164 Sjogren’s syndrome and localisedlocalized nodular cutaneous amyloidosis: new insights into the link between the two
Bibliographic record
Abstract
Background and aims Sjogren’s Syndrome (SS), a known complication of systemic lupus erythematosus, is associated with localised nodular cutaneous amyloidosis, AL type (AL-LNCA). The reason is unclear, but clues from studies of this rare variant of amyloidosis are emerging. Methods Six patients with AL-LNCA, 4 from Austria and 2 from Canada were identified. Clinical, demographic and histopathological data were recorded and outcome noted over a median period of 72 months (range 40–144). Results Of 3 men and 3 women (median age 57 years; range 36–72) 1 patient had diabetes mellitus and essential hypertension and another scleroderma. The skin lesions were tan plaques or nodules, 1.5–4.0 cm in size, on the legs (5) and arm (1). Microscopically, bulky deposits of AL amyloid in the dermis/subcutis were associated with light perivascular infiltrates of lymphocytes and monoclonal plasma cells (with kappa (3) or lambda (3) light chain restriction). Two patients developed local cutaneous recurrences of their AL-LNCA 4 and 5 years after presentation. None developed systemic amyloidosis. Conclusions The clinical phenotype and course of AL-LNCA in our series, like those in the literature, mirror those of primary cutaneous marginal zone lymphoma, lymphoplasmacytic variant. This is now included among the larger group of extranodal B-cell lymphomas of MALT. Patients with SS are at risk for the development of MALT lymphomas. These, in turn, are known to be associated with localised peritumoral amyloidosis in internal organs. We submit that AL-LNCA in SS is a manifestation of a MALT lymphoma in the skin.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.003 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".