Vasculitis Flashcards Preview

Year 1: Respiratory > Vasculitis > Flashcards

Flashcards in Vasculitis Deck (10):
1

define vasculitis

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

2

Symptoms of pulmonary vasculitis?

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

3

Vasculitis may be secondary to systemic disease. Name some.

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

4

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)

5

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.

6

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

7

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.

8

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.

9

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.

10

Lymphomatoid granulomatosis

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