(M)
n, number of subjects ; PKD+, Patients with polycystic kidney disease ; PKD-, Patients without polycystic kidney disease ; M, Months (median) ; CIS, Charlson Index Score
*, p<0.05
Clinical characteristics of patients included are summarized in Table 3 . Patients did not differ between the PKD and non PKD groups in terms of hypertension, access to kidney transplantation and transfer to hemodialysis. In one of the 9 studies [ 14 ], Charlson index was found to be higher in non PKD patients. Most of the patients were treated by continuous ambulatory peritoneal dialysis while only Yang et al. reported a higher prevalence of automated peritoneal dialysis in the PKD group [ 12 ].
Outcome | |||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Study, Year | Hypertension (%) | CIS | APD (%) | Kidney transplantation (%) | Transfer to hemodialysis (%) | ||||||||||
PKD+ | PKD- | PKD+ | PKD- | PKD+ | PKD- | PKD+ | PKD- | PKD+ | PKD- | ||||||
83 | 83.9 | 0.9 | - | - | - | 45.3 | 44.4 | 0.9 | 26.4 | 18.6 | 0.04 | 18.9 | 21.4 | 0.5 | |
14.7 | 23 | 0.21 | 4.27 (±1.58) | 5.27 (±2.5) | <0.001 | 43.4 | 33.7 | 0.1 | 47.2 | 30.7 | 0.004 | 17 | 20.3 | NS | |
71.2 | 73.1 | 0.66 | 2.9 (±1.4) | 3.0 (±1.4) | 0.5 | 46.8 | 37.6 | 0.03 | 9.4 | 9.8 | 0.87 | 24.5 | 22.5 | 0.64 | |
- | - | - | 3 (3–5) | 6 (3–7) | - | 54.9 | 34.5 | - | 52 | 30 | - | 32 | 30 | - | |
95.2 | 91.7 | 0.5 | 4.6 (±1.6) | 4.1 (±1.8) | 0.1 | - | - | - | 9.5 | 11.9 | - | 9.5 | 11.9 | - | |
- | - | - | - | - | - | 18.5 | 17.8 | - | 39 | 37 | NS | 30 | 25 | NS | |
100 | 93.3 | 0.152 | 3.3 (±1.1) | 3.1 (±1.0) | 0.351 | - | - | - | 16.7 | 16.7 | 1 | 16.7 | 20 | 0.506 | |
- | - | - | - | - | 0 | 0 | - | 60 | 41.2 | - | 25 | 29.4 | - | ||
- | - | - | - | - | - | 42.4 | 47 | 0.36 | 30.3 | 16.6 | - | 30.3 | 28.8 | - |
PKD+, Patients with Polycystic Kidney Disease; PKD-, Patients without Polycystic Kidney Disease; CIS, Charlson Index Score; APD, Automated Peritoneal Dialysis
Biological characteristics of patients included are shown in Table 4 . There was no difference in serum albumin level and dialysis adequacy in term of total weekly Kt/V urea between PKD and non PKD patients. In two studies [ 14 , 17 ], hemoglobin level was higher in PKD patients.
Study, Year | Hemoglobinemia [g/dL] | Albuminemia [g/L] | Kt/V | ||||||
---|---|---|---|---|---|---|---|---|---|
PKD+ | PKD- | PKD+ | PKD- | PKD+ | PKD- | ||||
11.4 (±1.5) | 11.2 (±1.7) | 0.2 | 37.3 (±6.0) | 36.0 (±7.3) | 0.1 | 2.22 (±0.59) | 2.33 (±0.71) | 0.2 | |
12.63 (±1.44) | 11.96 (±1.49) | 0.001 | - | - | - | 2.68 (±0.65) | 2.52 (±0.80) | 0.1 | |
- | - | - | - | - | - | - | - | - | |
- | - | - | - | - | - | - | - | - | |
10.4 (±1.7) | 9.0 (±1.3) | <0.001 | 32.0 (±5.9) | 33.0 (±5.0) | 0.4 | 1.93 (±0.39) | 1.99 (±0.37) | 0.4 | |
11.0 (±1.5) | 10.6 (±1.9) | NS | 39.8 (±3.9) | 38.1 (±4.9) | NS | 2.3 (±0.50) | 2.1 (±0.4) | NS | |
8.1 (±1.6) | 8.0 (±1.5) | 0.957 | 41.0 (±5.2) | 39.0 (±6.4) | 0.182 | 2.11 (±0.53) | 2.08 (±0.56) | 0.328 | |
- | - | - | - | - | - | - | - | - | |
10.7 (±2.0) | 10.3 (±2.0) | 0.38 | 38.6 (±5.1) | 35.6 (±6.6) | 0.03 | 2.15 (±0.40) | 2.03 (±0.60) | 0.47 |
PKD+, Patients with Polycystic Kidney Disease ; PKD-, Patients without Polycystic Kidney Disease; Kt/V, Total weekly Kt/V urea
There were 5 studies that reported hazard ratios for PKD and non PKD patient survival. 6,378 patients from Europe and China were included in the meta-analysis. PKD status was associated with a better global survival with a Hazard Ratio of 0.70 [95% CI, 0.54–0.92] ( Fig 2 ).
HR, Hazard ratio ; CI, Confidence interval .
Seven studies reported hazard ratios for PD technique survival in both groups, which included a total of 7,046 patients from Europe, China and Taiwan. There was no difference between PKD and non PKD groups in terms of PD technique survival (HR = 0.98 [95% CI, 0.83–1.16]) as shown in Fig 3 .
Seven studies reported odds ratios for the incidence of infectious peritonitis episodes in PKD and non PKD patients treated by PD. 6,767 patients from Europe, China, Taiwan and Turkey were included in the meta-analysis ( Fig 4 ). There was no statistical difference in occurrence of infectious peritonitis between PKD and non PKD patients (OR = 0.86 [95% CI, 0.66–1.12]) as shown in Fig 4 .
OR, Odds ratio ; CI, Confidence interval .
There were 7 studies that reported odds ratios for the incidence of abdominal wall hernias in PKD and non PKD patients treated by PD, including a total of 2,923 patients from Europe, China, Taiwan and Turkey ( Fig 5 ). PKD patients were found to have an increased risk of abdominal hernia with an Odds Ratio of 2.28 [95% CI, 1.26–4.12] as shown in Fig 5 .
Because the large weight of the study by Lobbedez et al. among the included studies might affect the entire analysis, we performed sensitivity analysis to check the stability of the previous results without including the study by Lobbedez et al. (except for abdominal wall hernias because this study was not included in analysis for this outcome). Results are summarized on Table 5 . Hazard ratios and Odds ratios showed a great stability even in the absence of the study by Lobbedez et al. The only observed change in the absence of the study by Lobbedez et al. was the absence of statistical relevance in the analysis of overall survival.
Hazard ratio or Odds Ratio | Confidence interval | Hazard ratio or Odds Ratio w/o Lobbedez et al. | Confidence interval w/o Lobbedez et al. | |
---|---|---|---|---|
HR = 0.70 | 0.54–0.92 | HR = 0.74 | 0.5–1.09 | |
HR = 0.98 | 0.83–1.16 | HR = 1.00 | 0.79–1.27 | |
OR = 0.86 | 0.66–1.12 | OR = 0.87 | 0.59–1.28 |
w/o, without ; HR, Hazard ratio ; OR, Odds ratio
To our knowledge this is the first meta-analysis designed to study the outcome of PKD patients on PD. We found that compared to non PKD patients, PKD patients on PD had i) better global survival, ii) no difference in PD technique survival, iii) no difference in peritonitis rate but increased risk of abdominal hernias. Our results should reassure nephrologists managing PKD patients regarding the choice of PD as renal replacement therapy.
We found that PKD patients had better survival compared with non PKD patients. This difference could be explained by lower comorbidities reported in PKD patients: younger age [ 14 , 20 ], lower Charlson index [ 14 ] and higher hemoglobinemia [ 14 , 17 ]. On the other hand, patients with this hereditary disease are usually diagnosed earlier and monitored with a better nephrology care and a planned access to PD in optimized conditions. Only one study reports this factor but it could certainly explain at least in part the good clinical outcome for PD group in other studies [ 14 ].In addition, PKD patients have less comorbidities and may therefore have better outcome and a relatively lower risk of death or transfer to hemodialysis because of a higher competing risk of being transplanted ( Table 3 ). Lastly, in one study, diabetes mellitus was found to be the only predictor of all-cause mortality independently from PKD status [ 13 ].
Furthermore, the meta-analysis strategy allowed us to include 7,046 patients for the analysis of PD technique survival. PD technique survival did not differ according to PKD status. Moreover, there were no statistical difference between PKD and non PKD patients in terms of access to kidney transplantation or transfer to hemodialysis. Koc et al. reported that causes of death and transfer to hemodialysis were not different between PKD and non PKD patients (p = 0.35 and 0.36, respectively) [ 16 ]. In the study of Kumar et al, in multivariate analysis [ 18 ], hypoalbuminemia at initiation of PD was found to be the main risk factor for PD therapy cessation independently from PKD status.
Previous reports have suggested an increased incidence of mechanical complications in PKD patients treated with PD, such as abdominal leak or hernia [ 5 , 12 ]. In these studies, abdominal hernias were not associated with an increased risk of PD discontinuation. In the present meta-analysis, we confirm that episodes of abdominal hernia appeared statistically more often in PKD patients treated with PD. However, abdominal wall complications have been found to be more frequent in PKD patients at all stages of kidney disease including before end stage renal disease. As a consequence, it is likely that hernias may not be directly related to increased intraperitoneal pressure but may be related to collagen defects and thus be observed in PKD patients treated with other renal replacement therapies [ 21 ]. Increased risk of abdominal hernias was not associated with a decrease in technique survival. This suggests that for these patients, the therapeutic intervention such as reducing intraabdominal pressure (reduced volume infused of peritoneal solution or using automated peritoneal dialysis) is successful for maintaining them on PD therapy. In addition, studies included in this meta-analysis did not show any difference in term of fluid leak episode incidence [ 13 , 14 , 18 , 20 ].
Some reports indicate that PKD may be associated with an increased risk of diverticulitis, leading to greater risk of peritoneal infection in patients treated with PD [ 5 ]. In the present meta-analysis, including 6,767 patients, we found that PKD was not associated with a higher risk of occurrence of peritoneal infection. Furthermore, incidence of peritonitis episode requiring catheter removal did not differ between groups in Kumar et al. and Xie et al. (25% in PKD group vs 21% in non PKD group and 6,7% in PKD group vs 3,3% in non PKD group respectively) [ 18 , 19 ]. Staphylococcus spp was the main causative micro-organism in both groups while gram-negative organisms incidence did not differ between PKD and non PKD patients [ 14 , 15 , 17 , 18 , 19 ].
PD treatment offers numerous advantages [ 22 , 23 ], however only 7 to 10% of end-stage renal disease patients are treated with PD [ 2 ]. This reflects, at least in part, the clinicians’ fear of technical failure [ 24 ]. PKD patients are probably more concerned by this issue, because of the common misconception that these patients will develop infectious and mechanical complications if treated with PD, due to increased intraperitoneal pressure and a higher incidence of diverticulitis. Our results suggest that PD is a safe renal replacement modality for PKD patients. To the best of our knowledge, there is no available study designed to assess the impact of different PD modalities (continuous ambulatory peritoneal dialysis or automated peritoneal dialysis) on PKD patients’ outcomes.
First limitation of our study is related to the small number of publications on this subject. Additionally, all the studies included in the meta-analysis are retrospective or registry based studies. Indeed, there is no randomized clinical trial available on this subject. Therefore, a potential selection bias may limit the relevance of our conclusions for the entire PKD population. However, we collected all the study available (ie 9) and a total of 7,197 patients across the 9 studies (n = 2,923 to 7,046 patients for each outcome). This was a sufficient prerequisite to perform a meta-analysis. Another limitation is related to the relative importance of the study by Lobbedez et al. among the included studies due to the large number of patients included (n = 4162) accounting for almost half of the total population and Furthermore the design of the Lobbedez study, was different it was the only one including survival analysis after exclusion of the diabetic patients. However our results show strong stability when analyzed with or without Lobbedez’s study.
In conclusion, this meta-analysis showed that PKD patients treated with PD seem to have an increased survival and an increased rate of abdominal hernia, without any impact on PD technique survival. There was no statistical difference in peritonitis rate between PKD and no PKD patients. Therefore, our data suggest that peritoneal dialysis is a safe modality to treat end stage renal disease of PKD patients and should be offered to these patients. However, properly designed controlled studies are needed to determine whether all PKD patients are eligible for PD or whether some specific criteria should be determined. A particular attention should be given to the impact of total kidney volume and liver size in the feasibility of PD.
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Objective: To perform a systematic literature review on definitions and instruments used to measure remission, relapse, and disease activity in polymyalgia rheumatica (PMR), to inform an OMERACT project to endorse instruments for these outcomes.
Methods: A search of Pubmed/MEDLINE, EMBASE, CINAHL, Cochrane, and Epistemonikos was performed May 2021 and updated August 2023. Qualitative and quantitative studies published in English were included if they recruited people with isolated PMR regardless of treatment. Study selection and data extraction was performed independently by two investigators and disagreement was resolved through discussion. Data extracted encompassed definitions of disease activity, remission and relapse, and details regarding the instruments used to measure these outcomes.
Results: From the 5,718 records, we included 26 articles on disease activity, 36 on remission, and 53 on relapse; 64 studies were observational and 15interventional, and none used qualitative methods. Some heterogeneity was found regarding definitions and instruments encompassing the domains pain, stiffness, fatigue, laboratory markers (mainly acute phase reactants), and patient and physician global assessment of disease activity. However, instruments for clinical signs were often poorly described. Whilst measurement properties of the polymyalgia rheumatica activity score (PMR-AS) have been assessed, data to support its use for measurement of remission and relapse is limited.
Conclusion: Remission, relapse, and disease activity have been defined heterogeneously in clinical studies. Instruments to measure these disease states still need to be validated. Qualitative research is needed to better understand the concepts of remission and relapse in PMR.
Review registration: PROSPERO identification: CRD42021255925.
Keywords: Polymyalgia rheumatica; disease activity; outcome measurement; relapse; remission; systematic literature review.
© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].
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IMAGES
COMMENTS
Polycystic kidney disease (PKD) is a multiorgan disorder resulting in fluid-filled cyst formation in the kidneys and other systems. The replacement of kidney parenchyma with an ever-increasing volume of cysts eventually leads to kidney failure. Recently, increased understanding of the pathophysiology of PKD and genetic advances have led to new ...
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of renal failure worldwide. ADPKD is a multisystem and progressive inherited disorder with renal cyst formation, kidney enlargement, and extrarenal organ involvement (eg, liver, pancreas, spleen, cardiac, and arachnoid membranes).
Autosomal dominant polycystic kidney disease (PKD) and autosomal recessive PKD are progressive cilia-related disorders that often lead to chronic kidney disease and end-stage renal disease. This ...
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited multisystem disorder, characterized by renal and extra-renal fluid-filled cyst formation and increased kidney volume that eventually leads to end-stage renal disease. ADPKD is considered the fourth leading cause of end-stage renal disease in the United States and globally.
Autosomal dominant polycystic kidney disease is a common cause of end stage kidney disease. It is a progressive and unfortunately incurable condition that can lead to significant morbidity and kidney failure. Many more patients are diagnosed with this disease without any symptoms as the population is increasingly undergoing imaging for other ...
Background Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterised by progressive renal cyst formation leading to renal failure in the majority of patients. The likelihood and rate of ADPKD progression is difficult to predict and there is a clear need to identify prognostic indicators that could be used to anticipate ADPKD progression, to aid the management of ...
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of end-stage renal disease (ESRD), affecting an estimated 1 in 1,000 people 1. Mutations in either PKD1 or ...
Women. Women with autosomal dominant polycystic kidney disease may have massive cystic enlargement of the liver, 1 a complication attributed to the role of estrogens in promoting the growth of ...
The prevalence of autosomal dominant polycystic kidney disease (ADPKD): A meta-analysis of European literature and prevalence evaluation in the Italian province of Modena suggest that ADPKD is a ...
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by cysts in the kidneys and, in many cases, is associated with cysts in the liver and pancreas that can be helpful in confirming the diagnosis. In addition, patients may have a variety of other abnormalities, many of which are consistent with a generalized defect in ...
INTRODUCTION Polycystic kidney disease (PKD) includes inherited diseases that cause an irreversible decline in kidney function. PKD is most commonly encountered as an autosomal dominant disease, while the rare autosomal recessive form represents a different entity. The autosomal dominant form (autosomal dominant PKD [ADPKD]) is the most common genetic cause of chronic kidney disease (CKD) [ 1 ...
Autosomal dominant polycystic kidney disease (ADPKD) is a slowly progressive kidney disease, and the effect of interventions on hard outcomes such as onset of end-stage kidney disease (ESKD) may be challenging to ascertain given the short duration of most clinical trials, where the statistical power may be low.1 We previously demonstrated a "legacy effect" of target BP interventions in ...
This JAMA Patient Page describes autosomal dominant polycystic kidney disease, its signs and symptoms, diagnosis, and treatment options.
Autosomal dominant polycystic kidney disease (ADPKD) is generally a late-onset multisystem disorder characterized by bilateral kidney cysts, liver cysts, and an increased risk of intracranial aneurysms. Other manifestations include: cysts in the pancreas, seminal vesicles, and arachnoid membrane; dilatation of the aortic root and dissection of the thoracic aorta; mitral valve prolapse; and ...
Abstract. Autosomal polycystic kidney disease is the most common inherited kidney disease determining 5% of all end-stage kidney disease. The only therapy approved for this condition is Tolvaptan, which, with its aquaretic effect, has a strong effect on patients' daily life. Recently, the literature has been enriched with new works that analyze ...
Methods: A systematic review and meta-analysis were performed examining all studies which included "Polycystic kidney disease" and "Peritoneal dialysis" in their titles, excluding commentaries, letters to the authors and abstracts.
BACKGROUND Polycystic renal disease is a frequent congenital anomaly of the kidneys, but research using chromosomal microarray analysis and exome sequencing in fetuses with polycystic renal disease remains sparse, with most studies focusing on the multisystem or genitourinary system.
Introduction: Polycystic kidney diseases are common disorders and are considered as common causes of end stage renal disease, both in children and in adults. General surgeons, neonatologists, pediatric urologists, nephrologists and general physicians frequently encounter patients with polycystic kidney diseases.
Polycystic kidney disease (PKD) is a multiorgan disorder resulting in fluid-filled cyst formation in the kidneys and other systems. The replacement of kidney parenchyma with an ever-increasing volume of cysts eventually leads to kidney failure. Recently, increased understanding of the pathophysiology of PKD and genetic advances have led to new approaches of treatment targeting physiologic ...
Cystic kidneys are common causes of end-stage renal disease, both in children and in adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are cilia-related disorders and the two main forms ...
Autosomal dominant polycystic kidney disease (ADPKD) is a genetic kidney disorder with multiple cyst formation that progresses to chronic kidney disease (CKD) and end-stage kidney disease. Plant-based diets have attracted considerable attention because they may prevent CKD development. This study investigated whether adherence to a plant-based diet is associated with kidney function in ...
The clinical manifestations of autosomal recessive polycystic kidney disease are multiple renal cyst, cyst of liver and liver fibrosis, intrahepatic bile duct dilatation. Two mutations (c.4274T > G, c.7973T > A) in PKHD1 gene may be pathogenic.
Tubal mesosalpinx cysts are paratubal cysts, that account for approximately 10% of adnexal masses, and the presence of these cysts combined with adnexal torsion is a rare acute abdominal condition, with few cases reported in the literature. We reported two cases of adolescent tubal mesosalpinx cysts combined with adnexal torsion and reviewed the literature to help improve the diagnosis of the ...
A literature review and meta-analysis of available data were performed from January 2000 to September 2023 to identify reported data of prevalence, clinical manifestation, and genetics anomalies of patients with polycystic kidney disease in the continent.
Adult polycystic kidney disease (APKD) is a frequent disease (1/1000) responsible for about 10% of chronic renal failure. It is an autosomic dominant disease due to mutation of one out of three genes: PKD1 (on the 16th chromosome), PKD2 (on the 4th chromosome) and PKD3 (still unmapped). In the past APKD diagnosis was normally done in fourteen ...
NIH Funding Opportunities and Notices in the NIH Guide for Grants and Contracts: Central Coordinating Site for the Polycystic Kidney Disease Research Core Centers (U24 Clinical Trial Not Allowed) RFA-DK-25-015. NIDDK
A systematic review and meta-analysis were performed examining all studies which included "Polycystic kidney disease" and "Peritoneal dialysis" in their titles, excluding commentaries, letters to the authors and abstracts. PubMed, Embase, Google scholar and Scopus were searched to December 31 st 2017.
Objective: To perform a systematic literature review on definitions and instruments used to measure remission, relapse, and disease activity in polymyalgia rheumatica (PMR), to inform an OMERACT project to endorse instruments for these outcomes.