Immune Related Multisystem Disorders Flashcards
Which autoimmune condition is organ specific with organ specific Ag?
Pernicious anaemia
Which autoimmune condition is organ specific without organ specific Ag?
Primary biliary cirrhosis
What are multisystem autoimmun conditions?
Rheumatoid Arthritis
Sjorgren’s syndrome
SLE
Which autoantibodies are related to SLE?
Anti-dsDNA
Anti-smith (against ribonucleoproteins)
What autoantibodies are used for drug-induced SLE?
Anti-histone (drug related e.g. hydralazine)
What is this?
SLE
The pink blobs are denatured nuclei. Here are two, with one seen being phagocytozed by a neutrophil.
What is this?
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).
What is this?
SLE - Skin IF
Immunofluorescence staining (antibody to IgG showing evidence for immune complexes at the dermal-epidermal junction).
What is this?
Normal Glomerulus
What is this?
SLE - Kidney
Thickened pink glomerular capillary loops (‘wire loops’) due to immune complex deposition.
What is this?
SLE - Immunofluorescence
Deposits of IgG and complement in the basement membrane.
What is this?
SLE - Electron microscopy
Electron dense deposit within the glomerular basement membrane.
What is this?
SLE - Libman-sacks
Strands of fibrin, neutrophils, lymphocytes, histiocytes
What is Scleroderma (systemic sclerosis)?
Fibrosis & excess collagen (localised form is called morphoea in the skin).
- Calcinosis
- Raynauds
- Esophageal dysmotility
- Sclerodactyly
- Telagiectasia
Nucleolar pattern immunofluorescence.
What are the diffuse and limited forms of scleroderma?
Diffuse form: Antibodies to DNA topoisomerase (Scl70).
Limited form: Anticentromere antibody.
What is this?
Scleroderma -Nucleolar IF
What is this?
Scleroderma
What is this?
Calcinosis
What is this?
Raynaud’s Phenomenon
What is this?
Nail-fold capillary dilatation
What is this?
Telangiecstasia
What is this?
Scleroderma - Skin
Increased dermal collagen resulting in reduced skin elasticity.
What is this?
Scleroderma - Stomach
Trichrome stain (2 acid dyes and a polyacid). Muscle: red, collage blue.
Fibrosis (collagen deposition in the submucosa).
Leading to dysmotility.
What is this?
Normal artery
What is this?
Scleroderma - onion skin
‘Onion skin’ intimal thickening of small arteries
What are mixed connective tissue disease?
SLE
Scleroderma
Polymyositis
Dermatomyositis
What is this?
Speckled pattern of ANA test
What is this?
Dermatomyositis
Gottron’s papules
What are clinical features of sarcoidosis?
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
What is this?
Sarcoidosis
What is this?
Lupus pernio
What is this?
Erythema nodosum
What is this?
Sarcoidosis
Non-necrotizing granulomas: Histiocytes (epithelioid cells), multinucleated giant cells of Langhans (peripheral nuclei) and lymphocytes.
What is the pathophysiology of sarcoidosis?
Hypergammaglobulinaemia
Raised ACE
Hypercalcaemia: Vit D hydroxylation by activated macrophages.
What are medium vessel vasculitis?
Polyarteritis Nodosa
Kawasaki Disease
What are immune complex small vessel vasculitis?
Cryoglobulinemic vasculitis
IgA Vasculitis (Henoch Scholein)
Hypocomplementermic Urticarial Vasculitis (Anti-C1q Vasculitis)
Anti-GBM Disease
What are large vessel vasculitis?
Takayasu ateritis
Giant cell arteritis
What are ANCA-Associated small vessel vasculitis?
Microscopic polyangiitis
Granulomatosis with Polyangiitis (Wegener’s)
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
What is this?
Vasculitis
What is this?
Temporal arteritis
What is this?
Temporal arteritis
Chronic lymphocytic inflammation in the media, giant cells, narrowing of the lumen.
What are clinical features of Kawasaki’s Disease?
- Fever
- Erythema of palms & soles, desquamation
- Conjunctivitis
- Lymphadenopathy
- Coronary arteries may be affected (MI)
- Otherwise disease is self limiting
What is polyarteritis nodosa?
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)
What is this?
Polyarteritis nodosa
What is this?
Granulomatosis with polyangiitis
- ENT
- Lung
- Kidneys
C-ANCA (cytoplasmic ANCA) directed against proteinase 3
What is this?
Churg-Strauss (Eosinophilic Granulomatosis with polyangiitis)
- Asthma
- Eosinophilia
- Vasculitis
P-ANCA (perinuclear ANCA) directed against myeloperoxidase.
