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Interstitial Lung Disease (restrictive) Cheat Sheet by

ACNP CheatSheet

Inters­titial Lung Disease (restr­ictive)

Inflam­matory process involving the alveolar wall (resulting in widespread fibroe­lastic • prolif­eration and collagen deposition).
• Can lead to irreve­rsible fibrosis, distortion of the lung archit­ecture, and impaired gas exchange.
• Prognosis is very variable and depends on diagnosis.
• Ask about medication history (esp. chemot­her­apeutic agents, gold, amiodarone, penici­lla­mine, and nitrofurantoin)
• Previous jobs (exposure to asbestos, silicone, beryllium, and coal).
Rales at the bases are common, digital clubbing is common (esp. in idiopathic pulmonary fibrosis), signs of pulmonary HTN and cyanosis in advanced disease.
• Dyspnea (at first with exertion then at rest), cough (nonpr­odu­ctive), fatigue, symptoms secondary to another condition.
• CXR, CT, PFTs, oxygen desatu­ration during exercise.
• Bronch­oal­veolar lavage for culture and cytology yields variable results.
• Tissue biopsy is often required and can be done via fiberoptic bronch­oscopy with transb­ron­chial biopsy (limited utility), open lung biopsy, or video-­ass­isted thorac­oscopic lung biopsy (VATS).
• UA can show signs of glomerular injury in Goodpa­sture's or Wegener's.

Causes ILD

• Coal worker's pneumo­con­iasis, silicosis, asbest­osis, beryll­iosis.
Granuloma Associated
Sarcoi­dosis (along with other organ involv­ement), Wegener's granul­oma­tosis, Churg-­Strauss syndrome.
Alveolar Filling Disease
• Goodpa­sture's syndrome, Idiopathic pulmonary hemosi­der­osis, alveolar protei­nosis.
• Hypers­ens­itivity pneumo­nitis, eosino­philic pneumo­nitis.
Drug Induced
Amiodarone, nitrof­ura­ntoin, bleomycin, phenytoin, illicit drugs.
Miscel­laneous Causes
• Idipathic pulmonary fibrosis, bronch­iolitis obliertans organizing penumonia (BOOP), ILD associated with connective tissue disorders (RA, sclero­derma, SLE, mixed connective tissue disease), ARDS, infection (fungal, TB, viral pneumo­nia), radiation pneumo­nitis.


• Typical diffuse changes are noted (reticular, reticu­lon­odular, ground glass, honeyc­ombing).
Honeyc­ombing refers to a scarred shrunken lung and is an end-stage finding with poor prognosis. Air spaces are dilated and there are fibrous scars in the inters­titial. Can arise from many causes of ILD.
Pulmonary Function Tests
• A restri­ctive pattern. FEV1/FVC is increased >80%. Both FEV1 and FVC are low. Decreased DLCO. Decreased TLC and RV.
CT Scan
• Shows the extent of fibrosis better than other imaging. Fibrosis, honeyc­ombing, or traction bronch­iec­tasis.


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