UNASSIGNED: We will describe the use of nasolabial Burow\'s advancement flaps (perialar crescentic advancements) to repair multi subunit defects of the nasal sidewall including the adjacent cheek, dorsum, tip, and ala without the need of additional flaps.
UNASSIGNED: This retrospective single centre study analyzed 6 month postoperative photographs using the Manchester Scar scale. The operative technique is described in detail.
UNASSIGNED: Of 355 cases, 336 were available for analysis. The median Manchester Scar scale was 7 for both sidewall defects and multi-subunit defects. There were low rates of infection or necrosis.
UNASSIGNED: With the correct technique, the nasolabial Burow\'s advancement alone is suitable to repair even large multi-subunit defects involving the nasal sidewall, cheek, dorsum, tip, and ala with high-level aesthetic and functional results.

BACKGROUND: Various immune cells, including T cells, B cells, macrophages, and neutrophils contribute to the development of crescentic glomerulonephritis. Previous animal studies have suggested that lymphangiogenesis is involved in the migration of inflammatory cells and the activation of adaptive immunity. However, the extent of the association between lymphatic vessels and crescentic glomerulonephritis severity and prognosis remains unknown.
RESULTS: In this study, we assessed lymphatic vessel density in 71 patients with crescentic glomerulonephritis who underwent renal biopsies between June 2017 and June 2022. By immunohistochemistry and immunofluorescence, we identified increased lymphatic vessel density in the kidneys of patients with crescentic glomerulonephritis compared to controls. Lymphatic vessels were categorized as total, periglomerular, and interstitial. Spearman\'s rank correlation analysis showed a positive correlation between total and periglomerular lymphatic vessel density and glomerular crescent proportion. High lymphatic vessel density (total and periglomerular) correlated with declining kidney function, increased proteinuria, and severe glomerular and interstitial pathology. Interstitial lymphatic vessel density had minimal relationship with renal lesions. After a median duration of 13 months of follow-up, higher total and periglomerular lymphatic vessel density was associated with poorer prognosis. Transcriptomic analysis revealed increased immune cell activation and migration in crescentic glomerulonephritis patients compared to healthy controls. Periglomerular lymphatic vessels might play a significant role in immune cell infiltration and renal injury.
CONCLUSIONS: Elevated lymphatic vessel density in patients with crescentic glomerulonephritis is associated with poor prognosis and may serve as a predictive factor for adverse outcomes in these patients.

UNASSIGNED: Pauci-immune crescentic glomerulonephritis (PICN) is an important cause of rapidly progressive renal failure. 10-40% of PICN cases have ANCA (antineutrophil cytoplasmic antibody) negative serology. The present study compared clinico-pathologic features, Brix\'s renal risk score, Berden\'s histopathological classes and differences in outcome between ANCAnegative vs ANCA positive PICN patients.
UNASSIGNED: Sixty-one patients of biopsy-proven PICN were studied. Biochemical findings and ANCA serology were recorded. Renal biopsy slides were reviewed along with direct immunofluorescence. Clinical and histological features were compared between ANCA negative and positive PICN using the Man Whitney U test and Chi-square test. Patients were compared for distribution in Berden\'s histological classes and Brix\'s renal risk categories. Patient and renal survival were compared using Kaplan-Meier survival analysis.
UNASSIGNED: ANCA negative PICN patients were younger (44.9 ± 16.5 years vs 53.6 ± 15.1 years, P = 0.049). Nasal (0 vs 18%, P = 0.035) and pulmonary involvement (9% vs 38%, P = 0.014) were lower in ANCA negative group. Both ANCA groups had similar renal biochemical profiles, percentage normal glomeruli, 16.3 ± 18.2 vs 21.7 ± 20.4 and percentage glomeruli with crescents, 64.5 ± 28.1 vs 64.3 ± 27.1. Twenty-seven per cent of ANCA negative cases fell in the sclerotic class in Berden\'s classification vs just 2.5% in ANCA positive group (p = 0.037) without significant difference in Brix\'s renal risk categories (p = 0.329). Thirteen per cent of ANCA negative patients achieved complete remission on treatment compared to 33% in ANCA positive patients. Patient survival and overall probability of progressing to ESRD were similar in the two groups.
UNASSIGNED: ANCA negative PICN cases present at younger ages. Nasal and pulmonary involvement is uncommon in these patients. Patient survival and progression to ESRD are similar in both ANCA groups.

Henoch-Schönlein purpura nephritis (HSPN) is one of the most common kidney diseases in children. The current diagnosis and classification of HSPN depend on pathological biopsy, which is seriously limited by its invasive and high-risk nature. The aim of the study was to explore the potential of radiomics model for evaluating the histopathological classification of HSPN based on the ultrasound (US) images. A total of 440 patients with Henoch-Schönlein purpura nephritis proved by biopsy were analyzed retrospectively. They were grouped according to two histopathological categories: those without glomerular crescent formation (ISKDC grades I-II) and those with glomerular crescent formation (ISKDC grades III-V). The patients were randomly assigned to either a training cohort (n = 308) or a validation cohort (n = 132) with a ratio of 7:3. The sonologist manually drew the regions of interest (ROI) on the ultrasound images of the right kidney including the cortex and medulla. Then, the ultrasound radiomics features were extracted using the Pyradiomics package. The dimensions of radiomics features were reduced by Spearman correlation coefficients and least absolute shrinkage and selection operator (LASSO) method. Finally, three radiomics models using k-nearest neighbor (KNN), logistic regression (LR), and support vector machine (SVM) were established, respectively. The predictive performance of such classifiers was assessed with receiver operating characteristic (ROC) curve. 105 radiomics features were extracted from derived US images of each patient and 14 features were ultimately selected for the machine learning analysis. Three machine learning models including k-nearest neighbor (KNN), logistic regression (LR), and support vector machine (SVM) were established for HSPN classification. Of the three classifiers, the SVM classifier performed the best in the validation cohort [area under the curve (AUC) =0.870 (95% CI, 0.795-0.944), sensitivity = 0.706, specificity = 0.950]. The US-based radiomics had good predictive value for HSPN classification, which can be served as a noninvasive tool to evaluate the severity of renal pathology and crescentic formation in children with HSPN.

OBJECTIVE: Accumulating evidence supports the use of antineutrophil cytoplasmic antibody (ANCA) type to classify different clinical entities. We aimed to evaluate whether the presence and type of ANCA determine different diseases, based on clinical phenotypes, renal involvement, and response to treatment.
METHODS: Differences in terms of clinical manifestations, disease activity, laboratory parameters, and histology were recorded between patients with focal necrotizing glomerulonephritis (FNGN) due to myeloperoxidase (MPO-), proteinase 3-ANCA(+) [PR3-ANCA(+)], and ANCA(-) disease at time of diagnosis. Patients were treated with the same protocol and followed-up for 24 months, in a scheduled basis of every month for the first year and every 3 months for the second year. Primary end points were: (i) Combined end-stage renal disease (ESRD) and/or death and (ii) The presence of major or minor relapse during follow-up and secondary endpoint was the combination of ESRD and reduction of estimated glomerular filtration rate (eGFR) ≥ 50%.
RESULTS: A total of 92 patients (M/F 39/53, mean age 59.1 ± 15 years) diagnosed with FNGN due to ANCA-associated vasculitis (AAV), 36 (39.1%) patients diagnosed with PR3-ANCA, 39 (42.4%) patients diagnosed with MPO-ANCA, and 17 (18.5%) patients diagnosed with ANCA(-) were included. Number of involved systems differed significantly between PR3-, MPO-ANCA, and ANCA(-), with only renal involvement in 3, 25.5, and 29% of patients, two systems involved in 33, 31, and 59% of patients, and > 3 systems involved in 64, 43.5, and 12% of patients, respectively (p = 0.002). Histology classification revealed focal, crescentic, mixed, and sclerotic type in 14, 64, 19, and 3% of PR3-ANCA(+), 8, 28, 18, and 46% of MPO-ANCA, and 41, 29, 6, and 24% of ANCA(-), respectively (p < 0.0001). Primary end point of ESRD ± Death was reached in 11 (30.6%), 16 (41%), and 6 (35.5%) patients with PR3-ANCA(+), MPO-ANCA(+), and ANCA(-), respectively (p = NS); similarly, ESRD± > 50% eGFR reduction in 8 (22.2%), 15 (38.5%), and 5 (29.4%) patients, respectively (p = NS), meaning that patients with MPO-ANCA(+) showed a propensity to decline renal function. Rate of relapse was increased in the presence of patients with PR3-ANCA(+), 14 (38.9%), 4 (11.8%), and 2 (10.3%) of patients with PR3-ANCA(+), MPO-ANCA(+), and ANCA(-), had at least one relapse during the two-year follow-up (p = 0.006).
CONCLUSIONS: Clinical phenotype and renal histology differ significantly between PR3-ANCA(+), MPO-ANCA(+), and ANCA(-) disease and FNGN; however, renal function outcome is similar, despite the increased rate of relapses in patients with PR3-ANCA(+).

BACKGROUND: Pauci-immune crescentic glomerulonephritis (PICGN) is rare form of glomerulonephritis that frequently presents as rapidly progressive renal failure. Several prior studies have evaluated role of various factors influencing outcomes in patients with PICGN. The histopathological classification proposed by Berden a decade earlier described difference in the outcomes of patients in the focal, crescentic, mixed and sclerotic category with best prognosis for focal and worst for sclerotic group. The newly proposed renal risk score of Brix takes into account both the histopathological parameters (% of normal glomeruli, tubular atrophy and interstitial fibrosis) and clinical parameter (eGFR) which influences outcome.
METHODS: Retrospective study was performed between 2014 to 2018. Biochemical parameters and ANCA details were recorded and renal histopathology slides were reviewed and classified according to Berden\'s histopathologic classes. All the cases were further characterized into three groups based on renal risk score (Brix et al). Univariate, multivariate analysis for risk factors predicting ESRD and Kaplan Meier Survival Analysis were done.
RESULTS: In the present study, we found eGFR (P 0.024), % of normal glomeruli (P 0.023) and IFTA (P 0.001) as important factors influencing renal outcome in patients with PICGN. More than 60% patients achieved complete remission with low renal risk score as compared to patients with high renal risk score in which 80% patients developed ESRD or death at follow up. We also found significant difference in survival among various renal risk categories (Log-Rank P = 0.001) as compared to Berden\'s international histological classification (Log-Rank P = 0.037) on Kaplan -Meier survival analysis.
CONCLUSIONS: PICGN is a significant cause of mortality and morbidity. Renal histological factors such as % normal glomeruli at time of biopsy, degree of IFTA and renal risk score play an important role in assessing prognosis in these patients.

A 60-year-old Polish male with a history of alcoholism, liver cirrhosis, and hepatocellular carcinoma presented via a referral from his primary medical doctor to the emergency room with respiratory distress, acute kidney injury (AKI), and a purpuric rash on both lower extremities. He had received a total of 16 doses of Nivolumab for hepatocellular carcinoma. He had a baseline serum creatinine of 1.5 and Nivolumab was skipped a month prior to presentation because of a rise in creatinine and the onset of the rash. Labs showed a blood urea nitrogen (BUN) level of 52 mg/dl and creatinine of 3.2 mg/dl. Urinalysis revealed 300 mg proteinuria and 25-50 red blood cells on a high-power field. He was subsequently placed on steroids for vasculitis manifesting as glomerulonephritis and dermatitis. Biopsy specimens of the kidney and skin were taken and showed focally crescentic diffuse proliferative glomerulonephritis with low-grade A IgA deposits and acute tubular necrosis. The skin biopsy revealed leukocytoclastic vasculitis. We hereby describe a case of focally crescentic diffuse proliferative glomerulonephritis with low-grade A IgA deposits and acute tubular necrosis in an individual with Nivolumab-treated hepatocellular carcinoma.

Antineutrophil cytoplasmic antibodies (ANCA) vasculitis is common after the age of 50 years but it can occur at any age. There is a slight male preponderance and it is more common in Whites than Blacks but the black race confers a worse prognosis. The clinical features of ANCA vasculitis vary considerably. The manifestation of the disease depends on the organs affected, the chronicity of the disease, and how quiescent it is. Non-specific symptoms of malaise, fatigue, fever, and weight loss are common. Crescentic glomerulonephritis with focal necrosis is usually the pathology underlying renal disease. Manifestations of renal disease include hematuria and proteinuria which may progress to renal failure. We present a case of a 75-year-old female who presented with acute worsening of renal function and nephrotic-range proteinuria with positive testing for p-ANCA after the recent commencement of treatment with tofacitinib. This prompted a suspicion of ANCA-vasculitis. The patient was started on pulse dose steroids and rituximab after kidney biopsy confirmation of ANCA-vasculitis with crescentic glomerulonephritis.

Pauci-immune necrotizing and crescentic glomerulonephritis (GN) is the most common etiology of rapidly progressive GN. Clinical presentation in those afflicted is usually related to rapid loss of kidney function. We report the case of a 70-year-old woman who came to medical attention for signs and symptoms related to lower-extremity deep vein thrombosis (DVT). At presentation, the patient had biochemical abnormalities consistent with active GN, which quickly progressed to rapid loss in kidney function requiring renal replacement therapy. Kidney biopsy revealed small-vessel vasculitis with glomerular crescents. Serologic studies were negative for antineutrophil cytoplasmic antibody antibodies and other causes of acute GN. Plasmapheresis, immunosuppressive, and anticoagulant therapies were prescribed. Absence of other apparent end-organ involvement with vasculitis pointed toward renal-limited small-vessel vasculitis, yet presence of unprovoked DVT argues for systemic vascular inflammation. This case illustrates that venous thrombosis can be the presenting manifestation in patients with vasculitis and silent, severe end-organ involvement. The epidemiology and pathophysiology of venous thromboembolism in small-vessel vasculitis are discussed in this report.

ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole. We suggest that MPO can cause both pauci-immune vasculitis and secondary membranous nephropathy in some cases, as in cases of levamisole-adulterated cocaine use.