Risk factors for relapse and long-term outcome of idiopathic retroperitoneal fibrosis

Moriconi, Diego; Giannese, Domenico; Capecchi, Riccardo; Cupisti, Adamasco; Barsotti, Simone; Morganti, Riccardo; Orsitto, E; Tavoni, Antonio; Egidi, Maria Francesca

doi:10.1007/s10157-019-01759-w

Cited by 18 publications

(21 citation statements)

References 19 publications

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“…As mentioned above, observation studies demonstrated that relapses during the observation periods were observed in approximately 15–50% of patients. 25–29 Although the suggested risk factors of relapse were inconsistent among the studies, they included higher baseline ESR, 25 a shorter course of glucocorticoid treatment, 25 ANA positivity, 27 smoking, 27 AKI, 27 and lumbar pain. 27 Reliable predictors of relapses are desired because relapse is suggested to result in a greater eGFR decline during the clinical course.…”

Section: Prognosismentioning

confidence: 99%

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Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.

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Section: Prognosismentioning

confidence: 99%

“… 27 Reliable predictors of relapses are desired because relapse is suggested to result in a greater eGFR decline during the clinical course. 27 …”

Section: Prognosismentioning

confidence: 99%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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“…Notably, recent reports of MMF and RTX treatment in RF show efficacy whether or not it is a manifestation of IgG4-RD (60,61). Some conditions, such as smoking habit, AKI at diagnosis, ANA positivity and lumbar pain, are associated to relapse, and all these factors represent a negative prognostic combination for renal outcome (62).…”

Section: Treatment and Follow-upmentioning

confidence: 99%

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

et al. 2021

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IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.

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“…5 Causes of secondary RPF include reactions to various medications, infectious organisms, malignant disease and exposure to radiation. 2,5,6 Risk factors for RPF in humans include hypertension, diabetes mellitus, neoplasia, coronary heart disease, dyslipidemia, infections, lymphoproliferative disease, cerebral vascular disease and tobacco exposure. 2,6 Renal transplant-associated RPF has also been reported in humans, and is suspected to be secondary to retroperitoneal insults such as surgical trauma, infection, urine extravasation or exposure to surgical glove talcum powder.…”

Section: Introductionmentioning

confidence: 99%

“…2,5,6 Risk factors for RPF in humans include hypertension, diabetes mellitus, neoplasia, coronary heart disease, dyslipidemia, infections, lymphoproliferative disease, cerebral vascular disease and tobacco exposure. 2,6 Renal transplant-associated RPF has also been reported in humans, and is suspected to be secondary to retroperitoneal insults such as surgical trauma, infection, urine extravasation or exposure to surgical glove talcum powder. 7,8 Additionally, acute and chronic transplant rejection episodes have been described as potential risk factors.…”

Section: Introductionmentioning

confidence: 99%

Retroperitoneal fibrosis as a postoperative complication following renal transplantation in cats

Byer

Hardie

McAnulty

2021

Journal of Feline Medicine and Surgery

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Objectives The aim of this report was to describe the clinical signs, diagnostic imaging findings, surgical management, histopathological findings, outcome and possible risk factors for cats that developed retroperitoneal fibrosis (RPF) following renal transplantation. Methods Medical records of cats that underwent renal transplantation and developed clinically significant RPF between 1995 and 2019 were reviewed. Results Eighty-one cats underwent 83 renal transplantations. Of these 81 cats, six developed clinically significant RPF. For all six cats, renal transplantation was performed using cold organ preservation solution and ureteral papilla implantation. Immunosuppression protocol included ciclosporin and prednisolone. All cats had at least one subtherapeutic trough ciclosporin level (<250 ng/ml) in the postoperative period. Cats presented with moderate-to-severe azotemia 39–210 days following renal transplantation. Abdominal ultrasonography and contrast pyelography revealed various degrees of hydroureter and hydronephrosis of the transplanted kidney. Surgical examination revealed a layer of dense fibrous tissue surrounding the transplanted kidney, ureter and bladder resulting in ureteral obstruction. Ureteral obstruction was managed by reimplantation of the proximal ureter or renal pelvis to the bladder. Histopathologic examination of the fibrous tissue and affected portion of the distal ureter revealed fibrous connective tissue with lymphoplasmacytic infiltration and perivascular inflammation suggestive of an autoimmune type reaction. Of the six cats, two died within 5 days after revision surgery, two developed signs consistent with recurrent partial ureteral obstruction (40 and 41 days after revision), one was euthanized 6 years later for an unrelated disease and one was lost to follow-up. Conclusions and relevance The incidence of RPF in this population of cats was relatively low (7%), but still represents a significant cause of morbidity and mortality. The cause of RPF remains unknown, although investigation into suboptimal immunosuppression as a potential cause for local rejection reaction is warranted.

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Risk factors for relapse and long-term outcome of idiopathic retroperitoneal fibrosis

Cited by 18 publications

References 19 publications

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

Retroperitoneal fibrosis as a postoperative complication following renal transplantation in cats

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