Presentation Symptoms – SOB on exertion – Lethargy – Dry cough – EAA – variability with work; IPF – progressive worsening – Sarcoid extrapulmonary features: – Anterior uveitis, conjunctivitis, arthralgia, erythema nodosum – SMOKING, OCCUPATION, PETS Signs – Tachypnoeic – Clubbing – IPF, EAA – Cyanosis – Fine end-inspiratory creps IPF – more at bases EAA – more at apices
Investigations Bedside – PEF, including work and home measurements – Sats, RR Blood tests – FBC, U&Es, LFTs, CRP, ESR – ABG - hypoxia – ANA and RF can be + in IPF – Calcium can be high in Sarcoidosis (serum ACE can be high but not always!) Imaging – CXR – fine reticulonodular shadowing – CT Ground glass appearance Honey combing
Investigations (2) Special tests – Spirometry Restrictive defect i.e. Lung volume reduced. FVC reduced, FEV1 reduced in proportion (or slightly less). Therefore FEV1:FVC is normal or high!! TLCO (transfer factor) reduced – due to fibrosis of alveolar walls. Means a thicker barrier to gas exchange and less effective transfer – N.B. Bronchoscopy, BAL, Biopsy
Honey combing and Ground Glass
Management Conservative – Smoking cessation – Change working conditions – Change work Medical – Depends on cause!! – IPF – very little besides oxygen! Steroids of little help – EAA – Steroids for acute episodes – Sarcoidosis – Only treat if extrapulmonary manifestations! Steroids Surgical – Lung transplant
Prognosis IPF – poor EAA – depends on extent of disease and ability to avoid the cause Industrial lung disease - variable Sarcoidosis - variable
Clinical scenario A 64 year old gentleman presents to his GP with increasing SOB over the last 6 months. His exercise tolerance has reduced to the point where walking to the corner shop makes him out of breath. He also complains of a dry cough. He has a past medical history of high blood pressure which is managed with Ramipril. He has never smoked and works as an office manager He has no pets On examination he is slightly short of breath with O2 sats 93% on air and he has clubbing. Auscultation reveals bilateral basal fine end inspiratory crepitations and no wheeze.
Clinical scenario Cont What are your main differentials for this gentleman? How would you investigate this gentleman? What is your management plan? Will anything help?
Summary ILD is more complicated than it needs to be Cardinal features are: – Fine end inspiratory creps – due to fibrosis – The cause of the fibrosis will usually be hinted at in occupation or hobbies! – If no cause obvious its probably IPF Restrictive spirometry with reduced gas transfer Treatment depends on cause – usual steroids