IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship? / Vasculite por IgA (púrpura de Henoch-Schönlein), nefrite e psoríase em uma criança: existe relação?

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

Acute renal failure by rapidly progressive glomerulonephritis with IgA deposition in a patient concomitantly diagnosed with multibacillary Hansen's disease: a case report / IRA por glomerulonefrite rapidamente progressiva com depósito de IgA em uma paciente com diagnóstico concomitante de hanseníase multibacilar: relato de caso

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.

Glomerulopatías combinadas: comunicación de 2 casos pediátricos / Combined glomerulopathies: two pediatric cases

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.

IgA Nephropathy in Salvador, Brazil. Clinical and laboratory presentation at diagnosis / Nefropatia por IgA em Salvador, Brasil. Apresentação clínica e laboratorial no momento do diagnóstico

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.

IgA nephropathy in systemic lupus erythematosus patients: case report and literature review / Nefropatia por IgA em paciente portadora de lúpus eritematoso sistêmico: relato de caso e revisão de literatura

Abstract Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

Resumo O lúpus eritematoso sistêmico (LES) é uma doença autoimune multissistêmica que tem como uma das manifestações mais marcantes a nefrite. Apesar de poder coexistir com outras doenças autoimunes e determinar a predisposição a diversas complicações infecciosas, o LES raramente é descrito em associação a nefropatias de etiologia não lúpica. Relatamos o caso da rara associação entre LES e nefropatia por IgA (NIgA) primária, a glomerulopatia primária mais frequente na população mundial. A paciente foi diagnosticada com LES pela ocorrência de eritema malar, alopecia, derrame pleural, proteinúria, pancitopenia, FAN 1:1.280 padrão nuclear pontilhado fino e anticardiolipina IgM 280 U/mL. A biópsia renal revelou hipercelularidade mesangial com depósitos isolados de IgA, compatível com NIgA primária. Foi tratada com antimalárico, prednisona e inibidor da enzima conversora de angiotensina e apresentou boa evolução. Por consistirem em doenças relativamente frequentes, a coexistência de LES e NIgA pode ser de fato um achado incomum por motivos desconhecidos ou uma condição subdiagnosticada. Este relato atenta para a importância da distinção entre a atividade de doença renal do LES e nefropatias não lúpicas, em especial a NIgA, definição que tem implicações importantes sobre o prognóstico renal e regimes terapêuticos a serem adotados em curto e longo prazo.

The Natural Course of Biopsy-Proven Isolated Microscopic Hematuria: a Single Center Experience of 350 Patients

The increasing interest in healthcare and health screening events is revealing additional cases of asymptomatic isolated microscopic hematuria (IMH). However, a consensus of the evaluation and explanation of the IMH prognosis is controversial among physicians. Here, we present the natural course of IMH together with the pathological diagnosis and features to provide supportive data when approaching patients with IMH. We retrospectively evaluated 350 patients with IMH who underwent a renal biopsy between 2002 and 2011, and the pathological diagnosis and chronic histopathological features (glomerulosclerosis, interstitial fibrosis, and tubular atrophy) were reviewed. Deterioration of renal function was examined during follow up. The patients with IMH were evaluated for a mean of 86 months. IgA nephropathy was the most common diagnosis in 164 patients (46.9%). Chronic histopathological changes were observed in 166 (47.4%) but was not correlated with proteinuria or a decline in renal function. Ten patients developed proteinuria, and all of them had IgA nephropathy. Three patients progressed to chronic kidney disease with an estimated glomerular filtration rate < 60 mL/min/1.73 m2 but none progressed to end stage renal disease. In conclusion, IMH had a generally benign course during 7-years of observation, although IgA nephropathy should be monitored if it progresses to proteinuria. Future prospective randomized studies may help conclude the long-term prognosis and lead to a consensus for managing IMH.

Effect of low-dose valsartan on proteinuria in normotensive immunoglobulin A nephropathy with minimal proteinuria: a randomized trial

BACKGROUND/AIMS: Immunoglobulin A nephropathy (IgAN) is a generally progressive disease, even in patients with favorable prognostic features. In this study, we aimed to investigate the antiproteinuric effect and tolerability of low-dose valsartan (an angiotensin II receptor blocker) therapy in normotensive IgAN patients with minimal proteinuria of less than 0.5 to 1.0 g/day. METHODS: Normotensive IgAN patients, who had persistent proteinuria with a spot urine protein-to-creatinine ratio of 0.3 to 1.0 mg/mg creatinine, were recruited from five hospitals and randomly assigned to either 40 mg of valsartan as the low-dose group or 80 mg of valsartan as the regular-dose group. Clinical and laboratory data were collected at baseline, and at 4, 8, 12, and 24 weeks after valsartan therapy. RESULTS: Forty-three patients (low-dose group, n = 23; regular-dose group, n = 20) were enrolled in the study. Proteinuria decreased significantly not only in the regular-dose group but also in the low-dose group. The change in urine protein-to-creatinine ratio at week 24 was -41.3% +/- 26.1% (p < 0.001) in the regular-dose group and -21.1% +/- 45.1% (p = 0.005) in the low-dose group. In the low-dose group, blood pressure was constant throughout the study period, and there was no symptomatic hypotension. In the regular-dose group, blood pressure decreased at weeks 8 and 12. No significant change in glomerular filtration rate, serum creatinine level, or serum potassium level was observed during the study period. CONCLUSIONS: Our results suggest that low-dose valsartan can significantly reduce proteinuria without causing any intolerability in normotensive IgAN patients with minimal proteinuria.

La nefropatía IgA, la glomerulopatía más frecuente en el mundo / IgA nephropathy, the most common glomerulopathy worldwide

La nefropatía inmunoglobulina A es una glomerulonefritis mediada por inmunocomplejos caracterizada por el depósito de inmunoglobulina A1 en el mesangio glomerular. Es la forma más frecuente de glomerulonefritis primaria en el mundo. La probabilidad de deterioro de la función renal a largo plazo, está aumentada por los hallazgos siguientes: hipertensión arterial, hematuria microscópica persistente, proteinuria mayor de 0,5 g/día, descenso de la función renal al comenzar las manifestaciones o hallazgos en la biopsia renal de esclerosis glomerular, esclerosis vascular, fibrosis intersticial, atrofia tubular, formación de crecientes o distribución de IgA en la pared de los capilares glomerulares en la inmunofluorescencia. Son manifestaciones clínicas en la nefropatía inmunoglobulina A la hematuria macroscópica en aproximadamente la mitad de los pacientes al primero o segundo día del inicio de síntomas de infección respiratoria, y está asociada con dolor en el flanco en pacientes menores de 40 años. En los más viejos, la hematuria microscópica es generalmente asintomática, y en ocasiones, detectada en análisis de orina de pesquisa. Entre 10 y 20 % de los pacientes, por lo general aquellos que tienen hematuria y proteinuria ligera, pueden lograr remisión espontánea; pero, entre 25 y 30 % pueden mostrar progresión hacia la enfermedad renal crónica terminal, y la progresión por lo general es lenta (5-20 años). La biopsia renal es la única prueba específica para confirmar el diagnóstico. Los pacientes con hematuria y proteinuria menor de 0,3 g/día que están normotensos, no requieren tratamiento específico con medicamentos, pero necesitan ser controlados periódicamente con análisis de orina, creatinina sérica y medida de la tensión arterial. Los pacientes con proteinuria o hipertensión deben ser tratados enérgicamente con inhibidores de la enzima convertidora. La hipertensión, la proteinuria significativa (> 0,5 g/día), la glomerulonefritis rápidamente progresiva (rara) y el síndrome nefrótico necesitan ser tratados inmediatamente. La amigdalectomía, frecuentemente realizada en Japón, puede ser de beneficio para los que se presentan con hematuria macroscópica y amigdalitis. Se consultaron varias fuentes para esta revisión.

IgA nephropathy is immunocomplex-mediated glomerulonephritis that is characterized by the A1 immunoglobulin deposition in the glomerular mesangium. It is the most frequent form of primary glomerulonephritis worldwide. The probabilities of long-term renal function deterioration increased due to the following findings: blood hypertension, persistent microscopic hematuria, proteinuria greater than 0.5 g/day, decrease of renal function when manifestations or findings of glomerular sclerosis are observed in the renal biopsy; vascular sclerosis, interstitial fibrosis, tubular atrophy, formation or distribution of IgA in the glomerular capillary walls in the immunofluorescence test. Among the clinical manifestations of IgA nephropathy is macroscopic hematuria in roughly half of patients in the first or second day after onset of the respiratory infection symptoms and is associated to flank pain in patients less than 40 years. In the oldest people, macroscopic hematuria is generally asymptomatic and occasionally detected in screening urinalysis. Ten to twenty percent of patients with mild proteinuria and hematuria may reach spontaneous remission, but 25 to 30 % of them may also progress into the terminal chronic renal disease at a general slow rate (5 to 20 years). Renal biopsy is the only specific test to confirm diagnosis. Those patients with hematuria and proteinuria of less than 0.3 g/day, whose blood pressure is normal, do not require specific drug treatment but they need to be systematically controlled through urinalysis, serum creatinine and blood hypertension taking. The patients suffering from proteinuria and hypertension must be strictly treated with converting enzyme inhibitors. Hypertension, significant proteinuria (0.5 g/day), rapidly progressive glomerulonephritis (rare) and nephrotic syndrome must be immediately managed. Tonsillectomy, frequent method in Japan, could be beneficial for those patients presenting with macroscopic hematuria and tonsillitis. Several sources were consulted to make this review.

Fabry disease previously diagnosed as Henoch-Schonlein purpura

No abstract available.

Nefropatia por IgA e polimiosite: uma rara associação / IgA nephropathy and polymyositis: a rare association

A polimiosite é uma miopatia inflamatória idiopática sistêmica que, além da manifestação muscular, pode eventualmente cursar com acometimento respiratório, do trato gastrintestinal e, raramente, renal. Neste último caso, há descrição de apenas dois casos de nefropatia por IgA em pacientes com miopatia, ambos em dermatomiosite. Em contrapartida, relatamos pela primeira vez esta rara associação em polimiosite.

Polymyositis is a systemic and idiopathic inflammatory myopathy that, besides muscle manifestation, may occur with respiratory involvement, gastrointestinal tract and rarely renal involvement. In this latter, there are only two cases of IgA nephropathy, but both in dermatomyositis. On the other hand, we reported, for the first time, a case of IgA nephropathy in polymyositis.

Urokinase, urokinase receptor, and plasminogen activator inhibitor-1 expression on podocytes in immunoglobulin A glomerulonephritis

BACKGROUND/AIMS: The purpose of this study was to investigate the expression of urokinase-type plasminogen activator (uPA), uPA receptor (uPAR), and plasminogen activator inhibitor (PAI)-1 on podocytes in immunoglobulin A (IgA) glomerulonephritis (GN). METHODS: Renal biopsy specimens from 52 IgA GN patients were deparaffinized and subjected to immunohistochemical staining for uPA, PAI-1, and uPAR. The biopsies were classified into three groups according to the expression of uPA and uPAR on podocytes: uPA, uPAR, and a negative group. The prevalences of the variables of the Oxford classification for IgA GN were compared among the groups. RESULTS: On podocytes, uPA was positive in 11 cases and uPAR was positive in 38 cases; by contrast, PAI-1 was negative in all cases. Expression of both uPA and uPAR on podocytes was less frequently accompanied by tubulointerstitial fibrosis. CONCLUSIONS: Our results suggest a possible protective effect of podocyte uPA/uPAR expression against interstitial fibrosis.

Immunoglobulin a nephropathy and malaria falciparum infection; a rare association

Glomerular involvement occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Here, we report a rare case of falciparum malaria-associated IgA nephropathy. A 28-year-old man was admitted because of fever and abdominal pain. Ultrasound and computed tomography [CT] showed right kidney pyonenphrosis. Despite placing a nephrostomy tube, fever continued. Repeated CT was in favor of focal pyelonephritis. In addition, peripheral blood smear suggested malaria. Anti-malarial drugs were initiated and right nephrectomy was performed. One year after recovery from malaria, a persistent rise in serum creatinine was detected. A left kidney biopsy showed mesangial proliferation and dominant IgA deposits in immunofluorescence study while C[1q] was not deposited. The impression was IgA nephropathy with M[1]E[0]S[0]T[0] of Oxford classification. The patient was prescribed a combination of low dose prednisolone and angiotensin converting enzyme inhibitor. Six months after treatment serum creatinine decreased from 1.6 mg/dL to 1.3mg/dL and urine abnormalities were disappeared. Our findings suggest that malaria infection might be associated with IgA nephropathy

Immunoglobulin A Nephropathy Associated with Plasmodium falciparum Malaria

Glomerulonephritis occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Herein, we report a case of falciparum malaria-associated IgA nephropathy for the first time. A 49-yr old male who had been to East Africa was diagnosed with Plasmodium falciparum malaria. Microhematuria and proteinuria along with acute kidney injury developed during the course of the disease. Kidney biopsy showed mesangial proliferation and IgA deposits with tubulointerstitial inflammation. Laboratory tests after recovery from malaria showed disappearance of urinary abnormalities and normalization of kidney function. Our findings suggest that malaria infection might be associated with IgA nephropathy.

A proton nuclear magnetic resonance-based metabonomics study of metabolic profiling in immunoglobulin a nephropathy

OBJECTIVES: Immunoglobulin A nephropathy is the most common cause of chronic renal failure among primary glomerulonephritis patients. The ability to diagnose immunoglobulin A nephropathy remains poor. However, renal biopsy is an inconvenient, invasive, and painful examination, and no reliable biomarkers have been developed for use in routine patient evaluations. The aims of the present study were to identify immunoglobulin A nephropathy patients, to identify useful biomarkers of immunoglobulin A nephropathy and to establish a human immunoglobulin A nephropathy metabolic profile. METHODS: Serum samples were collected from immunoglobulin A nephropathy patients who were not using immunosuppressants. A pilot study was undertaken to determine disease-specific metabolite biomarker profiles in three groups: healthy controls (N = 23), low-risk patients in whom immunoglobulin A nephropathy was confirmed as grades I-II by renal biopsy (N = 23), and high-risk patients with nephropathies of grades IV-V (N = 12). Serum samples were analyzed using proton nuclear magnetic resonance spectroscopy and by applying multivariate pattern recognition analysis for disease classification. RESULTS: Compared with the healthy controls, both the low-risk and high-risk patients had higher levels of phenylalanine, myo-Inositol, lactate, L6 lipids ( = CH-CH2-CH = O), L5 lipids (-CH2-C = O), and L3 lipids (-CH2-CH2-C = O) as well as lower levels of β -glucose, α-glucose, valine, tyrosine, phosphocholine, lysine, isoleucine, glycerolphosphocholine, glycine, glutamine, glutamate, alanine, acetate, 3-hydroxybutyrate, and 1-methylhistidine. CONCLUSIONS: These metabolites investigated in this study may serve as potential biomarkers of immunoglobulin A nephropathy. Point scoring of pattern recognition analysis was able to distinguish immunoglobulin A nephropathy patients from healthy controls. However, there were no obvious differences between the low-risk and high-risk groups in our research. These results offer new, sensitive and specific, noninvasive approaches that may be of great benefit to immunoglobulin A nephropathy patients by enabling earlier diagnosis.

Validation Study of the Oxford Classification for IgA Nephropathy in Korean Adults

No abstract available.

Nefropatía por Inmunoglobulina A: Guía de práctica clínica / Immunoglobulin A nephropathy: Clinical Practice Guidelines

La nefropatía por Inmunoglobulina A (N.IgA) es la causa más frecuente de enfermedad glomerular a nivel mundial, 15-50% de los pacientes presentan pérdida progresiva de la función renal en 10-20 años; el resto remisión clínica o hematuria/ proteinuria persistente. Su tratamiento óptimo es incierto. Nuestro objetivo fue desarrollar recomendaciones basadas en la evidencia a través de búsqueda en bases de datos Medline, Embase, Lilacs, Cochrane Trials Register. Los investigadores analizaron la calidad de los estudios independientemente, usando la Cochrane Renal Group checklist: aleatorización, carácter ciego, intención de tratar y pérdidas en el seguimiento. La evidencia se clasificó en niveles y la recomendación en grados, según el Centre for Evidence-Based Medicine, Oxford, con dos enfoques principales: Terapia inmunosupresora (corticoides, citostáticos, ciclosporina A y micofenolato mofetilo): Nivel I a, grado A. Terapia combinada con inmunosupresores en adultos: Nivel II b, grado B. Corticoides más ciclofosfamida o azatioprina en niños: Nivel II b, grado C. Ciclosporina y micofenolato-mofetilo: Nivel II b, grado B. Terapia no inmunosupresora: inhibidores del sistema renina-angiotensina (IEAC) y/o bloqueantes del receptor de angiotensina II (BRAII), aceite de pescado, estatinas, antiplaquetarios y tonsilectomía: Nivel I a, grado A. Niños: IECA y BRAII con monitoreo de función renal y de nivel sérico de potasio: Nivel I b, grado B. En nefropatía progresiva, antiplaquetarios como tratamiento coadyuvante: Nivel I, grado C. Aceite de pescado como soporte adicionado de BRAII e IECA en pacientes con lesiones histológicas leves y baja reducción de la filtración glomerular: Nivel II b, grado B (no en niños). No hay evidencias para recomendar estatinas en niños; en mayores de 5 años con síndrome nefrótico e hipercolesterolemia usar sólo con monitoreo de fosfocreatin-kinasa sérica. No hay evidencias para recomendar la tonsilectomía.

Immunoglobulin A nephropathy (N.IgA) is the world most common glomerular disease; 15-50% of patients develop loss of renal function in 10-20 years, and the rest remission or mild proteinuria/ hematuria. The optimal treatment is uncertain. Our aim was to develop evidence-based recommendations through research in Medline, Embasse, Lilacs and Cochrane Central Register of Controlled Trials. The study-quality was independently assessed by the reviewers following the Cochrane Renal Group checklist: randomization, blinding, intention-to-treat analysis and follow-up period. Levels of evidence and grades of recommendation were assigned according to Center for Evidence-Based Medicine, Oxford. Two approaches were considered: Immunosuppressive therapy (corticosteroids, cytostatics, cyclosporine A, mycophenolate-mofetil): Level I a, grade A. -Combined suppressive therapy in adults. Corticosteroids plus cytotoxics drugs (cyclophosphamide/azathioprine): Level II b, grade B. In children with severe IgA nephropathy: Level II b, grade D. Cyclosporine and mycophenolate- mophetil: Level II b, grade C. Cyclosporine and mycophenolate-mophetil: Level ll b, grade C. -Non immunosuppressive therapy: reninangiotensin converting enzyme inhibitors (ACEI) and/or angiotensin II receptor blockers (ARB), fish oil, statins, antiplatelets and tonsillectomy. ACEI and/or ARB, in patients with proteinuria =1 g: Level I a, grade A. In children with moderate proteinuria: ACEI and/or ARB with close monitoring of renal function and serum potassium level: Level II b, grade B. Antiplatelet as supportive treatment: Level I a, grade C. Fish oil in addition to ACEI or ARB in patients with mild histological lesions: Level II b, grade B (Not in children). Statins: no evidence to recommend these drugs in children. In patients > 5 years with nephrotic syndrome and hyper-cholesterolemia, use statins with close monitoring of serum creatine-kinase. There is no evidence to recommend tonsillectomy.

Clinical and histopathological spectrum of IgA nephropathy in Kuwait

Little is known about the nature and the course of IgA nephropathy [IgAN] in Arab countries. The aim of this work was to study the spectrum of clinical presentation and histopathological findings at our institution. Retrospective review, all renal biopsies at the Mubarak Al Kabeer Hospital between January 2000 and December 2004. Cases of IgA nephropathy were selected, and their medical records and biopsy findings were reviewed. Eighty patients [9.2% of all native kidney biopsies] were diagnosed to have IgAN nephropathy. Sixty-nine biopsies were included in the study; 11 were excluded. Forty-three [62.3%] patients were male and 26 [37.7] patients were female. Fifty [72.5%] patients were below the age of 40 years. Mean [SD] duration of follow-up was 3.6 [1.3] years. The first presentation included nephritic-range proteinuria [49.3%] and renal impairment [50.7%]. During the follow-up period, 56 [81.2%] patients were stable or improved. Hass classification of biopsies showed 36.2% had class I, 27.5% had class II, 13.0% had class III, 5.8% had class IV, and 17.4% had class V IgAN. Females had milder forms of the disease than males. Macroscopic hematuria and renal impairment at presentation were seen more in patients with class IV and V IgAN. The presenting serum creatinine and uric acid values were higher in those with Hass classes III to V. Deterioration of renal function during the follow-up period was more significant in the presence of hypertension, renal impairment, or macroscopic hematuria at the time of biopsy. The prevalence of IgAN in Kuwait is about 9.2%. Renal impairment or macroscopic hematuria at presentation was seen in patients with more aggressive renal lesions and contributed to poor outcome

Caso clínico-anatomopatológico: las diversas presentaciones de la nefropatía por IgA / Clinicopathological case: the various presentations of IgA nephropathy

Se trata de una paciente de 58 años, sexo femenino, que se presenta con hematuria, proteinuria severa y función renal normal. Pocas semanas después, ella desarrolla una trombosis de vena renal, embolia pulmonar secundaria y un episodio de insuficiencia renal aguda. Este caso clínico ilustra las distintas presentaciones clínicas de una nefropatía por IgA grave, incluyendo hematuria, síndrome nefrótico y trombosis de vena renal. Además muestra otras complicaciones serias, como embolia pulmonar y falla renal aguda. La paciente fue sometida a 2 biopsias renales, que permitieron una correlación adecuada entre las manifestaciones clínicas y la patología renal.

This is a female, 58 years old patient, who presented with hematuria, heavy proteinuria and normal kidney function. Few weeks later she developed a renal venous thrombosis, pulmonary embolism and acute kidney injury. This clinical case illustrates the variable presenting features of a severe IgA nephropathy including hematuria, nephrotic syndrome and renal venous thrombosis. Further it shows its possible severe complications such as lung embolism and acute renal failure. The patient was kidney biopsied in two opportunities, which allows assessing the correlation between the variable clinical characteristics and the renal pathology.

Hereditary spherocytosis with immunoglobulin a nephropathy

Hereditary spherocytosis is a familial hemolytic anemia. Immunoglobulin A [IgA] nephropathy associated with hereditary spherocytosis has not been reported in children. Here, we report a case of a 17-year-old boy with IgA nephropathy and hereditary spherocytosis. The patient was diagnosed with hereditary spherocytosis at the age of 12 years and splenectomy was done at the age of 15 years. Later, the patient presented with macroscopic hematuria and proteinuria. Kidney biopsy of the boy was consistent with IgA nephropathy. Treatment with angiotensin-converting enzyme inhibitor was started. The patient became free of proteinuria after the 6th month of therapy