Interstitial lung disease is a common case for the respiratory section of the MRCP PACES examination. Quite often they are cases of idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) or in the context of systemic (commonly rheumatological) disease. The above cases reflect these common scenarios. To make the diagnosis of interstitial lung disease is relatively straightforward, but presenting other physical signs of underlying aetiologies, mentioning important negatives, and appreciating and recognizing complications of therapy and the disease will impress examiners, not to mention gain considerable extra marks. 1. In approaching a respiratory patient, it is often useful in starting to present the case with a comment on functional status. They may be breathless at rest. They may be on oxygen therapy. Ask the patient to cough. The presence of a non-productive or a productive cough should give clues to underlying diagnosis. Patients with interstitial lung disease often have a non-productive cough, unless this has been complicated by infection. 2. Patients often have peripheral cyanosis. Central cyanosis may be present in advanced disease. 3. Clubbing may not always be present in cases of interstitial lung disease. If present, don’t miss it! 4. Spend a little extra time when examining hands and making general observations. There are many systemic disorders that are associated with pulmonary fibrosis. The presence of peripheral stigmata of systemic disease, usually connective tissue or rheumatological disease, will provide an important clue to the respiratory diagnosis. Look for • rheumatoid arthritis (symmetrical deforming arthropathy of the hands, rheumatoid nodules) • systemic sclerosis (tight and shiny skin, telangiectasia, sclerodactyly, calcinosis, atrophic nails, and Raynaud’s phenomenon) • SLE (petechial rash, livedo reticularis, purpura, arthropathy, butterfly skin rash) • dermatomyositis (Gottron’s papules, heliotrope rash of eyelids/periorbital areas, proximal myopathy) • ankylosing spondylitis (loss of lumber lordosis, fixed kyphosis, stooped posture) • neurofibromatoisis (neurofibromata, café au lait patches) • sarcoidosis (erythema nodosum, maculopapular skin lesions, lupus pernio, lympahdenopathy) • drugs, i.e. amiodarone (grey slate skin pigmentation—the irregular pulse of atrial fibrillation (AF) may be a clue) • radiation therapy (erythema and/or field markings on chest wall)
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