Langerhans' cell granulomatosis of the orbit was observed in a 7-year-old boy successfully treated by a simple biopsy. Recovery can be established one year after the biopsy on the basis of bone defect healing on the standard X-ray of the skull.
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Takayasu arteritis: a geographically distant but immunologically proximal MHC-I-opathy.
Lancet Rheumatol
January 2025
Section of Musculoskeletal Disease, NIHR Leeds Musculoskeletal Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Chapel Allerton Hospital, Leeds, UK. Electronic address:
Takayasu arteritis, a granulomatosis vasculitis with a pathogenesis that is poorly defined but known to be associated with HLA-B*52, shares many features with other MHC-I-opathies. In addition to the shared clinical features of inflammatory bowel diseases, cutaneous inflammation, and HLA-B*52, is shared association of an IL12B single- nucleotide polymorphism encoding the common IL-12 and IL-23 p40 subunit, which might affect not only type 17 cytokine responses, but also IFNγ and TNF production-the cardinal type 1 cytokines in granuloma formation. Considering the translational context of responses to TNF inhibition in Takayasu arteritis, in this Personal View we propose Takayasu arteritis as a type 1 MHC-I-opathy.
View Article and Find Full Text PDFAcute therapeutic effects and pathophysiology of eosinophilic granulomatosis with polyangiitis neuropathy.
BMJ Neurol Open
January 2025
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan.
Objective: This study investigated the effects of early treatment and pathophysiology on eosinophilic granulomatosis with polyangiitis neuropathy (EGPA-N).
Methods: Twenty-six consecutive patients with EGPA-N were diagnosed and treated within a day of admission and underwent clinical analysis. Peripheral nerve recovery rates were evaluated after early treatment by identifying the damaged peripheral nerve through detailed neurological findings.
From Fibrosis to Granuloma: Drug Induced Systemic Sarcoidosis-Like Reaction After Rituximab in a Patient with Primary Sjögren's Syndrome.
Eur J Case Rep Intern Med
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Clínica de Medicina, Serviço de Medicina Interna, Centro Hospitalar Universitário de Santo António, Porto, Portugal.
Unlabelled: Sarcoidosis is a multisystemic syndrome characterized by non-caseous granulomatous inflammation, although necrotizing sarcoid granulomatosis is considered part of the spectrum of the disease. Drug induced sarcoidosis-like reaction (DISR) is a systemic granulomatous reaction, which is histopathologically identical to primary sarcoidosis - mostly described after the use of biologics like tumour necrosis factor alpha antagonists but also anti-CD20 (rituximab). The authors present the very rare case of a woman with a primary Sjögren's syndrome (pSS) started on rituximab for disease control, which evolved with a 3-year indolent progressive systemic sarcoid reaction.
View Article and Find Full Text PDFProlonged SARS-CoV-2 Viremia in an Immunocompromised Patient.
J Med Cases
January 2025
Department of Clinical Sciences, Malmo, Lund University, Malmo, Sweden.
Immunocompromised patients, especially those receiving B-cell depleting therapies, are at risk for developing atypical presentation with regard to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, with the potential for diagnostic delay and adverse outcomes if such delay occurs. A 66-year-old female with history of granulomatosis with polyangiitis (GPA) with previous pulmonary involvement, treated with rituximab and low-dose prednisolone, presented with prolonged fever and cough after having been treated at home for a mild SARS-CoV-2 infection in early July 2023. The patient had a prolonged course over several months with constitutional symptoms such as fever, cough and malaise.
View Article and Find Full Text PDFA Unique Case Linking Rituximab to a Ureaplasma Infection and Life-Threatening Hyperammonemia.
Cureus
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Pulmonary & Critical Care, Indiana University Health Methodist Hospital, Indianapolis, USA.
Rituximab is an anti-CD20 monoclonal antibody medication used in treating various cancers like non-Hodgkin lymphomas as well as immunologic conditions like granulomatosis with polyangiitis. It disrupts and decreases the number of B-cells, which causes an immunosuppressive state. This can promote the growth of numerous rare and opportunistic pathogens, one of which is .
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