Microscopic polyangiitis (MPA) is an autoimmune systemic vasculitis of small vessels. The condition has been best characterized in older adults and little is known of the natural history of this disease in children and adolescents. In this report, a case of an adolescent presenting with symptomatic anemia and syncopal episodes is described. An extensive evaluation ultimately led to the diagnosis of MPA. The unique findings in this case and review of the literature are presented, outlining the variable clinical presentations and challenge of diagnosing this condition in pediatric patients.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s00296-009-1270-3 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Retinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis.
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 PDFPathology of Pulmonary Vascular Disease with Radiologic Correlation.
Radiol Clin North Am
March 2025
Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI, USA. Electronic address:
Pulmonary hypertensive changes are commonly seen by the surgical pathologist, but the majority represents secondary changes due to some process extrinsic to the lung. Some primary, or idiopathic, vascular diseases result in unique pathologic changes including the plexiform lesion and venous hypertensive changes. Thromboembolic disease also shows unique pathologic features.
View Article and Find Full Text PDFSTAT4 gene polymorphism may be associated with microscopic polyangiitis susceptibility in a Chinese Guangxi population: A case-control analysis based on propensity score matching.
Hum Immunol
January 2025
The Second Affiliated Hospital of Guangxi Medical University, Department of Nephrology, Nanning, Guangxi 530021, China. Electronic address:
Background: Microscopic polyangiitis (MPA) is a severe multisystem autoimmune disease featured by small-vessel vasculitis with few or no immune complex, also has a significant genetic predisposition. Growing evidence has confirmed that STAT4 gene is tightly associated with multiple autoimmune diseases, but its contribution to MPA onset is still elusive.
Objective: The aim was to investigated the association between STAT4 gene polymorphisms (rs7572482, rs7574865 and rs12991409) and MPA susceptibility in a Guangxi population of China.
Objective: 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.
Aneurysmal rupture in microscopic polyangiitis: a case-based review.
Clin Rheumatol
January 2025
Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Hokkaido, Japan.
Microscopic polyangiitis (MPA) affects small and medium vessel, which sometimes leads to arterial aneurysms. In English database, only 15 reports refer to ruptured aneurysms in MPA. We experienced a fatal case with MPA who developed multiple visceral aneurysms, resulting in rupture of the hepatic aneurysm.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!