The clinical and analytical manifestations and the evaluation of 6 patients diagnosed with Wegener's granulomatosis (WG) in the Germans Trias i Pujol Hospital between 1986-1990 are described. The most frequent clinical manifestation found were general and respiratory symptoms. Three patients presented an alteration in renal function and urinary sedimentation. Antibodies against the cytoplasm of the neutrophils (ANCA) detected by indirect immunofluorescence (IIF) were positive in 4 patients. All the patients underwent initial complete remission with immunodepressor treatment and one presented relapse of the disease at 19 months of diagnosis. Complications were: opportunistic infections obliging discontinuation of treatment in one patient, distal necrosis of the fingers requiring amputation in another patient and massive atelectasis of the right lung requiring pneumonectomy in a third patient.
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Reumatologia
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.
View Article and Find Full Text PDFMod Rheumatol Case Rep
January 2025
Department of Rheumatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotising vasculitis affecting small vessels accompanied by eosinophilic inflammation. Biological therapies, particularly anti-interleukin-5 (IL-5) monoclonal antibodies, have been shown to be effective in treating refractory EGPA. Mepolizumab, an anti-IL-5 monoclonal antibody, has been approved in Japan for the treatment of EGPA and has a significant glucocorticoid-sparing effect.
View Article and Find Full Text PDFObjective: The ADVOCATE trial demonstrated that treatment of active granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) with avacopan was noninferior in achieving remission at week 26 and superior for sustained remission at week 52 compared with a prednisone taper. This analysis of ADVOCATE evaluated the efficacy and safety of avacopan in patients with ear, nose, throat (ENT), or lung manifestations.
Methods: This post hoc analysis included patients enrolled in ADVOCATE with ENT or lung manifestations at baseline.
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