case_images: PMC11269126_01
case_images: The treatment regimen for the patient, from the onset of the condition until the end of the follow-up period, is shown at different time points (A). Histopathology of labial gland biopsy (B). The changes in serum AQP4-IgG levels performed by cell-based assay before and after immunoadsorption treatment (C, D). *GC, Glucocorticoid; CTX, Cyclophosphamide; MMF,Mycophenolate mofetil; VA,visual acuity. IVIG, intravenous immunoglobulin; MP, methylprednisolone;IA,immunoadsorption.
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case_images: Her EDSS score was six.  Figure 1A  shows the patient' treatment regimen from the onset of the condition until the end of the follow-up period. Auxiliary examinations: Salivary gland biopsy revealed two foci of lymphocyte invasion within the salivary gland tissue, with each focus containing more than 50 lymphocytes classified as grade IV. ( Figure 1B ). AQP4-IgG, as determined by the cell-based assay (CBA), was positive in the serum with a titer of 1:100 (Figure 1C), and turned negative after treatment (Figure 1D). Upon discharge, we re-examined the serum AQP4-IgG level, and found it to be negative ( Figure 1D ).
case_images: Neuroimaging changes of the patient with pSS-NMOSD patient. The MRI showed a T2 hyperintense lesion located at the cervical and thoracic spine levels of C5-T5 vertebrae (A) and also in the central portion of the spinal cord (B), in March 2016 (indicated by arrows). After treatment with protein-A immunoadsorption combined with immunosuppressive therapy, there was a significant improvement in the previously noted signal abnormalities. as observed in the follow-up scans in April 2018 (C-G) and April 2020 (F, G).
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case_images: Furthermore, MRI of the cervical and thoracic spine showed a long, extensive T2-hyperintense lesion at the C5-T5 vertebral levels, involving the central portion of the spinal cord ( Figures 2A, B ). Additionally, follow-up MRI scans conducted in April 2018 and April 2020 revealed a significant reduction in the intensity of high-signal areas of the spinal cord (Figures 2C-G).
case_images: The serum concentrations of IgA, IgG, IgM, RF, ESR, C3, and C4 were measured before and after each treatment session, as well as at each follow-up time point. The laboratory indices before treatment were normalized to a value of 1.
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case_images: Simultaneously, we regularly monitored the serological markers associated with disease activity, including routine blood tests, RF, ESR, complement C3/C4, and immunoglobulin levels, as outlined in Supplementary Table S1 and illustrated in Figure 3.