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

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

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

Give an example of an autoimmune disease which is:

organ specific with organ specific Ag
ogran specific without organ specific Ag
multisystem disease

organ specific with organ specific Ag - pernicious anaemia
ogran specific without organ specific Ag - primary biliary cirrhosis
multisystem disease - RhA, Sjogren’s syndrome, SLE

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What are the features of SLE?

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

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

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

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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What is seen on skin histology in SLE?

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

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

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

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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What is Libman-Sacks?

SLE related-endocarditis
non-infective
patients may present with emboli, HF and murmurs

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What are the vegetations composed of in Libman-Sacks?

Vegetations = lymphocytes, neutrophils, fibrin strands etc.

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What is scleroderma? What is the localised form called?

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

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What are the types of scleroderma?

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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What antibodies are present in limited vs diffuse scleroderma?

Anti-centromere antibodies = diffuse

Anti-topoisomerase (anti-Scl70) = limited

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

Nucleolar pattern of immunofluorescence in DIFFUSE

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What feature of scleroderma is shown?

Microstomia

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What does skin histology in scleroderma show?

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

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What GI problems may occur as a result of scleroderma?

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

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What is shown on vascular histology in scleroderma?

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

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

Shows a speckled pattern

This is suggestive of mixed connective tissue disease

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What is mixed connective tissue disease?

Characterised by overlap of several connective tissue diseases:

SLE
Scleroderma
Polymyositis
Dermatomyositis

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Name 3 features of dermatomyositis.

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

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What is shown and what is this a feature of?

Study These Flashcards

Gottron’s papules =erythematous rash over the knuckles
Seen in dermatomyositis

What organs are affected in sarcoidosis?

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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

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

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mumps,
alcohol,
Sjogren’s syndrome

What is a pathological hallmark of sarcoidosis?

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non-caseating granulomata (macrophage clustures)

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

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*

What is this type of sarcoidosis called?

Lupus pernio

What is this feature of sarcoidosis?

Erythema nodosum

What do laboratory investigations show in SLE?

* **Hypergammaglobulinaemia** * **Raised ACE** * **Hypercalcaemia** (vitamin D hydroxylation (1a-hydroxylase) by activated macrophages)

How are vasculitides classified?

Based on Chapel Hill Consensus Criteria **based on size of vessel**

Name the large vessel vasculitides.

1. Takayasu arteritis 2. Giant cell arteritis

Name the medium vessel vasculitides.

1. Polyarteritis nodosa 2. Kawasaki disease

Name the ANCA-associated small vessel vasculitides.

1. Microscopic polyangitis 2. Granulomatosis with polyangitis (Wegener's) 3. Eosinophilic granulomatosis with polyangitis (Churg-Strauss)

Name the immune complex small vessel vasculitides.

1. Cyroglobulinemic vasculitis 2. IgA vasculitis (Henoch-Schonlein) 3. Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis) **Anti-GBM** is another type of small vessel vasculitis.

What is this characteristic feature of vasculitis?

Palpable purpuric rash = characteristic of any vasculitis

What is this feature seen in vasculitis?

Nail fold infarcts

What is this feature of vasculitis?

Temporal arteritis - needs biopsy and measurement of ESR

What is meant by a primary vs secondary vasculitis?

**Primary** = conditions listed below **Secondary=** to another condition e.g. infective endocarditis, SLE

What is polyarteritis nodosa?

* Medium vessel vasculitis - usually causes inflammation of _gut/renal vessels_ * Polyarteritis - affects several vessels * A n**ecrotising polyarteritis** - heals by fibrosis Association with HBV

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

* **Polymorphs, lymphocytes and eosinophils** will infiltrate * Arteritis is **focal and sharply demarcated** Rosary beads appearance on angiogram i.e. aneurysms

How is temporal arteritis diagnosed?

_Definitive_ **- temporal artery biopsy** **ESR** - needs high dose prednisolone to treat

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

* Narrowing of the lumen * Lymphocytic infiltration of the tunica media (not intima)

What type of vasculitis is Kawasaki disease?

Medium vessel vasculitis Affects children

What are the clinical features of Kawasaki disease?

Clinical Features: * **C**onjunctivitis * **R**ash * **A**denopathy * **S**trawberry tongue * **H**ands - erythema of palms and soles, desquamation * **Burn** - Fever **+ coronary artery aneurysms** Self limiting.

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

aka Wegener's granulomatosis 1. **Upper respiratory (ENT)** – nosebleeds, sinusitis, saddle nose 2. **Lower respiratory (lungs)** – haemoptysis, SOB 3. **Kidneys** – haematuria

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