PMID- 38473038 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240315 IS - 2075-4418 (Print) IS - 2075-4418 (Electronic) IS - 2075-4418 (Linking) VI - 14 IP - 5 DP - 2024 Mar 6 TI - Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review. LID - 10.3390/diagnostics14050566 [doi] LID - 566 AB - BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. METHODS: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. RESULTS: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. CONCLUSIONS: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up. FAU - Iorio, Luca AU - Iorio L AD - Rheumatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Pizzi, Marco AU - Pizzi M AUID- ORCID: 0000-0003-4006-7317 AD - Surgical Pathology and Cytopathology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Cecchin, Diego AU - Cecchin D AUID- ORCID: 0000-0001-7956-1924 AD - Nuclear Medicine Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Davanzo, Federica AU - Davanzo F AD - Rheumatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Ghirardello, Anna AU - Ghirardello A AD - Rheumatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Dei Tos, Angelo Paolo AU - Dei Tos AP AD - Surgical Pathology and Cytopathology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - Rheumatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. FAU - Padoan, Roberto AU - Padoan R AUID- ORCID: 0000-0002-2356-375X AD - Rheumatology Unit, Department of Medicine DIMED, University of Padua, 35121 Padua, Italy. LA - eng PT - Journal Article PT - Review DEP - 20240306 PL - Switzerland TA - Diagnostics (Basel) JT - Diagnostics (Basel, Switzerland) JID - 101658402 PMC - PMC10930867 OTO - NOTNLM OT - ANCA vasculitis OT - Wegener granulomatosis OT - granuloma OT - granulomatosis with polyangiitis OT - pseudotumour OT - renal masses OT - renal tumour-like lesions COIS- The authors declare no financial support or potential conflicts of interest in this study. EDAT- 2024/03/13 06:47 MHDA- 2024/03/13 06:48 PMCR- 2024/03/06 CRDT- 2024/03/13 01:17 PHST- 2024/02/06 00:00 [received] PHST- 2024/03/03 00:00 [revised] PHST- 2024/03/04 00:00 [accepted] PHST- 2024/03/13 06:48 [medline] PHST- 2024/03/13 06:47 [pubmed] PHST- 2024/03/13 01:17 [entrez] PHST- 2024/03/06 00:00 [pmc-release] AID - diagnostics14050566 [pii] AID - diagnostics-14-00566 [pii] AID - 10.3390/diagnostics14050566 [doi] PST - epublish SO - Diagnostics (Basel). 2024 Mar 6;14(5):566. doi: 10.3390/diagnostics14050566.