The use of propylthiouracil (PTU) has been associated with various forms of vasculitis. We herein describe the case of a patient with Grave's disease who, after years of PTU therapy, developed a necrotizing vasculitis with anti-serine protease-3 antibodies. Despite treatment with corticosteroids and cyclophosphamide, the patient died of intra-alveolar hemorrhage secondary to her vasculitis. Based on the vessel size involved, the organ distribution of pathologic findings, and lack of granulomas, autopsy findings were felt to be more consistent with microscopic polyangiitis (MPA) than with her original clinical diagnosis of Wegener's granulomatosis. Her case satisfied both clinical and pathologic criteria for MPA. An MPA diagnosis is important to consider in similar clinical presentations because therapy may just need to be early withdrawal of an inciting drug, such as PTU, and the initiation of corticosteroids without cytotoxic therapy.
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http://dx.doi.org/10.1097/00124743-200106000-00007 | DOI Listing |
Cureus
January 2025
Adult Critical Care, St George's University Hospitals NHS Foundation Trust, London, GBR.
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are a group of rare diseases that cause inflammation of small to medium vessels. They comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). As the signs and symptoms are varied, the diagnosis of vasculitis is complex and challenging; however, there are type-specific clinical presentations that can be factored into the existing classification criteria.
View Article and Find Full Text PDFCureus
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 PDFRadiol 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 PDFHum 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.
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