IPF is one of the most common ILDs, and the prototypical progressive fibrosing ILD3

Infographic showing IPF prevalence of up to 200,000 people in the US and median survival of about 3 years Infographic showing IPF prevalence of up to 200,000 people in the US and median survival of about 3 years

IPF risk factors9

  • Male gender
  • >60 years old
  • History of smoking
  • Microbial agents
  • Environmental exposures
  • Family history

IPF claims ~40,000 US lives every year—almost as many as female breast cancer in 202410,11

ILD is a common, early, and potentially fatal manifestation of autoimmune diseases12

Infographic showing RA-ILD risk, with 15% developing ILD and 36% mortality within 5 yearsInfographic showing RA-ILD risk, with 15% developing ILD and 36% mortality within 5 years

Risk factors for ILD in patients with RA17:

  • High-titer RF
  • High-titer anti-CCP
  • History of smoking
  • Older age of RA onset
  • High disease activity
  • Male sex
  • Higher BMI
 
Infographic showing SSc-ILD risk, with 52% developing ILD and 23% mortality within 5 yearsInfographic showing SSc-ILD risk, with 52% developing ILD and 23% mortality within 5 years

Risk factors for ILD in patients with SSc17:

  • Anti-Scl-70 positivity, antinuclear antibody with nucleolar
  • Diagnosis of dcSSc
  • Male sex
  • African American
  • Early disease (first 5-7 years after onset)
  • Elevated acute phase reactants
Infographic showing myositis-ILD risk, with 50% developing ILD and 27% mortality within 5 yearsInfographic showing myositis-ILD risk, with 50% developing ILD and 27% mortality within 5 years

Risk factors for ILD in patients with myositis17:

  • Anti-synthetase (Jo-1, PL7, PL12, EJ, OJ, KS, Ha, Zo), anti–MDA5, anti-Ku, anti-Pm/Scl, anti-Ro52 antibody positivity
  • Mechanic’s hands, arthritis/arthralgia, ulcerating lesions
 
Infographic showing pSS-ILD risk, with 23% developing ILD and 18% mortality within 5 yearsInfographic showing pSS-ILD risk, with 23% developing ILD and 18% mortality within 5 years

Risk factors for ILD in patients with pSS17:

  • Presence of anti-Ro52 or antinuclear antibody
  • Raynaud’s phenomenon
  • Older age
  • Lymphopenia
  • Severe dental caries
Infographic showing MCTD-ILD risk, with 72% developing ILD and 59% mortality within 5 years Infographic showing MCTD-ILD risk, with 72% developing ILD and 59% mortality within 5 years

Risk factors for ILD in patients with MCTD17:

  • Dysphagia
  • Raynaud’s phenomenon
  • Other SSc clinical or laboratory features

Maintain a high level of suspicion for ILD in patients with known autoimmune diseases

Graphic stating that ILDs can become progressive Graphic stating that ILDs can become progressive Learn about the burden of ILD 
Alert graphic with message to sound the ILD alarm Alert graphic with message to sound the ILD alarm Learn about the role of PCPs 
Document graphic with message about resources for you and your patients Document graphic with message about resources for you and your patients View resources 
Magnifying glass graphic with message to discover more about ILD Visit the site 

ATA, anti-topoisomerase I antibodies; BMI, body mass index; CI, confidence interval; CCP, cyclic citrullinated peptide; dcSSc, diffuse cutaneous systemic sclerosis; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; MCTD, mixed connective tissue disease; MCTD-ILD, mixed connective tissue disease-associated interstitial lung disease; MDA5, melanoma differentiation-associated gene 5; pSS, primary Sjögren’s syndrome; pSS-ILD, primary Sjögren’s syndrome-associated interstitial lung disease; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; RF, rheumatoid factor; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

*Rates are reflective of 2 studies using a broad case definition (at least 1 diagnosis of IPF by a physician and no alternative diagnoses recorded within 6 months of IPF diagnosis).4,5 Study 1: Based on claims data from a US medical insurance claims database from January 2016 through December 2022, the rates of prevalence and incidence for IPF (adjusted for age and sex of the 2020 US census population) were 67.1 and 18.4 per 100,000 patients, respectively.4 Study 2: Based on claims data from another US medical insurance claims database from January 1, 2006 through September 30, 2012, the rates of prevalence and incidence for IPF (adjusted to the age distribution of the 2012 US census population and corrected for the positive predictive value) were 58.7 and 14.6 per 100,000 patients, respectively.5

†Prevalence of ILD in idiopathic inflammatory myositis (IIM) was 41% (95% Cl=35%, 50%). Prevalence of ILD in MCTD was 56% (95% Cl=39%, 72%).18

‡Overall survival was defined as the time in years from the date of progression to the date of death due to any cause. The cut-off date was fixed at December 31, 2017.22

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