胰岛素自身免疫综合征:不只是一种而是两种不同的疾病与治疗意义。

IF 0.7 Q4 ENDOCRINOLOGY & METABOLISM
Anne Cathrine Parelius Wammer, Ingrid Nermoen, Per Medbøe Thorsby, Nils Bolstad, Kari Lima, Hoa Tran, Ivar Følling
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引用次数: 0

摘要

摘要:我们报告了一位年轻女性治疗抵抗性胰岛素自身免疫综合征(IAS)的长期病程。她的血清胰岛素水平为6945 pmol/l(学习要点:IAS可视为两种不同的疾病,多克隆和单克隆。多克隆或单克隆抗体的存在应决定IAS的治疗选择。在多克隆IAS中,停用触发药物和治疗触发条件应该是治疗的支柱。单克隆IAS应接受针对浆细胞的治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Insulin autoimmune syndrome: not just one but two different diseases with therapeutic implications.

Summary: We present a young woman with treatment resistant insulin autoimmune syndrome (IAS) with a protracted course. Her serum insulin level was 6945 pmol/l (<160), C-peptide 4042 pmol/L (<1480), anti-insulin antibodies 5305 U/mL (<0.4) were monoclonal IgG kappa. After 12 h of fasting, her blood glucose fell to 1.2 mmol/L. Post-meal blood glucose peaked at 12.2 mmol/L with reactive hypoglycaemia below 2 mmol/L. Frequent meals and continuous blood glucose monitoring were helpful, but further treatments advocated in the literature with prednisolone, rituximab, plasmapheresis, cyclophosphamide and ciclosporin were without beneficial effect. Based on this case and a review of the literature, we propose that IAS is not one but two different diseases with different therapeutic strategies. The first disease, polyclonal IAS, predominates in Asia and is characterized by polyclonal anti-insulin antibodies, association with certain HLA genotypes and other autoimmune conditions, medications and viral infections possibly triggering the disease, a possible female predominance among young patients and a tendency towards spontaneous remission. The other disease, monoclonal IAS, predominates in Caucasians. Typical features are monoclonal anti-insulin antibodies, only weak HLA association, no drug predisposition, no sex difference, rare remission and conventional therapy often being without any clinical effect. We suggest that monoclonal IAS with IgG or IgA anti-insulin antibodies should receive therapy targeting plasma cells rather than lymphocytes.

Learning points: IAS may be considered as two separate diseases, polyclonal and monoclonal. The presence of either polyclonal or monoclonal antibodies should determine the choice of treatment for IAS. In polyclonal IAS, discontinuation of a triggering medication and treatment of triggering conditions should be the backbone of therapy. Monoclonal IAS should receive treatment targeting plasma cells.

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来源期刊
CiteScore
1.50
自引率
0.00%
发文量
142
审稿时长
9 weeks
期刊介绍: Endocrinology, Diabetes & Metabolism Case Reports publishes case reports on common and rare conditions in all areas of clinical endocrinology, diabetes and metabolism. Articles should include clear learning points which readers can use to inform medical education or clinical practice. The types of cases of interest to Endocrinology, Diabetes & Metabolism Case Reports include: -Insight into disease pathogenesis or mechanism of therapy - Novel diagnostic procedure - Novel treatment - Unique/unexpected symptoms or presentations of a disease - New disease or syndrome: presentations/diagnosis/management - Unusual effects of medical treatment - Error in diagnosis/pitfalls and caveats
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