PMC6886192_01
Lymph nodes from the patient (a, b) and normal control (c). Plasma cells staining with CD138 are shown in brown. a The patient has normal lymph node architecture. b Showing reduced numbers of plasma cells in the germinal centre in the patient. c The plasma cells are in the interfollicular areas in the control. CVID is typically associated with the absence of plasma cells and poorly formed germinal centres. The patient was not on immunosuppression at the time of the lymph node excision
13223_2019_383_Fig1_HTML.jpg
PMC6886192_01_13223_2019_383_Fig1_HTML.jpg
Fig1
Excision biopsy of a left inguinal lymph node showed the presence of discrete non-caseating granulomas, reactive follicles and overall normal lymph node architecture (Fig. 1a). Plasma cells were present although slightly reduced compared to a normal control (Fig. 1b, c).
MRI of the cauda equina. Enhancing lesions of the nerve roots are seen consistent with granulomatous inflammation. A biopsy was not possible, given the location
13223_2019_383_Fig2_HTML.jpg
PMC6886192_01_13223_2019_383_Fig2_HTML.jpg
Fig2
A repeat MRI scan of brain and spine revealed enhancing lesions in the cauda equina roots suggestive of granulomatous inflammation (Fig. 2).
IgG levels during immunosuppression. In mid-2018 the IgG normalised briefly between resolution of the cauda equine syndrome and treatment of renal disease. Cauda Cauda equine syndrome, Evans Evans syndrome, ITP immune thrombocytopenia, IVIG intravenous immunoglobulin, IS immunosuppression, Renal renal impairment
13223_2019_383_Fig3_HTML.jpg
PMC6886192_01_13223_2019_383_Fig3_HTML.jpg
Fig3
In mid-2018 when the immunosuppression was lifted, his IgG increased from 3.0 g/l to 8.8 g/l (Fig. 3) and the previously decreased IgA and IgM also normalised.