Vasculitis

Question 1

define vasculitis

Answer 1

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

Question 2

Symptoms of pulmonary vasculitis?

Answer 2

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

Question 3

Vasculitis may be secondary to systemic disease. Name some.

Answer 3

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

Question 4

Name some conditions in which there is primary vasculitides.

Answer 4

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)

Question 5

What are diagnostic markers for vasculitis?

Answer 5

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.

Question 6

Caplan’s syndrome AKA eosinophilic granulomatosis with polyangiitis

Answer 6

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

Question 7

Churg-Strauss syndrome

Answer 7

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.

Question 8

microscopic polyangiitis

Answer 8

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.

Question 9

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

Answer 9

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

Question 10

Lymphomatoid granulomatosis

Answer 10

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