Pulmonary vasculitis

Question 1

What are the (4) types of pulmonary vasculitis?

Answer 1

1. Granulomatosis with polyangiitis (Wegener’s granulomatosis)
2. Churg-Strauss syndrome (allergic granulomatosis and angiitis)
3. Goodpasture’s disease
4. Others: Systemic lupus erythematosus, Rheumatoid arthritis, Scleroderma)

Question 2

Define Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 2

Systemic vasculitis of medium and small arteries

Question 3

Pulmonary features of Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 3

Necrotizing
granulomatous lesions
of the upper and lower
respiratory tract

Question 4

Extra-pulmonary features of Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 4

Focal necrotizing
lesions of arteries
and veins; Crescentic
glomerulonephritis

Question 5

Ix for Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 5

1. CXR: nodules, cavities, and alveolar opacities
2. c-ANCA
3. Tissue confirmation

Question 6

Rx of Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Answer 6

Corticosteroids and
cyclophosphamide
or rituximab

Question 7

Define Churg-Strauss syndrome

Answer 7

Multisystem disorder

characterized by allergic rhinitis, asthma, and prominent peripheral eosinophilia

Question 8

Pulmonary features of Churg-Strauss syndrome

Answer 8

Asthma

Infiltrates

Question 9

Extra-pulmonary features of Churg-Strauss syndrome

Answer 9

Life-threatening systemic
vasculitis involving the
lungs, pericardium and heart, kidneys, skin, and PNS (mononeuritis multiplex)

Question 10

Ix of Churg-Strauss syndrome

Answer 10

Peripheral eosinophilia is
the most common finding

p-ANCA may be positive

Biopsy involved tissue

Question 11

Rx of Churg-Strauss syndrome

Answer 11

Corticosteroids

Question 12

Define Goodpasture’s disease

Answer 12

A disorder characterized by diffuse alveolar hemorrhage and glomerulonephritis caused by anti-GBM antibodies, which
cross-react with basement
membranes of the kidney and lung

Question 13

Pulmonary features of Goodpasture’s disease

Answer 13

Hemoptysis

May follow an influenza
infection

Question 14

Extra-pulmonary features of Goodpasture’s disease

Answer 14

Anemia

Question 15

Ix of Goodpasture’s disease

Answer 15

CXR: may see alveolar
infiltrates if hemorrhage is
profuse

ELISA test with anti-GBM
antibodies

Renal biopsy/indirect
immunofluorescence
shows linear staining

Question 16

Rx of Goodpasture’s disease

Answer 16

Acutely:
corticosteroids,
plasmapheresis
Immunosuppressive
therapy

Severe cases:
bilateral nephrectomy