Retroperitoneal fibrosis probably related to IgG4 disease and antiphospholipid positivity
DOI:
https://doi.org/10.32818/reccmi.a11n1a9Keywords:
IgG4 disease, retroperitoneal fibrosis, antiphospholipid syndromeAbstract
We present a case of retroperitoneal fibrosis (RPF) likely related to IgG4-related disease (IgG4-RD), associated with antiphospholipid
positivity not conclusive of antiphospholipid syndrome (APS). A 57-year-old woman with a history of
ischemic heart disease, psoriasis, and hypothyroidism presented with intermittent claudication and chronic low back
pain with signs of peripheral artery disease. CT angiography showed infrarenal aortic thrombosis and periaortitis,
and PET-CT was consistent with active RPF. Furthermore, autoimmunity testing showed a positive lupus anticoagulant
and weakly positive anticardiolipin IgM. Active RPF was likely associated with IgG4-RD, and the antiphospholipid
positivity was suggestive of APS. She received treatment with corticosteroids and rituximab with a good outcome.
This case illustrates the diagnostic complexity of systemic diseases and their associated vascular risk.
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Copyright (c) 2026 Sara Jiménez-González, Víctor José Vega-Rodríguez, Fabiola Campo-Lobato, Isabel González-Fernández, Pilar Sánchez-Latasa
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