Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis.
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ABSTRACT:
SUBMITTER: Yamakawa H
PROVIDER: S-EPMC5548681 | biostudies-other | 2017
REPOSITORIES: biostudies-other
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Project description:Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People's Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.
| S-EPMC7891782 | biostudies-literature
Project description:Retroperitoneal fibrosis (RPF) refers to a fibro-inflammatory lesion in the retroperitoneum, often anterolateral to the aorta. Most cases are due to IgG4-related disease (IgG4-RD) or are idiopathic. RPF can lead to severe morbidity. Treatment strategies remain poorly-defined. We evaluated the efficacy and safety of rituximab (RTX) for idiopathic or IgG4-related RPF.We retrospectively reviewed the records of patients who had RPF treated with RTX. Treatment response was determined by assessing changes in both clinical features, including symptoms and laboratory measurements, as well as in the radiographic dimensions of the lesion.Twenty-six patients with IgG4-related (n?=?19) or idiopathic RPF (n?=?7) were identified. Patients without histopathological evidence of IgG4-RD on either retroperitoneal biopsies or sampling of extra-retroperitoneal organs were considered to have idiopathic RPF. Of the 26 patients, 19 (73%) received RTX without additional glucocorticoids. All 19 patients who presented with pain reported symptomatic improvement following RTX. Among 25 patients with follow-up imaging, 22 (88%) had radiologic improvement. Among 10 patients with ureteral stents and/or percutaneous nephrostomy tubes, 4 (40%) underwent successful stent or tube removal. Responses to treatment were similar among those treated with RTX monotherapy and those treated with RTX and glucocorticoids. RTX was generally well tolerated, but 3 (12%) patients experienced severe infections.In this study, RTX for RPF led to resolution of symptoms in all patients and radiographic improvement in the majority. Prospective studies of RTX for RPF are indicated.
| S-EPMC6211888 | biostudies-other
Project description:A 51-year-old woman visited our clinic for orthopnea, chest discomfort, and facial and leg edema. Acute kidney injury and pleural effusion were diagnosed and she received hemodialysis. This is a rare case of IgG4-related disease diagnosed by renal biopsy in the patient with retroperitoneal fibrosis and severe acute kidney injury, which progressed rapidly and required hemodialysis without markedly elevated serum IgG4 level and urinary tract obstruction. Immunostaining of a kidney biopsy revealed IgG4-related renal disease and magnetic resonance imaging showed retroperitoneal fibrosis in the paraspinal and presacral areas. After 3 days of glucocorticoid treatment, renal function and bilateral pleural effusion were restored. Another 2 months of glucocorticoid treatment decreased the extent of retroperitoneal fibrosis on follow-up abdominal computed tomography. IgG4-related disease is a recently recognized fibro-inflammatory condition with a dense lymphoplasmacytic infiltration of IgG4-positive plasma cells. The kidney and retroperitoneum are the frequently associated organs in IgG4-related disease. This case shows that IgG4-related disease can involve the kidney and/or other organs and be diagnosed by kidney biopsy.
| S-EPMC7215037 | biostudies-literature
Project description:Introduction and importanceRetroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and inflammation. We present the case of a 40-years-old gentleman with RPF whose presenting complaints were bilateral flank pain and weight loss and was found to have IgG4 related disease. To the best of our knowledge, IgG4-related disease with isolated retroperitoneal involvement is a very rare occurrence.Case presentationThe diagnosis of IgG4-Related retroperitoneal fibrosis was made based on clinical, radiological and histopathological criteria. Imaging revealed isolated retroperitoneal involvement and the patient was started on oral steroids with a good clinical response after ten days. Repeated imaging months later showed significant regression in the fibrosis.Clinical discussionRPF can occur due to many etiologies and is categorized to idiopathic and secondary. Factors associated with secondary RPF include medications, autoimmune disease, malignancy and IgG4 related disease. Almost all the reported cases of IgG4 related disease had evidence of multifocal involvement such as the pancreas, aorta and kidneys. Our patient was diagnosed with isolated RPF due to IgG4 disease. He met all the suggested diagnostic criteria, was started on oral steroids and had an excellent clinical outcome.ConclusionIgG4 related disease can present with isolated retroperitoneal fibrosis without involvement of other organ systems. The diagnosis should be based on specific criteria. Treatment with corticosteroids can lead to remission both clinically and radiographically.
| S-EPMC7773681 | biostudies-literature
Project description:IntroductionIgG4-related disease (IgG4-RD) is a relapsing multisystem fibro-inflammatory disease, which may involve the kidney (IgG4-related kidney disease [IgG4-RKD]) and retroperitoneum (IgG4-related retroperitoneal fibrosis [IgG4-RPF]). The aim of this study was to describe IgG4-RKD and IgG4-RPF in the United Kingdom.MethodsWe conducted a retrospective observational study of patients with IgG4-RKD and IgG4-RPF in a multicenter IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings, treatment, and patient outcomes.ResultsOf 154 patients with IgG4-RD, 14 (9.1%) had IgG4-RKD, 10 (6.5%) had IgG4-RPF, and 4 (2.6%) had both. Patients were aged 58.2 ± 14.2 years, and 26 (92.9%) were male. Creatinine at presentation was worse in those with intrinsic renal disease (229 ?mol/l vs. 110 ?mol/l; P = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12-36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD).ConclusionIgG4-RKD and IgG4-RPF represent major organ manifestations of IgG4-RD, and should be identified early with prompt treatment to prevent progression to ESRD.
| S-EPMC6308386 | biostudies-literature
Project description:BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease that leads to hydronephrosis and renal failure. In a case-control study, we have recently shown that asbestos exposure was the most important risk factor for RPF in the Finnish population. The aim of this study was to evaluate the relation of asbestos exposure to radiologically confirmed lung and pleural fibrosis among patients with RPF. METHODS: Chest high-resolution computed tomography (HRCT) was performed on 16 unexposed and 22 asbestos-exposed RPF patients and 18 asbestos-exposed controls. Parietal pleural plaques (PPP), diffuse pleural thickening (DPT) and parenchymal fibrosis were scored separately. RESULTS: Most of the asbestos-exposed RPF patients and half of the asbestos-exposed controls had bilateral PPP, but only a few had lung fibrosis. Minor bilateral plaques were detected in two of the unexposed RPF patients, and none had lung fibrosis. DPT was most frequent and thickest in the asbestos-exposed RPF-patients. In three asbestos-exposed patients with RPF we observed exceptionally large pleural masses that were located anteriorly in the pleural space and continued into the anterior mediastinum.Asbestos exposure was associated with DPT in comparisons between RPF patients and controls (case-control analysis) as well as among RPF patients (case-case analysis). CONCLUSION: The most distinctive feature of the asbestos-exposed RPF patients was a thick DPT. An asbestos-related pleural finding was common in the asbestos-exposed RPF patients, but only a few of these patients had parenchymal lung fibrosis. RPF without asbestos exposure was not associated with pleural or lung fibrosis. The findings suggest a shared etiology for RPF and pleural fibrosis and furthermore possibly a similar pathogenetic mechanisms.
| S-EPMC2596089 | biostudies-other
Project description:Transcriptomic changes in longitudinal biopsies from a melanoma patient with spontaneous remission
| PRJEB35802 | ENA
Project description:Retroperitoneal fibrosis (RPF) is a very rare disease that is even more rare in the pediatric population. Even less common are idiopathic pediatric cases of retroperitoneal fibrosis, with a majority of reported pediatric retroperitoneal fibrosis cases being associated with secondary etiologies. We present an 11-year-old Caucasian female that was diagnosed with idiopathic retroperitoneal fibrosis using magnetic resonance imaging (MRI) to work-up severe bilateral hydronephrosis that was identified with retroperitoneal ultrasound. Given the uncommon nature of this serious condition, we present this case to illustrate the importance for physicians to include retroperitoneal fibrosis in the differential diagnosis of a pediatric patient presenting with obstructive urinary findings and understand the utility of using MRI to diagnosis and monitor this disease.
| S-EPMC6357545 | biostudies-literature
Project description: Not available
| S-EPMC8318656 | biostudies-literature
Project description:BACKGROUND:The aim of this study was to identify fibrosis-related serological surrogate outcome measures in patients with immunoglobulin G4-related disease (IgG4-RD). METHODS:This was a clinical observational study of 72 patients with untreated IgG4-RD from four institutions in Japan. The serum concentrations of growth differentiation factor 15 (GDF-15), CCL2, hyaluronic acid (HA), amino-terminal propeptide of type III procollagen (PIIINP), and tissue inhibitor of metalloproteinases 1 (TIMP-1) were measured by enzyme-linked immunosorbent assays. The enhanced liver fibrosis (ELF) score was calculated from the TIMP-1, PIIINP, and HA values. We evaluated associations between the values of these biomarkers and laboratory data, the IgG4-RD responder index (IgG4-RD RI) score, and organ involvements. RESULTS:Compared with the 44 healthy controls, the patients with IgG4-RD showed significantly elevated serum concentrations of GDF-15, MCP-1, HA, PIIINP, and TIMP-1 and ELF scores. The patients' serum concentrations of GDF-15, CCL2, HA, and TIMP-1 (but not PIIINP) were positively correlated with each other. Among them, serum GDF-15 most efficiently distinguished patients with IgG4-RD from healthy controls. Serum GDF-15 was not associated with the IgG4-RD RI score or the number of organ involvements but was independently associated with the presence of retroperitoneal fibrosis and with parotid gland involvement. CONCLUSIONS:We observed increased serological surrogate outcome measures of fibrosis in IgG4-RD. GDF-15 may precisely reflect the fibrotic degree in patients with IgG4-RD.
| S-EPMC6293522 | biostudies-literature