METHODS: The data were obtained from the National Health and Morbidity Survey (NHMS) which was conducted from September to October 2020. A cross-sectional survey with five structured questionnaires using the method of computer-assisted telephone interviews (CATI) was used to collect data. The socio-demographic characteristics such as age, gender, ethnicity, nationality, marital status, educational level, and occupation were recorded. Data were analysed using STATA SE Version 16. Associations between variables were tested using chi-square and logistic regression, with the level of statistical significance set at p
CASE REPORT: We report a case of a 45-year-old gentleman who presented with a painless anterior neck swelling and left supraglottic mass for six months. Computed tomography (CT) contrast imaging demonstrated a homogenous enhancing lesion at the left supraglottic and the midline of the anterior neck with erosive changes of the thyroid cartilage. A surgical resection of the anterior neck mass was performed. The diagnosis of Castleman disease plasma cell variant was made by histopathologic evaluation. The patient remained well post-resection.
CONCLUSION: Supraglottic multicentric Castleman disease is the least expected diagnosis in this case. Unicentric disease is treated with surgery. However, limited studies are available in determining the effectiveness of surgery in multicentric diseases. The plasma cell variant requires a multidisciplinary and multimodal approach due to an inclination towards malignancy. Research is needed to determine the role of surgery in multicentric disease and to develop optimum guidelines for managing cases. To date, there is unsubstantial literature describing supraglottic multicentric disease.
METHODS: A retrospective study of all NPSLE patients seen at the Pediatric Rheumatology Unit, Selayang Hospital from January 2004 to May 2017.
RESULTS: Twenty-eight (19.8%) of 141 JSLE patients had NPSLE with a median presenting age of 10 years (IQR 9 - 12), median follow-up of 7 years (IQR 4 - 11) and female: male ratio of 3.7:1. Twenty-three patients had single episodes of NPSLE and five patients had two distinct episodes each. The mean disease activity score (SLEDAI- 2K) was 24.9±11.8 at presentation with 81.8% having high disease activity (score > 12). Majority (60.6%) present with NPSLE within the first year of SLE diagnosis whilst the remainder occurred at a median of five years (IQR 3-7) post-SLE diagnosis. Majority (75.8%) had central nervous system (CNS) involvement commonly presenting with seizures, delirium and visual complaints whilst 24.2% had peripheral nervous system (PNS) involvement. Frequent accompanying features included hypocomplementemia, acute cutaneous lupus and lupus nephritis. Autoantibodies were common; ANA (100%), anti-dsDNA (78.8%) anti-RNP (39.4%) and anti-Sm (39.4%). Abnormalities were seen in 85.7% of the magnetic resonance imaging (MRI) studies performed, predominantly supratentorial white matter hyperintensities on T2 images whilst cerebrospinal fluid examination was normal in the majority. All patients with CNS involvement received corticosteroids with immunosuppressive therapy: Cyclophosphamide (20), Rituximab (2). Treatment for PNS involvement included corticosteroids with Azathioprine (6) or Mycophenolate mofetil (2). At 12 months post-NPSLE, majority (85.7%) recovered without any neurological sequelae.
CONCLUSIONS: Juvenile-onset NPSLE presents with a myriad of clinical features. It is associated with high disease activity and non-specific MRI features. With early diagnosis and treatment, the majority had good prognosis.