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Year 1: Respiratory > Vasculitis > Flashcards

Flashcards in Vasculitis Deck (10)
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define vasculitis

inflammation and necrosis of blood vessels. Includes a number of rare conditions


Symptoms of pulmonary vasculitis?

(Pulmonary vasculitis commonly occurs with systemic vasculitis)
wheeze, hypoxaemia, pulmonary infiltrates, masses, necrotising lesions, alveolar haemorrhage


Vasculitis may be secondary to systemic disease. Name some.

Collagen vascular disease (such as rheumatoid arthritis, scleroderma, SLE)


Name some conditions in which there is primary vasculitides.

Granulomatosis with polyangiitis (formerly Wegener's granulomatosis)
Churg-Strauss syndrome, microscopic polyangiitis ,angiitis
(Goodpasture's syndrome AKA anti-glomerular basement membrane disease - has similar presentations to pulmonary vasculitis)


What are diagnostic markers for vasculitis?

Autoantibodies against components of the cytoplasm of granulocytes and neutrophils
These are called "anti-neutrophil cytoplasmic antibodies". These can be differentially characterised by neutrophil staining: cytoplasmic ANCA and perinuclear ANCA.


Caplan's syndrome AKA eosinophilic granulomatosis with polyangiitis

combination of Rheumatoid arthritis and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogeneous and well-defined on CXR


Churg-Strauss syndrome

rare autoimmune condition that causes inflammation of small and medium sized blood vessels in people with a history of atopy. It has an allergic stage, eosinophilic stage, and then a vasculitic stage.


microscopic polyangiitis

ill-defined autoimmune disease characterised by systemic, necrotising, pauci-immune (minimal hypersensitivity), small vessel vasculitis without any evidence of necrotising granulomatous inflammation, There is production of p-ANCA : which binds to neutrophils, neutrophils degranulate - releasing toxins that cause endothelial injury.


Goodpasture's syndrome (anti-glomerular basement membrane disease)

rare autoimmune disease in which Ig attacks the basement membrane in lungs and kidneys - leading to bleeding form lungs, and kidney failure.


Lymphomatoid granulomatosis

very rare lymphoproliferative disorder (lympomatoid = lymphomalike, granulomatosis = polymorphic lymphoid infiltrates and a focal necrosis within it)