Upper lung fibrosis:
2-C cool-worker pneumoconiosis
4-A ankylosing spondylitis/ABBA
6-E extrinsic allergic alveolitis
Lower lung fibrosis:
2-R rheumatoid arthritis
5-I idoipathic pul fibrosis/intrinsic fibrosing alveolitis
6-O others: drug, irradiation
look for clues:RA,ankylosing spondylitis,systemic sclerosis,drug history
look for signs of pulmonary hypertension ('a' wave in JVP,left parasternal heave,loud P2)
present the case as:interstitial lung disease as evidant by...,the most like eatiology is...
remember...Cryptogenic fibrosing alveolitis:
Disease of unknown cause, characterized by inflammatory cell infiltrate and pulmonary fibrosis.
Eventually lead to respiratory failure and cor pulmonale from pulmonary hypertension.
Associated with other autoimmune diseases.
Malaise, Wt loss,
Fine end inspiratory crackles.
- ABG; low Po2, high pco2
- High ESR.
- Autoimmune serology:ANA +ve in 30%, RF +ve on 10%
- CXR; bilateral lower zone reticulonidular shadowing, honeycomb lung.
- PFT; restrictive, reduce lung volumes and transfer factor
- Bronchalveolar lavage: (not required for diagnosis; however, findings help exclude other diseases such as infection, alveolar proteinosis, eosinophilic pneumonia, and malignancy.) ↑ lymphocytes (good response/prognosis) ↑ neutrophills/esinophills (poor response/prognosis)
- Lung Bx (ideo-assisted thoracoscopic lung biopsy)
Prognosis; 50% 5 years survival