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There is very limited data on treatment and follow-up of smoking-related interstitial fibrosis. One study reported that corticosteroids were not beneficial. Smoking cessation resulted in stable/non-progressive disease after several years of follow-up. Improvements in pulmonary function tests with smoking cessation have also been documented. [7]
Smoking is a known cause of some types of lung fibrosis, such as smoking-related interstitial fibrosis (SRIF). [11] Some typical connective tissue diseases [7] such as rheumatoid arthritis, ankylosing spondylitis, SLE and scleroderma; Other diseases that involve connective tissue, such as sarcoidosis and granulomatosis with polyangiitis
When associated with disease, it is known as "Respiratory bronchiolitis-associated interstitial lung disease" or "RB-ILD". [4] Also, this disease is predominantly found in the upper lobe with centrilobar ground glass nodules. Importantly, no fibrosis is involved, just bronchial wall thickening. Treatment is to stop smoking.
The median survival in idiopathic pulmonary fibrosis is 3-3.5 years. In those who receive a lung transplant, the medial survival in idiopathic pulmonary fibrosis is 5.2 years, as compared to 6.7 years in those with other types of ILD. [8] ILD is associated with a 3-fold increased risk of lung cancer. [8]
Smoking-related interstitial fibrosis (SRIF) is another type of fibrosis that occurs in emphysematous lungs and can be identified by pathologists. Unlike CPFE, this type of fibrosis is usually clinically occult (i.e., does not cause symptoms or imaging abnormalities).
Differential diagnosis includes non-specific interstitial pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis-associated interstitial lung disease, and hypersensitivity pneumonia. DIP is primarily treated by quitting smoking, but it may not be enough in all cases.
First-line treatment is cefuroxime or co-amoxiclav. [7] Third-line treatment, as well as treatment in penicillin-allergic patients, is a fluoroquinolone such as ciprofloxacin. [7] An agent active against Streptococcus pneumoniae may have to be added. [7] Corticosteroids such as prednisolone reduce inflammation in the airways. [17]
Medical treatment for restrictive lung disease is normally limited to supportive care since both the intrinsic and extrinsic causes can have irreversible effects on lung compliance. [10] The supportive therapies focus on maximizing pulmonary function and preserving activity tolerance through oxygen therapy, bronchodilators, inhaled beta ...