Background: Kidney transplantation is an acceptable therapy end-stage kidney disease secondary to antineutrophil cytoplasmic antibody-associated vasculitis with risk of disease recurrence ranging from 3% to 17%. Standard posttransplant immunosuppression is the mainstay of therapy after recurrence. Recently, new medications focused on complement regulation and avoidance of steroids have been shown to be effective in treating antineutrophil cytoplasmic antibody (ANCA) vasculitis with no studies in the pediatric population.
Methods: We report a 5-year-old patient with immediate recurrence of positive myeloperoxidase (MPO)-ANCA vasculitis after deceased donor kidney transplant and the novel use of eculizumab to salvage the graft.
Results: Eculizumab and transition to ravulizumab has been successful in improving graft function and maintenance of disease remission after immediate MPO-ANCA vasculitis recurrence posttransplant.
Conclusions: Complement inhibitors may be used in addition to standard immunosuppression postkidney transplant in a pediatric patient with MPO-ANCA vasculitis recurrence without higher rates of infections.
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http://dx.doi.org/10.1111/petr.14760 | DOI Listing |
Korean J Neurotrauma
December 2024
Department of Neurosurgery, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
This case report describes a rare presentation of antineutrophil cytoplasmic antibodies-associated hypertrophic pachymeningitis in a 49-year-old woman with cranial and spinal lesions. Initial management comprised decompressive laminectomy and steroid therapy, with the subsequent addition of immunosuppressant therapy following symptom recurrence. After a ten-year follow-up, significant symptom improvement and return of motor function were noted.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Department of Internal Medicine, Division of Rheumatology, Mayo Clinic, Jacksonville, FL 32224, USA.
Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) using latent class analysis (LCA), and to evaluate their clinical characteristics and outcomes. We conducted a retrospective cohort study using electronic medical records of patients aged >18 years diagnosed with AAV and tracheobronchial disease between 1 January 2002 and 6 September 2022.
View Article and Find Full Text PDFActa Derm Venereol
January 2025
Dermatology Department, Unidade Local de Saúde Santa Maria, Lisbon, Portugal; Dermatology University Clinic, Faculty of Medicine, University of Lisbon, Lisbon, Portugal; Dermatology Research Unit, Instituto de Medicina Molecular, University of Lisbon, Lisbon, Portugal.
Sweet's syndrome (or acute febrile dermatosis) is a neutrophilic dermatosis with a characteristic presentation encompassing specific clinical (fever and erythemato-violaceous oedematous papules, plaques and nodules), laboratory (neutrophilia and increased inflammatory markers), and histological (dermal neutrophilic infiltrate without vasculitis) features. Its pathophysiology is poorly understood but there seems to be an auto-inflammatory component related to mutations in inflammasome genes. It has been subdivided into its classic form, malignancy-associated, and drug-induced, according to its aetiology.
View Article and Find Full Text PDFJ Clin Med
December 2024
Department of Internal Medicine, Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, 08036 Barcelona, Spain.
: Protracted febrile myalgia (PFM) is a rare but severe form of myalgia mainly occurring in pediatric patients with familial Mediterranean fever (FMF). PFM imaging and histopathological data remain scarce. : A comprehensive clinical, imaging, and histopathological characterization of PFM was performed by retrospectively analyzing a reference center cohort of adult patients with FMF and myalgia, and by a PubMed search of well-described cases with PFM.
View Article and Find Full Text PDFCureus
December 2024
Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Kuala Lumpur, MYS.
Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis.
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