Primary Sjögren's syndrome (chronic autoimmune exocrinopathy with characteristic anti-Ro/...

Детермінований перегляд YAML-сутності з джерельної бази. Клінічний авторитет лишається за вказаними source ID та статусом клінічного sign-off.

ID	`RF-SJOGREN-MALT-LYMPHOMA-PREVENTION`
Тип	Тривожна ознака
Статус	переглянуто 2026-05-18 \| очікує клінічного підпису
Хвороби	`DIS-HCV-MZL`
Джерела	`SRC-NCCN-BCELL-2025`

Походження тривожної ознаки

Визначення	Primary Sjögren's syndrome (chronic autoimmune exocrinopathy with characteristic anti-Ro/SSA ± anti-La/SSB autoantibodies and histologic focal lymphocytic infiltration of minor salivary glands; EULAR/ACR 2016 classification criteria) in an individual without a current lymphoma diagnosis. The strongest hematologic-cancer risk factor among rheumatic diseases — ~15-20x relative risk for marginal zone (MALT) lymphoma, predominantly arising in parotid and lacrimal glands, with lifetime incidence ~5-10%. Risk concentrated in patients with persistent parotid swelling, palpable purpura, cryoglobulinemia, low C4 complement, monoclonal gammopathy, lymph- node enlargement, or high EULAR Sjögren's Disease Activity Index (ESSDAI). Annual examination targeting parotid/lacrimal swelling + lab monitoring (cryoglobulins, C4, SPEP, immunoglobulins) enables earliest-stage MALT detection. Prevention-person...
Клінічний напрям	investigate
Категорія	other

Логіка спрацьовування

{
  "any_of": [
    {
      "finding": "primary_sjogren_syndrome_diagnosis_confirmed",
      "value": true
    },
    {
      "finding": "sjogren_with_persistent_parotid_swelling",
      "value": true
    },
    {
      "finding": "sjogren_with_cryoglobulinemia",
      "value": true
    },
    {
      "finding": "sjogren_with_low_c4",
      "value": true
    }
  ],
  "type": "lab_value"
}

Нотатки

v0.3 chronic-condition prevention pilot — Sjögren's syndrome / MALT lymphoma. Prevention-persona RedFlag (CHARTER §3 amended 2026-05-18 Path A, HCP-mediated). Fires when patient profile shows confirmed primary Sjögren's syndrome AND no confirmed lymphoma. Engine routes to PreventionPlan with 2 tracks: annual physical exam + lab monitoring + parotid US for any new/persistent swelling (IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-SURVEILLANCE) as standard; rheumatology-driven observation with lymphoma workup only on clinical suspicion (IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-OBSERVATION) as alternative. Evidence base: - **Lymphoma risk magnitude:** Theander et al. Ann Rheum Dis 2006 (Swedish cohort, SIR ~15.6); Solans-Laqué et al. Semin Arthritis Rheum 2011 (meta-analysis, RR ~13-19). MALT lymphoma comprises ~60-70% of Sjögren-related lymphomas; DLBCL ~20-25%; nodal marginal zone + others remainder. - **Predictive features:** Brito-Zerón et al. Ann Rheum Dis 2014 (multicenter cohort) — predictors: persistent salivary swelling (OR ~3), low C4 (OR ~2.5), cryoglobulinemia (OR ~2.5), palpable purpura (OR ~2), monoclonal gammopathy (OR ~2). Patients with ≥3 features carry markedly higher lymphoma r...

Де використовується

Indications

IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-OBSERVATION - IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-OBSERVATION
IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-SURVEILLANCE - IND-SJOGREN-MALT-LYMPHOMA-PREVENTION-SURVEILLANCE