Connective Tissue Flashcards
autoimmune multisystem disorder which causes
lymphocytic infiltration if the skin,
immune complex deposition
and thickened wire loop of capillary walls in the glomeruli
SLE
autoimmune multisystem disorder associated with anti-centromere abs. histology sshows increased collagen in skin and organs as well as onion skin thickening of the arterioles
limited scleroderma
autoimmune infla,mmatory dosirder of the muscles and skin, cutaneous features include heliotrope rash and gottrons papules.
Dermatomyositis
anti-Jo1 abs and myofibre damage
polymyositis
anti-scl70, anti fibrillarin and anti RNA pol. histology shows inflammation within or around muscle fibres
diffuse scleroderma
speckled pattern on ANA test
mixed connective tissue disease
pulseless disase with bruits and claudication. occurs in large vessels
Takayasu’s arteritis
occurs in the elderly with scalp tenderness, temporal headache, jaw claudication and raised ESR. histology shows granulomatous transmural inflamm, giant cells.
Giant cell arteritis (temporal arteritis)
vasculitis affection the medium sized vessels, with renal failure as main feature. 30% have underlying hepB. microaneurysm beads on angiography.
PAN polyarteritis nodosa
SPARES the lungs
red palms and soles with later desquamation, conjunctiviits, fever >5 days. coronary arteries may be involved in aneurysm formation.
Kawasaki
medium vessel disease resulting from inflammation of the arteries of the extremities - leads to pain and ulceration on extremities. common in heavy smokers <35.
Buergers disease (thromboangitis obliterans)
small vessel disease that presents as a triad of:
- URT sx - sinusitis, epistaxis, saddle nose
- LRT sx - cavitation, pulm haemorrhage
- kidney - crescenteric GN
Wegeners (GPA_
small vessel vasculitis with asthma and eosinophilia. can have later systemic involvement.
eGPA
Churg Strauss
small vessel vasculitis and pulmonary renal syndrome of 1. pulm haemorrhage 2. GN. p-ANCA positive.,
microscopic polyangiitis
IgA mediated small vessel vasculitis in choldren, preceded by an URTI. presents with a palpaple purpuric rash, glomerulonephritis and colicky abdo pain.
HSP
c-ANCA for proteinaise 3
Wegeners
we gunna cus we can-ca
p-anca for myeloperoxidaseq
churg strauss, MPA
multisystem disorder caused by abnormal folding of proteins that are deposited as amyloid fibrils in tissues, disrupting their normal function.
amyloidosis
deposition of AL protein, most commonly associated with plasma cell dyscrasias such as multiple myeloma.
most have monoclonal Ig and free light chains (bence jones proteins) in the urine.
primary amyloidosis - AL
protei found in secondary amyloidosis
AA protein
produced secondary to chronic infections and inflammation as the A protein is an acute phase protein.
common conditions causing secondary AA amyloidosis
- autoimmune - RA, ankspond, IBD
- chronic infections - TB, osteomyelitis, IVDU (skin infections)
- non-immune causes of inflamm - renal cell carcinoma, Hodgkins
deposition of b2-microglobulin
haemodialysis associated amyloidosis
most common cause of familial amyloidosis
familial mediterranean fever
AR defect causing incresed production of IL-1, attacks of fever and inflamma of serosal surfaces such as pluera, peritoneum, and synovium.
what protein is deposited most commonly in FMF and where
AA, renal
most common presentation of amyloidosis
nephrotic syndrome
also caues conduction defects, heart failure, macroglossia, HSM, carpal tunnel
histology of amyloidosis
apple green birefringence with congo red stain under polarised light
caused by beta pleated sheet configuration
non-caseating granulomas and histiocytes, schaumann and asteroid bodies (protein and ca inclusions)
sarcoidosis
skin manifestations of sarcoid
lupus pernio on nose, erthema nodosum, skin nodules
eye manifestations of sarcoid
anterior uveitis, progressive visual loss, keratoconjunctivitis
biochemical manifestations
high Ca, high ACE, high Ig, high ESR
common extrapulmonary manifestations
lymphadenopathy, arthrotis, HSM, leucopenia, renal calculi, meningitis, bilateral parotid enlargement.
