Multidisciplinary Approach to Diagnosis of IPF

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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

MDD, multidisciplinary discussion

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

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 diagnosis1,2

Prior to holding an MDD, there is often disagreement on a diagnosis, even among ILD experts.2,3

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
2. Martinez FJ et al. Lancet Respir Med. 2017;5(1):61-71.
3. Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910.

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

MDDs are the standard for diagnosing ILDs4
–An MDD is generally only performed in the context of an ILD center1

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
4. 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 definite UIP pattern, SLB is indicated to provide a definitive diagnosis of IPF or other ILD1
 
Cases that warrant taking a biopsy most likely will go through an MDD1

SLB comes with risks and can be contraindicated for much of this population.1

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

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 pathology1,5

Clinical findings + Radiologic findings + Histopathologic findings + Expert opinion

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
5. Larsen BT et al. Arch Pathol Lab Med. 2012;136(10):1234-1241.

Combinations of radiologic and pathologic data can point to a diagnosis

Clinical signs1,6,7
Dyspnea
Dry cough
Crackles
Clubbing
Low DLco
 
Wheezing
Productive cough
Signs of CTD

CTD, connective tissue disease

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
6. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64. 
7. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101.

Combinations of radiologic and pathologic data can point to a diagnosis

Clinical signs1,6,7 Radiologic1
Dyspnea
Dry cough
Crackles
Clubbing
Low DLco
Definite UIP
Possible UIP
Possible UIP
Inconsistent with UIP
 
Wheezing
Productive cough
Signs of CTD
Definite UIP
Possible UIP
Inconsistent with UIP

CTD, connective tissue disease

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
6. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64. 
7. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101.

Combinations of radiologic and pathologic data can point to a diagnosis1

Clinical signs1,6,7 Radiologic1 Pathologic1
Dyspnea
Dry cough
Crackles
Clubbing
Low DLco
Definite UIP Probable UIP, Possible UIP, Nonclassifiable fibrosis, Not UIP
Possible UIP Definite UIP, Probable UIP
Possible UIP Possible UIP, Nonclassified fibrosis
Inconsistent with UIP Definite UIP
 
Wheezing
Productive cough
Signs of CTD
Definite UIP Not UIP
Possible UIP
Inconsistent with UIP Probable UIP, Possible UIP, Nonclassifiable fibrosis, Not UIP

CTD, connective tissue disease

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
6. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64. 
7. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101.

Combinations of radiologic and pathologic data can point to a diagnosis1

Clinical signs1,6,7 Radiologic1 Pathologic1 Diagnosis1
Dyspnea
Dry cough
Crackles
Clubbing
Low DLco
Definite UIP Probable UIP, Possible UIP, Nonclassifiable fibrosis, Not UIP Definite UIP
Possible UIP Definite UIP, Probable UIP
Possible UIP Possible UIP, Nonclassified fibrosis Probable IPF
Inconsistent with UIP Definite UIP Possible IPF
 
Wheezing
Productive cough
Signs of CTD
Definite UIP Not UIP Inconsistent with IPF
Possible UIP
Inconsistent with UIP Probable UIP, Possible UIP, Nonclassifiable fibrosis, Not UIP

CTD, connective tissue disease

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
6. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64. 
7. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101.

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
  • 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 ILDs1
1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.

References

1. Raghu G et al. Am J Respir Crit Care Med. 2011; 183(6):788-824.
2. Martinez FJ et al. Lancet Respir Med. 2017;5(1):61-71.
3. Flaherty KR et al. Am J Respir Crit Care Med. 2004;170(8):904-910.
4. du Bois RM. Eur Respir Rev. 2012;21(124):141-146.
5. Larsen BT et al. Arch Pathol Lab Med. 2012;136(10):1234-1241.
6. ATS. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64.
7. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al, eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101.