Table 1 Recommendations adopted with minor changes and those added to the clinical referral pathway for Primary Care Management of Idiopathic Pulmonary Fibrosis

“For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable UIP, indeterminate for UIP, or an alternative diagnosis, we suggest cellular analysis of their BAL fluid (conditional recommendation, very low quality of evidence).” [28]

For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and do not have an HRCT pattern of definite UIP, we suggest cellular analysis of their BAL fluid (conditional recommendation, very low quality of evidence).

“For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and have an HRCT pattern of probable UIP, indeterminate for UIP, or an alternative diagnosis, we suggest SLB (conditional recommendation, very low quality of evidence).” [28]

For patients with newly detected ILD of apparently unknown cause who are clinically suspected of having IPF and do not have an HRCT pattern of definite UIP, we suggest SLB (conditional recommendation, very low quality of evidence)

Added Recommendation

Source

“Look for signs and symptoms (chronic dry cough, chest tightness, shortness of breath, mid to late inspiratory crackles, digital clubbing) that raise suspicion towards a diagnosis of IPF/ILD”

Medscape [32, 33]

“Assess risk factors – modifiable (environmental exposure of metal dust/wood particles, smoking history, exposure to pets) and non-modifiable (age over 50, male gender, family history) – for IPF”

Medscape [32, 34]

“Conduct rheumatological examination, along with general physical and respiratory examination in patients with suspicion of IPF.”

ATS/ERS [35, 36]

“For adult patients with suspicious signs and symptoms aligning with IPF, and a HRCT scan showing UIP pattern, refer to a pulmonologist or ILD clinic for specialized care.”

AAFP [37]

“For adult patients with suspicious signs and symptoms aligning with IPF, and a HRCT scan showing UIP pattern, recommend intervention for modifiable risk factors, if any. If comorbid GERD is present, start intervention.”

Medscape [32, 38]

“For adult patients with no suspicious signs and symptoms aligning with IPF, but with significant risk factors towards developing it, offer counselling and interventions for modifiable risk factors. If a patient is diagnosed with GERD, then start appropriate management.”

Medscape [32, 34]

“For adult patients with suspicious signs and symptoms aligning with IPF, HRCT scan showing UIP pattern, and patient hypoxemic (SpO2 < 88%), recommend home oxygen therapy to maintain saturation above 90% at rest, with sleep, and with exertion.”

Medscape [32, 33]