Work

Mass‐Forming Variants in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Diagnostic Complexities in Granulomatous Disease. A Case Report

Year: 2025

Type: article

Abstract: A middle-aged woman presented with a granulomatous breast lesion in 2018. By 2021, antibiotic-resistant pneumonia led to the discovery of granulomatous inflammation in the lung and thyroid. Initially misdiagnosed as Erdheim-Chester disease (ECD), she was treated with interferon without success. Histopathology later ruled out ECD, suggesting an unspecified granulomatous disease, with granulomatosis with polyangiitis (GPA) initially excluded due to negative antineutrophil cytoplasmic antibodies (ANCAs) and foamy histiocytes. In 2023, repeated lung biopsy and PR3-ANCA positivity led to a revised diagnosis of mass-forming GPA. Rituximab therapy resulted in remission. This case highlights the diagnostic complexity of GPA with atypical histopathological features.

Source: ACR Open Rheumatology

DOI: 10.1002/acr2.70038

Authors: Benedetta Fazzi, Elena Treppo, Simone Longhino, Maria Pillon, Luca Quartuccio

Institution: University of Udine

Language: English

Topic: Vasculitis and related conditions

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,550