Reporte de un caso de glomerulonefritis rápidamente progresiva por anticuerpos antimembrana basal glomerular / Case report of rapidly progressive glomerulonephritis due to anti-glomerular basement membrane antibodies

RESUMEN Una vez más en medicina interna no podemos, aún, prescindir de los métodos invasivos para alcanzar un diagnóstico. Los avances diarios en el hallazgo de nuevas herramientas paraclínicas no permiten reemplazar aquellos métodos de certeza como la anatomía patológica. El caso presentado es una muestra de ello. Se trata de una mujer de 27 años de edad, con antecedente de tiroiditis de Hashimoto que consulta por presentar severo deterioro de la función renal asociado a oligoanuria. Realizamos una revisión del tratamiento de las glomerulonefritis rápidamente progresivas por anticuerpos antimembrana basal glomerular, serológicamente negativas.

ABSTRACT Once again in internal medicine we cannot do a diagnosis without invasive methods. Daily advances in the finding of new paraclinical tools do not allow the replacement of certain methods such as pathological anatomy. The case presented is a sample of this. This is a 27-year-old woman with a history of Hashimoto's thyroiditis who consults for presenting severe impairment of kidney function associated with oligoanuria. We performed a review of the treatment of the rapidly progressive glomerulonephritis for serologically negative anti-GBM antibodies.

Glomerulonefritis rápidamente progresiva de etiología posinfecciosa. Caso pediátrico / Postinfectious rapidly progressive glomerulonephritis in a pediatric patient

La glomerulonefritis rápidamente progresiva de etiología posinfecciosa es rara en la infancia, con una prevalencia estimada del 1-3 %. La mayoría debuta como insuficiencia renal aguda y su tratamiento se basa en el uso de corticoides y ciclofosfamida. Si se realiza diagnóstico precoz, el 70 % presenta una recuperación temprana de la función renal. En los últimos años, se han descrito "glomerulopatías por C3", que presentan características que se superponen. Son útiles, en el diagnóstico diferencial, la inmunofluorescencia y la determinación del factor nefrítico. Se presenta un varón de 4 años que acude por fiebre y cuadro respiratorio. Se observa microhematuria, proteinuria, descenso de filtrado glomerular y descenso de C3, y se sospecha glomerulonefritis aguda. Se realiza una biopsia, cuya microscopía óptica muestra la presencia de semilunas epiteliales, y la electrónica, depósitos subepiteliales en forma de joroba, por lo que se diagnostica glomerulonefritis rápidamente progresiva de etiología posinfecciosa.

Postinfectious glomerulonephritis is rarely presented as rapidly progressive glomerulonephritis in children; the prevalence is approximately 1-3 %. Most children have acute onset of renal failure; initial treatment involves corticosteroids and immunosuppressive therapy. Early diagnosis improves prognosis. In recent years, an entity known as "C3 glomerulopathies" has been described, presenting characteristics that overlap. In the differential diagnosis, the immunofluorescence and the determination of the nephritic factor are useful. We report a 4-year-old boy with fever, respiratory symptoms and hyporexia. Microhematuria, proteinuria, decline in glomerular filtration and depressed C3 were found. Acute glomerulonephritis was suspected. Renal biopsy showed crescent formation, immunofluorescence staining for C3 and subepithelial humps. Therefore, postinfectious glomerulonephritis with crescent formations was diagnosed.

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.

Glomerulonefritis rápidamente progresiva asociada con vasculitis por ANCA / Rapidly progressive glomerulonephritis associated with ANCA-vasculitis

Resumen El glomérulo renal, estructura altamente vascularizada, se ve afectado por un grupo de vasculitis de pequeño vaso asociadas con anticuerpos anticitoplasma de neutrófilo (ANCA), descritas por su relativa escasez de depósitos inmunes como pauciinmunes. Estas vasculitis se clasifican según el consenso internacional de Chapel-Hill en poliangeítis microscópica, granulomatosis con poliangeítis, granulomatosis eosinofílica y la llamada vasculitis limitada al riñón. Desde el punto de vista anatomopatológico, la glomerulonefritis rápidamente progresiva puede dividirse en pauciinmune, antimembrana basal glomerular (GBM GN) y mediada por inmunocomplejos. El tratamiento, en general, es con inmunosupresor y terapia renal de soporte desde medidas conservadoras hasta tratamiento de sustitución renal. Se comunica un caso de glomerulonefritis rápidamente progresiva asociada con ANCA por confirmación anatomopatológica.

Abstract The renal glomerulus highly vascularized structure is affected by a small vessel vasculitis group associated with neutrophil cytoplasmic antibodies (ANCA) described by its relative scarcity of pauci-immune deposits as immune. This vasculitis is classified according to international consensus in Chapel-Hill microscopic poliangetitis, granulomatosis with polyangiitis, eosinophilic granulomatosis and the called vasculitis limited to the kidney. From the anatomopathological point of view, the rapidly progressive glomerulonephritis can be divided into pauci-immune, anti glomerular basement membrane and mediated by immune complexes. The treatment is generally based on immunosuppressive therapy and renal therapy support from conservative measures to renal replacement therapy. This paper reports a case of rapidly progressive glomerulonephritis associated with ANCA pathological confirmation.

ANCA-associated rapidly progressive glomerulonephritis in a patient with IgA nephropathy, case report / Glomerulonefritis rápidamente progresiva asociada a ANCA en un paciente con nefropatía por IgA, reporte de caso

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.

Síndrome pulmón riñón, reporte de caso y revisión de la literatura / Pulmonary-renal syndrome, case report and literature review / Síndrome Pulmão-Rim, relato de um caso e revisão da literatura

Resumen: El complejo del síndrome pulmón riñón (SPR) se define como la combinación de hemorragia alveolar difusa acompañada de glomerulonefritis rápidamente progresiva, dentro del protocolo de diagnóstico se requieren niveles de anticuerpos anticitoplasma de neutrófilos (ANCA), broncoscopia, tomografía de tórax y estudio de sedimento urinario, es más común que se manifieste en el sexo masculino entre la edad de 40 y 55 años, siendo infrecuente en población joven. Se han descrito tres formas principales, la granulomatosis con poliangeítis, síndrome de Churg Strauss y síndrome de Goodpasture (SGP). En cualquiera de sus presentaciones se asocia a una elevada tasa de mortalidad que requiere manejo multidisciplinario con soporte ventilatorio, sustitución de la función renal, manejo específico con esteroide sistémico y terapia inmunosupresora. La introducción de la ciclofosfamida en combinación con esteroides anunció una alternativa en el tratamiento de las vasculitis observando disminución en la mortalidad en comparación con los glucocorticoides como monoterapia. El objetivo de este artículo es presentar el caso de un paciente con SPR y revisar la patogénesis, el abordaje diagnóstico y tratamiento, haciendo énfasis en la importancia del manejo multidisciplinario en la unidad de cuidados intensivos.

Abstract: The complex syndrome lung-kidney or pulmonary renal syndrome (PRS) is defined as the combination of diffuse alveolar hemorrhage accompanied by rapidly progressive glomerulonephritis, within the diagnostic protocol levels of anti-cytoplasm (ANCA), bronchoscopy, chest tomography and study required urinary sediment, occurs more commonly in males between 40 and 55 years, being rare in young people, described three main forms granulomatosis with polyangiitis, Churg Strauss syndrome Goodpasture (SGP), in any of its presentations is associated with a high mortality rate requiring multidisciplinary management with ventilatory support, replacement of renal function, specific management with systemic steroid and immunosuppressive therapy. The introduction of cyclophosphamide in combination with steroids announced an alternative in the treatment of vasculitis finding decrease mortality compared with glucocorticoids as monotherapy. The aim of this paper is to present the case of a patient with PSR, review the pathogenesis, diagnosis and treatment approach, emphasizing the importance of multidisciplinary management in the Intensive Care Unit.

Resumo: A complexa síndrome do Pulmão-Rim (SPR) é definida como a combinação de hemorragia alveolar difusa e glomerulonefrite rapidamente progressiva, dentro do protocolo de diagnóstico é necessário níveis de anticorpos anti citoplasmáticos de neutrófilos (ANCA), broncoscopia, tomografia torácica e estudo do sedimento urinário. A síndrome é mais frequente em homens entre 40 e 55 anos, sendo raro na população jovem. Descreveu-se três formas principais: Granulomatose com Poliangeíte, Síndrome de Churg Strauss e síndrome de Good-Pasture (SGP), em qualquer das suas apresentações é associada com uma alta taxa de mortalidade, requerendo uma abordagem multidisciplinar com suporte respiratório, substituição da função renal, tratamento específico com esteróide sistêmico e terapia imunossupressora. A introdução da ciclofosfamida combinada com esteróides é uma alternativa no tratamento da vasculite, diminuindo a mortalidade, em comparação aos glucocorticóides como monoterapia. O objetivo deste artigo é apresentar o caso de um paciente com SPR, revisar a patogênese, a abordagem diagnóstica e o tratamento, enfatizando a importância do tratamento multidisciplinar na unidade de terapia intensiva.

Nursing care of two cases of anti-glomerular basement membrane disease / 现代临床护理

Objective To summarize the experience in nursing 2 patients with anti-glomerular basement membrane disease.Method The nursing measures such as close observation of disease condition,careful nursing of medication,nursing during the treatment of double filtrated plasmapheresis and mental care to the patients and their family members.Results After treatment,the concentration of anti-GBM antibody was declined and the level of serum creatinine was also decreased.The renal function got rccovered to a certain extent.Conclusion Such nursing points as close observation of renal and pulmonary pathgenesis,timely and accurate administration of medicine according to doctor's orders and active observation and prevention of complication from double filtrated plasmapheresis are key.

Eosinophilic granulomatosis with polyangiitis accompanied by rapidly progressive glomerulonephritis

Eosinophilic granulomatosis with polyangitis (EGPA) should be considered in asthmatic patients who present with severe systemic symptoms and eosinophilia. Progressive renal insufficiency can occur during the acute phase of EGPA accompanied by renovascular involvement. A 58-year-old man visited local clinic with complaints of malaise, weight loss, fever, and dyspnea. Eosinophilia was revealed in peripheral blood. Pulmonary function tests were carried out, which yielded decreased lung function with positive bronchodilator response. Kidney and skin biopsies were performed, and histological examination showed acute necrotizing crescentic glomerulonephritis and leukoclastic vasculitis in the skin, which led to a diagnosis of EGPA (Churg-Strauss syndrome) associated with rapidly progressive glomerulonephritis. The patient received pulse steroid therapy with parenteral methylprednisolone, followed by oral prednisolone. Clinical and laboratory findings improved dramatically, and remission was attained rapidly. The patient continued to be in remission for 5 months. Prompt and aggressive treatment with systemic corticosteroids is mandatory to control disease activity and to achieve remission.

Síndrome pulmón-rinón: serie de casosdel Hospital Universitario San Ignacio / Pulmonary - renal syndrome: case series Hospital Universitario San Ignacio

El síndrome pulmón-rinón ˜ (SPR) se define como la presencia de hemorragiaalveolar y glomerulonefritis rápidamente progresiva. Fue descrito, inicialmente, como síndromeantimembrana basal glomerular, aunque existen otros mecanismos más frecuentesimplicados en la lesión, tales como vasculitis ANCA (anticitoplasma de neutrófilo), lupuseritematoso sistémico, síndrome antifosfolípido, crioglobulinemia y microangiopatía trombótica.Objetivo: Describir la experiencia de nuestro centro en el manejo de pacientes con SPR,analizar las variables demográficas, clínicas y sus desenlaces.Métodos: Se revisaron las bases de datos de la Unidad de Nefrología y la historia clínicaelectrónica SAHI del Hospital Universitario San Ignacio.Resultados: Se identificaron un total de 14 pacientes que presentaron SPR, entre enero de2009 y agosto de 2011, todos, inicialmente, manejados en la unidad de cuidado intensivodel hospital, con un promedio de edad de 44 anos ˜ y 57% de sexo femenino. El diagnósticomás frecuente fue vasculitis ANCA positivo en 8 pacientes, lupus en 4, un caso decrioglobulinemia y uno de microangiopatía trombótica. La mayoría recibió tratamiento concorticosteroides, ciclofosfamida y plasmaféresis. La mortalidad fue de 66%.Conclusiones: Presentamos los resultados de 14 pacientes con SPR en nuestro centro. La causamás frecuente fue vasculitis ANCA. Su presentación suele ser agresiva y el tratamientoincluye uso de esteroides, ciclofosfamida y plasmaféresis...

Pulmonary- renal syndrome (PRS) is defined as the presence of alveolar hemorrhageand rapidly progressive glomerulonephritis. It was initially described as glomerularbasement membrane syndrome, although other more common causes and mechanisms areinvolved such as, anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA), systemiclupus erythematosus, antiphospholipid syndrome, cryoglobulinemia, and thromboticmicroangiopathy.Objective: To describe the experience in the treatment of patients with PRS in our centerand analyze the demographic, clinical variables, and outcomes.Methods: The databases of the Nephrology Unit and the electronic medical records of thehospital were reviewed, and a total of 14 patients with PRS between January 2009 and August2011 were identified.Results: A total of 14 cases managed in the intensive care unit of the hospital where analyze,of which 57% were woman and the mean age was 44 years. The most frequent diagnosiswas positive ANCA vasculitis in 8 patients, lupus in 4, one case of cryoglobulinemia, andother with thrombotic microangiopathy. Most of them were treated with corticosteroids andcyclophosphamide, with 10 patients also receiving plasmapheresis. The overall mortalitywas 66%.Conclusions: The results of 14 patients with PRS in our center are presented. The mostcommon cause of this was positive ANCA vasculitis. It is an aggressive disease and itstreatment included the use of steroids, cyclophosphamide and plasmapheresis...

A Case of Crescentic Glomerulonephritis and Marginal Zone B-cell Lymphoma / 대한내과학회지

Secondary rapidly progressive glomerulonephritis (RPGN) can be caused by many diseases and conditions, including vasculitis, systemic rheumatic diseases, infections, drugs and malignancies. Among the secondary RPGNs, malignancy-associated RPGN is extremely rare and causes renal function deterioration within several weeks to months. Thus, timely immunosuppressant therapy can improve renal outcome. Herein, we describe a case of RPGN detected simultaneously with marginal zone B-cell lymphoma. An 82-year-old male patient, who presented generalized edema and oliguria, was diagnosed with crescentic glomerulonephritis and marginal B-cell lymphoma. After the patient was given methylprednisolone pulse therapy, renal function was restored and hemodialysis was successfully discontinued without complications.

A Case of Crescentic Glomerulonephritis and Marginal Zone B-cell Lymphoma / 대한내과학회지

Secondary rapidly progressive glomerulonephritis (RPGN) can be caused by many diseases and conditions, including vasculitis, systemic rheumatic diseases, infections, drugs and malignancies. Among the secondary RPGNs, malignancy-associated RPGN is extremely rare and causes renal function deterioration within several weeks to months. Thus, timely immunosuppressant therapy can improve renal outcome. Herein, we describe a case of RPGN detected simultaneously with marginal zone B-cell lymphoma. An 82-year-old male patient, who presented generalized edema and oliguria, was diagnosed with crescentic glomerulonephritis and marginal B-cell lymphoma. After the patient was given methylprednisolone pulse therapy, renal function was restored and hemodialysis was successfully discontinued without complications.

Características clínicas e histológicas de la glomerulonefritis rápidamente progresiva en pacientes atendidos en un hospital general / Clinical and histological characteristics of rapidly progressive glomerulonephritis in patients treated at a general hospital

Objetivos:Determinar las características clínicas e histológicas, de los pacientes con glomerulonefritis rápidamente progresiva (GNRP) atendidos en un hospital general. Métodos: Se evaluaron las biopsias renales hechas en el Hospital Nacional Arzobispo Loayza entre 1998 y 2008. Histológicamente se determinó GNRP cuando la biopsia tenía más del 50% de crecientes epiteliales. Se registró: edad, sexo, síndromenefrológico de presentación (insuficiencia renal, síndrome nefrítico, síndrome nefrótico, hipertensión arterial); parámetros inmunológicos (anti cuerpos antinucleares, anti DNA, ANCA, complemento 3, anti MBG, VSG) e histológicos (porcentaje de semilunas epiteliales, fibroepite liales, infiltrado intersticial y atrofiatubular) . Para describir los resultados se utilizó medidas de tendencia central y desviación estándar. Resultados: La GNRP representó el 2,72% de las biopsias renales. La edad promedio fue 33,7 ± 11,7 años. La creatinina promedio al ingreso fue 4,5 ± 4,8 (0,5 – 26,0) mg/dl. El 59,3% tenía HTA, el 48.1% tenía síndrome nefrótico y el 48.1% síndrome nefrítico. El 52,7% de los casos tuvieron ANCA positivo.El 60% tuvo C3 disminuido. El porcentaje desemilunas fue 71,2%. El infiltrado intersticialy la atrofia tubular fueron de grado leve en el 63 y 74% de los pacientes respectivamente. Las causas más frecuentes de GNRP fueron lupus eritematoso sistémico (44,4%) y vasculitis (37%). La estancia hospitalaria promedio fue de 30 días. El 51,8% requirió hemodiálisis y 33% continuó con hemodiálisis al alta. Conclusiones: La GNRP es una patología poco frecuente en nuestro hospital y la causa más frecuente fue LES.

Objectives: Assessing the clinical and histological characteristics of patients with rapidly progressive glomerulonephritis (RPGN) treated at a general hospital. Methods: The kidney biopsies conducted at Hospital Nacional Arzobispo Loayza between 1998 and 2008 were evaluated. RPGN was histologically determined when the biopsy had more than 50% of epithelial crescents. The following information was recorded: age, gender, kidney syndrome present (kidney failure, nephritic syndrome, nephrotic syndrome, arterial hypertension); immune parameters (antinuclear antibodies, anti DNA antibodies, ANCA, complement 3, anti GBM antibodies and ESR) and histological parameters (epithelial or fibroepithelial crescent percentage, interstitial infiltrates and tubular atrophy). In order to describe the results, measures of central tendency and standard deviation were used. Results: RPGN accounted for 2.72% of kidney biopsies. The average age was 33.77 ± 11.7 years. The ave rage creatinine at entry was 4.5 ± 4.8 (0.5 – 26.0) mg/dl. There was a 59.3% of patients with HTN; 48.1% had nephrotic syndrome and 48.1% had nephritic syndrome. There was a 52.7% of ANCA-positive cases, and 60% experienced C3 decrease. The crescent percentage was 71.2%. The interstitial infiltrate and the tubular atrophy were mild in 63 and 74% of the patients, respectively. The most frequent causes for RPGN were systemic lupus erythematosus (44.4%) and vasculitis (37%). Average length of stay in hospital was 30 days. A 51.8% of the patients required hemodialysis and 33% continued with hemodialysis upon discharge. Conclusions: RPGN is a rare disease at our hospital, and the most frequent cause for it was SLE.

Glomerulonefritis rápidamente progresiva en ancianos / Rapidly progressive crescentic glomerulonephritis in the elderly

La glomerulonefritis rápidamente progresiva (GNRP) es un síndrome clínico que se caracteriza por la presencia de signos urinarios de enfermedad glomerular e insuficiencia renal de desarrollo en un lapso de días a pocos meses. La inmunofluorescencia permite clasificar a las GNRP en cuatro tipos según se identifiquen o no depósitos inmunes y, si están presentes, de acuerdo con su naturaleza. En la última década se ha demostrado un aumento constante en el promedio de edad de los pacientes con GNRP. Este fenómeno podría reflejar tanto una mayor incidencia de la enfermedad, como un incremento en la tasa de diagnóstico. Se presentan 3 casos de GNRP en adultos mayores de 65 años, diagnosticados en un periodo de 3 meses en nuestra institución.

Rapidly progressive glomerulonephritis (RPGN) is a syndrome characterized by glomerular lesions giving rise to acute renal injury that develops within a brief period of time, usually days or a few months. It is classified according to the underlying mechanism of injury and the immunofluorescence findings into four main disorders. In the last decade, nephrologists have witnessed a steady rise in the mean age of the patients diagnosed with RPGN. This observation may reflect an increase in the incidence of this entity and also a more timely diagnosis. We present 3 cases of RPGN in elderly patients, diagnosed within a 3-month period at our institution which illustrates the spectrum of these conditions.

Etiology and Outcome of Crescentic Glomerulonephritis.

Objective: To determine the etiology, course and predictors of outcome in children with crescentic glomerulonephritis (GN). Study design: Retrospective, descriptive study. Setting: Pediatric Nephrology Clinic at a referral center in Northern India. Methods: Clinic records of patients aged <18 year with crescentic GN diagnosed from 2001-2010 and followed at least 12-months were reviewed. Crescentic GN, defined as crescents in ≥50% glomeruli, was classified based on immunofluorescence findings and serology. Risk factors for renal loss (chronic kidney disease stage 4-5) were determined. Results: Of 36 patients, (median age 10 yr) 17 had immune complex GN and 19 had pauci-immune crescentic GN. The etiologies of the former were lupus nephritis (n=4), postinfectious GN (3), and IgA nephropathy, Henoch Schonlein purpura and membranoproliferative GN type II (2 each). Three patients with pauci-immune GN showed antineutrophil cytoplasmic antibodies (ANCA). Rapidly progressive GN was present in 33 patients, and required dialysis in 12. At median 34 (19-72) months, 2 patients with immune complex GN and 8 with pauci-immune GN showed renal loss. Renal survival was 94.1% at 3 yr, and 75.3% at 8 yr in immune complex GN; in pauci-immune GN survival was 63.2% and 54.1%, respectively (P=0.054). Risk factors for renal loss were oliguria at presentation (hazards ratio, HR 10.50; P=0.037) and need for dialysis (HR 6.33; P=0.024); there was inverse association with proportion of normal glomeruli (HR 0.91; P=0.042). Conclusions: Pauci-immune GN constitutes one-half of patients with crescentic GN at this center. Patients with pauci-immune GN, chiefly ANCA negative, show higher risk of disease progression. Renal loss is related to severity of initial presentation and extent of glomerular involvement.

Renal histology in pauci-immune rapidly progressive glomerulonephritis: 8-year retrospective study.

Context: The need to perform reporting of renal biopsies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides in a more uniform manner required relook at our eight-year data. Aims: To document detailed renal histopathology of pauci-immune rapidly progressive glomerulonephritis (RPGN) and also to seek any significant differences in renal histology of C-ANCA-positive, P-ANCA-positive, and ANCA-negative patients. Materials and Methods: A detailed analysis of the histopathologic features of renal biopsies of 48 patients in whom a diagnosis of pauci-immune glomerulonephritis was concluded on renal biopsy and who presented clinically as rapidly progressive renal failure was done. Statistical Analysis Used: One-way ANOVA and Pearson Chi square tests. Results: Compared with ANCA +ve patients, the ANCA -ve patients were much younger (46.85 ± 16.12 years vs 34.28±15.94 years). No significant differences were found between renal lesions of C-ANCA, P-ANCA, and ANCA-negative patients, except for diffuse tubular atrophy which was more severe and more frequently present with P-ANCA positivity (P value=0.013). Conclusions: Pauci-immune RPGN (irrespective of ANCA status) is a relatively rare disorder in patients who are undergoing the renal biopsy at our institute, constituting 2% of all renal biopsies submitted. It is mandatory to have ANCA serology status during reporting of a kidney biopsy showing pauci-immune crescentic or necrotizing glomerulonephritis. Also, if a uniform reporting strategy is followed throughout the country, the studies from this vast country will be comparable.

A Case of Rapidly Progressive Glomerulonephritis in a Hepatitis B Virus Carrier Successfully Treated with High dose Immunosuppressive Therapy and Prophylactic Lamivudine / 대한신장학회지

A 35-year-old man, previously hepatitis B surface antigen (HBsAg) carrier, presented with gross hematuria and heavy proteinuria that he had been suffering from for 1 month. Serum creatinine was 4.4 mg/dL. Renal biopsy showed pauci-immune crescentic glomerulonephritis. He received plasmapheresis and was treated with high-dose steroids and cyclophosphamide. Lamivudine was started for the prevention of hepatitis B virus (HBV) activation. Serum creatinine and proteinuria were ameliorated one week after the treatment. There was no sign of HBV activation after six months of treatment. We report a case of rapidly progressive glomerulonephritis in a HBV carrier successfully treated with high dose immunosuppressive therapy and prophylactic lamivudine.

A Case of Poststreptococcal Glomerulonephritis Representing Rapidly Progressive Glomerulonephritis in an Elderly Patient / 대한신장학회잡지

Postinfectious streptococcal glomerulonephritis (PSGN) presenting as a rapidly progressive glomerulonephritis (RPGN) and nephrotic syndrome (NS) is a rare disease in elderly patients. Here we report a case of PSGN with RPGN and NS in an elderly male patient with a complete recovery from his illness. A 73-year-old man was admitted for dyspnea, oliguria and generalized edema appearing after acute upper respiratory infection. On admission, he presented nephrotic range of proteinuria, decreased renal function with elevated ASO, and decreased C3 and CH50 concentrations. The renal biopsy showed marked cellular crescents in the glomeruli with collapsed glomerular tufts and inflammatory cell infiltration. There were prominent and various sizes of "humps" in subepithelial areas in electron microscopy. Under the diagnosis of PSGN with RPGN, we successfully treated the patient with steroid pulse therapy and hemodialysis. We would suggest that early diagnosis and aggressive steroid therapy should be indicated in the treatment of PSGN with RPGN.

Rapidly Progressive Glomerulonephritis Induced by Antithyroid Agents in Children / 实用儿科临床杂志

Objective To explore the clinical characteristic of rapidly progressive glomerulonephritis(RPGN) induced by the antithyroid agents[propylthiouracil(PTU) and methimazole(MMI)].Methods The analysis was made according to the drug histories,clinical manifestation,serology investigations,renal biopsy in 2 children.Results Two cases presented with gross hematuria,proteinuria and renal dysfunction.One case with antineutrophil cytoplasmic antibody(ANCA)-positive and vasculitis lesion with cresent in the renal biopsy;another presented with mesengial proliferation and cresent.They both had been diagnosed RPGN induced by antithyroid agents for 5 years because of Graves disease.The treatment included that PTU and MMI were withdrawn and then combined prednisone with mycophenolate mofetil(MMF)were applied.One year after treatment,the urine analysis and renal function returned to normal.Conclusions The antithyroid agents may induce RPGN.A early diagnosis and immunosuppressive therapy can improve its prognosis of the disease.

Insuficiencia renal rápidamente progresiva por enfermedad antimembrana basal glomerular / Rapidly progressive renal failure due to anti-glomerular basement membrane disease

Se presentó el caso de una paciente de 18 años en la que se diagnosticó una insuficiencia renal rápidamente progresiva como resultado de una glomerulonefritis crecéntica por enfermedad antimembrana basal glomerular. La severidad del fallo renal agudo y la presencia del 100 % de crecientes en el estudio histológico obtenido mediante la biopsia renal, constituyeron elementos de mal pronóstico en la recuperación de la función renal. La valoración de los riesgos y beneficios en continuar con la terapia inmunosupresora deben ser considerados oportunamente.

A case of an 18-year-old patient who was diagnosed a rapidly progressive renal failure as a result of a crescentic glomerulonephritis due to anti-glomerular basement membrane disease, was presented. The severity of the acute renal failure and the presence of 100 % of crescents in the histological study obtained by kidney biopsy were elements of poor prognosis in the recovery of the renal function. The assessment of the risks and benefits to continue with immunosuppressive therapy should be conveniently considered.

A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children

PURPOSE: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. METHODS: The clinical manifestations and pathological findings were reviewed retro spectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. RESULTS: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glome rular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. CONCLUSION: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.