Idiopathic interstitial fibrosis is a disease characterized by diffuse
interstitial fibrosis of unknown etiology and frequently occurs between the ages of 50-70.
- Patients present with dry cough and shortness of breath
and sometimes with fever, arthralgias and Raynaud's
- Clubbing (40-70%) tachypnea and persistent coarse basal crackles are
encountered on physical exam. Often they are using accessory muscles of inspiration for
Normal to reticulonodular to honeycombing. HRCT may show ground
glass appearance or honeycomb cysts. In Desquamative interstitial pneumonitis alveolar features can be
- PFT shows a restrictive and diffusion defects.
Patients are hypoxic and desaturate significantly with exercise.
- In 25-45%, serum cryoglobulins, RF and ANA are present.
- BAL: Predominance of neutrophils.
Desquamative Interstitial pneumonitis(DIP), Usual Interstitial Pneumonitis(UIP),
Lymphocytic Interstitial Pneumonitis( LIP) are various subgroups, probably of the same
Early IPF: Desquamation predominates where alveolar macrophages fill
alveolar spaces (desquamative interstitial pneumonitis or DIP).
Late IPF: Inflammation of alveolar walls continues and fibroblast
proliferation with collagen formation occurs (usual interstitial pneumonitis or
- Thearpy is useful in acute stages when there is significant inflammatory
- Once the fibrosis is chronic or if the chest X-ray has been stable for years or
if there is honeycombing, therapy is of no benefit.
- Corticosteroids + immunosupressants (cyclophosphamide or
azathioprine); anecdotal reports describing colchicine or methotrexate or
- Oxygen is often prescribed for these patients, probably not influencing pulmonary
- In some cases Plasmapheresis and Lung Transplant are
- Transplantation: 50% two year survival with single lung.
- In the acute progressive form "Hamman-Rich Syndrome" the prognosis is
- In the chronic form patients can live long with disability.