ArticleCureus2025
Curious Coexistence of Perivascula Epitheloid Cell Tumour (PEComa) Systemic Sclerosis-Associated Interstitial Lung Disease and Biopsy-Proven Lymphangioleiomyomatosis in a Middle-Aged Female: A Case Report.
Article in Cureus, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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5 authors.
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Abstract
We describe a rare case of an unusual coexistence of systemic sclerosis-associated interstitial lung disease (SSc-ILD), biopsy-proven lymphangioleiomyomatosis (LAM), and perivascular epithelioid cell tumor (PEComa) in a 41-year-old woman. Initial presentation with retroperitoneal angiomyolipoma preceded the later development of diffuse cystic lung disease and features of systemic sclerosis. Histopathology confirmed both LAM and interstitial pneumonitis. The patient improved on mycophenolate for SSc-ILD, with sirolimus planned for LAM. This unique case highlights the diagnostic challenges and the importance of multidisciplinary management in overlapping rare pulmonary and systemic diseases.
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