What is Pulmonary Fibrosis?

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What is Pulmonary Fibrosis?

Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.

What are the symptoms?

• Shortness of breath, particularly with exertion
• Chronic dry, hacking cough
• Fatigue and weakness
• Discomfort in the chest
• Loss of appetite
• Rapid weight loss

What is the prevalence of Pulmonary Fibrosis?

There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.

What are the causes?

Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung.

While the exact cause remains unknown, associations have been made with the following:

• Inhaled environmental and occupational pollutants
• Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
• Certain medications
• Therapeutic radiation

How is it treated?

There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress. Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.

The above information is courtesy of The Pulmonary Fibrosis Foundation

Important abbreviations...

Many more acronyms and abbreviations pertaining to interstitial lung disease (pulmonary fibrosis) can be found at http://noairtogo.tripod.com/gloss.htm

A1AD = Alpha1 Antitrypsin Deficiency Emphysema
ABG = Arterial Blood Gas
ARDS = Acute Respiratory Distress Syndrome
ATRA = All Trans-Retinoic Acid

BAL = Bronchoalveolar Lavage
BiPAP = Bi-level Positive Airway Pressure
BMD = Bone Mineral Density
BOOP = Bronchiolitis Obliterans Organizing Pneumonia
BP = Blood Pressure
BX = Biopsy

CF = Cystic Fibrosis
CHF = Congestive Heart Failure
CLD = Chronic Lung Disease
CT = CAT scan or Computerized Axial Tomography
CO = Carbon Monoxide
Co2 = Carbon Dioxide
COAD = Chronic Obstructive Airway Disease
COLD = Chronic Obstructive Lung Disease
CPAP = Continuous Positive Airway Pressure
CPR = Cardiopulmonary Resuscitation
CRT = Certified Respiratory Therapist
CXR = Chest X-ray

DAD = Diffuse Alveolar Damage
DLCO = Diffusing Capacity of Carbon Monoxide
DNR = Do Not Resuscitate
DOE = Dyspnea on Exertion
DPAP = Demand Positive Airway Pressure
DX = Diagnosis

ECG or EKG = Electrocardiogram
ECHO = Echocardiogram
ENT = Ears, Nose, Throat
ERV = Expiratory Reserve Volume
ET = Endotracheal Tube

FEF = Forced Expiratory Flow
FEFMAX = Forced Expiratory Flow at Maximum Effort
FEV = Forced Expiratory Volume
FEV1 = Forced Expiratory Volume in One Second
FPF = Familial Pulmonary Fibrosis
FRC = Functional Residual Capacity
FVC = Forced Vital Capacity

GERD = Gastroesophageal Reflux Disease
GI = Gastrointestinal
GP = General Practitioner

HEENT = Head, Eyes, Ears, Nose, Throat
HEPA = High Efficiency Particulate Air
HR = Heart Rate
HRCT = High Resolution CT Scan

IC = Inspiratory Capacity
ICU = Intensive Care Unit
ILD = Interstitial Lung Disease
IPF = Idiopathic Pulmonary Fibrosis
IRV = Inspiratory Reserve Volume

LOX = Liquid Oxygen
LPM = Liters per Minute Oxygen or o2 flow rate
LVRS = Lung Volume Reduction Surgery

MCS = Multiple Chemical Sensitivities
MDI = Metered Dose Inhaler
MRI = Magnetic Resonance Imaging
MVV = Maximal Voluntary Ventilation

NETT = National Emphysema Treatment Trial
NIV = Non Invasive Ventilator
NSIP = Non Specific Interstitial Pneumonitis

o2 = Oxygen
OA = Occupational Asthma
OAD = Obstructive Airway Disease
OLD = Occupational Lung Disease
OSA = Obstructive Sleep Apnea
OTC = Over the Counter

PAP = Positive Airway Pressure
PCP = Primary Care Physician
Pco2 = Partial Pressure of Carbon Dioxide in Blood
PDI = Powdered Dose Inhaler
PE = Physical Exam
PE = Pulmonary Edema
PE = Pulmonary Embolism
PEP = Positive Expiratory Pressure
PEEP = Positive End Expiratory Pressure
PEFR = Peak Expiratory Flow Rate
PF = Pulmonary Fibrosis
PFT = Pulmonary Function Test
PH = Pulmonary Hypertension Secondary
PI = Pulmonary Insufficiency
PLB = Pursed Lip Breathing
PND = Paroxysmal Nocturnal Dyspnea/Post Nasal Drip
Po2 = Oxygen Tension in Arterial Blood
PPH = Primary Pulmonary Hypertension
PPV = Positive Pressure Ventilation
PT = Physical Therapist/Pulmonary Therapy
PTX = Pneumothorax
PULM or PULMO = Pulmonary

QOL = Quality OF Life

R/O = Rule Out
RAD = Reactive Airway Disease
RDS = Respiratory Distress Syndrome
RLD = Restrictive Lung Disease
RLS = Restless Leg Syndrome
RRT = Registered Respiratory Therapist
RT = Respiratory Therapist/Respiratory Therapy
RV = Residual Volume
RX = Treatment/Therapy/Prescription

Sao2 = % Arterial Blood Saturation
SATS = Referring to o2 Saturation Level by Oximetry
SOB = Short Of Breath
Svo2 = % Venous Blood Oxygen Saturation

TLC = Total Lung Capacity
TTo2 = Transtracheal Oxygen
TV = Tidal Volume
TX = Transplant

UIP = Usual Interstitial Pneumonitis
URI = Upper Respiratory Infection
UTI = Urinary Tract Infection

VATS = Video Assisted Thoracic Surgery
VC = Vital Capacity
VQ = Ventilation Perfusion Scan

XP = Transplant