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Systemic sclerosis (scleroderma) per ACR/EULAR 2013 classification criteria (diffuse cuta...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDRF-SCLERODERMA-LUNG-PREVENTION
TypeRed flag
Statusreviewed 2026-05-18 | pending_clinical_signoff
DiseasesDIS-NSCLC
SourcesSRC-NCCN-BCELL-2025 SRC-NCCN-NSCLC-2025

Red Flag Origin

DefinitionSystemic sclerosis (scleroderma) per ACR/EULAR 2013 classification criteria (diffuse cutaneous or limited cutaneous form, characteristic Raynaud's, sclerodactyly, fingertip pitting/ulceration, abnormal nailfold capillaroscopy, ± organ involvement) in an individual without a current lung cancer diagnosis. Established risk factor for lung cancer (~2-3x relative risk overall; further elevated in the scleroderma-associated interstitial lung disease [SSc-ILD] subset to ~5-7x, and in anti-topoisomerase-I-positive patients). Risk attributable to chronic pulmonary inflammation, fibrosis, immunosuppressant exposure, and the underlying scleroderma immunobiology. Smokers with SSc-ILD compound risk multiplicatively. HRCT chest q1-2y in SSc-ILD subset enables detection of incidental pulmonary nodules; PFTs q6-12mo guide ILD management and identify decompensation that may flag malignancy. Prevention-...
Clinical directioninvestigate
Categoryother

Trigger Logic

{
  "any_of": [
    {
      "finding": "systemic_sclerosis_diagnosis_confirmed",
      "value": true
    },
    {
      "finding": "scleroderma_with_interstitial_lung_disease",
      "value": true
    },
    {
      "finding": "anti_scl70_topoisomerase_positive",
      "value": true
    }
  ],
  "type": "lab_value"
}

Notes

v0.3 chronic-condition prevention pilot — systemic sclerosis / lung cancer. Prevention-persona RedFlag (CHARTER §3 amended 2026-05-18 Path A, HCP-mediated). Fires when patient profile shows confirmed systemic sclerosis (especially SSc-ILD or anti-Scl70 subset) AND no confirmed lung cancer. Engine routes to PreventionPlan with 2 tracks: HRCT chest q1-2y if ILD present + PFTs q6-12mo + smoking cessation (IND-SCLERODERMA-LUNG-PREVENTION-SURVEILLANCE) as standard; rheumatology-driven observation with chest imaging only on symptom trigger (IND-SCLERODERMA-LUNG-PREVENTION-OBSERVATION) as alternative. Evidence base: - **Lung cancer risk magnitude:** Bonifazi et al. Arthritis Care Res 2013 meta-analysis (cancer in SSc, SIR 4.4 for lung); SSc-ILD subset SIR ~5-7. Pearson et al. Arthritis Rheumatol 2018 cohort study showing temporal clustering at SSc onset suggestive of paraneoplastic mechanisms in some cases. - **Autoantibody risk:** anti-RNA polymerase III associated with paraneoplastic-presentation cancers (breast, others) at SSc onset; anti-Scl70 (topoisomerase I) associated with SSc-ILD and elevated lung-cancer risk. - **Surveillance recommendations:** EULAR 2023 update — HRCT baseline...

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