Idiopathic pulmonary fibrosis is a chronic and progressive lung disease with a poor prognosis. Idiopathic basically means that the cause is not known.
     Pulmonary fibrosis is characterized by lung inflammation and abnormal tissue repair, resulting in the replacement of normal functional tissue with an abnormal accumulation of fibroblasts and deposition of collagen in the lung. This process involves cellular interactions via a complex cytokine-signaling mechanism and heightened collagen gene expression, ultimately resulting in its abnormal collagen deposition in the lung. Our current understanding of the pathogenesis of pulmonary fibrosis suggests that in addition to inflammatory cells, the fibroblast and signaling events that mediate fibroblast proliferation and myofibroblasts, play important roles in the diverse processes that constitute fibrosis. Increasing knowledge of cytokine biology, cytokine-signaling and cell matrix interactions have shed some light on the genesis of pulmonary fibrosis; however, the importance of inflammation in pulmonary fibrosis remains controversial. This remains true because the inflammatory component is variable at the time of diagnosis, and the most potent anti-inflammatory drugs that have been widely used in the treatment of pulmonary fibrosis do not seem to interfere with the fibrotic disease progression. Consider signing up to a free natural medicine newsletter sent by email once or twice a month. I will discuss new pulmonary fibrosis studies as they become available. See Newsletter.

Pulmonary fibrosis treatment
At present there is no good medical treatment for pulmonary fibrosis. Lung transplantation is a last resort. acetylcysteine is a promising nutrient that may slow the progression of the disease.

Natural Treatment for Pulmonary Fibrosis
Acetylcysteine - A double-blind, randomized, placebo-controlled multicenter study was conducted that assessed the effectiveness over one year of a high oral dose of acetylcysteine (600 mg three times daily) added to standard therapy with prednisone plus azathioprine. The primary end points were changes between baseline and month 12 in vital capacity and in single-breath carbon monoxide diffusing capacity (DLCO). Conclusions: Therapy with acetylcysteine at a dose of 600 mg three times daily, added to prednisone and azathioprine, preserves vital capacity and DLCO in patients with idiopathic pulmonary fibrosis better than does standard therapy alone.

Pulmonary fibrosis symptom
Symptoms of pulmonary fibrosis include shortness of breath, coughing and diminished exercise tolerance.

End stage pulmonary fibrosis
End stage pulmonary fibrosis is characterized by failure of alveolar re-epithelization, persistence of fibroblasts, deposition of extracellular matrix, and distortion of lung architecture which ultimately results in respiratory failure. Pulmonary fibrosis is a highly lethal disorder, which continues to pose major clinical challenges because an effective therapeutic regimen is yet to be determined.

Cause of pulmonary fibrosis
Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes (sarcoidosis, Wegener’s granulomatosis ), infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to radiation, chronic conditions (lupus, rheumatoid arthritis), and certain prescription medications. Silicosis is a cause of pulmonary fibrosis.

Pulmonary fibrosis questions
Q. Does alpha lipoic acid help with pulmonary fibrosis?
   A. I have not seen such research regarding the role of alpha lipoic acid and pulmonary fibrosis.

For more information, visit the pulmonary fibrosis foundation