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Background: To describe a case of pediatric Sjogren syndrome (SS) which initially presented as edema and extensive pleural effusion without sicca symptoms. Case presentation: A 16-year-old female presented to the pediatric nephrology clinic with complaint of bilateral eyelid swelling for approximately 2 months. The laboratory examinations and labial glands biopsy were consistent with SS, for which she was started on a oral prednisone regimen. Despite initial improvement, she re-presented with significantly worsened eyelid swelling and edema of the lower limbs. Abdomen distention and shifting dullness was palpable at this time. Thoracentesis was performed and analysis of hydrothorax indicated exudates. The patient also developed neurological disorder manifesting as static tremor in the course. Intravenous methylprednisolone and immunosuppression therapy was initiated. Reduction of hydrothorax and alleviation of anasarca was observed. She was able to wean off prednisone 7 months later and still remains on mycophenolate mofetil (MMF) therapy after 31 months post diagnosis. Her illness remained stable without any symptoms until now.Conclusions: Sjogren syndrome in the pediatric population may present in diverse ways in the absence of sicca symptoms. We report a case of pediatric SS, presenting with edema and extensive pleural effusion without sicca symptoms, which was successfully treated with immunosuppression. Given the wide spectrum manifestations upon disease onset, it is of great significance to improve our understanding and awareness of this presumably under-diagnosed disease.

Background: Secondary vasculitis is encountered in about one third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported few times in previous literature. Case Presentation: We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. Conclusions: Presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.

To prepare a novel MRI targeted contrast agent Gd-DTPA-Granzyme B monoclonal antibody (mAb) and to test its reaction conditions.The Granzyme B mAb was coupled with DTPA,and then conjugated with Gd. The Gd-DTPA antibody was characterized using MALDI-TOF-MS. Cytotoxicity test was performed with MTT assay, and immune activation was examined with immunohistochemistry.MALDI-TOF-MS demonstrated that the molecular weight shifted from granzyme B mAb (133986) to Gd-DTPA-GB mAb (139736), which indicated the conjugation of the antibody with Gd-DTPA. The molar ratio of Gd per IgG molecule was about 20. MTT assay showed that Gd, DTPA, Gd-DTPA and Gd-DTPA-GB mAb groups did not make an impact on cell viability, and there were no significant differences among 4 groups (P0.05). Immunohistochemistry results showed that compared with the positive control group the targeted contrast agent had a high immune activity.The novel contrast agent Gd-DTPA-Granzyme B mAb prepared in this study keeps a good immune activity and has no significant cytotoxicity.