Publications by authors named "S Grootenboer-Mignot"
Article Synopsis
- Lichen planus pemphigoides (LPP) is a rare autoimmune disease that causes blisters and can affect mucous membranes, though this aspect has not been thoroughly studied before.* -
- The study analyzed 12 cases of LPP primarily involving mucous membranes, confirming the diagnosis through specific histological and immunological tests.* -
- Treatment typically involves corticosteroids, which work better in cases with skin involvement; however, for those with significant mucous membrane issues, immunomodulators may be more effective as an initial treatment.*
Article Synopsis
- The study evaluated the effectiveness and safety of the drug omalizumab for treating bullous pemphigoid (BP) in patients who did not respond to standard treatments, analyzing data from 100 patients across 18 medical centers in France from 2014 to 2021.
- 77% of patients achieved complete remission (CR), and 9% achieved partial remission, with a median time to CR of approximately 3 months, although 14% experienced relapses within a median follow-up of 12 months.
- Higher baseline levels of anti-BP180-NC16A IgE were linked to better CR outcomes, while other factors like urticarial lesions or eosinophil
Unicentric Castleman disease (UCD) is a lymphoproliferative disease of unknown cause. Paraneoplastic pemphigus (PNP) is a major complication shown to be associated with a poor prognosis, with particular severity in patients with bronchiolitis obliterans (BO). This study describes the clinical and biological characteristics of UCD-PNP patients in a large Western cohort.
View Article and Find Full Text PDFSeveral reports have highlighted a potential role of autoreactive B-cells and autoantibodies that correlates with increased disease severity in patients with idiopathic pulmonary fibrosis (IPF). Here we show that patients with IPF have an altered B-cell phenotype and that those subjects who have autoantibodies against the intermediate filament protein periplakin (PPL) have a significantly worse outcome in terms of progression-free survival. Using a mouse model of lung fibrosis, we demonstrate that introducing antibodies targeting the endogenous protein PPL (mimicking naturally occurring autoantibodies seen in patients) directly in the lung increases lung injury, inflammation, collagen and fibronectin expression through direct activation of follicular dendritic cells, which in turn activates and drives proliferation of fibroblasts.
View Article and Find Full Text PDFMucous membrane pemphigoid (MMP) is a heterogeneous group of rare, chronic, subepithelial autoimmune blistering diseases (AIBDs) with predominant involvement of mucous membranes that can be sight-threatening and life-threatening. Rituximab (RTX) has demonstrated its efficacy in severe MMP refractory to conventional immunosuppressants in small series that differed in RTX scheme, concomitant therapies, and outcome definitions. In a meta-analysis involving 112 patients with MMP treated with RTX, complete remission (CR) was reported in 70.
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