Wegener's granulomatosis is a disease characterized by necrotizing vasculitis of the upper and lower respiratory tracts, necrotizing glomerulonephritis, and varying degrees of disseminated small vessel vasculitis. Patients can present to an otolaryngologist head and neck surgeon with ear, nose, throat, lung, orbit, salivary gland, or cutaneous lesions. The disease is variable in its presentation and progression. Tissue biopsies may be non-diagnostic even in the presence of active disease. Although the disease was rapidly fatal as recently at 1970, it can now be effectively treated. This paper reviews the diagnosis and management of Wegener's granulomatosis at a major university hospital between 1965 and 1979. during this period there was a significant evolution of treatment methods.
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Ann Rheum Dis
January 2025
Department of Rheumatology and Clincal Immunology, Charite Universitatsmedizin Berlin, Berlin, Germany. Electronic address:
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de Coimbra, Coimbra, PRT.
Microscopic polyangiitis (MPA) is a rare, autoimmune, small-vessel vasculitis usually described with the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). It encompasses a broad spectrum of clinical features, including fatigue, weight loss, fever, arthralgia, skin lesions, and involvement of the lungs or kidneys. Ocular manifestations, however, are extremely rare.
View Article and Find Full Text PDFReumatologia
December 2024
Department of Rheumatology and Immunology, Jagiellonian University Medical College, Krakow, Poland.
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic granulomatous vasculitis. Typical symptoms include late-onset bronchial asthma and blood and tissue eosinophilia. In addition to these characteristic symptoms, EGPA can affect important organs such as the skin, kidneys, heart, sinuses, gastrointestinal tract, and nervous system.
View Article and Find Full Text PDFAME Case Rep
December 2024
The PLA Center of Respiratory and Allergic Disease Diagnosing Management, General Hospital of Northern Theater Command, Shenyang, China.
Background: Patients with asthma exhibit a significantly heightened susceptibility to eosinophilic granulomatosis with polyangiitis (EGPA) when compared to the general population. Vigilance for EGPA manifestations is crucial, especially in cases where asthma remains poorly controlled despite high-dose corticosteroid therapy or when eosinophil counts exceed 5%. The diagnosis of EGPA can be complex due to the absence of definitive biomarkers, as indicated by the American College of Rheumatology (ACR)'s 1990 classification criteria.
View Article and Find Full Text PDFDiagnostics (Basel)
January 2025
Department of Internal Medicine and Gastroenterology, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. : We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain.
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