Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disease requiring treatment with immunosuppressive medications; however, finding a therapy that has a sustained durable response and an acceptable side effect profile has been challenging.
Objective: Our study aimed to evaluate the clinical outcomes of patients with BP treated with rituximab therapy at a single academic center.
Methods: A retrospective chart review was performed on 20 patients who received at least 1 dose of rituximab therapy, either as initial therapy for severe BP or as therapy for recalcitrant disease after having failed conventional immunotherapies.
Results: Within our cohort, 75% of patients (n = 15) achieved remission an average of 169 days following rituximab therapy. There were no rituximab-related deaths and significantly fewer adverse events following rituximab therapy.
Limitations: This study was limited by its retrospective nature, focus on a single academic center, and small sample size.
Conclusion: Use of rituximab therapy demonstrated high rates of remission, steroid-sparing activity, and an acceptable safety profile in our cohort of patients with severe BP or disease refractory to conventional therapies.
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http://dx.doi.org/10.1016/j.jaad.2019.03.049 | DOI Listing |
Neurol Neuroimmunol Neuroinflamm
March 2025
Department of Neurology, Mayo Clinic, Rochester, MN.
Background And Objectives: While it is well characterized in adults, little is known about the clinical features of neurofascin 155-IgG4 autoimmune nodopathy (NF155-IgG4 AN) in the pediatric population. In this study, we aimed to describe the clinical features and treatment outcomes in children diagnosed with neurofascin 155-IgG4 autoimmune nodopathy (NF155-IgG4 AN).
Methods: Pediatric and adult patients with NF155-IgG4 AN were identified retrospectively through the Mayo Clinic Neuroimmunology Laboratory database.
Curr Treatm Opt Rheumatol
December 2024
Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, MA USA.
Purpose Of Review: To summarize the current treatment landscape of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) in the context of the recent 2023 American College of Rheumatology/American College of Chest Physicians guideline for ILD treatment in systemic autoimmune rheumatic diseases.
Recent Findings: The guideline conditionally recommends mycophenolate, azathioprine, and rituximab for first-line RA-ILD therapy, with cyclophosphamide and short-term glucocorticoids as additional options. For RA-ILD progression after first line, mycophenolate, rituximab, nintedanib, tocilizumab, cyclophosphamide, and pirfenidone are conditionally recommended, while long-term glucocorticoids are conditionally recommended against.
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de São José, Lisbon, PRT.
Anti-melanoma differentiation-associated protein 5 (anti-MDA5) clinically linked amyopathic dermatomyositis (CADM) is a rare autoimmune condition strongly linked to rapidly progressive interstitial lung disease (RP-ILD), a life-threatening complication. We present a 63-year-old female patient with anti-MDA5-positive CADM, who developed RP-ILD with an imaging pattern consistent with organizing pneumonia. She presented with Gottron's papules, periungual erythema, progressive dyspnea, and anorexia.
View Article and Find Full Text PDFAnn Hematol
January 2025
Department of Clinical Pathology-Hematology and AinShams Medical Research Institute (MASRI), Faculty of Medicine, Ain Shams University, Cairo, 11566, Egypt.
Refractory Diffuse Large B-cell Lymphoma (DLBCL) presents a major therapeutic challenge due to its resistance to standard treatments. Engineered T-cells, especially Chimeric Antigen Receptor (CAR) T-cells, have shown promise in overcoming drug resistance. This study investigates the effectiveness of WEE1-engineered T-cells in targeting and eliminating refractory DLBCL in vitro.
View Article and Find Full Text PDFNeurology
February 2025
Department of Preventive Medicine and Epidemiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
Background And Objectives: Hepatitis B vaccination (HBV) requires 6 months to complete and is recommended for patients with multiple sclerosis (PWMS), particularly those who are candidates for anti-CD20 therapy. However, limited data exist on HBV immunogenicity in PWMS receiving disease-modifying therapies (DMTs) and the impact of starting anti-CD20 therapy during immunization. We aimed to evaluate HBV immunogenicity in PWMS starting anti-CD20 therapy during vaccination, focusing on the number of doses received before anti-CD20 initiation.
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