Clinical practice guidelines · Interactive education module
ERS/EULAR CTD-ILD Guidelines 2026
Evidence-based recommendations on screening, diagnosis, monitoring and treatment of interstitial lung disease in connective tissue diseases — developed by a joint ERS/EULAR task force using GRADE methodology.
Insufficient evidence; very low ILD prevalence in SLE prevents a firm recommendation.
Very limited data — 2 observational studies, 170 patients
Should PFTs replace HRCT for screening? (PICO 1–4)
Against (Strong)
Do not replace HRCT with PFTs (FVC/DLCO) for ILD screening in any CTD. PFTs missed 6–88% of ILD cases in SSc. Sensitivity varied 34–99% across CTDs — unacceptably variable.
Low certainty for SSc, RA, IIM · Very low for other CTDs · 9 SSc studies (n=3191), 4 RA studies (n=287)
PFTs and symptom assessment remain important for functional evaluation — they cannot serve as standalone ILD screening tools. Missing ILD = lost treatment opportunity + increased morbidity and mortality.
Should lung ultrasound replace HRCT? (PICO 5–11)
Against (Conditional)
Do not replace HRCT with LUS — either extended (>18 intercostal spaces) or short protocol (≤18 spaces). Overall LUS sensitivity 94%, specificity 86% across 12 studies (n=971), but false-negatives still cause diagnostic delays and LUS is highly operator-dependent.
Low certainty (short protocol) · Very low certainty (extended protocol) · B-lines non-specific for ILD
Baseline risk assessment at ILD diagnosis (NQ 8–11)
SSc
RA
IIM
Other CTDs
Conditional
Perform global assessment including HRCT extent, FVC, DLCO, ATA-I antibodies, skin involvement (mRSS and progression), CRP/ESR, and African American ethnicity to identify high-risk SSc-ILD patients.
Low certainty · 14 observational studies, n=5268
Conditional
Perform global assessment including UIP/probable-UIP pattern on HRCT, extent of ILD, FVC and DLCO. UIP pattern carries highest mortality risk. Anti-CCP and RF positivity also linked to poor outcomes.
Low certainty · 25 studies, n=7587
Conditional
Perform global assessment including HRCT, FVC, DLCO, anti-MDA-5, anti-synthetase, and anti-Ro52 antibodies. Anti-MDA-5 positivity with ferritin >1600 ng/mL signals highest mortality risk.
Low certainty · 21 studies, n=1985 · Mainly Asian cohort data — ethnicity extrapolation caution warranted
No recommendation
Very low evidence for SjD; no evidence for SLE and MCTD. Risk factors are disease-specific — extrapolation not appropriate.
Only 1 retrospective single-centre study for SjD (n=33)
Role of BAL and lung biopsy at diagnosis (NQ 12–13)
Conditional
BAL may be considered when infection is suspected or to exclude alternative diagnoses. Pathogens detected in 22–25% of CTD-ILD patients on BAL in published studies.
Very low certainty · 21 studies, n=1196 · MDT discussion advised before performing BAL
Against (Conditional)
Lung biopsy should not be routinely performed at CTD-ILD diagnosis. TBLB identified CTD-ILD in only 13.8% of unclassifiable ILD cases. Pneumothorax occurred in 4 patients across studies reviewed.
Very low certainty · Consider only in MDT discussion if atypical HRCT features or malignancy suspected
Functional and patient-reported assessment (NQ 14–19)
Conditional
6MWT — use to assess severity and prognosis across all CTD-ILD subtypes. Lower 6MWD and SpO₂ desaturation predict progression and mortality. Caveats: avoid in RA with lower limb joint damage; in IIM with significant muscle involvement.
Low certainty for SSc · Very low for RA, IIM, other CTDs · 12 SSc studies (n=1724)
Conditional
PROMs (SGRQ, K-BILD, MRC, FACIT-dyspnoea, LCQ) capture symptom burden and daily life impact that objective measures miss. Weak but significant correlations with FVC, DLCO and 6MWD.
Low certainty SSc · Very low for other CTDs · No single PROM is specifically recommended over others
PFT monitoring frequency (NQ 20–23)
SSc
RA
IIM
Other CTDs
3–6 mo
First 3–5 years
6–12 mo
Thereafter (minimum)
Monitor FVC and DLCO. FVC is more responsive to treatment. Isolated DLCO fall should raise suspicion for emerging pulmonary hypertension. Repeat anytime progression is clinically suspected.
Monitor FVC, DLCO and FEV₁ — include FEV₁ given possible concomitant airway disease component in RA.
Very low certainty · 16 studies, n=1717
3–6 mo
First year
6–12 mo
Thereafter
Monitor FVC and DLCO. In rapidly progressive ILD, significant FVC changes can appear within 1 month. DLCO is less reliable than FVC in IIM.
Very low certainty · 16 studies, n=1176
3–6 mo
First year (SjD)
6–12 mo
Thereafter
For SjD-ILD: monitor FVC, DLCO and FEV₁. No recommendation for SLE and MCTD due to absence of evidence.
Very low certainty · 1 retrospective study, n=19
HRCT repeat schedule (NQ 24–27)
Conditional
SSc-ILD and RA-ILD — repeat HRCT after 1–2 years, particularly in high-risk patients. Earlier if progression suspected.
Very low certainty for both
Conditional
IIM-ILD — repeat HRCT at 3–6 months in high-risk or rapidly progressive ILD, then annually for first 2 years. Quantitative HRCT measures outperform visual scoring for detecting treatment response.
Very low certainty · 13 studies, n=596
Conditional
Other CTD-ILD (SjD, MCTD) — repeat HRCT after 1–2 years, extrapolated from SSc/RA/IIM data.
Very low certainty · Indirect evidence only · Low-dose CT protocols recommended for routine follow-up
FVC is the primary monitoring outcome. HRCT is particularly useful when PFTs cannot be performed, or to rule out other causes of worsening such as infection or malignancy.
Early dcSSc + elevated inflammatory markers or recent skin fibrosis progression
Strong · Moderate
Rituximab
SSc-ILD (esp. severe or multi-organ involvement)
Conditional · VL
Cyclophosphamide
SSc-ILD · Limited by cumulative toxicity
Conditional · Low
Nintedanib
SSc-ILD with ≥10% fibrosis extent on HRCT
Conditional · Moderate
Nintedanib + MMF
Add nintedanib on top of stable MMF (SENSCIS design)
Conditional · Low
Tocilizumab is the only strong treatment recommendation in SSc-ILD — restricted to the inflammatory, early diffuse cutaneous, skin-progressive subgroup. Biologics not currently combined. Avoid high-dose glucocorticoids. Refer for lung transplant if progressive and severe.
Immunosuppression
MMF, azathioprine, tocilizumab, abatacept, rituximab, JAK inhibitors
Conditional · VL
Pirfenidone
RA-ILD with UIP pattern specifically (TRAIL-1 trial data)
Conditional · VL
Nintedanib
RA-ILD with progressive pulmonary fibrosis (INBUILD trial)
Conditional · Low
For RA-ILD: treat active arthritis to target. TNF inhibitors may aggravate ILD — use case-by-case in MDT. Methotrexate now shown beneficial in RA-ILD despite historical pneumonitis concerns. No recommendation for pirfenidone in CTD-ILD other than RA-ILD (PICO 22).
Strong
Immunosuppressive treatment is recommended for all IIM-ILD. Narrow treatment window and rapid progression mandate prompt intervention.
Strong recommendation despite very low certainty — disease severity and high ICU admission rates justify this
Immunosuppressive treatment suggested for SjD-ILD, MCTD-ILD and SLE-ILD. Evidence is entirely indirect — no disease-specific RCTs identified for these subgroups.
Very low certainty · Indirect evidence from RECITAL and EVER-ILD trials
Conditional
Nintedanib (± immunosuppressant) suggested for progressive pulmonary fibrosis phenotype in any CTD-ILD including SjD/MCTD/SLE.
Low certainty · INBUILD trial subgroup data
Mycophenolate
First-line option
Azathioprine
Alternative immunosuppressant
Glucocorticoids
Adjunct — avoid long-term high dose
Rituximab / CYC
Combination or severe disease
Overarching treatment principles (NQ 28)
1
Prescribe off-label/highly specialised therapy only in expert ILD + CTD centres
2
Multidisciplinary discussion including pulmonologist and rheumatologist is advised for all cases
3
Use RCT inclusion criteria to guide agent selection — efficacy demonstrated in selected patient populations (Conditional · VL)
4
Early and/or aggressive treatment for patients at risk of progressive or severe ILD, especially anti-MDA-5 positive patients
5
Consider lung transplant referral for progressive, severe ILD unresponsive to therapy
6
Non-pharmacological management (oxygen, rehabilitation, palliative care) is important but not addressed in this guideline — follow disease-specific EULAR recommendations
Key research priorities identified by the ERS/EULAR task force
A
AI and digital monitoring — assess role of AI for disease trajectory prediction; validate home spirometry/oximetry tools; evaluate remote 6MWT using mobile devices
B
Risk stratification tools — develop composite tools to tailor upfront treatment; define uniform CTD-ILD progression criteria; clarify impact of RA articular disease activity on ILD prognosis
C
Novel therapies — evaluate CAR-T cells, bi/tri-specific antibodies, plasmapheresis in IIM-ILD; conduct IVIG vs placebo RCT for IIM-ILD; evaluate tofacitinib in prospective trials
D
Biomarkers and genetics — assess MUC5B promoter variant as RA screening tool; validate IIM-ILD prognostic biomarkers across ethnicities; evaluate liquid biopsy and genomic transbronchial biopsy
E
Anti-fibrotic research — assess pirfenidone and nintedanib effects on long-term mortality; assess pirfenidone efficacy across different HRCT patterns (UIP vs NSIP)
F
Screening frequency — no recommendation currently possible; multicentre prospective studies needed to define optimal re-screening intervals
G
Non-pharmacological management — evaluate lung transplantation, supplemental oxygen, and palliative care across CTD-ILD populations — none addressed in current guideline
H
SjD / MCTD / SLE-ILD — prospective collaborative studies urgently needed; no disease-specific RCTs exist; outcomes must include PFTs, HRCT, survival, symptoms and quality of life
Source: Antoniou KM, Distler O, Gheorghiu A-M, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J 2026; 67: 2402533. DOI: 10.1183/13993003.02533-2024. Endorsed by the ERS Executive Committee (9 June 2025), EULAR Council (16 December 2024), and ERN-LUNG (5 June 2025). Open access under CC BY-NC-ND licence.
Disclaimer: This interactive module is an educational summary prepared for clinical self-education purposes only. It does not replace the full published guideline, clinical judgement, or individualised patient assessment. Recommendations must be interpreted in the context of local reimbursement policies, drug availability, regulatory approvals, and each patient's clinical circumstances. Developed by Vamsipedia® · Precision Pulmonary Diagnostics.