Interstitial Lung Disease Flashcards
sx of Idiopathic Pulmonary Fibrosis
Fatigue, Anorexia, Weight loss.
Gradual onset of non-productive cough and progressive dyspnea.
Dry crackle during inspiration.
Advanced stages-> cyanosis, clubbing, cor pulmonale
Lung Biopsy
Gold standard in helping in confirming the diagnosis, usually by VATS procedure.
diagnosis of IPF
Surgical lung biopsy is Gold Standard. Presence of Usual Interstitial Pneumonia (UIP) is characteristic-> Honey comb lung and fibrosis in late stages.
dx of Acute Interstitial Pneumonia (Hamman-Rich syndrome)
Diffuse bilateral oacification on CXR.
HRCT shows bilateral patchy ground-glass attenuation.
tx is largely inneffective. Transplant is successful but majority die while waiting.
Pulmonary Alveolar Proteinosis
Dx: CT scan typically shows “Crazy-paving” lung.
Tx: whole lung lavage.
Pulmonary Lymphangioleiomyomatosis (LAM)
Pre-menopausal women.
dx: HRCT shows THIN walled cysts surrounded by normal lung.
Chylothorax.
Goodpasture syndrome
Tx;
Plasmapheresis remove the offending antibody (plasma exchange).
Corticosteroids.
Immunosuppressive therapy with azithioprine or cyclophosphamide.
Renal transplantation is sometimes needed.
Sarcoidosis
dx: Lymphopenia, Elevated ESR, Elevated ACE (cannot use angiotensin converting enzyme), Hypergammaglobulinemia, Hypercalcemia, Hypercalciuria.
Stage1: Bilateral hilar lymphadenopathy +/- Right paratracheal adenopathy
Stage2: Adenopathy and diffuse infiltrates
Stage3: Infiltrates alone
Stage4: Honey comb appearance
Tx: 1st line; systemic corticosteroids. 2nd line; Methotrexate
Wegener’s granulomatosis
Tx: Glucocorticoids-> symptomatic relief with no survival benefit.
ANCA levels cannot be used to assess disease activity.
Induction of remission: High dose Cyclophosphamide with glucocorticoids with adjunctive Plasmapheresis for RPGN. 6-9months of induction tx.
maintenance after Remission: Methotrexate or Azathioprine for 2yrs and then tapered over 6-12months.
TMP-SMX induction/maintenance for URT disease.
