Miscellaneous Lung Diseases
·
Goodpastures Syndrome (=
Antibasement Membrane Antibody Disease – ABMA):
o Simultaneous rapidly progressive glomerulonephritis and haemorrhagic
pneumonitis
o Present with renal failure and haemoptysis
o Due to anti-basement membrane antibodies deposited along alveolar walls
and in glomeruli (type II immune reaction)
o Usually occurs in young men, treated with steroids and cytotoxic drugs
· Goodpasture’s Syndrome
· GN +/- pulmonary involvement (ranging from pulmonary infiltrate on x-ray to frank haemoptysis)
· Pathogenesis: antibodies against an antigen in the glomerular basement membrane and pulmonary tissue
·
Biopsy: Crescents + linear
immunoflourescence on the basement membrane
·
Can measure serum anti-GBM
antibody
· Treatment: immunosuppression (steroids, cyclophosphamide) +/- plasmapheresis
·
Alveolar Proteinosis: Peripheral
alveoli filled with pink, granular, acellular material. No treatment, variable
course, clears in a few years. Prone to infection so steroids worsen the
condition by depressing the immune system. Bronchial lavage effective in acute
episodes
·
A number of vasculitis affect the
lung. Eg Wegner‟s granulomatosis, allergic angiitis and granulomatosis. Also
collagen diseases such as SLE and Rheumatoid arthritis
·
Pulmonary Hamartoma: benign
localised proliferation of normal tissue components (hyaline cartilage with
respiratory epithelium, maybe fibrous tissue, fat, blood vessels). Usually
found as incidental findings on X-ray
·
Primary Pulmonary Hypertension:
rare, usually in young women. Usually secondary to COPD, congenital or acquired
heart disease, etc
· Langerhans cell: diagnostic cell seen in eosinophilic granuloma. = histiocytosis X and pulmonary eosinophilic granuloma. A large histiocyte with one bland folded nucleus, abundant eosinophilic cytoplasm with indistinct cell borders. Proliferative disorder of histiocytes. X-rays show multiple nodules scattered through both lungs
·
Langhans giant cell: (not the
same as Langerhans cell) multinucleated giant cell in granulomas, with nuclei
arranged around the periphery of the cell in a horseshoe pattern
· Sequestration:
o Extralobular: Congenital. Mass of lung tissue not connected to bronchial
tree and outside the visceral pleura
o Intralobar sequestration: usually acquired. Within the visceral pleura
but not connected to the bronchial tree
·
Differential of Solitary lung
nodule:
o Tumour: benign (bronchial adenoma or pulmonary hamartoma) or malignant
o Tb
o Sarcoidosis
o Other granuloma: eg fungal
o Haematoma (ie blood clot, eg in cavity following lung contusion)
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