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Lung tissue consists of millions of air sacs surrounded by blood vessels and connective tissue. It is a very thin and delicate tissue. "Interstitium" is the portion of the lungs surrounding the air sacs. If there are evidences of scarring in the interstitium than this disease is called interstitial fibrosis. If asbestos fibers get into the interstitium after inhalation they can cause fibrosis.
Asbestosis, is an interstitial fibrosis from inhaled asbestos. Bus asbestos is not the only cause of interstitial fibrosis. It can occur from many diseases and products, so we can say that it is a non-specific disorder. It can be caused by: infections, drug reactions, chronic heart failure and arthritis-related diseases.
Because of the poorly understood of these diseases still there are controversies about terminology and classification. The word "interstitial" means that the disease development occurs in the alveolar wall between the alveolar epithelium and capillary endothelium. Yet there are a lot of evidences of intra-alveolar pathology development. Sometimes it may be more marked than in the interstitium. So in general, pulmonary fibrosis can develop due to quite a vast group of diseases of lung parenchyma.
Another kind of fibrosis is idiopathic interstitial fibrosis which is characterized by diffuse interstitial fibrosis of unknown etiology. It often occurs between the ages of 50-70.
Clinical Picture of interstitial fibrosis
- Persistent coarse basal crackles and clubbing (40-70%) tachypnea can be disclosed during physical exam.
- The most common symptoms interstitial fibrosis are shortness of breath and dry cough and sometimes arthralgias, fever and Raynaud's phenomenon.
- CXR: Normal to reticulonodular to honeycombing. HRCT may show ground glass appearance or honeycomb cysts. In Desquamative interstitial pneumonitis alveolar signs can be found out.
- PFT displays diffusion and restrictive defects.
- In some cases ANA, serum cryoglobulins and RF are present.
- BAL: Predominance of neutrophils.
Pathology of interstitial fibrosis
Various subgroups of the same diseases process can be defined: Lymphocytic Interstitial Pneumonitis( LIP), Desquamative Interstitial pneumonitis(DIP), Usual Interstitial Pneumonitis(UIP).
Diagnosis of interstitial fibrosis
During diagnosis it is necessary to rule out other possible causes of fibrosis, for example drugs or Silicosis.
Interstitial fibrosis can be identified by transbronchial lung biopsy. Open lung biopsy is usually necessary to confirm.
Treatment of interstitial fibrosis
Therapy can be useful when there is significant inflammatory process, i.e. in acute stages. When the fibrosis is chronic or if there is honeycombing or if the chest X-ray has been stable for years, there is no need of therapy. As therapy immunosupressants can be prescribed. In some cases the only considerable solution can be Plasmapheresis and Lung Transplant. The mediate survival with single lung is 50%.
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