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The Unknowns of Idiopathic Pulmonary Fibrosis – Part 1

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Idiopathic Pulmonary Fibrosis (hereafter referred to as IPF for short), is a chronic and progressive disease of the lung wherein the lung tissues thicken, stiffen and scar without a definitive cause. Recent studies believe that scarring or an inflammatory response of the lung tissues begins when the tissues are repeatedly injured by some chemical or an unknown pathogen.

In IPF, the interstitium (especially the walls of the alveoli therein) of the lungs undergo abnormal and excessive collagen deposition with minimum associated inflammation (Source: "Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment". American Journal of Respiratory and Critical Care Medicine 161 (2): 646–664. 1 February 2000. PMID 10673212). Thus, the homeostasis of the alveolar epithelial cells is disturbed by some external or endogenous stimuli. The factors could be anything from cigarette smoking, infections, environmental pollutants, gastro-esophageal reflux disease, drugs, or any other unknown cause. The disruption of homeostasis then causes overproduction of collagen and fibronectin. Eventually as the cause of the fibrosis continues to be present, abnormal epithelial healing sets the stage for progressive fibrosis and architectural distortion of the lung sets in.

As per the American Thoracic Society/European Respiratory Society (ATS/ERS) consensus in 2002, IPF is the most common of the seven idiopathic interstitial pneumonias classified.

Symptoms of IPF are common with many infectious lung conditions and thus, differential diagnosis approach is required at arriving at a conclusion. The symptoms are usually:

• Dry cough
• Dyspnoea (shortness of breath), labored breathing
• Easy fatigue
• Occasional chest pain
• Rasping sounds heard from the lungs while breathing, through a stethoscope
• Weight loss
• Pulmonary hypertension
• Cyanosis (bluish skin color due to deficient oxygen levels in blood)
• Clubbing of fingers

As you can see the symptoms above are common with so many other lung diseases that differential diagnosis needs to be done for the following conditions to rule them out and zero in on IPF. These diseases could be:

• Asbestosis
• Bacterial, fungal or viral pneumonia
• Aspiration Pneumonia
• Silicosis
• Pneumococcal infections
• Farmer’s Lung
• Eosinophillic Pneumonia
• Drug induced pulmonary toxicity
• Restrictive lung disease
• Cardiogenic, Neurogenic Pulmonary Edema
• Lung cancer
• Recurrent intra-alveolar hemorrhage.

As with other lung conditions, diagnosis is made by running a combination of tests to correctly ascertain the disease and extent of damage, prognosis and line of treatment to be followed. Some diagnostic tests are outlined below:

1. Differential diagnosis
2. Transbronchial Lung Biopsy
3. Pulmonary function test
4. Chest X-ray to view the grid-like pattern in the lower lobes of the lung
5. CAT scan to show Bibasilar reticular abnormalities with minimal ground glass, honeycombing/ bronchiectasis
6. Measurement of blood oxygen level, hemoglobin and leukocyte values
7. Tests for connective tissue diseases like rheumatoid arthritis, lupus or scleroderma
8. ECG
9. Broncho-alveolar lavage

Our next post on Idiopathic Pulmonary Fibrosis will cover treatment options, prognosis for this chronic and progressive lung condition.

Mamta Singh is a published author of the books Migraines for the Informed Woman (Publisher: Rupa & Co.) and the upcoming Rev Up Your Life! (Publisher: Hay House India). She is also a seasoned business, creative and academic writer. She is a certified fitness instructor, personal trainer & sports nutritionist through IFA, Florida USA. Mamta is an NCFE-certified Holistic Health Therapist SAC Dip U.K. She is the lead writer and holds Expert Author status in many well-received health, fitness and nutrition sites. She runs her own popular blogs on migraines in women and holistic health. Mamta holds a double Master's Degree in Commerce and Business. She is a registered practitioner with the UN recognised Art of Living Foundation. Link: http://www.migrainingjenny.wordpress.com and http://www.footstrike.wordpress.com

Add a Comment4 Comments

Thank you for the informational article, Mamta. It's so good to actually see EmpowHER provide good and accurate information on this disease. TJ

March 20, 2010 - 10:28pm
(reply to teejaysee)

You are kind with your comments Teejaysee:) Thank you so much for the encouragement!

March 28, 2010 - 11:25am
EmpowHER Guest

Thank you for your article about this lung disease that has no FDA approved treatment or cure. The more knowledge that can be shared the better!
Jennifer BUlandr
Director of Community Outreach
Pulmonary Fibrosis Foundation

March 4, 2010 - 10:17am
(reply to Anonymous)

Hi Jennifer,

You are welcome.
Thank you for such an encouraging feedback.
Hope you have an enjoyable day at work!
Best Regards,

March 4, 2010 - 12:23pm
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We value and respect our HERWriters' experiences, but everyone is different. Many of our writers are speaking from personal experience, and what's worked for them may not work for you. Their articles are not a substitute for medical advice, although we hope you can gain knowledge from their insight.

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