Histopathology 18 - Immune disorders Flashcards

(33 cards)

1
Q

What does SOAP BRAIN MD staind for?

A

SLE features

Serositis

Oral ulcers

Arthritis

Photosensitive

Blood (pancytopoenia)

Renal (proteinuria)

ANA

Immunological (anti-dsDNA)

Neurological (psych, seizures)

Malar rash

Discoid rash

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2
Q

Which autoantibodies are present in SLE?

A

ANA - anti-dsDNa, anti-Sm, anti-histone

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3
Q

Which autoantibody is most specific for SLE

A

anti-dsDNA

anti-histone also raised in drug related - e.g. hydralazine

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4
Q

What method is used to measure anti-dsDNA?

A

Incubate patient’s serum with Crithidia Luciliae (a protozoa)

also ELISA

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5
Q

What is titre and how do you interpret it?

A

titre - dilution value (e.g. 1:10) - highest dilution at which you can still see fluorescence is titre (i.e. 1:1000 > 1:40)

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6
Q

What will skin histology show in SLE?

A

Lymphocytic infiltration of dermis

Vacuolisation (dissolution of cells) of basal epidermis

Extravasation of RBCs → rash

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

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7
Q

What will renal histology show in SLE?

A

Glomerular capillaries thick (“wire-loop”) due to immune complexes in BM

immunofluorescence can be used to visualise the immune complex deposition (electron microscopy will also show dark areas of immune complex deposition)

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8
Q

What type of endocarditis is associated with SLE?

A

Libman-Sacks, non infective

s/s: emboli, heart failure or murmurs

Vegetations = lymphocytes, neutrophils, fibrin strands etc.

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9
Q

What is the localised form of scleroderma called?

A

morphoea in skin

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10
Q

Which antibodies are involved in diffuse vs limited cutaneous systemic sclerosis (scleroderma)?

A

diffuse - Anti-topoisomerase (anti-Scl70) - nucleolar pattern

limited - anti-centromere

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11
Q

What are the differences between diffuse versus limited systemic sclerosis?

A

diffuse - involves trunk

limited - no trunk involvement

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12
Q

Recall the features of CREST syndrome

A

associated with LIMITED systemic sclerosis

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13
Q

What are the skin histological features of scleroderma?

A

↑ collagen → ↓ skin elasticity

N.B. difficulty swallowing and stomach dysmotility due to excess collagen within lining → reduced elasticity

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14
Q

What are the vascular histological features of scleroderma ?

A

renal crisis (v high BP)

intimal proliferation → onion skin

Lumen effectively obliterated and some small thrombi form

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15
Q

What does a speckled pattern of ANA on immunofluorescence suggest?

A

mixed connective tissue disease

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16
Q

What are the clinical features of dermatomyositis?

A

proximal muscle pain and weakness

High CK

Gottron’s papules (erythematous rash over the knuckles)

17
Q

Recall the clinical features of sarcoidosis

A

Joints

Skin (lupus pernio, erythema nodosum)

Lungs (fibrosis, lymphocytosis (increased CD4+ cells in BAL)

Lymphadenopathy

Heart (any layer: pericardium, myocardium, endocardium)

Eyes (uveitis, keratoconjunctivitis)

CNS (meningitis, cranial nerve lesions)

Liver (hepatitis, cirrhosis, cholestasis)

Parotids (bilateral enlargement)

18
Q

What is the histological features pf sarcoidosis?

A

non-caseating granulomata (ball of activated macrophages);

composed of:

Histiocytes (epithelioid cells)

Multinucleated giant cells of Langerhans (peripheral nuclei) – fused macrophages → horseshoe appearance

Lymphocytes

19
Q

What are the biochemical features of sarcoidosis ?

A

Hypergammaglobulinaemia

↑ ACE

Hypercalcaemia (vitamin D hydroxylation (1a-hydroxylase) by activated macrophages)

21
Q

What are some examples of small vessel vasculitis?

A
  • IgA vasculitis (HSP)
  • anti-GBM
  • microscopic polyangitis
  • granulomatosis with polyangitis (Wegner’s)
  • eosinophilic granulomatosis with polyangitis (Churg Strauss)
22
Q

Recall two examples of medium vessel vasculitis

A

Polyarteritis Nodosa

Kawasaki

23
Q

Recall two examples of large vessel vasculitis

A

Takayasu

Giant cell

24
Q

What rash is characteristic of vasculitis?

A

palpable purpuric rash characteristic of vasculitis

Nail fold infarcts are also a feature of vasculitis

25
26
What is polyarteritis nodosa?
inflammation of gut/renal vessels Necrotising arteritis → heals by fibrosis
27
Which type of arteritis is associated with rosary beads appearance on angiogram and HBV?
polyarteritis nodosa rosary beads = aneurysms
28
What is the management temporal arteritis?
ESR → needs high dose prednisolone
29
What is the definitive diagnosis for temporal arteritis and what will it show?
temporal artery biopsy narrowing of lumen and lymphocytic infiltration of tunica media (not intima)
30
What are the three hallmarks of granulomatosis with polyangiitis ?
Wegener's Granulomatosis Upper respiratory (ENT) – nosebleeds, sinusitis, saddle nose Lower respiratory (lungs) – haemoptysis, SOB Kidneys – haematuria
31
Which antibody is implicated in granulomatosis with polyangiitis ?
C-ANCA (directed against proteinase 3)
32
What are the three hallmarks of Churg-Strauss syndrome?
Asthma Eosinophilia Vasculitis
33
Which antibodies implicated in Churg Strauss syndrome?
PERInuclear ANCA P-ANCA (directed against myeloperoxidase)