Challenges in the diagnosis of idiopathic pulmonary fibrosis: the importance of a multidisciplinary approach

Figures & data

Table 1. The role of MDD in the diagnosis of IPF according to the guidelines.

ATS/ERS/JRS/ALAT 2011 [5]	ATS/ERS/JRS/ALAT 2018 [10]	ATS/ERS/JRS/ALAT 2022 [17]
MDD should be performed after SLB	MDD should be performed once clinical and radiological data are available to determine the need for more invasive procedures (BAL, SLB)	MDD should be performed once clinical and radiological data are available to determine the need for more invasive tests (BAL, TBLC, SLB)

Abbreviations: ATS/ERS/JRS/ALAT: American Thoracic Society/European Thoracic Society/Japanese Respiratory Society/Associatión LatinoAmericana de Tórax; MDD: multidisciplinary discussion, BAL: bronchoalveolar lavage; SLB: surgical lung biopsy; TBLC: transbronchial lung cryobiopsy.

Table 2. Radiological criteria of disease progression in Progressive Pulmonary Fibrosis (PPF).

Increased extent or severity of bronchiectasis or/and bronchiolectasis

New ground-glass areas with bronchiectasis

New reticulations

Increase extent or increase coarseness of preexisting reticulations

Evidence of new honeycombing or more extensive honeycombing

Volume loss

Table 3. Useful clinical information to share during multidisciplinary discussion.

General information (age, gender)

Respiratory signs and symptoms and their onset and evolution (exertional dyspnea, dry cough, digital clubbing, peripheral cyanosis, ‘velcro-like’ crackles)

Signs and symptoms of a systemic autoimmune disease (arthralgia, joint swollen, Raynaud’s phenomenon, sclerodactyly, mechanics hands, muscle weakness, myalgia, sicca syndrome, rash)

Serological tests to exclude CTDs (CRP, ESR, RF, ACPA, ANA, CPK)

Pulmonary function tests (FVC, FEV1, TLC, DLco, 6MWT)

Smoking history

Occupational/environmental exposures (asbestos, metal or wood dust, birds, farming)

Drug history (consider use of medications known to cause pulmonary toxicity, such as chemotherapy agents, antiarrhythmic drugs, immunosuppressive drugs)

Family history of ILDs

HRCT features

Histological features, if available

Abbreviations: CTDs = Connective tissue disease; CRP = C-reactive protein; ESR = erythrocyte sedimentation rate; RF = rheumatoid factor; ACPA anti-cyclic-citrullinated peptides antibodies; ANA = antinuclear antibodies; CPK = creatine phosphokinase; FVC = forced vital capacity; FEV1 = forced expiratory volume in one second; TLC = total lung capacity; DLco = diffusion capacity of the lung for carbon monoxide; 6MWT = six-minutes walking test; ILDs = Interstitial lung diseases; HRCT = high resolutions computed tomography.

Table 4. Essential features and desirable features of MDDs.

Essential features	Desirable features
Identification of clinical cases requiring MDD	Having more than one member from each discipline
Participation of at least one pulmonologist and chest radiologist. One pathologist and rheumatologist on a case-by-case basis.	Allowing the attendance of external physicians either in person or via videoconferencing
Clinical data and accurate medical history	Report the degree of confidence of the diagnosis
HRCT scan	List differential diagnosis if a confident diagnosis is not possible
Histological data, if available	Following a standardized protocol annually reviewed
Interaction among specialists involved	Identification of priority cases to discuss

Abbreviations: MDD: multidisciplinary discussion, HRCT: High Resolution Computed Tomography.