Angioedema Due to Acquired C1-Inhibitor Deficiency Without Hematological Condition: A Multicenter French Cohort Study of 34 Patients
Bibliographic record
Abstract
BACKGROUND: Angioedema (AE) due to acquired C1-inhibitor deficiency (AAE-C1-INH) is a rare disease associating recurrent edema of the mucosa and skin. Several underlying diseases have been reported, mainly lymphoproliferative diseases and monoclonal gammopathy. However, 15% to 20% of patients never exhibit such a hematological condition. OBJECTIVE: To analyze specific features of patients with AAE-C1-INH without a hematological condition. METHODS: This is a multicenter, retrospective cohort study of patients with AAE-C1-INH without a hematological condition included from January 1999 to May 2024 in the French national CREAK (Centre de reference des angioedèmes à kinine) registry. The clinical and biological characteristics of patients were detailed and then compared with those of patients with AAE-C1-INH associated with lymphoid hemopathies or monoclonal gammopathy. RESULTS: Thirty-four patients were included. All patients displayed a functional C1-INH below 50% of the reference value, 26 (76%) also had a decreased C1-INH antigen level, and 26 (76%) displayed anti-C1-INH antibodies. After a median follow-up of 65 months, 4 (12%) patients were in spontaneous complete remission of AE; 15 (44%) were in complete response under long-term prophylactic treatment. Compared with 75 patients with lymphoma-associated AAE-C1-INH, patients with AAE-C1-INH without a hematological condition displayed a higher incidence of anti-C1-INH antibodies and received more frequently symptomatic or prophylactic treatment with a lower remission rate at last follow-up. Clinical and biological features of patients with AAE-C1-INH without a hematological condition were similar to those of 30 patients with monoclonal gammopathy-associated AAE-C1-INH. CONCLUSIONS: AAE-C1-INH without a hematological condition displays a different clinical and biological presentation from lymphoma-associated AAE-C1-INH. No autoimmune disease was identified. Unlike rituximab, long-term prophylaxis seems to prevent AE attacks among these patients.
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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 | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".