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APSR in Bali (November 2014)

<Acute Excerbation of IPF>

1) Practical questions and answers from experts

 (underline: our answers

・Do you use BAL for Dx ? ⇒Yes, unless a high chance of triggering the need for MV.

・Do you use empirical ABTs Tx ? ⇒Yes, broad-spectrum antibiotic therapy.

・Do you treat with antivirals ? ⇒No, unless severely lymphopenic.

Usually No. but. Yes, at flu season.

・Do you treat for pneumocystis ? ⇒Yes, co-trimoxazole.

・Do you use anticoagulation ? ⇒No, because warfarin do harm fot IPF (PANTHER trial)

Yes, LMWH or rh-Thrombomodulin (0.06 mg/kg/day x 6days), unless known bleeding disorders.

・Do you use corticosteroid ? ⇒Yes, pulse Tx: mPSL 1g/d, for 3d.

・Do you use immunosuppressant ? ⇒No, due to lack of evidence.

Yes, unless overt infection.

To prevent 2nd AEX during tapering CS is crucial.

(Antoniou KM and Wells AU. Respiration 2013; 88: 285-274.)

 

2) Summary of management of AE-IPF

・Early diagnosis and intervention are important.

・Exclude pulmonary embolism and heart failure.

・Evaluate infectious etiology.

・Pulse CS therapy, broad ABTs, PPI, co-trimoxazole are recommended therapies for AE.

・NPPV should be introduced early.

<Comorbidity of IPF>

・Comorbidity:

1) Ischemic heart disease, 2) Acute Excerbation, 3) Infection, 4) Cancer, 5) GERD,
6) DM, 7) PAH

 

・Hypoxia less than 88% during and after Exercise:

IPF; 77%,Old Tbc; 23%, Emphysema; 38.2%.

 

・Scheme of CT patterns of AE-IPF (AJRCCM 2008; 178: 372-378)

A) Peripheral pattern, B) multifocal pattern, C) diffuse pattern.

・Trigger Factors for Acute Exacerbation of IPF:

1) Non-bacterial infections of unknown etiology

2) Inappropriate tapering corticosteroid

3) Combinational use of cytotoxic drugs

4) Irradiation

5) Invasive surgical procedures (biopsy, treatment operations)

6) Inhalation of high concentration of oxygen

7) Mechanical ventilation

8) Pneumothorax

 

・PMX-DHP improves survival rate of AE-IPF: 30days⇒70.1%, 90days⇒34.5%.

 

・Summary of PAH in IPF

1) PAH is higher prevalent according to severity of IPF.

2) mPAP > 25mmHg induces higher mortality.

3) But, vasodilator i.e. ET1R inhibitor rather worsened prognosis of IPF.

4) How to prevent PAH is therapeutic target of IPF.

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