Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease that develops with necrotizing granulomatous inflammation and is characterized by eosinophilia, asthma, and small vessel vasculitis. We report the case of a 74-year-old woman with a history of asthma, admitted to the Emergency Room with fever, headache, general malaise, weight loss and night sweats with one-month evolution, previously medicated with antibiotics without improvement. She presented with sinus palpation tenderness and lower leg bilateral sensitivity impairment. Laboratory tests showed neutrophilia and eosinophilia, normocytic anemia and elevated erythrocyte sedimentation rate and C-reactive protein. A computed tomography revealed sphenoid and maxillary sinusitis. Blood cultures and lumbar puncture were innocent. An extended autoimmune panel exposed a strong positive perinuclear anti-neutrophil cytoplasmic antibody - myeloperoxidase (pANCA-MPO). Sinus biopsy showed tissue infiltration by eosinophils, confirming EGPA. Corticosteroid (1 mg/kg/day) treatment was started with gradual improvement. Six months later there were no signs of active disease under prednisolone 10 mg and azathioprine 50 mg/day. This case highlights that refractory sinusitis in the presence of constitutional syndrome and peripheral eosinophilia should alert clinicians to the possibility of EGPA, particularly in patients with late-onset asthma.
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http://dx.doi.org/10.7759/cureus.35512 | DOI Listing |
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 PDFAm J Hum Genet
January 2025
Institute of Human Genetics, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany; Institute of Human Genetics, University of Regensburg, 93053 Regensburg, Germany; Institute of Clinical Human Genetics, University Hospital Regensburg, 93053 Regensburg, Germany. Electronic address:
BCL11B is a Cys2-His2 zinc-finger (C2H2-ZnF) domain-containing, DNA-binding, transcription factor with established roles in the development of various organs and tissues, primarily the immune and nervous systems. BCL11B germline variants have been associated with a variety of developmental syndromes. However, genotype-phenotype correlations along with pathophysiologic mechanisms of selected variants mostly remain elusive.
View Article and Find Full Text PDFClin Transl Allergy
December 2024
Department of Internal Medicine and Clinical Nutrition, Krefting Research Centre, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Although asthma is more frequently diagnosed in childhood, a substantial proportion of cases manifests in adulthood. Nonetheless, few studies have comprehensively examined asthma incidence across different ages, genders, and asthma phenotypes. We conducted a retrospective evaluation of asthma incidence from birth to late adulthood, stratified by age, gender, and the presence or absence of allergies.
View Article and Find Full Text PDFPediatr Allergy Immunol
December 2024
National Heart and Lung Institute, Imperial College London, London, UK.
Background: Wheezing is common in early life, but most children stop wheezing by school age. However, the prediction of course of wheezing through childhood is difficult.
Objective: To investigate whether urinary EPX (a marker of eosinophil activation) in children at age 3 years may be useful for the prediction of wheeze persistence and future asthma diagnosis.
J Asthma
December 2024
Department of Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, SP, Brazil.
Background: Asthma in the elderly is usually considered homogeneous and non-atopic.
Objective: To compare clinical, functional and immunological features between elderly asthmatics with long-standing asthma (LSA) and those with late-onset asthma (LOA).
Methods: Eighty-two asthmatics older than 64 were included into LSA (asthma onset before age 40; = 46) and LOA (asthma onset from 40 years of age on; = 36) groups.
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