Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 4

Idiopathic Pulmonary Fibrosis

Acute Exacerbation (AE):

– It is an unexplained worsening or development of dyspnoea within 30 days with new radiological infiltrates at HRCT abnormality often super imposed on background consistent with UIP pattern.

– The yearly incident of AE-IPF is poor, with mortality ranging from 78% to 96%.
Other causes of AEIPF such as pulmonary embolism, congestive heart failure, pneumothorax or infectin need to be excluded.

– Pulmonary infection has to be ruled out by endotracheal aspirate or BAL.

– Many patients experiencing acute deterioration require intensive care treatment, particularly when respiratory failure is associated with hemodynamic instability significant co-morbidity or severe hypoxemia.

– Mortality during hospitalization is high.

– Mechanical ventilation should be introduced only after carefully weighing the patients long-term prognosis and whenever possible, the patient’s wishes.


Clinical causes of IPF are unpredictable.
– Prognosis of IPF is associated with estimated median survival time 2-5 years after diagnosis.
5 years survival for IPF ranges between 20-40 %.

– Mortality rate higher than that of a number of malignancies including colon cancer, multiple myeloma and bladder cancer.

– Recently a multidimensional index and staging system has been proposed to predict mortality in IPF.

– The name of the index is GAP and is based on gender (G) age (A), and two lung physiology variable (P) that are commonly measured in clinical practice to predict mortality in IPF.

– The highest stage of GAP (Stage III) has been found to be associated with a 39% risk of mortality at 1 year.
This model has been evaluated in IPF and other ILDs and shown good performance in predicting mortality in all main ILD subtypes.

– In addition to clinical and physiological parameters to predict how rapidly patients with IPF might progress genetic and molecular features are also associated with IPF mortality.

– It has been shown that IPF patients who have a specific genotype in the mucin MUC 58 gene polymorphism experience slower decline in FVC and significantly improved survival.

– Even if such data are interesting from a scientific point of view, the application in the clinical routine of a prognostic model based on the specific genotypes is still impossible.


– IPF is the most common form of IIP.

– The prevalence of IPF has been estimated between 14 and 42.7 per 100,000 persons based on a USA analysis of health care claims date with a variation depending on the case definitions used in this analysis.

– IPF is more common in men than in women and is usually diagnosed in people over 50 years of age.
The incidence of IPF is 6.8-16.3 per 100,000 persons.

– Approximately 30,000-35,000 new patients will be diagnosed with IPH each year.

– IPF was the most common diagnosis (28%) followed by connective tissue disease – related ILD (14%), hypersensitivity pneumonitis (7%) and non-specific interstitial pneumonia (NSIP) (7%).

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