Immune Related Multisystem Disorders

Question 1

Which autoimmune condition is organ specific with organ specific Ag?

Answer 1

Pernicious anaemia

Question 2

Which autoimmune condition is organ specific without organ specific Ag?

Answer 2

Primary biliary cirrhosis

Question 3

What are multisystem autoimmun conditions?

Answer 3

Rheumatoid Arthritis

Sjorgren’s syndrome

SLE

Question 4

Which autoantibodies are related to SLE?

Answer 4

Anti-dsDNA

Anti-smith (against ribonucleoproteins)

Question 5

What autoantibodies are used for drug-induced SLE?

Answer 5

Anti-histone (drug related e.g. hydralazine)

Question 6

What is this?

Answer 6

SLE

The pink blobs are denatured nuclei. Here are two, with one seen being phagocytozed by a neutrophil.

Question 7

What is this?

Answer 7

SLE

Lymphocyte infiltration in the upper dermis; vacuolization of the basal layer of epidermis; RBCs extravasated into the upper dermis (which are the reasons for the rash).

Question 8

What is this?

Answer 8

SLE - Skin IF

Immunofluorescence staining (antibody to IgG showing evidence for immune complexes at the dermal-epidermal junction).

Question 9

What is this?

Answer 9

Normal Glomerulus

Question 10

What is this?

Answer 10

SLE - Kidney

Thickened pink glomerular capillary loops (‘wire loops’) due to immune complex deposition.

Question 11

What is this?

Answer 11

SLE - Immunofluorescence

Deposits of IgG and complement in the basement membrane.

Question 12

What is this?

Answer 12

SLE - Electron microscopy

Electron dense deposit within the glomerular basement membrane.

Question 13

What is this?

Answer 13

SLE - Libman-sacks

Strands of fibrin, neutrophils, lymphocytes, histiocytes

Question 14

What is Scleroderma (systemic sclerosis)?

Answer 14

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

• Calcinosis
• Raynauds
• Esophageal dysmotility
• Sclerodactyly
• Telagiectasia

Nucleolar pattern immunofluorescence.

Question 15

What are the diffuse and limited forms of scleroderma?

Answer 15

Diffuse form: Antibodies to DNA topoisomerase (Scl70).

Limited form: Anticentromere antibody.

Question 16

What is this?

Answer 16

Scleroderma -Nucleolar IF

Question 17

What is this?

Answer 17

Scleroderma

Question 18

What is this?

Answer 18

Calcinosis

Question 19

What is this?

Answer 19

Raynaud’s Phenomenon

Question 20

What is this?

Answer 20

Nail-fold capillary dilatation

Question 21

What is this?

Answer 21

Telangiecstasia

Question 22

What is this?

Answer 22

Scleroderma - Skin

Increased dermal collagen resulting in reduced skin elasticity.

Question 23

What is this?

Answer 23

Scleroderma - Stomach

Trichrome stain (2 acid dyes and a polyacid). Muscle: red, collage blue.

Fibrosis (collagen deposition in the submucosa).

Leading to dysmotility.

Question 24

What is this?

Answer 24

Normal artery

Question 25

What is this?

Answer 25

Scleroderma - onion skin

‘Onion skin’ intimal thickening of small arteries

Question 26

What are mixed connective tissue disease?

Answer 26

SLE

Scleroderma

Polymyositis

Dermatomyositis

Question 27

What is this?

Answer 27

Speckled pattern of ANA test

Question 28

What is this?

Answer 28

Dermatomyositis

Gottron’s papules

Question 29

What are clinical features of sarcoidosis?

Answer 29

Skin: Lupus pernio, erythema nodosum

CNS: Meningitis, cranial nerve lesions

Eyes: Uveitis, keratoconjunctivitis

Parotids: Bilateral enlargement

Lungs: BHL, fibrosis, lymphocytosis (CD4+ in BAL)

Liver: Hepatitis, cholestasis & cirrhosis

Question 30

What is this?

Answer 30

Sarcoidosis

Question 31

What is this?

Answer 31

Lupus pernio

Question 32

What is this?

Answer 32

Erythema nodosum

Question 33

What is this?

Answer 33

Sarcoidosis

Non-necrotizing granulomas: Histiocytes (epithelioid cells), multinucleated giant cells of Langhans (peripheral nuclei) and lymphocytes.

Question 34

What is the pathophysiology of sarcoidosis?

Answer 34

Hypergammaglobulinaemia

Raised ACE

Hypercalcaemia: Vit D hydroxylation by activated macrophages.

Question 35

What are medium vessel vasculitis?

Answer 35

Polyarteritis Nodosa

Kawasaki Disease

Question 36

What are immune complex small vessel vasculitis?

Answer 36

Cryoglobulinemic vasculitis

IgA Vasculitis (Henoch Scholein)

Hypocomplementermic Urticarial Vasculitis (Anti-C1q Vasculitis)

Anti-GBM Disease

Question 37

What are large vessel vasculitis?

Answer 37

Takayasu ateritis

Giant cell arteritis

Question 38

What are ANCA-Associated small vessel vasculitis?

Answer 38

Microscopic polyangiitis

Granulomatosis with Polyangiitis (Wegener’s)

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

Question 39

What is this?

Answer 39

Vasculitis

Question 40

What is this?

Answer 40

Temporal arteritis

Question 41

What is this?

Answer 41

Temporal arteritis

Chronic lymphocytic inflammation in the media, giant cells, narrowing of the lumen.

Question 42

What are clinical features of Kawasaki’s Disease?

Answer 42

• Fever
• Erythema of palms & soles, desquamation
• Conjunctivitis
• Lymphadenopathy
• Coronary arteries may be affected (MI)
• Otherwise disease is self limiting

Question 43

What is polyarteritis nodosa?

Answer 43

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)

Question 44

What is this?

Answer 44

Polyarteritis nodosa

Question 45

What is this?

Answer 45

Granulomatosis with polyangiitis

• ENT
• Lung
• Kidneys

C-ANCA (cytoplasmic ANCA) directed against proteinase 3

Question 46

What is this?

Answer 46

Churg-Strauss (Eosinophilic Granulomatosis with polyangiitis)

• Asthma
• Eosinophilia
• Vasculitis

P-ANCA (perinuclear ANCA) directed against myeloperoxidase.