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Relapsing ANCA-associated vasculitis presenting as a mediastinal mass.
BMJ Case Rep
January 2025
Department of Nephrology, Barts Health NHS Trust, London, UK.
Anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV) represent a heterogeneous multisystem group of disorders typified by necrotising inflammation of smaller blood vessels, classically yielding a pauci-immune, crescentic glomerulonephritis. Without prompt treatment, there is a significant risk of irreversible damage and ensuing renal impairment.Diagnosis is often challenging, exacerbated by the disorder's often vague and insidious presentation.
View Article and Find Full Text PDFPolyarteritis nodosa with life-threatening intracranial aneurysms in a child, and treatment with infliximab.
Turk J Pediatr
December 2024
Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye.
Background: Polyarteritis nodosa (PAN) is a rare and serious form of systemic necrotizing vasculitis that predominantly affects medium and small-sized arteries, with central nervous system involvement being particularly uncommon. Treatment strategies are tailored according to the extent and severity of the disease. While conventional therapy includes glucocorticoids and conventional disease-modifying-rheumatic drugs (cDMARDs), biologic agents may be critical for severe and refractory cases.
View Article and Find Full Text PDFFrom 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
December 2024
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 PDFUnusual presentation of necrotising sarcoid granulomatosis in the liver, spleen and bone.
BMJ Case Rep
January 2025
Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon
Sarcoidosis is an inflammatory multisystem disease characterised by non-necrotising granulomas that typically affect the lungs, lymph nodes, eyes, skin, liver, spleen, heart, bones and joints. Although rare, necrotising granulomas can also occur. In this report, we present a case of a healthy woman in her 60s who presented with a 1-year history of fatigue and generalised body aches.
View Article and Find Full Text PDFPost-procedural necrotizing fasciitis following femoral coronary angiography in patient with chronic endocarditis: A case report.
ARYA Atheroscler
January 2024
Cardiology Department, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran,Iran.
Background: Necrotizing fasciitis is a life-threatening soft tissue infection characterized by rapid tissue necrosis, often leading to sepsis and multisystem organ failure. Necrotizing fasciitis can rarely occur as a post-procedural complication, particularly following cardiac catheterization or angiography. This case report presents the clinical presentation and management of a 64-year-old female with a history of chronic endocarditis and valvular involvement who developed necrotizing fasciitis after femoral coronary angiography.
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