about the disease and an research article based on it

1. Name- Neera Katyayni
Section- B108
Reg no- 11717308
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2. • Idiopathic disease for which the cause is unknown.
• Absence of proper diagnosis
• Article ; Randomised Trial Of Acetylcysteine in IPF
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3. • An idiopathic disease is any disease with an unknown
cause
• The term ‘idiopathic’ literally means ‘of no known cause’
known that its common in smokers.
• There are many disease which are idiopathic in nature like;
idiopathic pulmonary fibrosis, intestinal pneumonia,
pellucid marginal degeneration, condylar resorption etc
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4. Idiopathic pulmonary fibrosis
• Idiopathic pulmonary fibrosis (IPF) is a type of
chronic lung disease characterized by a progressive and
irreversible decline in lung function.
• It is a type of interstitial lung disease (ILD).
• Symptoms may not be perticular.
• The underlying mechanism involves scarring of the
lungs.
• Complications may include pulmonary
hypertension, heart failure, pneumonia, or pulmonary
embolism.
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8. CAUSE
• The cause of IPF is unknown but certain
environmental factors and exposures have been
shown to increase the risk of getting IPF.
• Cigarette smoking is the most accepted risk factor
for IPF
• Other environmental and occupation exposures
such as exposure to metal dust, wood, coal, silica,
biologic dusts coming from hay dust also show the
increase risk for IPF.
• Viral infections may be associated with IPF and
other fibrotic lung diseases
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9. Symptoms
• Loss of elasticity leading to breathlessness, and a
crackling “Velcrolike” sound when breathing
• Dry cough(persistent cough )
• Tiredness
• Weight loss
• Finger clubbing.
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11. Drawback
• the general approach to treatment is to attempt to
suppress progression with corticosteroids, alone or
in combination with cytotoxic agents such as
azathioprine or cyclophosphamide.
• these compounds only slow the progression of the
disease, they do not act as a cure and cannot halt or
reverse the decline in lung function
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12. discussion
• Diagnosis may be supported by a CT scan or lung
biopsy.
• About 5 million people are affected globally
• Laboratory Tests - No specific serum laboratory
tests are available to diagnose IPF,
• but use of available tests can aid in
differentiating IPF from other diseases and
identifying complications and coexisting entities.
• Routine laboratory tests in the evaluation,
including complete blood cell count, may support
the presence of an infection.
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13. Developing new IPF therapies
• The world urgently needs improved
treatment of IPF. A range of agents are
currently being developed to combat IPF,
targeting various pathways
• One such research Article published on May
18,2014 by Fernando J . Martinez, MD, New
York
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14. Randomized Trial of Acetylcysteine in
Idiopathic Pulmonary Fibrosis
• Background - Acetylcysteine has been
suggested as a beneficial treatment for
idiopathic pulmonary fibrosis.
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15. Method
• - In our initial placebo-controlled trial, we randomly
assigned patients who had IPF with mild-to-
moderate impairment in pulmonary function to
receive acetylcysteine alone; or placebo.
• The trial continued as a two-group study
(acetylcysteine vs. placebo) without other changes;
133 and 131 patients were enrolled in the
Acetylcysteine and placebo groups, respectively.
• The primary outcome was the change in forced
vital capacity (FVC) over a 60-week period.
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17. Result
• At 60 weeks, there was no significant difference in
the change in FVC between the Acetylcysteine
group and the placebo group
(−0.18 liters and −0.19 liters, respectively; P = 0.77).
• In addition, there were no significant differences
between the acetylcysteine group and the placebo
group in the rates of death (4.9% vs. 2.5%, P = 0.30
by the log-rank test) or acute exacerbation (2.3% in
each group, P>0.99).
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18. Conclusion
• As compared with placebo, Acetylcysteine offered
no significant benefit with respect to the
preservation of FVC in patients with idiopathic
pulmonary fibrosis with mild to-moderate
impairment in lung function.
(Funded by the National Heart, Lung, and Blood
Institute and others
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