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Pendahuluan: Sindrom hiperimunoglobulin E adalah sindrom imunodefisiensi langka yang melibatkan banyak sistem organ, antara lain sistem muskuloskeletal, jaringan ikat, serta sistem imun. Disregulasi imun pada sindrom hiperimunoglobulin E juga kerap disertai penyakit autoimun, seperti systemic lupus erythematosus. Patogenesis systemic lupus erythematosus yang dipahami akibat adanya autoantibodi subtipe IgG, ternyata juga diperantarai oleh adanya autoantibodi subtipe lain, termasuk autoantibodi IgE. Konsentrasi IgE yang tinggi, baik jenis nonspesifik maupun jenis autoreaktif, seperti pada keadaan hiperimunoglobulin E, memiliki peran potensial dalam proses patogenesis hingga ke progresivitas penyakit autoimun seperti lupus. Kasus ini memaparkan terduga penyandang sindrom hiperimunoglobulin E awitan dewasa yang kemudian didiagnosis systemic lupus erythematosus pada pemeriksaan lanjutan.

Hyperimmunoglobulin E syndrome (HIES) is a rare multisystem syndrome that affects musculoskeletal system, connective tissue, and immune system. Immune dysregulation in hyperimmunoglobulin E syndrome often accompanied by autoimmune diseases, such as systemic lupus erythematosus. While the role of IgG autoantibody is well known in the pathogenesis of systemic lupus erythematosus, other subtypes of autoantibodies, IgE in particular, are now known to be involved in autoimmune process. A female with known elevated IgE was diagnosed with adult onset HIES. Atypical chest pain and otherwise normal cardiac imaging studies prompted her for further lab testings. Positive antinuclear antibody immunofluorescence test and elevated anti ds-DNA led to SLE diagnosis. Lack of characteristic clinical manifestations complicates establishing HIES while showing the extent to which IgE elevation could be related to SLE.

Background: This study aimed to assess the comparison between two HbA1c analyzers, Alere Afinion AS100, a boronate affinity based point of care testing (POCT), and High-Performance Liquid Chromatography (HPLC) based analyzer Bio-Rad Variant II Turbo HbA1c kit-2.0 as reference method.Methods: This study involved 120 samples of peripheral K3EDTA whole blood sent to Clinical Pathology Laboratory of Cipto Mangunkusumo National Hospital for HbA1c measurement. Based on reference method, 40 samples with HbA1c ≤ 6.4%, 40 samples with HbA1c > 6.4%, and 40 samples with variant hemoglobin or hemoglobinopathy were included. Precision and accuracy of both analyzers were assessed using control materials. The goodness of fit between these methods were assessed by Bland-Altman plot and Passing-Bablok regression test. Results were compared to National Glycohemoglobin Standardization Program (NGSP) recommendations.Results: Reference method had total error (TE) ranging from 3.15% to 4.9%, while Afinion ranged from 2.16% to 3.24%. Both methods correlated well with Passing-Bablok regression showing no proportional or systematic differences. Linearity between tests was proven by Cusum test value of p > 0.05. Bland-Altman plot yielded 91.74% goodness of fit. No significant differences were observed in hemoglobinopathy and variant hemoglobin analysis of HbA1c.Conclusion: Afinion was precise, accurate, and linear to HPLC reference method. Both methods exhibited no systematic or proportional differences. Despite Bland-Altman plot of less than 95% goodness of fit, no clinically significant result was found based on NGSP criteria.