ILD Multidisciplinary Discussion Support Tool

Adult ILD evaluation and diagnosis support.

Structured intake modeled on key exposure domains, CTD serologies, and guideline-style HRCT patterning. The goal is to surface a transparent working diagnosis, plausible differential, uncertainty level, and the next tests most likely to change the diagnosis.

Load sample cases:

1. Patient context

Case label Age Sex at birth Known connective tissue disease (clinician-established, using current rheumatologic criteria where applicable) Smoking status Pack-years Symptom tempo Cough pattern Dyspnea burden (mMRC-like) Weight loss Fever / inflammatory systemic symptoms

2. Familial and genetic context

Genetic information can modify both classification and management. Pathogenic TRG or SRG findings may support a familial or genetically mediated fibrosis framework, influence transplant planning and surveillance, and reduce the incremental value of surgical lung biopsy when histology would not change care.

TRG examples include TERT, TERC, RTEL1, PARN, DKC1, NAF1, and ZCCHC8.
SRG examples include SFTPC, SFTPA1, and SFTPA2; SFTPA1/2 findings may also affect lung cancer surveillance planning.
Very short age-adjusted telomere length supports a short telomere syndrome framework even when a pathogenic variant is not identified.

Family history of pulmonary fibrosis / ILD Telomere-related gene testing (TRG) TRG gene identified Surfactant-related gene testing (SRG) SRG gene identified Age-adjusted leukocyte telomere length Short telomere syndrome features present (for example premature graying, cytopenias, liver disease) Genetic notes

3. Exposure history

Birds / feathers / avian exposure Mold or visible mildew exposure Water damage in home or workplace Humidifier exposure Hot tub / Jacuzzi / sauna exposure Farming / hay / compost / grain exposure Heavy smoke or general dust exposure Metal or hard mineral exposure Silica exposure Asbestos exposure Wood dust / cork / organic dust exposure Paint / isocyanate / industrial cleaning exposure Travel / residence context relevant to exposure or endemic infection Exposure notes

4. Medication and toxin screen

Amiodarone exposure Nitrofurantoin exposure Methotrexate exposure Bleomycin or other cytotoxic therapy Cyclophosphamide or radiation exposure Other pneumotoxic medication concern Medication / toxin notes

5. CTD and autoimmune clinical screen

Raynaud phenomenon Sicca (dry eyes / dry mouth) Reflux / significant GERD Dysphagia / esophageal symptoms Inflammatory arthritis / morning stiffness Skin thickening / sclerodactyly Mechanic hands Proximal muscle weakness CTD-compatible rash / heliotrope / Gottron-like features Mouth ulcers / photosensitive mucocutaneous disease Hematuria / renal vasculitic signal Hand ulcers / digital ischemia Autoimmune phenotype notes

6. CTD laboratories and BAL

ANA titer denominator ANA pattern RF status Anti-CCP Anti-Scl-70 / topoisomerase I SSA / Ro SSB / La Anti-Jo-1 Extended myositis panel (e.g., PL-7, PL-12, EJ, OJ, SRP, Mi-2, MDA5, TIF1-gamma, NXP2, SAE1, Ro52) MPO-ANCA PR3-ANCA CK and/or aldolase elevated Ferritin elevated ESR or CRP elevated Low complement (C3/C4) BAL lymphocytes (%) Laboratory notes

7. HRCT descriptors

The application derives UIP-family and HP-family HRCT categories from the entered descriptors so the clinician does not have to assign those pattern labels manually.

UIP-family derivation is driven mainly by lower-lung subpleural fibrosis, honeycombing, traction bronchiectasis, and the presence or absence of alternative-pattern features.
HP-family derivation is driven mainly by air trapping, centrilobular nodules, fibrosis, distribution, and other compatible non-UIP descriptors.
These are app-derived helper categories based on the structured inputs below and should be reviewed against the full radiology interpretation.

App-derived UIP family

Awaiting HRCT descriptors

Enter distribution, fibrosis, and competing HRCT features.

App-derived HP family

Awaiting HRCT descriptors

Enter air-trapping, nodules, distribution, fibrosis, and compatible secondary findings.

Honeycombing Traction bronchiectasis / bronchiolectasis Ground-glass opacity extent Consolidation extent Dominant distribution Mosaic attenuation / expiratory air trapping Centrilobular nodules Bilateral hilar and/or mediastinal lymphadenopathy Perilymphatic nodules / sarcoid-type nodularity Thin-walled cysts Emphysema Subpleural sparing Fibrosis extent HRCT notes

8. Pathology and granulomatous disease context

Use pathology as a structured process rather than a single yes/no toggle: first document whether tissue exists and what specimen was reviewed, then whether a thoracic pathology review and clinicoradiologic correlation occurred, and only then record the dominant histopathologic pattern and companion features.

UIP-family disease is best supported by a UIP-like pattern with fibroblast foci, architectural distortion, and no competing bronchiolocentric or granulomatous process.
HP rises when exposure history aligns with bronchiolocentric or airway-centered pathology, minor lymphocytic bronchiolitis, and poorly formed granulomas.
Sarcoidosis is more convincing when granulomas are well formed and the overall presentation also fits a perilymphatic or multisystem sarcoid pattern after alternative granulomatous causes are excluded.
Lymphoid follicles or plasma cell-rich infiltrates, NSIP-like change, and organizing pneumonia can push the discussion toward CTD-ILD or overlap patterns.
Use Unknown / not sampled rather than Absent when tissue was not obtained; that preserves uncertainty instead of falsely arguing against a diagnosis.

Pathology material available Thoracic pathology review documented Pathology-clinical-radiology correlation completed at MDD Dominant histopathologic impression Well-formed nonnecrotizing granulomas / sarcoid-type granulomas Airway-centered fibrosis Minor lymphocytic bronchiolitis Poorly formed nonnecrotizing granulomas Fibroblast foci Organizing pneumonia / Masson bodies Lymphoid follicles / plasma cell-rich infiltrate Extrapulmonary sarcoid-compatible features (for example eye, skin, cardiac, or lymph node involvement) Pathology or granulomatous disease notes

9. Progression overlay and MDD context

FVC decline over about 12 months (% predicted) DLCO decline over about 12 months (% predicted) Symptoms progressing within a year Radiographic progression within a year Rest / exertional oxygen requirement 6MWT or exertional desaturation concern Alternative granulomatous causes reasonably excluded (for example infection, HP, pneumoconiosis, vasculitis, or drug reaction) Specific MDD question Narrative context

Ranked differential diagnosis

MDD note draft

Case Summary for External AI

Copy and paste this prompt into OpenEvidence (or similar tool) to request an AI-supported multidisciplinary discussion summary and management-oriented review.

About The Application

This tool uses a transparent rule-based scoring process to generate relative diagnostic probabilities. It does not use a black-box image model, and the displayed percentages should be interpreted as within-tool ranking estimates rather than prospectively validated disease probabilities.

The application first collects structured inputs across familial/genetic context, exposure history, medication and toxin history, CTD symptoms, core serologies, and HRCT pattern descriptors. Those domains are grounded in the CHEST ILD questionnaire and contemporary ILD diagnostic frameworks. CHEST ILD clinician toolkit (2025), ERS/EULAR CTD-ILD guideline (2026)
It then derives clinically meaningful signals from those inputs, such as likely antigen exposure, possible pneumotoxic drug exposure, autoimmune signal strength, UIP-family patterning, HP-family patterning, and progression overlays. This mirrors guideline-based ILD reasoning, where known causes are assessed before idiopathic labels are applied and progression is handled separately from etiology. ATS/ERS/JRS/ALAT IPF and PPF update (2022), ERS/EULAR CTD-ILD guideline (2026)
Certain diagnoses are strengthened by hard rule gates. For example, the HP branch is strongly favored when exposure history, a typical HP HRCT category, and BAL lymphocytosis align or when exposure history and HP-compatible pathology cohere; the IPF branch is only strengthened when the intake supports exclusion of major alternate causes and the HRCT pattern is UIP-family; and the sarcoidosis branch is only hard-gated when compatible thoracic or extrapulmonary presentation, non-necrotizing granulomatous inflammation, and exclusion of alternative granulomatous causes align. Autoimmune and CTD-ILD branches are up-weighted by disease-specific clinical and serologic cues. ATS/JRS/ALAT HP guideline (2020), ATS/ERS/JRS/ALAT IPF and PPF update (2022), ERS/ATS IPAF research statement (2015), ATS sarcoidosis diagnosis and detection guideline (2020)
For every candidate diagnosis, the current version applies an additive point-based score: supportive findings add weight and conflicting findings subtract weight. This keeps the logic inspectable and easier to review in multidisciplinary discussion. Pathology and granulomatous inputs are active parts of this score, especially when they increase or decrease confidence in HP or sarcoidosis and help avoid premature idiopathic labeling.
Familial and genetic data are also incorporated as diagnostic and prognostic modifiers. Pathogenic TRG or SRG findings, very short telomere length, and strong familial context do not automatically erase clinicoradiologic phenotypes, but they can reframe the case as familial or genetically mediated fibrosis and change how the final MDD diagnosis is worded, how strongly surgical biopsy is pursued, and how follow-up and transplant planning are prioritized. ERS/ATS interstitial pneumonias statement update (2025), geneMDD study (2025), Telomeres in ILD review
Those raw scores are then converted into relative percentages using a softmax transform. The percentages therefore express how strongly each candidate diagnosis is favored relative to the others in the current rule set, not an externally calibrated absolute risk.
Formal CTD diagnoses are not auto-classified from first principles inside the tool. If a clinician marks a known CTD, the application treats that as externally established using current rheumatologic classification criteria where applicable, while the tool itself uses structured autoimmune features and serologies to support CTD-ILD and IPAF branches. EULAR/ACR criteria index, 2019 EULAR/ACR SLE criteria
Diagnostic certainty is assigned separately from probability. High-certainty output requires either a hard-gated branch or a well-supported leading diagnosis with adequate data completeness. Low completeness lowers certainty even if one diagnosis remains the top-ranked choice.
The application only prints competing differential diagnoses when their estimated probability is at least 5%, to keep the output focused on clinically meaningful competing possibilities.
MDD-style outputs are designed to support case review by documenting a leading diagnosis, confidence, competing differential, expected disease behavior, and suggested next tests or management steps, consistent with published ILD multidisciplinary meeting recommendations. LFA/TSANZ ILD MDM position statement (2017), Glenn et al. ILD MDM review (2022)

Guideline Basis And Current Practice Anchors

The diagnostic logic is intentionally transparent and aligned to major current ILD practice frameworks rather than an opaque model.

ATS/ERS/JRS/ALAT IPF and PPF update (2022) for UIP-family diagnostic framing, exclusion of known causes, multidisciplinary discussion, and separate progression overlay logic.
ERS/ATS interstitial pneumonias statement update (2025) for the broader interstitial pneumonia framework used by the application, while preserving practical MDD-facing labels such as IPF, NSIP, OP-spectrum disease, smoking-related ILD, sarcoidosis, and unclassifiable ILD.
Impact of a Multidisciplinary Team Discussion for Genetic Lung Fibrosis (2025), Genetic Evaluation and Testing of Patients and Families with IPF, and Telomeres in Interstitial Lung Disease: The Short and the Long of It for familial pulmonary fibrosis framing, TRG/SRG testing, telomere-length interpretation, transplant and surveillance implications, and the role of gene-focused MDD review.
ATS/JRS/ALAT hypersensitivity pneumonitis guideline (2020) for exposure-first evaluation, HP HRCT categories, BAL integration, and HP pathology features such as airway-centered fibrosis, giant cells, and bronchiolitis.
ERS/ATS IPAF research statement (2015) for structured autoimmune-feature criteria when defined CTD is uncertain.
ERS/EULAR CTD-ILD guideline (2026) for CTD-ILD screening, baseline assessment, BAL and biopsy positioning, and monitoring considerations.
ATS sarcoidosis diagnosis and detection guideline (2020) for the sarcoidosis framework of compatible presentation, non-necrotizing granulomatous inflammation, and exclusion of alternative granulomatous causes, with WASOG organ assessment referenced by the ATS guideline.
High risk of lung cancer in surfactant-related gene variant carriers for the added lung cancer surveillance concern in SFTPA1 and SFTPA2 variant carriers.
CHEST ILD clinician toolkit (2025) for the structured exposure, medication, and symptom intake domains used in this interface.
LFA/TSANZ ILD MDM position statement (2017) and Glenn et al. ILD MDM review (2022) for recommended MDD inputs, participants, documentation, and outputs.