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Interstitial Lung Disease (restrictive) Cheat Sheet by

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Inters­titial Lung Disease (restr­ictive)

Pathop­hys­iology
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.
History
• Ask about medication history (esp. chemot­her­apeutic agents, gold, amio­dar­one, penici­lla­mine, and nitrofurantoin)
• Previous jobs (exposure to asbestos, silicone, beryllium, and coal).
Signs
Rales at the bases are common, digital clubbing is common (esp. in idiopathic pulmonary fibrosis), signs of pulm­onary HTN and cyanosis in advanced disease.
Symptoms
• Dyspnea (at first with exertion then at rest), cough (nonpr­odu­cti­ve), fatigue, symptoms secondary to another condition.
Diagnosis
• 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

Enviro­nmental
• 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
• 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.
.
 

Tests

CXR
• 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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