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Multidisciplinary Approach to Diagnosis of IPF

Diagnosing IPF: Multidisciplinary discussions

Multidisciplinary discussions are recommended for diagnosing IPF

The ATS/ERS/JRS/ALAT guidelines recommend using an MDD to evaluate all cases of suspected IPF that do not have a definite diagnosis by evaluating clinical and radiologic evidence alone1,2

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

MDD, multidisciplinary discussion.

Multidisciplinary discussions improve diagnostic confidence

An MDD involves a panel of ILD experts including clinicians, radiologists, and pathologists familiar with IPF who discuss the full array of findings to come to a consensus diagnosis2-4

Prior to holding an MDD, there is often disagreement on a diagnosis, even among ILD experts5,6

2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 3. ATS. Am J Respir Crit Care Med. 2002;165:277-304. 4. Lynch DA et al. Lancet. 2018;6(2):138-153. 5. Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910. 6. Tomassetti S et al. Am J Respir Crit Care Med. 2016; 193(7):745-752.

ILD, interstitial lung disease.

Multidisciplinary discussions are only used once a patient has a suspected ILD

MDDs are the standard for diagnosing ILDs7

–An MDD is generally only performed in the context of an ILD center2

2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 7. du Bois RM. Eur Respir Rev. 2012;21(124):141-146.

When clinical and radiologic evidence alone are not conclusive, biopsy and MDD are critical for a diagnosis

When HRCT scans do not show a UIP pattern, SLB is indicated to provide a definitive diagnosis of IPF or other ILD1,2,4

Cases that warrant taking a biopsy should be evaluated in an MDD1,2,4

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Lynch DA et al. Lancet. 2018;6(2):138-153.

SLB comes with risks and can be contraindicated for much of this population1

HRCT, high-resolution computed tomography; SLB, surgical lung biopsy.

MDDs synthesize all the data to produce a consensus diagnosis

An MDD involves taking the full sum of evidence (clinical, HRCT, and histopathologic findings) into account to help the panel determine whether the findings point to IPF or another pathology1,2,4,8

Clinical

findings

Radiologic

findings

Histopathologic

findings

Expert opinion

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Lynch DA et al. Lancet. 2018;6(2):138-153.

8. Larsen BT et al. Arch Pathol Lab Med. 2012;136(10):1234-1241.

Suspected Case of IPF

Histopathology

UIP

Probable UIP

Indeterminate for UIP

Alternative Diagnosis

HRCT results

UIP

IPF

Not IPF

Probable UIP

IPF

IPF Likely

Not IPF

Indeterminate for UIP

IPF

IPF Likely

Indeterminate

Not IPF

Alternative Diagnosis

IPF (likely) or non-IPF

Not IPF

Combinations of radiologic and pathologic data can point to a diagnosis1

Patients with clinical signs of IPF, including dyspnea, dry cough, crackles, low DLco, and digital clubbing, should undergo evaluation HRCT and potentially SLB to make a definitive diagnosis1,2,4

It is important to consider signs and symptoms that point to other diagnoses (eg, wheezing, productive cough, signs of CTD)1,4

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Lynch DA et al. Lancet. 2018;6(2):138-153. 9. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64.

Summary

All cases of suspected ILD that do not have a definitive diagnosis with clinical and radiologic evidence alone should be discussed in an MDD1,2,4

In practices where an MDD is not available or feasible, patients with suspected ILD should be referred to regional centers that have expertise in evaluating and managing ILDs2

1. Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Lynch DA et al. Lancet. 2018;6(2):138-153.

References

1.Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.

2.Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

3.ATS. Am J Respir Crit Care Med. 2002;165:277-304.

4.Lynch DA et al. Lancet. 2018;6(2):138-153.

5.Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910.

6.Tomassetti S et al. Am J Respir Crit Care Med. 2016; 193(7):745-752.

7.du Bois RM. Eur Respir Rev. 2012;21(124):141-146.

8.Larsen BT et al. Arch Pathol Lab Med. 2012;136(10):1234-1241.

9.ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64.