Histopath - connective tissue diseases Flashcards
Pernicious anemia - pathophys?
High MCV
Anti-parietal cell antibodies, thus low IF –> poor B12 absorption
11 features of SLE
SOAP-BRAIN MD Serositis Oral ulcers Arthritis Photosensitivity
Blood (pancytopenia, AIHA, ITP) Renal involvement ANA +ve Immune(anti-dsDNA, anti-smith, anti-histone) Neuro (psychosis)
Malar rash
Discoid rash
What is the titre in the ANA test?
The highest dilution of pt serum at which the test is still positive
3 autoantibodies seen in SLE?
Which one is most specific for SLE?
Anti-dsDNA
Anti-smith
Anti-histone
Which drug can cause SLE? how?
Hydralazine –>anti-histone antibody formation
2 ways to measure anti-dsDNA antibodies
- Crithidia luciliae (big mitochondrion with dsDNA)
2. ELISA
One cell type seen in SLE? what is it?
LE cells = neutrophils which have phagocytosed denatured nuclei
Skin histology in malar rash of SLE?
lymphocyte infiltration, RBCs extravasation, vacuolisation
Renal histology in SLE? What gives this appearance?
Thickened glomerular capillaires = wire loops
- Immune complex deposition in BM
Heart involvement in SLE?
Libman-sacks: non infective endocarditis
Scleroderma aka..?
What causes the tight skin? What is the localised form called?
Systemic sclerosis
Excess collagen + fibrosis.
Morphoea
2 forms of scleroderma? what is the difference?
Diffuse and limited
Diffuse= involves the skin of the trunk
Antibodies responsible for diffuse vs limited scleroderma
Limited = anticentromere antibody Diffuse = antibodies ot DNA topoisomerase
Features of limited form of scleroderma
CREST Calcinoshs (painful Ca deposits in fingertips) Raynauds Oesophageal dysmotility Sclerodactyly Telangiectasia
Why Oesophageal dysmotility in scleroderma?
Fibrosis in submucosa
What is seen on screening in suspected scleroderma
ANA test - nucleolar immunofluorescence
Nail changes in scleroderma
Nail-fold capillary dilatation
Histology in scleroderma?
Onion skin appearance of small arteries = intimal thickening
What is mixed connective tissue disease?
SLE
Scleroderma
Polymyositis
Dermatomyositis
What is seen in screening for mixed connective tissue disease?
Speckled pattern
Abnormal Ix result in polymyositis + dermatomyositis
Raised CK
Cutaneous feature of polymyositis
Gottron’s papules
Most commonly involved organ in sarcoidosis? manifestations?
Skin manifestations?
Lung involvement (fibrosis + BHL + lymphocytosis)
Lupus pernio, erythema nodosum
Sarcoidosis - histology?
Non-Caseating (non necrotic) granulomas
+ schaumann and asteroid bodies
Eye and CNS and gland involvement in sarcoidosis?
Uveitis, keratoconjunctivitis
Meningitis, CN lesions
Bilateral parotid gland enlargement
3 abnormal investigation results seen in sarcoidosis
hypergammaglobulinaemia
Raised ACE
Hypercalcemia (Vit D hydroxylation from macrophages)
Which vasculitis is assoc with a certain virus? how does it sometimes present?
Polyarteritis nodosa
Hep B
Gut ischemia
Weird Ix with temporal arteritis
Raised ESR
Characteristic rash in vasculitis?
Purpuric + PALPABLE
Kawasaki’s disease
Features?
Main concern
Persistent fever >5days Conjunctivitis Lymphadenopathy Desquamation of palms and soles Mucous membranes (strawberry tongue)
Coronary artery involvement –> aneurysms
Features of polyarteritis nodosa?
Appearance on angiography
Necrotising
Inflammation is focal + well demarcated = mostly renal + mesenteric arteries
Nodular appearance (micro aneurysms)
Wegener’s aka…?
Granulomatosis with polyangiitis
Granulomatosis with polyangiitis = 3 main things?
Useful Ix?
ENT
Lungs
Kidney
cANCA directed against proteinase 3
Chung Strauss - 3 main things? useful Ix?
Asthma
Eosinophilia
Vasculitis
pANCA against myeloperoxidase
