Multidisciplinary Approach to IPF Diagnosis
Multidisciplinary Approach
Multidisciplinary discussions (MDDs) 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 alone.1,2
Multidisciplinary discussions improve diagnostic confidence.
An MDD involves a panel of interstitial lung disease (ILD) experts including clinicians, radiologists, and pathologists familiar with IPF who discuss the full array of findings to come to a consensus diagnosis.2-4
- Prior to holding an MDD, there is often disagreement on a diagnosis, even among ILD experts.5,6
Multidisciplinary discussions are only used once a patient has a suspected ILD.
MDDs are the standard for diagnosing ILDs.7
- An MDD is generally only performed in the context of an ILD center.2
When clinical and radiologic evidence alone are not conclusive, biopsy and MDD are critical for a diagnosis.
When high-resolution computed tomography (HRCT) scans do not show a usual interstitial pneumonia (UIP) pattern, surgical lung biopsy (SLB) is indicated to provide a definitive diagnosis of IPF or other ILD.1,2,4 Cases that warrant taking a biopsy should be evaluated in an MDD.1,2,4
- SLB comes with risks and can be contraindicated for much of this population.1
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 pathology.1,2,4,8
Clinical findings + Radiologic findings + Histopathologic findings + Expert opinion
Combinations of radiologic and pathologic data can point to a diagnosis.1
| Suspected Case of IPF | Histopathology | ||||
| UIP | Probable UIP | Indeterminate UIP | Alternative Diagnosis | ||
| HRCT Results | UIP | IPF | IPF | IPF | Not IPF |
| Probable UIP | IPF | IPF | IPF Likely | Not IPF | |
| Indeterminate UIP | IPF | IPF Likely | Indeterminate | Not IPF | |
| Alternative Diagnosis | IPF (likely) or non-IPF | Not IPF | Not IPF | Not IPF | |
- 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 (e.g., wheezing, productive cough, signs of connective tissue disease)1,4
REFERENCES
- Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST.
- Raghu G, Collard HR, Jim J Egan, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. doi: 10.1164/rccm.2009-040GL.
- ATS. American Thoracic Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165(2):277-304. doi: 10.1164/ajrccm.165.2.ats01.
- Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet. 2018;6(2):138-153. doi: 10.1016/S2213-2600(17)30433-2.
- Flaherty KR, King TE, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170(8):904—910. doi: 10.1164/rccm.200402-147OC.
- Tomassetti S, Wells AU, Costabel U, et al. Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2016;193(7):745-752. doi: 10.1164/rccm.201504-0711OC.
- du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev. 2012;21(124):141-6. doi: 10.1183/09059180.00000812.
- Larsen BT, Colby TV. Update for pathologists on idiopathic interstitial pneumonias. Arch Pathol Lab Med. 2012;136(10):1234-1241. doi: 10.5858/arpa.2012-0225-RA.
- ATS. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000;161(2 Pt 1):646-64. doi: 10.1164/ajrccm.161.2.ats3-00.
