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Chapter: Medicine Study Notes : Respiratory

Miscellaneous Lung Diseases

Goodpastures Syndrome (= Antibasement Membrane Antibody Disease – ABMA):

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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