MO303URINARY ALBUMIN-TO-CREATININE RATIO INDICATES NECROTIZING AND CRESCENTIC GLOMERULONEPHRITIS IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Samy Hakroush ◽  
Björn Tampe

Abstract Background and Aims Renal involvement is a common and severe complication of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) as it can cause acute kidney injury (AKI), end-stage renal disease (ESRD) or death. We have previously reported that elevated urinary albumin-to-creatinine ratio (uACR) correlates with rapid deterioration of kidney function in ANCA GN. Therefore, we here aimed to describe the association between proteinuric findings and histopathological diagnosis of necrotizing and crescentic ANCA GN in 50 urinary samples at admission and corresponding renal biopsies of patients with AAV. Method A total number of 50 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included between 2015 till 2020 in a single-center observational study. Results Renal involvement of AAV revealed variable proteinuria ranging from low-range to nephrotic syndromes, however most patients presented with subnephrotic proteinuria predominated by albumin (uACR). Severely increased uACR levels >300 mg/g correlated with reduction of normal glomeruli (P<0.001), attributed to increased glomerular crescents (P<0.001) and necrosis (P=0.008). By contrast, no such association was observed for global sclerotic glomeruli (P=0.58), revealing that uACR reflects necrotizing and crescentic ANCA GN rather than adaptive glomerular hyperfilitration in chronic sclerosing stage. These findings were additionnaly bolstered by histopathological subgrouping and ARRS: patients with uACR levels >300 mg/g were classified either into Berden’s crescentic class (P=0.002) or ANCA renal risk score (ARRS) high/intermediate risk (P=0.003). No association between uACR levels and extrarenal manifestation of AAV disease could be observered, suggesting that uACR levels reflected specific renal involvement with ANCA GN and further confirmed by survival analysis for cumulative incidence of RRT during the short-term course of disease. In summary, uACR measurements at admission were associated with renal biopsy findings thereafter. Levels of uACR >300 mg/g were more frequently observed in necrotizing and crescentic ANCA GN with classification either into Berden’s crescentic class or ARRS high/intermediate risk and specific for renal involvement in AAV. Conclusion Early identification of patients who mostly benefit from aggressive immunosuppressive therapy is of clinical importance. Our observation that uACR levels at disease onset predict necrotizing and crescentic ANCA GN requires further investigation for therapeutic decision especially in patients with severe deterioration of kidney function.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1228-1228
Author(s):  
S. Hakroush ◽  
B. Tampe

Background:Renal involvement is a common and severe complication of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) as it can cause acute kidney injury (AKI), end-stage renal disease (ESRD) or death.Objectives:We have previously reported that elevated urinary albumin-to-creatinine ratio (uACR) correlates with rapid deterioration of kidney function in ANCA GN. Therefore, we here aimed to describe the association between proteinuric findings and histopathological diagnosis of necrotizing and crescentic ANCA GN in 50 urinary samples at admission and corresponding renal biopsies of patients with AAV.Methods:A total number of 50 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included between 2015 till 2020 in a single-center observational study.Results:Renal involvement of AAV revealed variable proteinuria ranging from low-range to nephrotic syndromes, however most patients presented with subnephrotic proteinuria. Severe deterioration of kidney function requiring RRT within 30 days after admission was associated with elevated levels of nonselective proteinuria, mostly attributed to albuminuria (uACR). Because we have previously shown that histologically confirmed ANCA GN with glomerular crescents and necrosis is associated with AKI and requirement of RRT during short-term disease course and elevated uACR levels were equally associated with AKI and requirement of RRT during the short-term course after disease onset, we next analyzed the association between uACR measurements at admission and histopathological findings within renal biopsies performed thereafter. Severely increased uACR levels >300 mg/g correlated with reduction of normal glomeruli, attributed to increased glomerular crescents and necrosis. By contrast, no such association was observed for global sclerotic glomeruli, revealing that uACR reflects crescentic ANCA GN rather than adaptive glomerular hyperfilitration in chronic sclerosing stage. Since uACR levels could reflect both, either a specific renal involvement with necrotizing and crescentic ANCA GN or severity of systemic AAV disease, we next correlated uACR levels assessed at admission with extrarenal disease manifestation. We observed no association between uACR levels and extrarenal manifestation of AAV disease including pulmonary hemorrhage, skin involvement and BVAS assessment, suggesting that uACR levels reflected specific renal involvement in AAV. These observations were further confirmed by survival analysis for cumulative incidence of RRT during the short-term course of disease.Conclusion:Early identification of patients who mostly benefit from aggressive immunosuppressive therapy is of clinical importance. Our observation that uACR levels at disease onset predict necrotizing and crescentic ANCA GN requires further investigation for therapeutic decision especially in patients with severe deterioration of kidney function.Disclosure of Interests:None declared


2021 ◽  
Vol 10 (7) ◽  
pp. 1538
Author(s):  
Désirée Tampe ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Samy Hakroush ◽  
Björn Tampe

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Methods: A total number of 53 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included from 2015 to 2021 in a single-center study. Results: Proteinuria correlated with myeloperoxidase (MPO) subtype, diagnosis of microscopic polyangiitis (MPA) and severe deterioration of kidney function. Proteinuria was most prominent in sclerotic class ANCA GN and ANCA renal risk score (ARRS) high risk attributed to nonselective proteinuria, including both glomerular and tubular proteinuria. Finally, there was no association between proteinuria and systemic disease activity, suggesting that proteinuria reflected specific renal involvement in AAV rather that systemic disease activity. Conclusions: In conclusion, proteinuria correlated with distinct clinicopathological characteristics in ANCA GN, mostly attributed to a reduced fraction of normal glomeruli. Furthermore, proteinuria in ANCA GN reflected specific renal involvement in AAV rather than systemic disease activity. Therefore, urinary findings could further improve our understanding of mechanisms promoting kidney injury and progression of ANCA GN.


Author(s):  
Claudius Speer ◽  
Christine Altenmüller-Walther ◽  
Jan Splitthoff ◽  
Christian Nusshag ◽  
Florian Kälble ◽  
...  

AbstractTo study the impact of glucocorticoid maintenance dose and treatment duration on outcomes in patients with AAV (ANCA-associated vasculitis) with emphasis on infectious complications. A total of 130 AAV patients from two German vasculitis centers diagnosed between August 2004 and January 2019 treated with cyclophosphamide and glucocorticoids for induction therapy and glucocorticoids for maintenance therapy were retrospectively enrolled. We investigated the influence of glucocorticoid maintenance therapy on patient survival, time to relapse, kidney function, infectious complications and irreversible physical damage. The patients were divided into the following groups: patients treated according to the predefined reduction scheme (< 7.5 mg) or patients treated with glucocorticoids ≥ 7.5 mg after 6 months. Compared to patients receiving < 7.5 mg glucocorticoids after 6 months, patients receiving $$\ge $$ ≥ 7.5 mg had an increased rate of infectious episodes per patient (1.7 vs. 0.6; p < 0.001), including urinary tract infection (p = 0.007), pneumonia (p = 0.003), opportunistic pneumonia (p = 0.022) and sepsis (p = 0.008). Especially pneumonia during the first 24 months after disease onset [hazard ratio, 3.0 (95% CI 1.5 − 6.1)] led to more deaths from infection (p = 0.034). Glucocorticoid maintenance therapy after 6 months had no impact on relapse rate or patient survival and decline in kidney function was comparable. Glucocorticoid maintenance therapy with $$\ge $$ ≥ 7.5 mg after 6 months is associated with more severe infectious complications leading to an increased frequency of deaths from infection. Glucocorticoid maintenance therapy has no effect on time to relapse or patient survival and should therefore be critically revised throughout the aftercare of AAV patients.


2021 ◽  
Author(s):  
Mustafa Zafer Temiz ◽  
Ibrahim Hacibey ◽  
Ramazan Omer Yazar ◽  
Mehmet Salih Sevdi ◽  
Suat Hayri Kucuk ◽  
...  

AbstractBackgroundThe central role in the pathogenesis of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), called as coronavirus disease 2019 (COVID-19), infection is attributed to angiotensin-converting enzyme 2 (ACE-2). ACE-2 expressing respiratory system involvement is the main clinical manifestation of the infection. However, literature about the association between the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and higher ACE-2 expressing kidney is very limited. In this study, we primarily aimed to investigate whether there is a kidney injury during the course of SARS-CoV-2 infection. The predictive value of kidney injury for survival was also determined.MethodsA total of 47 participants who met the inclusion criteria were included in the study. The participants were classified as ‘‘COVID-19 patients before treatment’’ ‘‘COVID-19 patients after treatment’’, ‘‘COVID-19 patients under treatment in ICU’’ and ‘‘controls’’. The parameters comorbidity, serum creatinine and cystatin C levels, CKD-EPI eGFR levels, KIM-1 and NGAL levels, urine KIM-1/creatinine and NGAL/creatinine ratios were statistically compared between the groups. The associations between covariates including kidney disease indicators and death from COVID-19 were examined using Cox proportional hazard regression analysis.ResultsSerum creatinine and cystatin C levels, urine KIM-1/creatinine levels, and CKD-EPI, CKD-EPI cystatin C and CKD-EPI creatinine-cystatin C eGFR levels exhibited significant difference in the groups. The causes of the difference were more altered kidney function and increased acute kidney damage in COVID-19 patients before treatment and under treatment in ICU. Additionally, incidences of comorbidity and proteinuria in the urine analysis were higher in the COVID-19 patients under treatment in ICU group. Urine KIM-1/creatinine ratio and proteinuria were associated with COVID-19 specific death.ConclusionsWe found that COVID-19 patients under treatment in ICU exhibited extremely higher levels of serum cystatin C, and urine KIM-1/creatinine and urine NGAL/creatinine ratios. These results clearly described the acute kidney damage by COVID-19 using molecular kidney damage markers for the first time in the literature. Lowered CKD-EPI, CKD-EPI cystatin C and CKD-EPI creatinine-cystatin C eGFR levels were determined in them, as well. Urine KIM-1/creatinine ratio and proteinuria were associated with COVID-19 specific death. In this regard, considering kidney function and kidney damage markers must not be ignored in the COVID-19 patients, and serial monitoring of them should be considered.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Björn Tampe ◽  
Samy Hakroush

Abstract Background and Aims Renal involvement is a common and severe complication of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We have previously reported that severe deterioration of kidney function is associated with necrotizing and crescentic ANCA glomerulonephritis (GN), classified into Berden’s crescentic class or ANCA renal risk score (ARRS) high risk. However, tubulointerstitial inflammation associated with either histopathological subgrouping or ARRS remains elusive. Furthermore, clinical and laboratory markers of AAV disease severity or deterioration of kidney function in association with inflammatory findings in the kidney have not been described yet. Since aggressive immunosuppressive therapy is recommended for remission induction especially in severe cases of AAV, we here aimed to expand our current knowledge with regard to histopathological classification of tubulointerstitial injury and inflammatory findings analogous to the Banff classification. Method A total number of 50 renal biopsies with confirmed renal involvement of AAV were retrospectively included between 2015 till 2020 in a single-center observational study. Renal biopsies were evaluated for either focal, crescentic, mixed or sclerotic class (according to Berden et al.) and ARRS low, intermediate or high risk (according to Brix et al.). Inflammatory and fibrotic tubulointerstital alterations were evaluated analogous to Banff scoring system for allograft pathology. Results We here show that distinct inflammatory lesions are associated with glomerular findings classified into either histopathological subgrouping or ARRS. Furthermore, interstitial inflammation and tubulitis correlate with disease severity and decline of kidney function in AAV. Finally, we provide data that tubulointerstitial injury and inflammatory findings correlate with short-term outcome in response to aggressive immunosuppression and remission induction therapy. Conclusion In summary, we here provide evidence that a systematic scoring of inflammatory and degenerative tubulointerstitial lesions correlate with severe renal impairment and short-term response to remission induction therapy. Since aggressive immunosuppressive therapy is recommended for remission induction especially in severe cases of AAV, systematic histopathological scoring of tubuloinsterstital lesions could further improve our current knowledge of ANCA GN progression.


2021 ◽  
Vol 10 (12) ◽  
pp. 2682
Author(s):  
Samy Hakroush ◽  
Désirée Tampe ◽  
Peter Korsten ◽  
Philipp Ströbel ◽  
Björn Tampe

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Acute tubular injury with the presence of tubulitis was previously reported to be of prognostic value in ANCA glomerulonephritis (GN). In particular, distinct tubular injury lesions were associated with the deterioration of kidney function at AAV disease onset, as well as renal resistance to treatment, and higher risk of progression to composite outcome in patients with AAV. To expand our knowledge regarding distinct tubular lesions in AAV, we aimed to describe acute tubular injury patterns in association with glomerular lesions in ANCA GN by systematic histological scoring. Methods: A total number of 48 renal biopsies with confirmed renal involvement of AAV admitted to the University Medical Center Göttingen from 2015 to 2020 were retrospectively examined. By systematic scoring of tubular injury lesions, the association between clinical parameters, laboratory markers, and histopathological findings was explored. Results: We have shown that cellular casts in renal biopsies were frequently observed in the majority of cases with ANCA GN. Furthermore, we showed that tubular epithelial simplification with dilatation correlated with MPA and MPO subtypes, C3c hypocomplementemia, severe renal involvement, and uACR. Red blood cell (RBC) casts were associated with increased levels of C-reactive protein (CRP), leukocyturia, and hematuria. Finally, we found that hyaline casts were associated with an increased fraction of glomeruli with global glomerular sclerosis. Conclusions: Acute tubular injury patterns were correlated with active ANCA GN, whereas tubular injury lesions reflecting the later stages of kidney disease correlated with chronic glomerular lesions. These results suggest an interplay between different renal compartments.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
David Jayne ◽  
Peter Merkel ◽  
Pirow Bekker ◽  
Jeffrey McMahon ◽  
Thomas J Schall ◽  
...  

Abstract Background and Aims Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life- or organ-threatening condition in which patients experience severe inflammation of small arteries. Renal involvement is common in ANCA-associated vasculitis and is correlated with high morbidity and mortality. Current treatment regimens have limited efficacy for renal disease in patients presenting with organ- or life-threatening ANCA-associated vasculitis. Avacopan, a novel orally-administered antagonist of the complement fragment C5a receptor (C5aR), was evaluated through a Phase 3 trial in patients with ANCA vasculitis. Efficacy and safety results have been previously reported; this abstract provides details of the effects on renal function in patients with renal involvement. Method The ADVOCATE trial was a randomized, double-blind, active controlled, double-dummy, 52-week treatment Phase 3 trial of 331 patients with ANCA-associated vasculitis. Patients were randomized 1:1 and received either a standard daily prednisone dosing with taper (i.e., starting at 60 mg / day tapered to 0 mg by Week 21), or daily avacopan. Background therapy included either: a) cyclophosphamide (oral or IV) followed by azathioprine, or, b) rituximab (four IV infusions). Patients with active glomerulonephritis at baseline were included in this analysis. Kidney function was analyzed based on the following parameters, which were assessed at pre-specified time-points: changes in the urine albumin-to-creatinine ratio (UACR), estimated glomerular filtration rate (eGFR), and Urinary Monocyte Chemoattractant Protein-1 (MCP-1):Creatinine ratio. Results At the baseline visit, 265 patients had renal disease. eGFR improved more in the avacopan group (n=131) compared to the prednisone group (n=134). At Week 26, eGFR increased 5.8 mL/min/1.73 m2 (from a baseline of 44.6 mL/min/1.73 m2), compared to 2.9 mL/min/1.73 m2 in the prednisone group (from a baseline of 45.6 mL/min/m2), P=0.046. At Week 52, the increases in eGFR were 7.3 mL/min/1.73 m2 and 4.1 mL/min/1.73 m2, respectively, P=0.029. The improvement was most prominent in subjects with Stage 4 kidney disease at baseline (eGFR &lt; 30 mL/min/1.73 m2), in whom eGFR improved 13.7 mL/min/1.73 m2 at Week 52 in the avacopan group (from a baseline of 21.1 mL/min/1.73 m2) compared to 8.2 mL/min/1.73 m2 in the prednisone group (from a baseline of 21.6 mL/min/1.73 m2), P=0.005. In addition to the differences in eGFR, a more rapid decrease in UACR was observed with avacopan; by Week 3 this difference was statistically significant, and at Week 4, a 40% decrease from baseline occurred in the avacopan group vs no change from baseline in the prednisone group (P&lt;0.0001). By Week 52, both groups showed a similar decrease in UACR from baseline. The urinary MCP-1:creatinine ratio decreased 59% in the avacopan group by Week 13 vs 52% in the prednisone group, P=0.03, but there was a similar decrease in the two treatment groups by Week 52. Conclusion Treatment with avacopan in patients with ANCA-associated vasculitis with renal disease led to greater recovery in eGFR when compared to standard prednisone therapy, especially in patients with Stage 4 kidney disease (eGFR &lt;30 mL/min/1.73 m2). Avacopan also led to more rapid improvement in the UACR and urinary MCP-1:creatinine ratio than prednisone. Since albuminuria is an independent risk factor for progression of renal disease (in addition to eGFR decline), the more rapid improvement in albuminuria with avacopan may also provide long-term benefit. These findings have important implications for the health of patients with ANCA-associated vasculitis.


2018 ◽  
Vol 5 (3) ◽  
pp. 681
Author(s):  
Swarna Gupta ◽  
Punit Gupta ◽  
Vishal Jain

Background: Acute kidney injury previously known as acute renal failure, is characterized by the sudden impairment of kidney function resulting in the retention of nitrogenous and other waste products normally cleared by the kidneys.   Acute Kidney Injury is usually manifested as multiorgan failure syndrome and extracorporeal support may also target fluid overload and heart failure, extracorporeal CO2 removal for combined kidney and lung support, albumin dialysis for liver support. Haemodialysis is more effective than peritoneal dialysis for management of Acute Kidney injury as Peritoneal dialysis is associated with clearance limitation and difficulties with fluid removal and is thus rarely used in adults in developed countries.Methods: The study was conducted in the Department of Medicine, Pt. J.N.M. Medical College and Dr. B.R.A.M. Hospital, Raipur (CG), India, from 2010 to 2012. All patients of both the sexes who were diagnosed as a case of Acute Kidney Injury due to Acute Gastroenteritis and Malaria and who were advised for Hemodialysis were included in the study. In our study, 32 patients of Acute Kidney Injury were included. The criteria used for AKI in the study was RIFLE criteria. Hemodialysis was done in all the cases. Quantitative variables are reported as means±SD and qualitative variables as percentage. Factor(s) determining outcome of AKI were tested by univariate analysis using “fisher’s exact test”. All variables with a P value <0.05 in the univariate analysis were defined statistically significant.Results: Out of 32 patients of Acute Kidney Injury in our study, 50% (n=16) were of Malaria associated AKI cases and other 50% (n=16) patients were of Acute Gastroenteritis associated AKI in which 87.5% males,12.5% Females were of Malaria and 75% male,25% Female were in AGE associated AKI. Maximum number of patients presented with features of AKI within first 3days of disease onset i.e. 56.25% (n=9) of malaria patients and 68.75% (n=11) of AGE patients. Mortality due to MOD was more common in Malaria patients as compared to AGE patients. AGE associated AKI patients had different level of deranged SOFA score.Conclusions: Acute kidney injury due to acute gastroenteritis differs from other causes of AKI by frequent occurrence of hypokalemia. Early diagnosis and prompt management can restore the kidney function.


2018 ◽  
Vol 90 (6) ◽  
pp. 15-21 ◽  
Author(s):  
N M Bulanov ◽  
E A Makarov ◽  
E M Shchegoleva ◽  
A S Zykova ◽  
E S Vinogradova ◽  
...  

Objective. To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA). Materials and methods. In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016). Results. Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Marina Frleta-Gilchrist ◽  
Oshorenua Aiyegbusi ◽  
Malcolm MacKinnon ◽  
Jamie Traynor ◽  
Emily McQuarrie ◽  
...  

Abstract Background and Aims Seasonal variation of ANCA associated vasculitis (AAV) and a possible link to extrinsic infective triggers is predominantly based on single centre epidemiological data. Despite frequent confounding factors including difficulty in identifying the precise time of disease onset, seasonality may be associated with the type of vasculitis and may impact the incidence of renal involvement. Therefore, the aim of this study was to explore if there is an association between seasonality, severity and incidence of biopsy-proven renal vasculitis in the Scottish population. Method Using the Scottish renal biopsy registry, we identified all adult native renal biopsies performed across Scotland between 2014 and 2018 with a diagnosis of AAV, including microscopic polyangiitis (MPA) and granulomatosis with polyangitis (GPA). Demographic data including ANCA antibody status, histological diagnosis, estimated glomerular filtration rate (eGFR) and proteinuria at presentation were recorded. Seasons were defined as autumn (September – November), winter (December-February), spring (March – May) and summer (June - August). Statistical analysis was performed using multivariate ANOVA analysis and Student’s t-test in parametric data. Results 339 cases of biopsy proven AAV were identified and included in the analysis. In this cohort, 53% were female with mean patient age of 65.6 years (± 13). Mean estimated glomerular filtration rate (eGFR) at the time of diagnosis of AAV was 32 (± 27.2) mL/min/1.73m2 and median urinary protein creatinine ratio (uPCR) was 146mg/mmol (IQR 79.8 – 271.3). Diagnosis of MPA n=209(62%) was more common than GPA n=130(38%) and patients with MPA were significantly younger at presentation (63.5 ± 13.6 ‘vs’ 67 ± 12.7 years, p = 0.017). Otherwise, these groups did not differ in mean eGFR (MPA 29.6 ± 25.7 ‘vs’ GPA 34.8 ± 27.6 mL/min/1.73m2) or median uPCR (MPA 147, IQR 78.6 – 286.5 ‘vs’ GPA 139, IQR 80.5 – 261 mg/mmol) at onset. We observed a mean of 3.5 (± 1) new cases of MPA and 2.1 (± 0.7) new cases of GPA per month, with no significant difference observed in month-to-month comparison. Seasonal analysis showed mean occurrence of 11.4 (± 4.5) cases of MPA in autumn, 11.2 (± 4.9) in winter, 10.6 (± 1.5) in spring and 8.6 (± 1.9) in summer months. In GPA, mean 6.6 (±2.7) cases occurred each autumn, 5.4 (± 3) in winter, 7.2 (± 2.9) in spring and 6.4 (± 0.9) in summer months. Overall, no significant differences in monthly or seasonal incidence across 5 years of monitoring were detected. Similarly, we observed no difference in renal function at presentation during different seasons for MPA (mean eGFR range 21.8 – 37.6 mL/min/1.73m2, uPCR median range 112 – 167.5 mg/mmol) or GPA (mean eGFR range 32-37 mL/min/1.73m2; uPCR median range 110 – 244 mg/mmol). Conclusion Our data suggest that there is no seasonal variation in the incidence of AAV diagnosed on kidney biopsy in patients living in Scotland. Additionally, patients present with similar levels of kidney function regardless of season. Thus traditional holiday periods i.e. Easter/Christmas do not seem to lead to a delay in diagnosis. This is the first study to consider seasonality in a complete national cohort and suggests that seasonal extrinsic factors do not play a major role in the pathogenesis leading to AAV onset.


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