised form is called morphoea in the skin)

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11
Q

What are the types of scleroderma?

A

Diffuse = involves the trunk

Limited = does NOT involve the trunk (remembered as CREST)

  • Calcinosis (i.e. calcium deposit on tip of thumb)
  • Raynaud’s phenomenon (white –> blue –> red)
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia (Other: nail fold dilatation)
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12
Q

What antibodies are present in limited vs diffuse scleroderma?

A

Anti-centromere antibodies = diffuse

Anti-topoisomerase (anti-Scl70) = limited

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13
Q

What is seen on immunofluorescence when ANA test is done in diffuse scleroderma?

A

Nucleolar pattern of immunofluorescence in DIFFUSE

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14
Q

What feature of scleroderma is shown?

A

Microstomia

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15
Q

What does skin histology in scleroderma show?

A

There is increased depth and amount of collagen –> reduced skin elasticity.

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16
Q

What GI problems may occur as a result of scleroderma?

A

Difficulty swallowing and stomach dysmotility due to excess collagen within the lining –> reduced elasticity

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17
Q

What is shown on vascular histology in scleroderma?

A
  • There is intimal proliferation giving an onion skin appearance
  • Lumen effectively obliterated and some small thrombi form
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18
Q

What is seen on immunofluorescence when an ANA test is done in mixed connective tissue disease?

A

Shows a speckled pattern

This is suggestive of mixed connective tissue disease

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19
Q

What is mixed connective tissue disease?

A

Characterised by overlap of several connective tissue diseases:

  • SLE
  • Scleroderma
  • Polymyositis
  • Dermatomyositis
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20
Q

Name 3 features of dermatomyositis.

A
  • Proximal muscle pain and weakness
  • High CK
  • Gottron’s papules (erythematous rash over the knuckles)
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21
Q

What is shown and what is this a feature of?

A
  • Gottron’s papules =erythematous rash over the knuckles
  • Seen in dermatomyositis
22
Q

What organs are affected in sarcoidosis?

A

Joints

Skin e.g. lupus pernio- lesions affecting the nose, erythema nodosum

Lungs e.g. BHL, fibrosis, lymphocytosis (increased CD4+ cells in BAL)

Lymphadenopathy

Heart - any layer: pericardium, myocardium, endocardium

Eyes e.g. uveitis, keratoconjunctivitis

Neuro e.g. meningitis, cranial nerve lesions

Liver e.g. hepatitis, cirrhosis, cholestasis

Parotids - bilateral enlargement

23
Q

Other than sarcoidosis, what are 3 causes of bilateral parotid enlargement?

A
  • mumps,
  • alcohol,
  • Sjogren’s syndrome
24
Q

What is a pathological hallmark of sarcoidosis?

A

non-caseating granulomata (macrophage clustures)

25
Q

What are non-caseating granulomas composed of in sarcoidosis? What does non-caseating mean?

A
  1. Histiocytes (epithelioid cells)
  2. Multinucleated giant cells of Langerhans (peripheral nuclei) – fused macrophages form a horseshoe appearance
  3. Lymphocytes

Non-caseating = there is NO necrosis within the granuloma NB: Caseating are seen in TB

26
Q

What is this type of sarcoidosis called?

A

Lupus pernio

27
Q

What is this feature of sarcoidosis?

A

Erythema nodosum

28
Q

What do laboratory investigations show in SLE?

A
  • Hypergammaglobulinaemia
  • Raised ACE
  • Hypercalcaemia (vitamin D hydroxylation (1a-hydroxylase) by activated macrophages)
29
Q

How are vasculitides classified?

A

Based on Chapel Hill Consensus Criteria based on size of vessel

30
Q

Name the large vessel vasculitides.

A
  1. Takayasu arteritis
  2. Giant cell arteritis
31
Q

Name the medium vessel vasculitides.

A
  1. Polyarteritis nodosa
  2. Kawasaki disease
32
Q

Name the ANCA-associated small vessel vasculitides.

A
  1. Microscopic polyangitis
  2. Granulomatosis with polyangitis (Wegener’s)
  3. Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
33
Q

Name the immune complex small vessel vasculitides.

A
  1. Cyroglobulinemic vasculitis
  2. IgA vasculitis (Henoch-Schonlein)
  3. Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Anti-GBM is another type of small vessel vasculitis.

34
Q

What is this characteristic feature of vasculitis?

A

Palpable purpuric rash = characteristic of any vasculitis

35
Q

What is this feature seen in vasculitis?

A

Nail fold infarcts

36
Q

What is this feature of vasculitis?

A

Temporal arteritis - needs biopsy and measurement of ESR

37
Q

What is meant by a primary vs secondary vasculitis?

A

Primary = conditions listed below

Secondary= to another condition e.g. infective endocarditis, SLE

38
Q

What is polyarteritis nodosa?

A
  • Medium vessel vasculitis - usually causes inflammation of gut/renal vessels
  • Polyarteritis - affects several vessels
  • A necrotising polyarteritis - heals by fibrosis

Association with HBV

39
Q

What are the histological features of polyarteritis nodosa? What is seen on angiography?

A
  • Polymorphs, lymphocytes and eosinophils will infiltrate
  • Arteritis is focal and sharply demarcated

Rosary beads appearance on angiogram i.e. aneurysms

40
Q

How is temporal arteritis diagnosed?

A

Definitive - temporal artery biopsy

ESR - needs high dose prednisolone to treat

41
Q

What is seen on histology in temporal arteritis? Which layers are affected?

A
  • Narrowing of the lumen
  • Lymphocytic infiltration of the tunica media (not intima)
42
Q

What type of vasculitis is Kawasaki disease?

A

Medium vessel vasculitis

Affects children

43
Q

What are the clinical features of Kawasaki disease?

A

Clinical Features:

  • Conjunctivitis
  • Rash
  • Adenopathy
  • Strawberry tongue
  • Hands - erythema of palms and soles, desquamation
  • Burn - Fever

+ coronary artery aneurysms

Self limiting.

44
Q

What are the hallmarks of granulomatosis with polyangiitis (GPA)?

A

aka Wegener’s granulomatosis

  1. Upper respiratory (ENT) – nosebleeds, sinusitis, saddle nose
  2. Lower respiratory (lungs) – haemoptysis, SOB
  3. Kidneys – haematuria
45
Q

What antibody is diagnostic in GPA? What is it directed against?

A

C-ANCA = cytoplasmic ANCA, directed against proteinase 3

46
Q

What are the hallmarks of eosinophilic granulomatosis with polyangitis (EGPA)?

A
  1. Asthma
  2. Eosinophilia
  3. Vasculitis
47
Q

What antibody is diagnostic in EGPA? What is it targeted against?

A

P-ANCA = perinuclear ANCA, directed against myeloperoxidase

48
Q

This histology is consistent with:

  1. SLE
  2. Scleroderma
  3. Sarcoidosis
A

SLE

49
Q

This histology is consistent with:

  1. SLE
  2. Scleroderma
  3. Sarcoidosis
A

Scleroderma

50
Q

This histology is consistent with:

  1. SLE
  2. Scleroderma
  3. Sarcoidosis
A

Sarcoidosis

a path 3 (89 decks)

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