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Arq. bras. oftalmol ; 84(1): 74-77, Jan.-Feb. 2021. graf
Article in English | LILACS | ID: biblio-1153096


ABSTRACT A 45-year-old female patient presented with a complaint of right eye redness and pain for 7 days. She was under investigation for urinary abnormalities and reported a previous history of recurrent oral ulcers and ocular hyperemia in both eyes. Best-corrected visual acuity was 20/30 and 20/20 in the right and left eyes, respectively. Slit-lamp biomicroscopy of the ocular surface of the right eye revealed nasal scleral hyperemia that persisted after instillation of topical phenylephrine 10%, reinforcing the diagnosis of anterior scleritis. Renal biopsy showed immunoglobulin A immune complexes and confirmed the suspected diagnosis of Berger's disease. Maintenance immunosuppressive therapy with azathioprine following a 6-month induction of remission with cyclophosphamide was necessary after pulse therapy with methylprednisolone. Scleritis is usually related to systemic autoimmune diseases, such as rheumatoid arthritis, and polyangiitis. Herein, we describe a rare case of unilateral anterior scleritis associated with Berger's disease.

RESUMO Paciente de 45 anos, sexo feminino queixava-se de hiperemia e dor no olho direito há sete dias. Encontrava-se sob investigação de alterações urinárias e relatou história pregressa de úlceras orais e hiperemia ocular bilateral recorrentes. A acuidade visual corrigida era de 20/30 no olho direito e 20/20 no esquerdo. A biomicroscopia da superfície ocular do olho direito revelou intensa hiperemia escleral em região nasal que persistiu após a instilação de fenilefrina tópica a 10%, reforçando o diagnóstico clínico de esclerite anterior unilateral. A biópsia renal revelou a presença de imunocomplexos de IgA e confirmou a hipótese de doença de Berger. Uma terapia imunossupressora de manutenção com azatioprina após 6 meses de indução de remissão com ciclofosfamida foi necessária após pulsoterapia com metilprednisolona. A esclerite geralmente está relacionada a doenças autoimunes sistêmicas, como artrite reumatoide e poliangeite. Descrevemos aqui um caso raro de esclerite anterior unilateral associada à doença de Berger.

Humans , Female , Middle Aged , Arthritis, Rheumatoid , Glomerulonephritis, IGA , Scleritis , Scleritis/diagnosis , Scleritis/etiology , Scleritis/drug therapy , Immunosuppressive Agents/therapeutic use
Med. lab ; 25(3): 569-580, 2021. ilus
Article in Spanish | LILACS | ID: biblio-1343146


La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad

Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options

Glomerulonephritis , Purpura , Purpura, Schoenlein-Henoch , Steroids , Biopsy , ISCOMs , Glomerulonephritis, IGA , Kidney Failure, Chronic
Article in Chinese | WPRIM | ID: wpr-828654


OBJECTIVE@#To systematically evaluate the efficacy and safety of steroid combined with immunosuppressants in the treatment of primary IgA nephropathy in children.@*METHODS@#English and Chinese electronic databases were searched to include the studies on the efficacy and safety of steroid combined with immunosuppressants versus steroid alone in the treatment of primary IgA nephropathy in children. Outcome measures included proteinuria remission rate, urinary protein quantification, incidence of adverse events, estimated glomerular filtration rate, and incidence of renal dysfunction. Review Manager 5.3 software was used for data analysis.@*RESULTS@#A total of 7 studies with 381 children were included. The children had moderate to severe proteinuria. The Meta analysis showed that compared with the steroid alone group, the steroid combined with immunosuppressants group achieved a significantly higher rate of proteinuria remission (RR=1.36, 95%CI: 1.19-1.55, P<0.001) and significantly lower urinary protein quantification (SMD=-0.82, 95%CI: -1.23 to -0.41, P<0.001). There was no significant difference in the incidence rate of adverse events between the two groups (RR=1.28, 95%CI: 0.92-1.77, P=0.14).@*CONCLUSIONS@#The current evidence shows that for children with primary IgA nephropathy who have moderate to severe proteinuria, steroid combined with immunosuppressants has a better effect than steroid alone and does not increase the incidence rate of adverse events.

Child , Glomerular Filtration Rate , Glomerulonephritis, IGA , Humans , Immunosuppressive Agents , Proteinuria
J. bras. nefrol ; 41(1): 152-156, Jan.-Mar. 2019. tab, graf
Article in English | LILACS | ID: biblio-1002430


ABSTRACT Rapidly progressive glomerulonephritis (RPGN) is a renal disease with an extensive differential diagnosis. This paper reports the case of a 55-year-old female patient diagnosed with Hansen's disease with acute progressive renal impairment after developing lower limb pyoderma. The association between Hansen's and kidney disease has been well documented, with glomerulonephritis (GN) ranked as the most common form of renal involvement. Post-infectious glomerulonephritis (PIGN) in adults has been associated with a number of pathogens occurring in diverse sites. The patient described in this case report had RPGN and biopsy findings suggestive of PIGN with C3 and IgA detected on immunofluorescence and kidney injury secondary to recent infection by Staphylococcus, a well-documented manifestation of renal impairment in patients with Hansen's disease.

RESUMO A Glomerulonefrite Rapidamente Progressiva (GNRP) é um padrão de doença renal com amplo diagnóstico diferencial. O caso reporta uma paciente de 55 anos com deterioração aguda e progressiva da função renal após quadro de piodermite em membro inferior com diagnóstico concomitante de hanseníase. Associação da hanseníase com doença renal é bem descrita, sendo a GN a forma de acometimento renal mais comum. As glomerulonefrites pós-infecciosas (GNPIs) em adultos ocorrem devido a um grande número de patógenos, nos mais diversos sítios. A paciente do caso relatado apresentava quadro de GNRP e achados de biópsia que sugerem GNPI com marcação de C3 e IgA na imunofluorescência, sugestiva de lesão renal secundária a infecção recente por Staphylococcus, uma manifestação bem descrita de doença renal em pacientes com hanseníase.

Humans , Middle Aged , Complement C3/metabolism , Leprosy, Multibacillary/diagnosis , Acute Kidney Injury/diagnosis , Glomerulonephritis, IGA/diagnosis , Rifampin/therapeutic use , Biopsy , Blood Urea Nitrogen , Fluorescent Antibody Technique , Clofazimine/therapeutic use , Creatinine/blood , Dapsone/therapeutic use , Diagnosis, Differential , Acute Kidney Injury/drug therapy , Glomerulonephritis, IGA/drug therapy , Glucocorticoids/administration & dosage , Glucocorticoids/therapeutic use
Chinese Medical Journal ; (24): 647-652, 2019.
Article in English | WPRIM | ID: wpr-774775


BACKGROUND@#Immunoglobulin A nephropathy (IgAN) is the most common pathological type of glomerular disease. Kidney biopsy, the gold standard for IgAN diagnosis, has not been routinely applied in hospitals worldwide due to its invasion nature. Thus, we aim to establish a non-invasive diagnostic model and determine markers to evaluate disease severity by analyzing the serological parameters and pathological stages of patients with IgAN.@*METHODS@#A total of 272 biopsy-diagnosed IgAN inpatients and 518 non-IgA nephropathy inpatients from the Department of Nephrology of Chinese People's Liberation Army General Hospital were recruited for this study. Routine blood examination, blood coagulation testing, immunoglobulin-complement testing, and clinical biochemistry testing were conducted and pathological stages were analyzed according to Lee grading system. The serological parameters and pathological stages were analyzed. The receiver operating characteristic (ROC) analysis was performed to estimate the diagnostic value of the clinical factors. Logistic regression was used to establish the diagnostic model.@*RESULTS@#There were 15 significantly different serological parameters between the IgAN and non-IgAN groups (all P < 0.05). The ROC analysis was performed to measure the diagnostic value for IgAN of these parameters and the results showed that the area under the ROC curve (AUC) of total protein (TP), total cholesterol (TC), fibrinogen (FIB), D-dimer (D2), immunoglobulin A (IgA), and immunoglobulin G (IgG) were more than 0.70. The AUC of the "TC + FIB + D2 + IgA + age" combination was 0.86, with a sensitivity of 85.98% and a specificity of 73.85%. Pathological grades of I, II, III, IV, and V accounted for 2.21%, 17.65%, 62.50%, 11.76%, and 5.88%, respectively, with grade III being the most prevalent. The levels of urea nitrogen (UN) (13.57 ± 5.95 vs. 6.06 ± 3.63, 5.92 ± 2.97, 5.41 ± 1.73, and 8.41 ± 3.72 mmol/L, respectively) and creatinine (Cr) (292.19 ± 162.21 vs. 80.42 ± 24.75, 103.79 ± 72.72, 96.41 ± 33.79, and 163.04 ± 47.51 μmol/L, respectively) were significantly higher in grade V than in the other grades, and the levels of TP (64.45 ± 7.56, 67.16 ± 6.94, 63.22 ± 8.56, and 61.41 ± 10.86 vs. 37.47 ± 5.6 mg/d, respectively), direct bilirubin (DB) (2.34 ± 1.23, 2.58 ± 1.40, 1.91 ± 0.97, and 1.81 ± 1.44 vs. 0.74 ± 0.57 μmol/L, respectively), and IgA (310.35 ± 103.78, 318.48 ± 107.54, 292.58 ± 81.85, and 323.29 ± 181.67 vs. 227.17 ± 68.12 g/L, respectively) were significantly increased in grades II-V compared with grade I (all P < 0.05).@*CONCLUSIONS@#The established diagnostic model that combined multiple factors (TC, FIB, D2, IgA, and age) might be used for IgAN non-invasive diagnosis. TP, DB, IgA, Cr, and UN have the potential to be used to evaluate IgAN disease severity.

Adult , Biomarkers , Blood , Blood Urea Nitrogen , Cholesterol , Blood , Creatinine , Blood , Female , Fibrinogen , Metabolism , Glomerulonephritis, IGA , Blood , Diagnosis , Pathology , Humans , Immunoglobulin A , Blood , Logistic Models , Male , Middle Aged , Multivariate Analysis , ROC Curve
Article in Chinese | WPRIM | ID: wpr-774107


OBJECTIVE@#To explore the value of galactose-deficient IgA1 (Gd-IgA1) in the early diagnosis of Henoch-Schönlein purpura nephritis (HSPN) in children.@*METHODS@#A total of 67 hospitalized children who were definitely diagnosed with HSPN between January and April 2018 and 58 hospitalized children with Henoch-Schönlein purpura (HSP) were enrolled in the study. Twenty children undergoing routine physical examinations served as controls. The levels of serum and urine Gd-IgA1 were determined using ELISA. The receiver operating characteristic curve was used to analyze the value of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in the diagnosis of HSPN.@*RESULTS@#The level of serum Gd-IgA1 and urine Gd-IgA1/urine creatinine ratio in children with HSP or HSPN were significantly higher than those in healthy control group (P<0.01), with a significantly greater increase observed in children with HSPN (P<0.01). Serum Gd-IgA1 ≥1 485.57 U/mL and/or urine Gd-IgA1/urine creatinine ratio ≥105.74 were of favorable value in the diagnosis of HSPN. During the six-month follow-up of the 49 children with HSP, the incidence of HSPN was 47% (23/49), which included a 100% incidence in children with serum Gd-IgA1 ≥1 485.57 U/mL and a 73% incidence in children with urine Gd-IgA1/urine creatinine ratio ≥105.74.@*CONCLUSIONS@#Serum and urine Gd-IgA1 is of favorable clinical value in the early diagnosis of HSPN.

Child , Early Diagnosis , Galactose , Glomerulonephritis, IGA , Humans , Immunoglobulin A , Purpura, Schoenlein-Henoch
Article in Chinese | WPRIM | ID: wpr-774088


OBJECTIVE@#To study the clinical effect and safety of tacrolimus (TAC) combined with glucocorticoid (GC) versus mycophenolate mofetil (MMF) combined with GC in the treatment of primary IgA nephropathy (IgAN) in children.@*METHODS@#A retrospective analysis was performed for the clinical data of children with primary IgAN confirmed by renal pathology between January 2012 and December 2017. These children were divided into TAC group and MMF group according to the treatment regimen. Their clinical data before treatment and at 1, 3, and 6 months of treatment were collected, and the remission status of IgAN and adverse reactions were compared between the two groups.@*RESULTS@#A total of 43 children who met the inclusion criteria were enrolled, with 15 children in the TAC group and 28 children in the MMF group. At 1 month of treatment, there was no significant difference in the remission status between the two groups (P>0.05). At 3 and 6 months of treatment, the TAC group had a significantly better remission status than the MMF group (P0.05), but fungal infection was observed in one child from the TAC group.@*CONCLUSIONS@#TAC combined with GC can effectively reduce urinary protein in children with primary IgAN, and it has a better short-term clinical effect than MMF combined with GC, with good safety.

Child , Drug Therapy, Combination , Glomerulonephritis, IGA , Drug Therapy , Glucocorticoids , Therapeutic Uses , Humans , Immunosuppressive Agents , Mycophenolic Acid , Retrospective Studies , Tacrolimus , Therapeutic Uses
Article in English | WPRIM | ID: wpr-786124


Anti–glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. A renal biopsy showed crescentic GN with linear IgG deposition along the GBM and mesangial IgA deposition. The patient was diagnosed as concurrent anti-GBM nephritis and IgA nephropathy. Therapies with pulse methylprednisolone and cyclophosphamide administration were effective. Concurrent cases of both anti-GBM nephritis and IgA nephropathy are rare among cases of anti-GBM diseases with deposition of immune complexes. This rare case of concurrent anti-GBM nephritis and IgA nephropathy with literature review is noteworthy.

Adult , Anti-Glomerular Basement Membrane Disease , Antibodies , Antigen-Antibody Complex , Azotemia , Basement Membrane , Biopsy , Cyclophosphamide , Female , Glomerulonephritis , Glomerulonephritis, IGA , Hematuria , Humans , Immunoglobulin A , Immunoglobulin G , Methylprednisolone , Nephritis , Proteinuria
Article in Chinese | WPRIM | ID: wpr-813255


To investigate the correlation of different types of urinary abnormalities or different proteinuria and hematuria with the pathological injury of kidney in IgA nephropathy with isolated hematuria and/or mild proteinuria.
 Methods: Patients with primary IgA nephropathy, isolated hematuria and/or mild proteinuria were enrolled in the Department of Nephrology, the Second Xiangya Hospital, Central South University from January 2013 to January 2018. According to the difference of red blood cell count in urinary sediment and quantitative of 24-hour urinary protein (24 h-UP) during renal biopsy, the patients were grouped in 3 ways: a simple hematuria group, a hematuria and proteinuria group, and a simple proteinuria group; a proteinuria I group, a proteinuria II group, and a proteinuria III group; a hematuria I group, a hematuria II group, and a hematuria III group. The clinical parameters such as age, mean arterial pressure, blood urea nitrogen, serum creatinine, blood uric acid, 24 h-UP, and renal pathological damage were compared.
 Results: A total of 157 patients met the inclusion criteria, including 71 males and 86 females. The most common pathological type was focal and/or segmental glomerulosclerosis. The Lee's classification were dominated by grade III and IV, and the renal pathological injury was heavy. Immunoglobulin deposition was dominated by simple IgA deposition. The most common fluorescence intensity of IgA deposition was +++. 97 (61.78%) patients were accompanied by complement deposition and were mainly composed of simple complement C3 deposition. There were 18 patients (11.47%) in the simple hematuria group, 111 patients (70.70%) in the hematuria and proteinuria group, and 28 patients (17.83%) in the simple proteinuria group. Compared with the simple hematuria group, the proportion of patients with mild injury was lower in the simple proteinuria group, and the proportion of patients with moderate-to-severe injuries was increased (χ2=7.053, P=0.008). Compared with the hematuria and proteinuria group, the proportion of patients with mild injury was lower in the simple proteinuria group, and the proportion of patients with moderate-to-severe injury was increased (χ2=4.294, P=0.038). Compared with the proteinuria I group, the proportion of patients with mild injury was lower in the proteinuria III group, and the proportion of patients with moderate-to-severe injury was increased (χ2=5.433, P=0.020). There was no significant difference in the proportion of patients with renal pathological injury among different hematuria groups (P>0.05).
 Conclusion: The clinical manifestations of patients with IgA nephropathy with hematuria and/or mild proteinuria are inconsistent with renal pathological damage. Some patients with mild clinical manifestations have severe renal pathological damage and the renal pathological damage is more serious in simple proteinuria. The more proteinuria, the heavier the renal pathological damage.

Creatinine , Female , Glomerulonephritis, IGA , Hematuria , Humans , Kidney , Male , Proteinuria
Iatreia ; 31(4): 400-406, oct.-dic. 2018. tab, graf
Article in Spanish | LILACS | ID: biblio-975490


RESUMEN El síndrome de cascanueces se produce por la obstrucción de la vena renal izquierda, debido al atrapamiento entre la aorta y la arteria mesentérica superior. Se manifiesta por dolor, hematuria, varicocele o síntomas urinarios bajos o pelvianos. La prevalencia de este síndrome es desconocida, pero se ha incrementado con el advenimiento de estudios no invasivos para su diagnóstico. Por su parte, la nefropatía IgA es la glomerulopatía más común en el mundo y representa cerca del 15 % de las causas de hematuria en niños. La combinación de nefropatía IgA y el síndrome de cascanueces es inusual, con pocos casos descritos en la literatura. Describimos el caso de un escolar de 9 años con hematuria y proteinuria persistente en el curso de una púrpura de Henoch-Schönlein, en quien se realiza una biopsia renal documentando nefropatía IgA. Sin embargo, durante el seguimiento, por la persistencia de hematuria y proteinuria aunadas a otros síntomas, se realizan estudios adicionales que confirman un síndrome de cascanueces. Es clave en los pacientes con la persistencia de estos síntomas descartar la asociación de estas dos enfermedades para evitar intervenciones innecesarias.

SUMMARY The nutcracker syndrome is caused by the obstruction of the left renal vein secondary to its compression between the superior mesenteric artery and the aorta. Clinically, this syndrome manifests with pain, hematuria, varicocele or lower urinary tract symptoms. The prevalence of this syndrome is currently unknown; however, the diagnosis of this condition has increased thanks to the availability of non-invasive studies that allow its recognition. On the other hand, IgA nephropathy is the most common type of glomerular disease worldwide. Almost 15% of the causes of hematuria in children are secondary to this condition. The combination of IgA nephropathy and the nutcracker syndrome is rare. In the current literature, few cases have been described. We described the case of a 9-year-old scholar with hematuria and persistent proteinuria in the course of a purpura of Henoch-Schonlein in whom renal biopsy documented IgA nephropathy. However, during follow-up, due to the persistence of hematuria and proteinuria, in addition to other symptoms, additional studies are carried out confirming a Nutcracker syndrome. It is key in patients with persistent symptoms to rule out the association of these two diseases to avoid unnecessary interventions.

Humans , Renal Nutcracker Syndrome , Proteinuria , Glomerulonephritis, IGA , Hematuria
Rev. colomb. obstet. ginecol ; 69(4): 303-310, Oct.-Dec. 2018. graf
Article in English | LILACS | ID: biblio-985513


ABSTRACT Objective: To report the case of a placental site tumour which presented clinically in the form of a nephrotic syndrome secondary to IgA nephropathy. Case presentation: A 24-year-old woman, primigravida, referred to a Level IV complexity institution in Bogotá (Colombia), where she was diagnosed with IgA nephropathy as a paraneoplastic manifestation of a placental site trophoblastic tumour. After total hysterectomy as treatment, the patient evolved well, with oedema resolution and a drop in proteinuria. The patient completed one year of medical follow-up, with no evidence of disease. Discussion: Placental site trophoblastic tumour is rare, is part of the differential diagnosis for gestational trophoblastic disease and may present as a renal paraneoplastic manifestation which usually resolves once the tumour is treated. IgA nephropathy may be secondary to chronic inflammatory diseases and neoplastic diseases, as in this case.

RESUMEN Objetivo: reportar un caso de tumor del lecho placentario, cuya manifestación clínica fue síndrome nefrótico secundario a nefropatía IgA. Presentación del caso: se describe el caso de una paciente de 24 años, primigestante, quien fue remitida a una institución de cuarto nivel de complejidad localizada en Bogotá (Colombia), en donde se le hizo diagnóstico de nefropatía por IgA como manifestación paraneoplásica de un tumor trofoblástico del lecho placentario. Se realizó histerectomía total como tratamiento, con evolución satisfactoria, con resolución de los edemas y descenso de proteinuria; completó un año en seguimiento médico, sin evidencia de enfermedad. Discusión: el tumor trofoblástico del lecho placentario es infrecuente, hace parte del diagnóstico diferencial de la enfermedad trofoblástica gestacional (ETG), y puede manifestarse con fenómenos paraneoplásicos a nivel renal, que generalmente se resuelven con el tratamiento del tumor. La nefropatía por IgA puede ser secundaria a enfermedades inflamatorias crónicas y a patologías neoplásicas, como en este caso.

Humans , Nephrotic Syndrome , Trophoblastic Neoplasms , Gestational Trophoblastic Disease , Glomerulonephritis, IGA
Arch. argent. pediatr ; 116(5): 688-691, oct. 2018. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-973675


La combinación de glomerulopatías es infrecuente en la población pediátrica. Su presencia debe ser sospechada en aquellos pacientes con una enfermedad glomerular de curso clínico atípico. La influencia a largo plazo sobre el deterioro funcional renal permanece incierta. Se presentan dos niños con características histológicas de glomerulopatía combinada.

Combined glomerulopathy is infrequent in pediatric patients. Its presence should be suspected in those patients with glomerulophaties with atypical course. The influence on the long-term renal impairment remains uncertain. Here we report two children with histological findings of combined glomerulopathy.

Humans , Male , Female , Child, Preschool , Glomerulonephritis, Membranous/diagnosis , Glomerulonephritis, IGA/diagnosis , Glomerulonephritis, Membranous/psychology , Glomerulonephritis, IGA/physiopathology
J. bras. nefrol ; 40(3): 242-247, July-Sept. 2018. tab, graf
Article in English | LILACS | ID: biblio-975903


ABSTRACT Introduction: IgA nephropathy (IgAN) is the most prevalent primary glomerulopathy in the world, but great variation is reported in different countries. In Brazil, the reported prevalence is high in the Southeastern States and low in Salvador, Bahia State, Brazil. Objectives: This study investigated the clinical and histological patterns of patients with IgAN in Salvador, Brazil. Methods: This is a descriptive study that included all patients with a diagnosis of IgAN performed in native kidney biopsies collected from referral nephrology services of public hospitals in Salvador between 2010 and 2015. Results: Thirty-two cases of IgAN were identified, corresponding to 6% of primary glomerulopathies. There was a slight male predominance (56%) and the median age was 30 [22-40] years. Hematuria was present in 79%, non-nephrotic proteinuria was present in 61%, and hypertension was present in 69% of patients. Segmental sclerosis (S1 lesions) was present in 81% of cases, and chronic tubulo-interstitial lesions (T1 and T2 lesions) were present in 44% of cases. Patients with M1 and T2 MEST-C scores exhibited higher serum urea and creatinine than other patients. Conclusion: The prevalence of IgAN was lower in Salvador than other regions of Brazil. Chronic histological lesions and laboratory markers of severe disease were frequent. M1 and T2 MEST-C scores were correlated with markers of renal dysfunction.

RESUMO Introdução: A nefropatia por IgA (NIgA) é a glomerulopatia primária mais prevalente no mundo, mas grande variação é relatada em diferentes países. No Brasil, a prevalência relatada é alta nos estados do Sudeste e baixa em Salvador, Bahia, Brasil. Objetivos: Este estudo investigou os padrões clínicos e histológicos de pacientes com NIgA em Salvador, Brasil. Métodos: Trata-se de um estudo descritivo que incluiu todos os pacientes com diagnóstico de NIgA, realizados em biópsias de rins nativos, coletados nos serviços de referência em nefrologia dos hospitais públicos de Salvador, entre 2010 e 2015. Resultados: Foram identificados 32 casos de NIgA, correspondendo a 6% de glomerulopatias primárias. Houve uma ligeira predominância do sexo masculino (56%) e a mediana da idade foi de 30 [22-40] anos. Hematúria esteve presente em 79%, proteinúria não nefrótica esteve presente em 61% e hipertensão esteve presente em 69% dos pacientes. A esclerose segmentar (lesão S1) estava presente em 81% dos casos, e lesões túbulo-intersticiais crônicas (lesões T1 e T2) estavam presentes em 44% dos casos. Pacientes com escores M1 e T2 MEST-C exibiram maior ureia e creatinina séricas que outros pacientes. Conclusão: A prevalência de NIgA foi menor em Salvador do que em outras regiões do Brasil. Lesões histológicas crônicas e marcadores laboratoriais de doença grave foram frequentes. Os escores M1 e T2 MEST-C foram correlacionados com marcadores de disfunção renal.

Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Young Adult , Glomerulonephritis, IGA/diagnosis , Brazil
Article in English | WPRIM | ID: wpr-717618


PURPOSE: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. METHODS: We retrospectively reviewed the medical records of 456 children and adolescents aged < 20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. RESULTS: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. CONCLUSION: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.

Adolescent , Biopsy , Child , Colitis, Ulcerative , Crohn Disease , Female , Glomerulonephritis, IGA , Hematuria , Humans , Incidence , Inflammatory Bowel Diseases , Kidney , Mass Screening , Medical Records , Proteinuria , Retrospective Studies
Kosin Medical Journal ; : 85-90, 2018.
Article in English | WPRIM | ID: wpr-715146


Ankylosing spondylitis (AS) can involve the eye, gastrointestinal system, cardiopulmonary system, skin, kidneys, and spinal and peripheral joints. It is rarely accompanied by immunoglobulin A (IgA) nephropathy. Although IgA is involved in both AS and IgA nephropathy, the relationship between these diseases remains unclear. We detected hematuria and proteinuria in a 32-year-old male patient with ankylosing spondylitis that remained stable for 4 years through treatment with etanercept, a tumor necrosis factor-α (TNF-α) inhibitor, and diagnosed IgA nephropathy through a renal biopsy. IgA nephropathy seems to be less commonly associated with AS disease activity or specific treatment such as TNF-α inhibitor use.

Adult , Biopsy , Etanercept , Glomerulonephritis, IGA , Hematuria , Humans , Immunoglobulin A , Joints , Kidney , Male , Necrosis , Proteinuria , Skin , Spondylitis, Ankylosing , Tumor Necrosis Factor-alpha
Chinese Medical Journal ; (24): 2953-2959, 2018.
Article in English | WPRIM | ID: wpr-772888


Background@#Diabetes mellitus (DM) has become the leading cause of chronic kidney disease (CKD). Nondiabetic renal diseases (NDRDs) have different clinicopathological features and prognosis from those of diabetic nephropathy. Our study sought to analyze the clinical and pathological features of NDRDs, in different age groups through a cross-sectional study.@*Methods@#All patients with type 2 DM at our center who underwent renal biopsy between March 1997 and March 2017 were screened and divided into three groups by age: Group 1 (youth group), 18-44 years old; Group 2 (middle-aged group), 45-59 years old; and Group 3 (elderly group), ≥60 years old. We analyzed the clinicopathological data and risk factors by univariate and multivariate logistic regression for NDRD of the patients to identify the features of NDRD in different age groups.@*Results@#We included 982 patients in the final analysis. Patients with NDRD accounted for 64.4% of all patients. IgA nephropathy (IgAN) was the most common pathological pattern in young patients with NDRD, accounting for 26.3%. In the middle-aged group, the two most common pathological patterns were IgAN and membranous nephropathy. Membranous nephropathy was the most common pathological pattern in elderly patients with NDRD, accounting for 29.3%. Consistent with pathological features, glomerular hematuria is a risk factor for NDRD in Group 1 (odds ratio [OR], 26.514; 95% confidence interval [CI], 2.503-280.910; P = 0.006). On the other hand, rapidly increasing proteinuria or nephrotic syndrome is a risk factor for NDRD in Group 2 (OR, 5.921; 95% CI, 2.061-17.013; P = 0.001) and Group 3 (OR, 90.409; 95% CI, 6.198-1318.826; P = 0.001).@*Conclusions@#This single-center study showed that the proportion and composition of NDRD differ among different age groups. Consistent with pathological features, some clinical indices such as hematuria and proteinuria showed different features among different age groups.

Adult , Age Factors , Aged , Cross-Sectional Studies , Diabetic Nephropathies , Pathology , Female , Glomerulonephritis, IGA , Pathology , Humans , Kidney Diseases , Pathology , Logistic Models , Male , Middle Aged
Article in English | WPRIM | ID: wpr-739187


Thymic stromal lymphopoietin (TSLP) is an interleukin-7-like cytokine that is an important trigger and initiator of many allergic diseases. TSLP promotes a T-helper type 2 (Th2) cytokine response that can be pathological. A relationship is formed both at the induction phase of the Th2 response through polarization of dendritic cells to drive Th2 cell differentiation and at the effector phase of the response, by promoting the expansion of activated T cells and their secretion of Th2 cytokines and TSLP. In transgenic mice with TSLP overexpression, it has been reported that TSLP leads to the development of mixed cryoglobulinemic membranoproliferative glomerulonephritis. In addition, TSLP can play an important role in the pathogenesis of IgA nephropathy and systemic lupus erythematosus-related nephritis. From our knowledge of the role of TSLP in the kidney, further studies including the discovery of new therapies need to be considered based on the relationship between TSLP and glomerulonephritis.

Animals , Cytokines , Dendritic Cells , Glomerulonephritis , Glomerulonephritis, IGA , Glomerulonephritis, Membranoproliferative , Kidney , Mice , Mice, Transgenic , Nephritis , T-Lymphocytes , Th2 Cells
Rev. colomb. nefrol. (En línea) ; 4(2): 200-209, July-Dec. 2017. tab, graf
Article in English | LILACS, COLNAL | ID: biblio-1092996


Abstract IgA nephropathy is the most common glomerulonephritis, in which less than 10% of patients have a rapid decline of renal function. The histological findings of this group resemble those of vasculitis, with presence of crescents and fibrinoid necrosis. The coexistence of IgA nephropathy and neutrophil anti-cytoplasmic antibodies is infrequent, and the pathogenic role of these antibodies in IgA nephropathy is unclear. Here we describe a case of a patient with IgA nephropathy, rapidly progressive glomerulonephritis and neutrophil positive anti-cytoplasmic antibodies, and literature review is presented.

Resumen La nefropatía por IgA es la glomerulonefritis más frecuente, en la cual menos del 10% de los pacientes cursan con deterioro rápido de la función renal. Los hallazgos histológicos de este grupo semejan los de vasculitis, con presencia de semilunas y necrosis fibrinoide. La coexistencia de nefropatía por IgA y anticuerpos anticitoplasma de neutrófilo es infrecuente y no está claro el rol patogénico de estos anticuerpos en la nefropatía por IgA. A continuación, se describe un caso de un paciente con nefropatía por IgA, glomerulonefritis rápidamente progresiva y anticuerpos anticitoplasma de neutrófilo positivos y se realiza una revisión de la literatura referente al tema.

Humans , Male , Female , Antibodies, Antineutrophil Cytoplasmic , Glomerulonephritis , Glomerulonephritis, IGA , Vasculitis , Colombia