Sjögren's disease & overlap diseases
Progression and prognosis of interstitial pneumonia with autoimmune features: a longitudinal, prospective, multi-centre study
Authors' conclusion
IPAF patients had a rate of progression towards SAD similar to that reported in previous studies on undifferentiated connective tissue diseases, thus including some patients in which lung involvement could represent the first or even the sole clinical manifestation of a SAD.
Study context
- Population
- Patients classifiable as interstitial pneumonia with autoimmune features (IPAF) by research criteria, jointly followed by rheumatologists and pulmonologists for at least one year95 IPAF criteria combinations
- Participants
- 191 participants
- Exposure
- IPAF patients who progressed to SAD vs stable IPAF patients
- Outcome
- Rate of progression towards specific autoimmune diseases (SADs)
Methodological detailslongitudinal, multicentre
- Longitudinal
- Multicentre
Main finding
As reported in the abstract
Progressed towards SAD, mainly connective tissue diseases but also MPA
24.1%
Observational data: associations, not effects.
Further findings
Age
P = 0.002
Follow-up duration
P = 0.007
Severity
- Similar severity in progressed and stable patients
Predictors of overall progression
- No parameters associated with overall progression
Parameters associated with Sjögren's syndrome
- SSA positivity
- P = 0.007, χ2 7.4
Parameters associated with IIM
- Mechanic's hands
- P = <0.0001, χ2 12.6
- Organizing pneumonia pattern
- P = 0.01, χ2 6.1
- Anti-Pm/scl positivity
- P = 0.04 χ2 4.1
- Anti-MDA5 positivity
- P = 0.04, χ2 4.2
Parameters associated with SSc
- Palmar telangiectasias
- P = <0.0001 2 18.3
- Anti-Scl70 positivity
- P = <0.0001 χ2 12.5
- Anti-PM/Scl positivity
- P = 0.001 χ2 10.1
Original publication
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