The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy

Abstract

The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. In conclusion, we believe this case is extremely rare and clinically suggestive, and that these findings can be applied to a future phenotype-tailored treatment strategy for EGPA.

Eosinophilic granulomatosis polyangiitis (EGPA) complicated with periaortitis, precipitating role of dupilumab? A case report a review of the literature

“…1 However, although rare, large Vasculitis Vasculitis Vasculitis vessels involvement, including aortitis and periaortitis, has been described in AAVs, mainly in GPA and MPA, 2 3 and is usually non-stenosing, consisting of perivascular soft tissue masses, aneurysms, dissection and rupture. 3 With regard to EGPA, to our knowledge, only four cases of periaortitis have been reported in the literature, [4][5][6][7] and are summarised in table 1. The suggested underlying pathophysiology is a vasa-vasoritis of the aorta.…”

“…2 3 With regard to EGPA, only four cases of periaortitis have been reported in the literature. [4][5][6][7] Dupilumab is a human monoclonal IgG 4 antibody that targets the interleukin-4 receptor subunit α of the IL-4 and the IL-13 receptors. It is used in the treatment of atopic dermatitis, uncontrolled asthma with hypereosinophilia and chronic sinusitis with nasal polyposis.…”

ANCA-Negative Vasculitis in Eosinophilic Granulomatosis with Polyangiitis Complicated with Membranous Nephropathy: A Case Report and Brief Literature Review

“…We compared the clinicopathological features described in the six previous case reports of EGPA complicated with MN, including both ANCA-positive [4][5][6] and negative cases [7,8], with our case (Table 1). We found that those with rapidly progressing renal deficiency demonstrated NCGN histologically, except for our case, and that renal function was recovered in almost all cases.…”

“…However, it was recently reported that 10% of EGPA was complicated with non-NCGN, such as membranous nephropathy (MN), of which most were ANCA negative [3]. Furthermore, MN complicated in EGPA has been previously reported for both ANCA-positive [4][5][6] and -negative [7,8] cases. Although ANCA is recognized as a useful marker to differentiate the heterogeneous clinical spectrum of EGPA [9], it is difficult to clarify all clinical findings from ANCA results given the aforementioned exceptions.…”