Nefropatía por IgA con microangiopatía trombótica / IgA nephropathy with thrombotic microangiopathy

INTRODUCTION: IgA nephropathy is the most common glomerulopathy in the world, it has a wide clinical expression, from asymptomatic to rapidly progressive glomerulonephritis. The definitive diagnosis is renal biopsy, within which the IgA pattern can be identified, including thrombotic microangiopathy. CLINICAL CASE: 28-year-old female patient, with a history of preeclampsia in the last pregnancy, presents high blood pressure, hematuria and proteinuria. Study begins with initially negative results. Renal biopsy confirms IgA nephropathy with thrombotic microangiopathy. DISCUSSION: Vascular damage is underestimated in IgA nephropathy. Thrombotic microangiopathy can be associated with various clinical manifestations, however when it is associated with IgA Nephropathy it is usually associated with proteinuria, arterial hypertension and elevation of creatinine. In the presence of microangiopathy, secondary causes must be ruled out. In general, there is no pathognomonic serological marker. Eventually patients could benefit from the use of eculizumab. CONCLUSION: IgA nephropathy is the most common glomerulopathy worldwide; there is a wide range of clinical presentations, among which thrombotic microangiopathy can be found. This presentation is associated with a higher risk of progression to end-stage renal disease.

INTRODUCCIÓN: La nefropatía por IgA es la glomerulopatía más frecuente en el mundo, tiene una amplia expresión clínica, desde asintomática hasta glomerulonefritis rápidamente progresivas. El diagnóstico definitivo es la biopsia renal, dentro de las cuales se puede identificar el patrón de la IgA, dentro de los cuales está la microangiopatía trombótica. CASO CLÍNICO: Paciente femenina 28 años, con antecedentes de preeclampsia en último embarazo, presenta hipertensión arterial, hematuria y proteinuria. Se inicia estudio con resultados inicialmente negativos. Biopsia renal confirma nefropatía por IgA con microangiopatía trombótica. DISCUSIÓN: En la nefropatía por IgA se subestima el daño vascular. La microangiopatía trombótica se puede asociar con varias manifestaciones clínicas, sin embargo, cuando está asociada a NIgA suele estar asociado con proteinuria, hipertensión arterial y elevación y creatinina. Ante la presencia de microangiopatía, se deben descartar causas secundarias de la misma. En general no existe un marcador serológico patognomónico. Eventualmente los pacientes se podrían beneficiar del uso de eculizumab. CONCLUSIÓN: La nefropatía por IgA es la glomerulopatía más frecuente a nivel mundial, existe una gran gama de presentaciones clínicas, dentro de las cuales se puede encontrar microangiopatía trombótica. Esta última presentación se asocia con mayor riesgo de progresión a enfermedad renal en etapa terminal.

Caracterización de enfermedades glomerulares: análisis de 22 años de biopsias renales / Frequency of glomerular diseases in an analysis of 550 kidney biopsies

BACKGROUND: The frequency of glomerular diseases is dynamic and varies according to geographic area. AIM: To evaluate the frequency of primary and secondary glomerulopathies, their demographic profile and main clinical characteristics. MATERIAL AND METHODS: Renal biopsies from native kidneys performed between 1999 and 2020 were retrospectively reviewed. Demographic characteristics, clinical presentation, most relevant laboratory tests, frequency of primary and secondary glomerulopathies were analyzed. RESULTS: We analyzed 550 kidney biopsies from patients with a median age of 48 years (64% females). Nephrotic syndrome was the main indication for renal biopsy. Primary and secondary glomerulopathies occurred with similar frequency. Within the primary glomerulopathies, membranous nephropathy (34.1%) was the most common, followed by IgA nephropathy (31.1%) and focal segmental glomerulosclerosis (14.1%). Among the secondary glomerulopathies, lupus nephropathy was the most common (41.7%), followed by pauciimmune glomerulonephritis (27.1%) and diabetic nephropathy (6.4%). When comparing the results with other regions, significant differences were observed with reported frequencies in United States, Europe, Asia and the rest of Latin America. CONCLUSIONS: The most common primary glomerulopathies were membranous nephropathy and IgA nephropathy. Among the secondary glomerulopathies lupus nephropathy and pauci-immune glomerulonephritis were the most common. Compared to international registries, we observed a high proportion of membranous nephropathy and pauci-immune glomerulonephritis.

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.

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.

Nefropatia por IgA em portadores de espondiloartrites acompanhados no Serviço de Reumatologia do Hospital das Clínicas da UFMG / IgA nephropathy in patients with spondyloarthritis followed-up at the Rheumatology Service of Hospital das Clínicas/UFMG

OBJETIVO: Determinar a frequência das glomerulonefrites nos pacientes espondiloartríticos acompanhados em Serviço de Reumatologia Brasileiro e avaliar variáveis clínicas correlacionadas. PACIENTES E MÉTODOS: Os pacientes foram avaliados quanto às características sociodemográfi cas, tipo de espondiloartrite, tempo e atividade da doença, uso de anti-infl amatórios não esteroides, presença do HLA-B27, níveis de creatinina e ureia séricas, presença de comorbidades e presença de hematúria e/ou proteinúria. Os pacientes com hematúria foram submetidos à pesquisa de dismorfi smo eritrocitário, e aqueles com proteinúria submeteram-se à quantifi cação da proteína na urina de 24 horas. Biópsia renal foi indicada para aqueles com hematúria de origem glomerular e/ou proteinúria maior que 3,5 g. RESULTADOS: Foram avaliados 76 pacientes. A alteração mais frequente no exame de urina de rotina foi a hematúria microscópica (44,7 por cento), geralmente intermitente e em amostra isolada de urina durante o seguimento do paciente. Em oito (10,5 por cento) dos pacientes a hematúria sugeriu origem glomerular. A biópsia renal foi realizada em cinco deles, e mostrou nefropatia por IgA em quatro (5,3 por cento) e doença da membrana fi na em um paciente. CONCLUSÕES: Notou-se alta frequência de alterações no exame de urina desse subgrupo de pacientes, assim como alta prevalência de nefropatia por IgA. Apesar de mais estudos sobre o assunto serem necessários para melhor esclarecimento desses resultados, a realização periódica de exames de urina deveria ser recomendável.

OBJECTIVE: To determine the frequency of glomerulonephritis in patients with spondyloarthritis followed-up at a Brazilian Rheumatology Service, and to evaluate the clinical variables associated. PATIENTS AND METHODS: Patients were assessed for sociodemographic characteristics, type of spondyloarthritis, time since diagnosis and disease activity, non-steroidal anti-infl ammatory drug use, HLA-B27 positivity, creatinine and urea serum levels, major comorbidities, hematuria and proteinuria. Patients with hematuria were subsequently assessed for the presence of dysmorphic red blood cells in urine, and those with proteinuria underwent 24-hour urine protein measurement. Renal biopsy was performed in patients with glomerular hematuria and/or proteinuria over 3.5 g/24-hour. RESULTS: Seventy-six patients were assessed. Microscopic hematuria was the most frequently found abnormality in urinalysis (44.7 percent), usually intermittent and in spot urine samples during patients' follow-up. In eight patients (10.5 percent), glomerular hematuria was suspected. Renal biopsy was performed in fi ve of them, showing IgA nephropathy in four (5.3 percent) and thin membrane disease in one patient. CONCLUSIONS: A high frequency of urinalysis alterations was observed in that subgroup of patients, as well as a high prevalence of IgA nephropathy. Although further studies on this subject are needed to better clarify these results, periodic urinalysis of patients with spondyloarthritis should be recommended.

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

Profile of glomerular diseases in a public hospital of Federal District, Brazil / Perfil das doenças glomerulares em um hospital público do Distrito Federal

INTRODUÇÃO: As doenças glomerulares são uma causa frequente de doença renal crônica, sobretudo nos países em desenvolvimento. OBJETIVO: O objetivo deste estudo foi determinar o perfil destas glomerulopatias em um hospital público da cidade de Brasília, Distrito Federal. MÉTODOS: Foram realizadas 121 biopsias renais pela equipe de nefrologia do Hospital Regional da Asa Norte (HRAN) entre agosto de 2005 e maio de 2009. Foram excluídas oito biopsias realizadas em pacientes transplantados renais e analisados os prontuários dos 113 pacientes restantes. Dados analisados: sexo, idade, exames laboratoriais, síndrome glomerular, diagnóstico clínico, grau de fibrose intersticial, uso de imunossupressores, necessidade de diálise e desfecho clínico. RESULTADOS: A média de idade foi 34,9 ± 16,2 anos, com predomínio masculino (51,3 por cento). As principais síndromes glomerulares foram: síndrome nefrótica (41,6 por cento) e glomerulonefrite rapidamente progressiva (35,4 por cento). Entre as glomerulopatias primárias, houve predomínio da glomeruloesclerose segmentar e focal (26,9 por cento) e da nefropatia por IgA (25 por cento) e entre as secundárias a nefrite lúpica (50 por cento) e a glomerulonefrite proliferativa exsudativa difusa (34,2 por cento). A maioria dos pacientes fez uso de imunossupressores (68,1 por cento) e quase um terço deles (29,2 por cento) necessitou de diálise durante a internação. Evoluíram para terapia dialítica crônica 13,3 por cento dos pacientes e 10,6 por cento evoluíram a óbito. CONCLUSÃO: Este estudo poderá contribuir para melhor entendimento epidemiológico das doenças glomerulares no Distrito Federal, orientando na adoção de políticas públicas visando permitir rápido diagnóstico e manejo clínico das mesmas.

INTRODUCTION: Glomerular diseases are a frequent etiology of chronic kidney disease, especially in the developing countries. OBJECTIVE: To determine the profile of such glomerulopathies in a public hospital located in the city of Brasilia, Federal District. METHODS: 121 renal biopsies in different patients were performed by the Renal Division of Hospital Regional da Asa Norte (HRAN) between August 2005 and May 2009. Eight renal biopsies in renal-transplant patients were excluded and the medical records of 113 remaining patients were analyzed. Analyzed data: sex, age, laboratory exams, glomerular syndrome, clinical diagnosis, degree of interstitial fibrosis, immunosuppressants use, need for dialysis and clinical outcome. RESULTS: The age average was 34.9 ± 16.2 years-old, a predominance of male patients (51.3 percent). Major glomerular syndromes were: nephrotic syndrome (41.6 percent) and the rapidly- progressive glomerulonephritis (35.4 percent). Among primary glomerulopathies focal glomerulosclerosis (26.8 percent) followed by IgA nephropathy (25 percent) were predominant; and among the most prevalent secondary glomerulopathies we had lupus nephritis (50 percent) and diffuse exudative proliferative glomerulonephritis (34.2 percent).The majority of the patients used immunosuppressants (68.1 percent) and almost one third of them (29.2 percent) needed dialysis during their hospitalization. Progressed to chronic dialysis therapy 13.3 percent of the patients and 10.6 percent died. CONCLUSION: This study may contribute to better epidemiological understanding of glomerular diseases in the Federal District, guiding the adoption of public policies aiming the quick clinical treatment of such diseases.

IgA nephropathy in Kerala, India: a retrospective study.

This study was conducted to assess the prevalence of IgA nephropathy in Kerala. No published data are available in literature from this part of India. This study included 1592 renal biopsies received from all parts of Kerala over a 2-year period. The age of the subjects ranged from 5 to 78 years old with a peak incidence in the third decade of life. The male:female ratio was 1.2:1. A total of 60% of the cases had presented with hematuria. Direct immunoflourescence was performed using specific antibodies for IgA, IgG, IgM, C3 and C1q. Light microscopic studies with special stains namely PAS, silver and trichrome were also done. A total of 227 cases (14.26% of all renal biopsies) were diagnosed as IgA excluding 12 cases of Henoch-Schonlein Purpura (12.74% in the first year and 15.52% in the second year). The prevalence of IgA nephropathy appears to be increasing in Kerala.

Glomerular diseases in a Hispanic population: review of a regional renal biopsy database / Enfermedades glomerulares en una población hispánica: resultados de un registro regional de biopsias renales

CONTEXT AND OBJECTIVE: Epidemiological data provide useful information for clinical practice and investigations. This study aimed to determine glomerular disease frequencies in a region of Colombia and it represents the basis for future studies. DESIGN AND SETTING: Single-center retrospective analysis at the University of Antioquia, Colombia. METHODS: All native renal biopsies (July 1998 to December 2007) were reviewed, but only glomerular diseases were analyzed. The diagnosis of each case was based on histological, immunopathological and clinical features. RESULTS: A total of 1,040 biopsies were included. In 302 cases (29.0 percent), the patient's age was < 15 years. Primary glomerular diseases were diagnosed in 828 biopsies (79.6 percent) and secondary in 212 (20.4 percent). The most common primary diseases were focal and segmental glomerulosclerosis (FSGS) (34.8 percent), immunoglobulin A (IgA) nephropathy (IgAN) (11.8 percent), membranous glomerulonephritis (MGN) (10.6 percent), minimal change disease (MCD) (10.6 percent), crescentic glomerulonephritis (GN) (5.6 percent), and non-IgA mesangial proliferative GN (5.6 percent). Postinfectious GN represented 10.7 percent of the diagnoses if included as primary GN. Lupus nephritis corresponded to 17.8 percent of the entire series. In adults, the order of the most frequent primary diseases was: FSGS, IgAN, MGN, crescentic GN and MCD. In children (< 15 years), the most frequent were: FSGS, postinfectious GN, MCD, non-IgA mesangial proliferative GN, endocapillary diffuse GN and IgAN. CONCLUSIONS: As among Afro-Americans, FSGS is the most frequent type of glomerulopathy in our population, but in our group, there are more cases of IgAN. The reasons for these findings are unclear. This information is an important contribution towards understanding the prevalence of renal diseases in Latin America.

CONTEXTO Y OBJETIVO: Los datos epidemiológicos dan información útil en clínica e investigación. Nuestro objetivo fue determinar frecuencias de enfermedad glomerular en una región de Colombia y representa la base para trabajos futuros. DISEÑO Y UBICACIÓN: Análisis retrospectivo en un único centro: Universidad de Antioquia, Colombia. MÉTODOS: Todas las biopsias de riñón nativo fueron revisadas (1998 - 2007), pero solo analizamos enfermedades glomerulares. El diagnóstico en cada caso estuvo basado en histología, inmunopatología y características clínicas. RESULTS: 1.040 biopsias fueron incluidas. En 302 casos (29,0 por ciento) la edad del paciente fue < 15 años. El diagnóstico fue enfermedad primaria en 828 biopsias (79,6 por ciento) y secundaria en 212 (20,4 por ciento). Las enfermedades primarias más frecuentes fueron glomeruloesclerosis focal y segmentaria (GEFyS) (34,8 por ciento), nefropatía IgA (NIgA) (11,8 por ciento), glomerulonefritis membranosa (GNM) (10,6 por ciento), enfermedad de cambios mínimos (ECM) (10,6 por ciento), glomerulonefritis (GN) extracapilar (5,6 por ciento) y GN proliferativa mesangial no-IgA (5,6 por ciento). La GN postinfecciosa representa el 10,7 por ciento de glomerulopatías primarias. La nefritis lúpica corresponde al 17,8 por ciento de todos los casos. En adultos el orden de frecuencia de enfermedades primarias es: GEFyS, NIgA, GNM, GN extracapilar y ECM. En niños (< 15 años) las más frecuentes fueron: GEFyS, GN postinfecciosa, ECM, GN mesangial no-IgA, GN endocapilar difusa y NIgA. CONCLUSIONES: Al igual que en afro-americanos, en nuestra población la GEFyS es la glomerulopatía más frecuente, pero en nuestro grupo hay más NIgA. Las razones para estos hallazgos no se conocen. La información presentada aquí es una contribución importante para el entendimiento de la prevalencia de enfermedades renales en Latinoamérica.

Non-neoplastic renal diseases in Kerala, India--analysis of 1592 cases, a two year retrospective study.

A retrospective analysis of 1592 renal biopsies received from various hospitals all over Kerala over a period of two years was done. Of the 1544 native kidney biopsies majority of cases (300; 18.84%) were focal segmental glomerulosclerosis. Next infrequency was IgA nephropathy (227; 14.26%). The other diagnoses included post infectious glomerulonephritis (GN) (150; 9.42%), lupus nephritis (131; 8.23%), membranous glomerulonephritis (118; 7.41%), minimal change disease (94; 5.94%), interstitial nephritis 87(5.46%), membranoproliferative glomerulonephritis (MPGN) and severe glomerulosclerosis (74; 4.64% each), IgM nephropathy (72; 4.52%), acute tubular necrosis and diabetic glomerulopathy (24; 1.5% each), ANCA-related GN (21;1.32%), amyloidosis (13; 0.82%), Henoch Schonlien purpura (12; 0.75%) atd thrombotic microangiopathy (10; 0.63%). Afew others like myeloma cast nephropathy, cholesterol embolism, Clq nephropathy, oxalosis, mixed connective tissue disorder (MCTD, Wegner's granulomatosis etc. also were seen occasionally. 48 cases were allograft biopsies (3.02%).

Clinical characteristics and histopathological findings in 120 IgA nephropathy patients in Thailand.

The authors reviewed the clinical and pathological findings in 120 IgA-nephropathy (IgAN) patients diagnosed at King Chulalongkorn University Hospital from 1994 to 2005. The average age of the patients was 36 years. Male and female ratio was 1:1.2. Thirty percent of the patients had hypertension. Thirty-four percent of the patients had serum creatinine greater than 1.5 mg/dl and had urine protein greater than 3 g/day. The most common presentation was asymptomatic urinary abnormalities (43%) followed by nephrotic syndrome (36%). Of note, many of the presented patients had advanced pathological classification and high tubulointerstitial (TI) fibrotic score. The clinico-pathological correlation was found significantly between serum creatinine and degree of TI fibrotic score by univariate analyses. Compared to other reports from Asian countries, the presented population had many worse prognostic markers including a decline in renal function, advanced pathologic findings, and high TI fibrotic scores. Further study on prognosis in Thai patients should be performed to help decision making in management of IgAN patients.

A glomerulopatia da imunoglobulina a na síndrome de anormalidades urinárias / The IGA glomerulopathy in the syndrome of urinary abnormalities

Foram analisadas 32 biópsias renais realizadas no Serviço de Nefrologia do HU-U.F.J.F. A glomerulopatia da imunoglobulina A (GlgA) foi a doença glomerular mais freqüente (25%), manifestando-se principalmente como síndrome de anormalidades urinárias (SAU) assintomática. Demonstrou-se ainda que os indivíduos com GlgA apresentaram tempo conhecido de doença até a realização da biópsia renal significativamente maior (p<0,05), traduzindo uma demora na elucidação da doença renal. Por outro lado, a depuração da creatinina plasmática nos casos de GlgA foi significativamente maior (p<0,05) do que aquela de outras doenças glomerulares. Observou-se que nos sete casos de GlgA com SAU, a esclerose focal e segmentar foi a glomerulopatia presente em quatro casos. A GlgA é uma doença glomerular freqüente em nosso meio, manifestando-se principalmente como síndrome de anormalidades urinárias assintomáticas. Estratégias clínicas e terapêuticas deverão ser traçadas com o objetivo de retardar a progressão da GlgA para insuficiência renal crônica.

The authors assessed 32 renal biopsies done at our Unit, and found that Immunoglobulin A (IgA) glomerulopathy was the most frequent glomerulonephritis, particularly among patients who presented with abnormal urinary syndrome. It was observed that the time course between clinical presentation and renal biopsy was longer time to confirm the diagnosis of the disease. On ther hand, creatinine clearance was higher in patients with IgA gomerulopathy (p<0,05) when compared to other histological patterns. The IgA glomerulopathy is frequent in our region, and its clinical prescritation is mainly as abnormal urinary syndrome. Clinical and therapeutic strategies should be used aiming to delay the progression of IgA nephroparhy to end stage renal failure.

Hipotiroidismo autoinmune asociado a nefropatía por IgA

La nefropatía por IgA, cuya incidencia en la población pediátrica oscila entre 1.5 y 56.21 según reportes publicados en el sur de Europa, Australia y Japón, se reporta como primera causa de glomerulonefritis primaria en niños. La nefropatía por IgA puede ser primaria o secundaria, se diagnostica por demostración de IgA predominantemente mesangial o IgA codominante en el glomérulo sin evidencia de lupus eritematoso. La mayoría de los pacientes presenta hematuria macroscópica y el pronóstico depende de los marcadores genéticos y la severidad de la lesión histológica renal. A continuación presentamos el reporte de un caso de una paciente con evidencia de hipotiroidismo primario autoinmune y nefropatía por IgA.

Glomerulonephritis in Kelantan, Malaysia: a review of the histological pattern.

Renal biopsy is essential in the management of renal parenchymal diseases. Thus far there is no publish report on the pattern of glomerulonephritis in Kelantan. We decided to establish the pattern of glomerulonephritis in Kelantan and use this information as our reference in future studies. Records of patients who had proven glomerulonephritis histologically were analysed. Their biological data, clinical presentation, etiology and clinicopathological pattern were studied. Where appropriate mean and standard deviation were calculated. A total of 74 biopsies were performed during the study period (between January 1991 and December 1993), out of which 72 biopsies (97.3%) were considered suitable for analysis. The male to female ratio was 1:1.1. Mean age at presentation was 27.6 +/- 12.2 years. Nephrotic syndrome was the commonest clinical presentation (65.3%). The main underlying cause was systemic lupus erythematosus (50%) followed by primary glomerulonephritis. Histologically, IgA nephropathy and minimal change disease were the main patterns among patients with primary glomerulonephritis while diffuse proliferative glomerulonephritis was the commonest pattern among patients with lupus nephritis. Hence the pattern of glomerulonephritis is similar to other reported series. The procedure is considered safe and has a high success rate.

IgA associated glomerulonephritis.

Immunohistological analysis of 1146 renal biopsies revealed IgA associated glomerulonephritis (IgAGN) in 83 (7.24%) patients (33 children, 50 adults). Clinical features were unusually severe in a high proportion. Nephrotic syndrome (NS) responding poorly to prednisolone was found in 24%, hypertension (HT) in 39%, and azotemia in 34% of patients. NS was slightly more frequent in children than in adults, but HT and azotemia occurred twice as often in adults as in children. Histologically, extensive glomerular crescents and sclerosis were prominent. In addition, moderate arteriolitis and arteriolosclerosis and marked tubulointerstitial nephropathy were notable features. Thus, a low incidence and marked severity characterized IgAGN in this study.

IgA nephropathy in Malaysia.

A total of 164 patients with IgA nephropathy were diagnosed at the Department of Medicine, Universiti Kebangsaan Malaysia and the Department of Nephrology, General Hospital, Kuala Lumpur between 1981-1988. This represented an incidence of 20.1% of all primary glomerulopathies seen in both units. The 3 major ethnic groups were equally affected with 59.7% occurring between the ages of 20-36 years. It was not uncommon in females. The high prevalence of hypertension, renal failure, heavy proteinuria at presentation and the increased chronicity index in the biopsy, suggest that IgA nephropathy is progressive disease leading to chronic renal failure.

Nefropatía IgA / IgA nephropathy

Se revisa la nefropatía por IgA en sus diferentes aspectos. Se destaca su alta prevalencia entre el conjunto de las glomerulopatías, así como la dificultad en establecer diagnóstico en muchos casos paucisintomáticos por la no realización de biopsia renal con inmunofluorescencia. Se expone la clasificación histopatológica de la OMS, que reconoce 5 variantes y los criterios diagnósticos exigidos en la inmunofluorescencia. Se analizan algunos aspectos del sistema inmunitario de la IgA y ciertas posibilidades patogénicas planteadas en la nefropatía por IgA. Se describen las distintas formas de inicio clínico (sobre todo la hematuria macroscópica recidivante y las alteraciones asintomáticas del sedimento urinario), así como la evolución prolongada, en general, hacia la insuficiencia renal. Se discuten los factores pronósticos reconocidos, tanto clínicos como histológicos. Se presenta además una serie nacional de 15 pacientes analizando su presentación y evolución clínica