Posterior Reversible Encephalopathy Syndrome, not so Uncommon in Pediatric Patients with Renal Involvement: A Case Series.

Introduction: Posterior reversible encephalopathy syndrome (PRES) primarily shows neurological symptoms and is more frequent in males, often occurring in oncological patients. It can also be associated with renal conditions like post-streptococcal glomerulonephritis, a common cause of pediatric hypertension. Management involves blood pressure and seizure treatment. In some cases, it may lead to irreversible and severe complications. Early treatment is essential for prevention. Presentation of case series: In the past six months, we have documented the cases of two patients, aged 15 and 10, both of whom presented with PRES and renal disease. These patients were admitted because of general malaise, headaches, nausea, vomiting, visual disturbances, and elevated blood pressure. Subsequently, both patients experienced epileptic episodes. Only the first patient required transfer to the Pediatric Intensive Care Unit (PICU). Cerebral magnetic resonance imaging (MRI) scans revealed distinct PRES lesions in both cases. Following comprehensive investigations, both cases were diagnosed with PRES in the context of acute post-streptococcal glomerulonephritis. Conclusions: The patients showed improvement following the administration of antihypertensive and anticonvulsant medications, along with treatment for the underlying renal condition.

Precision medicine in lupus nephritis.

Lupus nephritis (LN) is a prominent manifestation of systemic lupus erythematosus (SLE), characterized by diverse clinical and histopathological features, imposing a substantial burden on patients. Although the exact cause of SLE remain undetermined, several genetic, epigenetics, hormonal, and other factors are implicated in LN pathogenesis. The management of LN rely on invasive renal biopsies, while the standard therapy of the proliferative form of LN remains empirical and relies on indiscriminate immunosuppressants (IS). These treatments exhibit unsatisfactory remission rates, trigger recurrent renal flares, and entail grave adverse effects (ADEs). The advent of precision medicine into LN entails a concentrated effort to pinpoint essential biomarkers, reshaping the landscape of LN management. The primary objective of this review is to synthesize and summarize existing research findings by elucidating the most prevalent immunological, genetic, and epigenetic alterations and deliberate on management strategies that can pave the way for precision medicine in tackling LN. Novel clinical biomarker such as serum anti-complement component 1q (anti-C1q), with urinary markers including neutrophil gelatinase-associated lipocalin (NGAL), monocyte chemoattractant protein-1 (MCP1) and tumour necrosis-like weak inducers of apoptosis (TWEAK) are strongly correlated with LN. These biomarkers have good sensitivity and specificity and perform better than conventional biomarkers in assessing LN activity. Similarly, more renal-specific genetic and epigenetic alteration have been correlated with LN susceptibility and severity. This includes variants of hyaluronan synthase 2 (HAS2), and platelet-derived growth factor receptor alpha (PDGFRA). In the future, integrating clinical, genetic, epigenetic, and targeted therapies holds promise for guiding precision medicine and improving LN outcomes.

Hypocitraturia as a biomarker of renal tubular acidosis in patients with Sjögren's disease.

INTRODUCTION: Sjögren's disease (SD) is an immune-mediated chronic inflammatory disease that affects epithelial tissues, mainly salivary and lacrimal glands. It also presents extraglandular manifestations. The main renal manifestation is tubulointerstitial nephritis (TIN), which can manifest as renal tubular acidosis (RTA). Urinary citrate may be a biomarker of RTA in these patients. The objective of this study was to evaluate whether hypocitraturia is a predictive biomarker of RTA in a sample of patients with SD in a tertiary hospital in southern Brazil. METHODS: All patients with SD who met the inclusion criteria and who participated in the rheumatology outpatient clinic of the Irmandade Santa Casa de Misericórdia de Porto Alegre were included. Demographic, SD, serological and urinary data were obtained. RTA was considered in those patients who persistently presented urinary pH above 5.5 and serum pH below 7.35. Patients who persistently had urinary pH above 5.5 underwent a urinary acidification test with furosemide and fludrocortisone. These patients received 1 mg of fludrocortisone and 40 mg of furosemide and had their urine samples tested 2, 4 and 6 h after taking the medications. The test was stopped at any urine sample with pH 5.5 or less. The variables were expressed as mean and standard deviation or interquartile range. The association between hypocitraturia and RTA was assessed using the chi-square. RESULTS: Forty-two patients were included, 95.2% female with a median age of 61.73 years. The prevalence of complete distal RTA was 4.88%. Twenty-eight patients underwent urine acidification testing. Five patients had hypocitraturia, and two of them had complete distal RTA. The association between hypocitraturia and RTA was statistically significant (p < 0.012), with a sensitivity of 100%, specificity of 91.2% and accuracy of 91.7%. The negative predictive value was 100%. The global renal assessment of the population demonstrated two patients with RTA, one patient with decreased renal function and six patients with proteinuria greater than 0.5 g/24 h. CONCLUSION: The prevalence of RTA in the studied population was 4.88%. Hypocitraturia had high sensitivity and accuracy for the diagnosis of RTA.

Aberrant phenotypes of circulating γδ-T cells may be involved in the onset of systemic lupus erythematosus.

OBJECTIVE: Human gamma-delta T cells (γδ-T cells) play crucial roles in both innate and adaptive immune responses. However, much less is known about the immune status of γδT cells in systemic lupus erythematosus (SLE) patients. The objective of this study was to explore potential relationships between the frequency of γδ-T-cell subpopulations and disease activity, autoantibody titres and renal involvement in patients with SLE. METHODS: Circulating γδ-T cells and their subsets (Vδ1+ T cells, Vδ2+ T cells and γδ-T-cell subpopulations defined by expression of surface receptors, including NKG2D, NKp30, NKp46 and PD-1), were identified via flow cytometry. Sixty active SLE patients were selected, including 41 new-onset and 19 relapsing cases. One hundred healthy controls (HCs) were enrolled as the control group. Percentages of these cell subsets in SLE patients and HCs and their relationships with disease activity were analysed. Twenty-two of the 41 new-onset SLE patients were assessed before and after treatment. Changes in the frequencies of these cell subsets and their relationships with renal involvement were also analysed. RESULTS: Compared with that in HCs, the percentage of total γδ-T cells among CD3+ T cells in SLE patients was significantly lower. An imbalance in the proportions of Vδ1+ and Vδ2+ T cells among γδ-T cells was observed. The proportion of Vδ1+ T cells among γδ-T cells was significantly greater in SLE patients than in HCs, while the proportion of Vδ2+ T cells was significantly lower. Expression levels of PD-1, NKG2D, NKp30 and NKp46 in Vδ1+ T cells and Vδ2+ T cells from SLE patients were generally significantly increased, except for expression of NKG2D in Vδ2+ T cells. Moreover, Vδ2+ T cells, Vδ1+ T cells and Vδ1+PD-1+ T cells were associated with disease activity, and an increase in Vδ2+ T-cell frequency and a decrease in PD-1 expression by γδ-T cells might be associated with effective treatment. Interestingly, our results indicated that Vδ2+ T cells and their Vδ2+NKp30+ T-cell subpopulation might be associated with renal involvement in SLE. CONCLUSION: A broad range of anomalies in the proportions of γδ-T-cell subsets and γδ-T cells in SLE patients may be involved in the pathogenesis of SLE. There is a strong association between Vδ2+ T cells and their Vδ2+NKp30+ T-cell subpopulation and LN occurrence. Our results indicate that γδ-T cells and their subpopulations might be key players in disease immunopathology and renal involvement in SLE.

Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature.

Juvenile primary Sjögren syndrome (pSS) with renal involvement is extremely rare, reported approximately in 50 children, predominantly girls. Here, we present the first reported case of a male child with juvenile pSS with ocular surface disease (previously keratoconjunctivitis sicca), submandibular salivary gland involvement, and tubulointerstitial nephritis. First, two symptoms were clinically apparent at presentation. We illustrate here that kidney involvement in pSS should be actively looked for, as juvenile pSS may be associated with asymptomatic renal involvement. Immunophenotyping of peripheral blood cells using multicolor flow cytometry revealed at the time of diagnosis changes in both adaptive (T memory cells and B memory cells), and innate immunity (an increased activation of natural killer cells, as well as monocytes and neutrophils, and an increased representation of intermediate monocytes). Our case report points to the importance of kidney examination, early diagnosis and therapy in juvenile pSS, as well as highlights international collaboration to obtain more data for this rare disease.

Kikuchi-Fujimoto's Disease: A Rare and Underdiagnosed Condition with Possible Renal Involvement.

Kikuchi-Fujimoto disease (KFD), or Histiocytic Necrotizing Lymphadenitis, is a rare disease, with worldwide distribution but is best known in Japan and South Asia. The most common feature is cervical lymphadenopathy, accompanied by tenderness or high fever, with night sweats, but it can also be asymptomatic or with a very wide range of symptoms. The diagnosis is histopathological, on excisional biopsy. The Kikuchi-Fujmoto disease can mimic lymphoma but also tuberculosis and some autoimmune diseases, or be associated with them. Nephrologists need to be aware of it, considering the potential renal involvement. The association with systemic lupus erythematosus (SLE) is the most frequent but not the only one. Early diagnosis of this disease can prevent unnecessary investigations and aggressive therapies.

Autosomal Dominant Polycystic Kidney Disease: Is There a Role for Autophagy?

Autosomal-Dominant Polycystic Kidney Disease (ADPKD) is a monogenic disorder initiated by mutations in either PKD1 or PKD2 genes, responsible for encoding polycystin 1 and polycystin 2, respectively. These proteins are primarily located within the primary cilia. The disease follows an inexorable progression, leading most patients to severe renal failure around the age of 50, and extra-renal complications are frequent. A cure for ADPKD remains elusive, but some measures can be employed to manage symptoms and slow cyst growth. Tolvaptan, a vasopressin V2 receptor antagonist, is the only drug that has been proven to attenuate ADPKD progression. Recently, autophagy, a cellular recycling system that facilitates the breakdown and reuse of aged or damaged cellular components, has emerged as a potential contributor to the pathogenesis of ADPKD. However, the precise role of autophagy in ADPKD remains a subject of investigation, displaying a potentially twofold impact. On the one hand, impaired autophagy may promote cyst formation by inducing apoptosis, while on the other hand, excessive autophagy may lead to fibrosis through epithelial to mesenchymal transition. Promising results of autophagy inducers have been observed in preclinical studies. Clinical trials are warranted to thoroughly assess the long-term safety and efficacy of a combination of autophagy inducers with metabolic and/or aquaferetic drugs. This research aims to shed light on the complex involvement of autophagy in ADPKD, explore the regulation of autophagy in disease progression, and highlight the potential of combination therapies as a promising avenue for future investigations.

The Immunodiagnostic Utility of Antinuclear Antibody Patterns: A Prediction for Renal Involvement in Systemic Lupus Erythematosus Patients in the Western Region of Saudi Arabia.

Objectives Previous studies have noted associations between the immunofluorescence patterns of antinuclear autoantibodies (ANA) and the autoimmune responses seen in systemic lupus erythematosus (SLE). In this study, the authors tested the hypothesis of whether ANA patterns predict renal involvement in SLE patients. Method A retrospective study was carried out on consecutive SLE patients who had ANA staining pattern data and who were screened for renal involvement defined as all-stage proteinuria or chronic kidney disease (CKD) at a referral tertiary center in western Saudi Arabia from December 2021 to February 2022. Demographic data and levels of lupus immune markers including ANA titers, anti-double-stranded deoxyribonucleic acid antibodies (anti-dsDNA), complements C3 and C4, anticardiolipin (aCL) immunoglobulin (Ig) G and IgM, anti-ß2 glycoprotein (ß2-IgM and ß2-IgG), and lupus anticoagulant (LA) antibodies were collected. Result Among 243 patients included, 25.1% had renal involvement (95% confidence interval {CI}=19.8-31.0). A mixed ANA pattern was associated with a higher prevalence of renal involvement (46.2%), followed by homogenous (26.5%) and speckled (25.6%) patterns, compared with 4.5% for the other patterns (p=0.044). No further association of renal involvement was observed with other biological markers. Adjusted logistic regression showed age (odds ratio {OR}=0.95; 95% CI=0.92-0.97) and mixed ANA pattern (OR=26.66; 95% CI=2.53-281.11) to be independently associated with renal involvement, explaining 12.6% of the variance. Conclusion A mixed homogenous/speckled ANA staining pattern is associated with an increased risk of renal involvement, independent of ANA titer or other lupus immune markers. The potential clinical applications of the ANA staining pattern in SLE should be explored in various subtypes of SLE and patient groups.

Focal Segmental Glomerulosclerosis and Hyalinosis in a Patient with Spondyloarthritis: A Rare Renal Involvement Case Report.

Background: During its course, spondyloarthritis (SpA) may be associated with extra-articular manifestations affecting several organs. Renal involvement is one of the most common extra-articular manifestations and is dominated by secondary amyloidosis (AA), immunoglobulin A (IgA) nephropathy, and urolithiasis. Other nephropathies such as Focal segmental glomerulosclerosis and hyalinosis (FSGS) are less common and are limited to few case reports. Case: We report the case of a patient followed for axial SpA, who consulted, after being lost to follow-up for 3 years, for elevated blood pressure and edema of both lower limbs associated with an hydrocele and bilateral pleural effusion. Biological examinations showed hypoproteinemia, hypoalbuminemia, and proteinuria. In this context of nephrotic syndrome, the diagnosis of FSGS was confirmed by renal biopsy. Furthermore, the etiological investigation ruled out the causes of secondary FSGS. Conclusion: Renal involvement is a sign of severity in SpA. Its detection and management should be part of the overall management of SpA.

Limited Granulomatosis With Polyangiitis of Nose and Paranasal Sinuses - a Case Report.

Granulomatosis with polyangiitis is a rare disease with involvement of multiple organ system. It classically affects the upper respiratory tract, lower respiratory tract and kidneys. 90% of patients present with upper respiratory diseases like rhinosinusitis, nasal polyposis, nasal septal perforation, serous otitis media, impaired hearing and stridor due to subglottic stenosis. Disease manifests in two forms as limited disease i.e. without renal involvement and generalized disease i.e. with renal involvement. It needs to be differentiated from chronic granulomatous diseases like tuberculosis and delay in the treatment should be prevented. Here we present a case with disease limited to Nose and Paranasal sinuses.

Risk factors associated with recurrence of Henoch-Schonlein purpura: a retrospective study.

Background: Recurrence is considered a vital problem for assessing the prognosis of Henoch-Schonlein purpura (HSP). The objective of this study was to evaluate factors affecting the recurrence in children with HSP. Methods: We retrospectively reviewed records of 368 patients under the age of 16 years diagnosed with HSP from October 2019 to December 2020 in Beijing Children's Hospital. Patients were divided into a non-recurrence group and a recurrence group according to whether there was a recurrence. Incidence of manifestation, possible cause, age, and treatment were retrospectively analyzed. Univariate and multivariate logistic regression analyses were used to determine the risk factors of recurrence in HSP. Results: Percentages of patients were 65.2% for the non-recurrence group and 34.8% for the recurrence group. The percentage of patients with renal involvement was significantly higher in the recurrence group (40.6%) than in the non-recurrence group (26.3%). Respiratory tract infection was the most frequent trigger: 67.5% in the non-recurrence group and 66.4% in the recurrence group. Recurrence was more likely to occur in patients aged >6 years (53.3% vs. 71.9%). Logistic regression analysis revealed that hematuria plus proteinuria was an independent risk factor for the recurrence of HSP. Conversely, animal protein, exercise restriction, and age ≤6 years were independent favorable factors for the non-recurrence of HSP. Conclusion: These results suggest that organ involvement, exercise, and diet management during the initial episode of HSP should be strictly monitored for children with HSP. Adequate clinical intervention for these risk factors may limit or prevent HSP recurrence. Moreover, renal involvement is associated with the long-term prognosis of HSP.

Adult Henoch-Schönlein Purpura: Comprehensive Assessment of Demographic, Clinical, and Histopathological Features as Predictors for Systemic Involvement.

BACKGROUND: Henoch-Schönlein purpura (HSP) is a small-vessel IgA-predominant vasculitis. A major challenge in managing adult HSP is the difficulty assessing the risk of systemic involvement. There is currently a paucity of data in this area. OBJECTIVE: The objective of this study was to determine demographic, clinical, and histopathological features associated with systemic involvement in adult HSP. METHODS: In this retrospective study, we reviewed demographical features and clinical and pathology data of 112 adult HSP patients seen at Emek Medical Center between January 2008 and December 2020. RESULTS: Of these patients, 41 (36.6%) had renal involvement, 24 (21.4%) had gastrointestinal tract involvement, and 31 (27.7%) had joint involvement. Age >30 years (p = 0.006) at diagnosis was an independent predictor of renal involvement. Platelet count (<150 K/µL) (p = 0.020) and apoptosis of keratinocytes on skin biopsy (p = 0.031) were also associated with renal involvement. History of autoimmune disease (p = 0.001), positive c-antineutrophil cytoplasmic antibody (p = 0.018), positive rheumatoid factor (p = 0.029), and elevated erythrocyte sedimentation rate (p = 0.04) were associated with joint involvement. Female sex (p = 0.003), Arab race (p = 0.036), and positive pANCA (p = 0.011) were associated with gastrointestinal tract involvement. LIMITATIONS: This study is retrospective. CONCLUSION: These findings may serve as a guide to stratify risk in adult HSP patients so that those at higher risk can be monitored more closely.

The evolution of the initial manifestations and renal involvement of chinese patients with classical and late-onset Fabry disease at different sexes and ages.

BACKGROUND: Fabry disease is a rare hereditary disease involving multiple organs, and there are few reports on how the initial manifestations and renal involvement of these patients with classical and late-onset phenotype evolve with sexes and ages. To improve clinicians' understanding of Fabry disease and avoid misdiagnoses by discussing the initial manifestations, first medical specialties visited and renal involvement development in patients. METHODS: This study collected relevant data from 311 Chinese Fabry disease patients (200 males, 111 females) and descriptive statistical analysis was used to analyze the evolution of the initial manifestations and renal involvement of patients with classical and late-onset phenotype at different sexes and ages. RESULTS: Regarding the age at manifestation onset, age at the first medical specialty visited and age at the diagnosis of Fabry disease, males were earlier than females, and males with classical phenotype were earlier than males with late-onset and females with classical phenotype. In both male and female patients, the initial manifestations of classical patients were mainly acroparesthesia, and the first medical specialty visited were mainly pediatrics and neurology. The initial manifestations of late-onset patients were mainly renal and cardiovascular involvement, and the first medical specialty visited were mainly nephrology and cardiology. In classical patients, both male and female, the initial manifestations of the preschool and the juvenile groups were mainly acroparesthesia, and the frequency of renal and cardiovascular involvement in the young group was higher than that in the preschool and juvenile groups. There was no obvious renal involvement in the preschool group, renal involvement was most common in the young group and the middle-aged and elderly group. Proteinuria can appear in classical male patients as early as approximately 20 years, and renal insufficiency can occur at approximately 25 years. With age, over 50% of classical male patients can develop varying degrees of proteinuria at the age of 25 and renal insufficiency at the age of 40. 15.94% of the patients progressed to dialysis or kidney transplantation, mainly classical males. CONCLUSIONS: The initial manifestation of Fabry disease is affected by sex, age and classical/late-onset phenotype. The initial manifestations were mainly acroparesthesia and the frequency and degree of renal involvement increased gradually with aging in classical male patients.

Renal involvement, presence of amyloidosis, and genotype-phenotype relationship in pediatric patients with Familial Mediterranean fever: a single center study.

The aim of the study is to investigate how renal involvement is correlated with frequency of amyloidosis, risk factors, and demographic and clinical characteristics in pediatric patients with Familial Mediterranean fever (FMF). Demographic and clinical characteristics and laboratory data of the pediatric patients diagnosed with FMF between 1990 and 2018 were recorded from their files. The diagnosis of patients with amyloidosis (AA) was proven by renal biopsy, and as for patients with non-amyloidosis renal involvement (RI wo AA), amyloidosis could not be detected but they were followed up with the diagnosis of proteinuria and/or hematuria. A total of 1929 FMF pediatric patients were included in the study. About 962 (49.9%) participants were male. There were 134 (6.9%) patients with RI wo AA and 23 (1.2%) patients with AA diagnosed by biopsy. The most common M694V heterozygous/homozygous(het/hom) (31%) mutation was observed. Delay in diagnosis and presence of colchicine resistance were more in patients with RI wo AA and AA (p < 0.05). M694V het/hom mutation was high in both RI wo AA and AA, while the presence of compound heterozygous with M694V mutation was high in RI wo AA (p < 0.01, p = 0.02, p = 0.048, respectively). There was a positive correlation between M694V mutation and monoarthritis/polyarthritis, between compound heterozygous with M694V mutations and presence of chest pain, and between V726A mutation and constipation. Also a negative correlation was found between E148Q and chest pain and between R202Q mutation and monoarthritis/polyarthritis. While M694V mutation increased the risk 2.6 times for AA and 1.7 times for RI wo AA, colchicine resistance increased the risk 33 times for AA and 25 times for RI wo AA.    Concluson: It was concluded in the present study that M694V mutation and colchicine resistance were two important risk factors for RI wo AA (6.9%) and amyloidosis (1.2%) in FMF patients. It should be kept in mind that compound heterozygous with M694V mutations may be associated with chest pain and R202Q mutation may be negatively correlated with arthritis, unlike M694V. The genetic results and clinical findings of the patients should be evaluated together and followed up closely. What is Known: • M694V mutation and colchicine resistance were two important risk factors for RI wo AA and amyloidosis in FMF patients. What is New: • Compound heterozygous with M694V mutations were associated with chest pain and may be more serious than thought. • Another point is that while R202Q mutations were negatively correlated with arthritis, M694V mutations were positively correlated.

Rapid Clinical Improvement of Multicentric Castleman Disease (MCD) with Renal Involvement Following Treatment with Tocilizumab: AA Amyloidosis as a Possible Renal Involvement of MCD.

Castleman disease (CD) is a lymphoproliferative disorder that manifests as hypergammaglobulinemia and severe inflammation with multiorgan involvement. However, renal involvement has been infrequently described in CD. We present a case of a 63-year-old Japanese male patient with multicentric CD (MCD) in whom kidney involvement, including impaired renal function and massive proteinuria, is present. He had a 2-year history of inflammatory arthritis and was referred to our clinic with newly developed proteinuria, renal dysfunction, and elevated levels of acute-phase proteins. Abdominal computed tomography scan revealed hepatosplenomegaly, including mesenteric and inguinal lymph node enlargements. The patient underwent inguinal lymph node resection. Excisional biopsy of the inguinal lymph node showed multiple lymphoid follicles and expansion of interfollicular areas by marked plasmacytosis consistent with mixed type CD. The patient was diagnosed with human herpes virus 8-negative MCD according to the international diagnostic criteria for CD. Diagnostic renal biopsy was not performed following the medical viewpoint. Tocilizumab (TCZ) treatment was highly effective in reducing proteinuria and stabilizing renal function, as well as improving other clinical symptoms. The patient responded to TCZ treatment, and the renal involvement was rapidly improved. Our preliminary immunohistochemical analysis indicated AA amyloid deposits in urinary epithelial cells suggesting a possible renal involvement of AA amyloidosis. TCZ could potentially be one of the therapeutic options in patients with MCD with renal involvement.

Assessment of semaphorin 3A and semaphorin 7A levels in primary Sjogren's syndrome.

Sjogren's syndrome (SS) is a chronic autoimmune connective tissue disease. Varying rates of system involvements may be seen in the course of the disease. Semaphorins has multifunctions in several physiological and pathological processes such as immune system regulation. The association of Semaphorin 3A (Sema3A) and Semaphorin 7A (Sema7A), which are immune semaphorins, with autoimmune diseases is interesting for researchers. We aimed to compare serum Sema3A and Sema7A levels between primary SS and control subjects, and investigated Sema3A and Sema7A levels in disease subgroups and associated system involvements. 50 consecutive primary SS patients and 40 healthy subjects followed in the Rheumatology clinic of Cumhuriyet University Medical Faculty between 2017 and 2018 were included in the study. Inclusion criteria of patients were diagnosis of primary SS according to the 2016 ACR/EULAR classification criteria. Serum Sema3A and Sema7A levels were measured by commercial ELISA kit. Both groups were similar in terms of age, gender, and body mass index. Serum Sema3A and Sema7A levels were significantly lower in SS than in the controls (p = 0.001 and p = 0.005, respectively). Serum Sema3A levels were significantly lower in patients with renal involvement than in patients without (p = 0.03). Sema3A and Sema7A may play a role in the etiopathogenesis of SS and may be a potential serological marker for the diagnosis of SS and may be a target for treatment.

Development and validation of a nomogram for renal involvement in primary Sjögren syndrome patients: A retrospective analysis.

OBJECTIVE: To establish and validate a nomogram for individualized prediction of renal involvement in pSS patients. METHODS: A total of 1293 patients with pSS from the First Affiliated Hospital of Wenzhou Medical University between January 2008 and January 2020 were recruited and analyzed retrospectively. The patients were randomly divided into development set (70%, n = 910) and validation set (30%, n = 383). The univariable and multivariate logistic regression were performed to analyze the risk factors of renal involvement in pSS. Based on the regression ß coefficients derived from multivariate logistic analysis, an individualized nomogram prediction model was developed and subsequently evaluated by AUC and calibration plot. RESULTS: Multivariate logistic analysis showed that hypertension, anemia, albumin, uric acid, anti-Ro52, hematuria, and ChisholmMason grade were independent risk factors of renal involvement in pSS. The AUC were 0.797 and 0.750, respectively, in development set and validation set. The calibration plot showed nomogram had a strong concordance performance between the prediction probability and the actual probability. CONCLUSION: The individualized nomogram for pSS patients those who had renal involvement could be used by clinicians to predict the risk of pSS patients developing into renal involvement and improve early screening and intervention.

A Case of Insidious Onset of Kimura Disease-associated Immunoglobulin A Nephropathy without Eosinophil Infiltration in the Renal Tissue.

Kimura disease (KD), also known as eosinophilic lymphogranuloma, is a rare chronic inflammatory or allergic disease. It can present with immune-related diseases such as nephrotic syndrome, asthma, and ankylosing spondylitis. In this study, we report a case of KD combined with immunoglobulin A nephropathy that first presented as a mass in the inguinal region, followed by recurrent renal involvement. Previous reports suggested that renal involvement caused by KD was due to direct infiltration of eosinophils; however, in this case, no eosinophil infiltration was found in the renal tissue after renal biopsy. This observation reminds us to approach the case from an immune-related molecular perspective to investigate the exact cause of renal damage due to KD.

Arterial hypertension in systemic sclerosis.

Introduction: Arterial hypertension (AH) is common in systemic connective tissue diseases. Aim: To evaluate the incidence of AH in patients with systemic sclerosis (SSc) and to present clinical characteristics of the group diagnosed with AH. Material and methods: The study involved 108 patients with SSc divided into two groups: with AH (+) - 45 and AH (-) - 63. Moreover, the serological profile, scleroderma renal crisis, involvement of internal organs and mortality were determined. The kidney function was assessed based on creatinine concentration and the estimated glomerular filtration rate (eGFR). Results: AH was diagnosed in 47/108 SSc patients (41.7%). The age difference among patients was statistically significant and was higher in the AH (+) SSc group (p = 0.026). The incidences of oesophageal involvement (p = 0.011), digital ulcerations (p = 0.017), and mortality (p = 0.019) were found to be significantly higher in the AH (+) SSc group. Scleroderma renal crisis was observed in 9/108 patients (8.3%). The incidence of chronic kidney disease (CKD) was higher in the AH (+) SSc group, both of stage 2 (p = 0.013) and 3 (p = 0.07). Stages 4 and 5 of CKD were found only in the group with AH. Moreover, this group had a higher incidence of elevated uric acid (p = 0.007). Conclusions: AH is relatively common in patients with SSc and is associated with a significantly more severe course of the disease and higher frequency of renal involvement.

Rhupus Syndrome: A Diagnostic Dilemma.

Rhupus syndrome, a rare coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis, is characterized by symmetrical erosive polyarthritis and permanent deformities in addition to the clinical and serological characteristics of SLE. Its prognosis is further complicated by neurological and hematological involvement, which dramatically lowers patients' perceptions of their quality of life in terms of their health. Rhupus individuals have significantly less kidney involvement than SLE patients do. We present a case of a young female who had symmetric, bilateral, erosive polyarthritis for one and a half years preceding the signs and symptoms of SLE, which occurred about six months later.