Interstitial lung disease

Question 1

Pulmonary fibrosis

Answer 1

end stage of many diseases
DDX - Cryptogenic fibrosing alveolitis, Extrinsic allergic alveolitis, sarcoidosis, wegener’s granulomatosis, Good pasture’s syndrome
Q - occupation, PmHx, travel, environment including pets and hobbies, drugs and smokes
Ex - Resp and CVS, of systemic disease e.g. pyrexia, skin involvement, joints.
Ix - FBC, Eosinophil count, CRP/ESR, ANCA, ANAB and RF, U and E, LFT, Ca (sarcoidosis)
CXR, CT
RFT
Transbronchial biopsy if Dx unclear

Question 2

Cryptogenic fibrosing alveolitis

Answer 2

Cause - Unknown
Epi - middle age M greater then F
Presentation - gradual onset of breathlessness and dry cough
Ex - Clubbing, fine late inspiratory crackles at bases, increase RR, cyanosis
IX - RFT - restrictive pattern. Blood gases - low O2 and CO2. CXR - bilateral basal shadowing with honeycombing. RF positive
Dx - on lung biopsy
Tx - corticosteroids and immunosuppressives

Question 3

Extrinsic allergic alveolitis

Answer 3

Bird fancier’s lung, pigeon fancier’s lung
Formation of granuloma then fibrosis
Presentation - fever myalgia, cough and breathlessness with crackles on ex. finger clubbing uncommon.
CXR - every upper lobe fibrosis with cavitation
RFT - restrictive pattern
Tx - remove trigger and may need steroids.

Question 4

Sarcoidosis

Answer 4

cause - unknown
affects the hilar LN and lungs most commonly
Hypercalciuria may be present
Restrictive pattern
Tx - Steroids or other immunosuppressants e.g. methotrexate

Question 5

Wegener’s granulomatosis

Answer 5

Granulomatous vasculitis of lungs, kidneys and upper respiratory tract. Medium to small arteries and veins.
Presentation - haemoptysis, breathlessness, pleuritic pain, general malaise and low grade fever.
Ix - CXR - bilateral pulmonary nodules that may cavities. No hilar lymphadenopathy. ANCA
Tx - corticosteroids and cyclophosphamide

Question 6

Good pasture’s syndrome

Answer 6

usually young
Presentation - pulmonary haemorrhage and glomerulonephritis
DX by presence of antiglomerular basement membrane antibody and deposit of immune complex in basement membrane on renal biopsy
Tx - dialysis, plasma exchange to remove antibody and cytotoxic therapy.