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Pathology
Inflammatory disorder with accumulation of T-cells and granulomas that can affect many systems, most commonly the pulmonary system
Aetiology
Idiopathic. Most commonly affects younger adults (30-40 years), female> male
Signs
Idiopathic. Most commonly affects younger adults (30-40 years), female> male.
Symptoms
Up to 40% may be asymptomatic, dry cough, increasing dyspnoea, rash, neuropathy
Investigations
Chest X-Ray: Bilateral hilar lymphadenopathy (90%), infiltrates
Bloods: ↑serum ACE, ↑ESR, ↑Ca2+
Spirometry: reduced lung volumes and/or a restrictive defect.
Biopsy: Non-caseating granulomata
Treatment
May resolve spontaneously
If symptoms persist then treatment with 6-12 months of oral steroids (prednisolone
Prognosis
60% recover in <2 years. Monitor with Chest X-rays, lung function and clinical review
Can progress to lung fibrosis, respiratory failure, cor-pulmonale
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