Organizing Pneumonia in A Patient Double-Positive for ANCA and Anti-GBM Antibodies: A Case Report / 中国医学科学杂志(英文版)

Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies.

Síndrome de Goodpasture en una preescolar / Goodpasture´s Syndrome in a pre-school girl

Introducción: El síndrome de Goodpasture es una rara enfermedad autoinmune que forma parte del espectro de síndrome pulmón-riñón Objetivo: Presentar un paciente pediátrico con síndrome de Goodpasture atípico. Presentación del caso: Paciente de seis años seguida por el servicio de nefrología; ingresó a los cuatro años de edad por presentar hematuria macroscópica asociada a manifestaciones respiratorias y antecedente de títulos elevados de anticuerpos antimembrana basal glomerular. Fue motivo de investigación y se diagnosticó el síndrome de Goodpasture. Después de tratamiento inmunosupresor con ciclofosfamida y metilprednisolona seguido de prednisona oral, la paciente presentó descenso de los títulos de anticuerpos antimembrana basal glomerular, así como mejoría de los síntomas respiratorios, sin embargo, la proteinuria se mantuvo con incremento en los últimos meses. Para el diagnóstico se tuvieron en cuenta las manifestaciones clínicas en la niña, título elevado de anticuerpos antimembrana basal glomerular y la confirmación por biopsia renal de glomerulopatía. El tratamiento fue rápidamente efectivo con una disminución inmediata en los títulos de anticuerpos antimembrana basal y mejoría evidente de la condición clínica de la paciente. Se trata del primer caso pediátrico con síndrome de Goodpasture publicado en Cuba. Conclusiones: Por tratarse de una entidad rara en pediatría se requiere un diagnóstico temprano y tratamiento agresivo para mejorar el pronóstico del paciente. En su seguimiento son necesarias una terapia farmacológica prolongada, una adecuada adherencia al tratamiento propuesto y un estrecho monitoreo clínico y analítico de lo cual dependerá la progresión de la injuria renal y pulmonar(AU)

Introduction: Goodpasture´s syndrome is a rare autoimmune disease that is part of the lung-kidney syndrome's sprectrum. Objective: To present the case of a pediatric patient with an atypical Goodpasture´s syndrome. Case presentation: Six years old female patient under follow-up in the Nephrology service. She was admitted when she was four years old by presenting macroscopic hematuria, respiratory symptoms and a history of high titers of anti-glomerular basement membrane antibodies. She was under research and was diagnosed with the Goodpasture´s symdrome. After being under immunosupressive treatment with cyclophosphamide and methylprednisolone followed by oral prednisone, the patient presented a decrease in the titers of anti-glomerular basement membrane antibodies, and an improvement of the respiratory symptoms; however, proteinuria kept increasing in the last months. For the diagnosis, there were taken into account the clinical manifestations of the girl, the high titers of anti-glomerular basement membrane antibodies and the confirmations of glumerulopathy by renal biopsy. The treatment was effective quickly with an inmmediate decrease of the titers of anti-glomerular basement membrane antibodies and an evident improvement of the clinical condition of the patient. This is the first pediatric case presenting Goodpasture´s syndrome that has been published in Cuba. Conclusions: As this is a rare entity in Pediatrics, it is required an early diagnosis and an aggressive treatment to improve the patient's prognosis. In the follow-up are needed a prolonged pharmacological therapy, an adequate adherence to the proposed treatment and a close clinical and analytic monitoring from which will depend the progression of the lung and renal injury(AU)

Síndrome pulmón-riñón: serie de 12 casos / Pulmonary renal syndrome: series of 12 cases

El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)

Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)

Recurrencia de Síndrome de Goodpasture con negativización de anticuerpos anti-membrana basal glomerular: caso clínico / Recurrence of goodpasture syndrome with negative antiglomerular basement antibodies: report of one case

Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Recurrence with negative anti-GBM antibodies in plasma is even less frequent We report a 63 years old male in whom anti-GBM disease recurred without detectable anti-GBM antibodies in plasma, despite having positive antibodies at the onset.

ANCA associated vasculitis: experience of a tertiary care referral center / Vasculite associada a ANCA: experiência de um centro de referência de atendimento terciário

ABSTRACT Background and objectives: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Outcomes of interest were: demographics, ANCA antibody positivity, patient and renal survival, and regional trends. Results: 986 biopsies were reviewed, 41 cases met the criteria for inclusion: 18 GPA, 19 MPA, and 4 double positive (anti-GBM disease plus ANCA vasculitis). Mean age at presentation was 52.4 years (SD 23.7), 23 (56%) were male and median creatinine was 2.6 mg/dL. The median patient follow up was 77 weeks (IQR 10 - 263 weeks), with a 3-month mortality rate of 5.7% and a 1-year estimated mortality rate of 12%. Thirteen patients required hemodialysis at the time of diagnosis; 7 patients came off dialysis, with median time to renal recovery of 4.86 weeks (IQR 1.57 - 23.85 weeks). C-ANCA positivity (p < 0.001) and C-ANCA plus PR3 antibody pairing (p = 0.005) was statistically significant in GPA versus MPA. P-ANCA positivity was observed in MPA versus GPA (p = 0.02) and double positive versus GPA (p = 0.002), with P-ANCA and MPO antibody pairing in MPA versus GPA (p = 0.044). Thirty-seven of the 41 cases were referred locally, 16 cases were from within a 15-mile radius of Albany, Schenectady, and Saratoga counties. Conclusions: ANCA vasculitis is associated with end stage renal disease and increased mortality. Our study suggests the possibility of higher regional incidence of pauci-immune GN in Upstate New York. Further studies should investigate the causes of clustering of cases to specific regions.

RESUMO Introdução e objetivos: A vasculite associada a anticorpos anticitoplasma de neutrófilo (ANCA) é uma vasculite de pequenos vasos com estimativas epidemiológicas insuficientes nos Estados Unidos. Nosso objetivo foi determinar características demográficas e clínicas de pacientes com vasculite associada à ANCA, apresentando-se a um grande centro de referência de atendimento terciário em Upstate New York. Formato, cenário, participantes e medidas: Foi realizada uma análise retrospectiva dos casos de GN pauci-imune em biópsias renais e diagnóstico clínico de vasculite ANCA por mais de 11 anos. Os resultados de interesse foram: dados demográficos, positividade de anticorpos ANCA, sobrevidas renal e de pacientes e tendências regionais. Resultados: 986 biópsias foram revisadas, 41 casos preencheram os critérios de inclusão: 18 GPA, 19 PAM, e 4 duplo-positivos (doença anti-MBG com vasculite ANCA). A média de idade na apresentação foi de 52,4 anos (DP 23,7), 23 (56%) eram do sexo masculino e mediana de creatinina de 2,6 mg/dL. O acompanhamento mediano dos pacientes foi de 77 semanas (IQR 10 - 263 semanas), com uma taxa de mortalidade de 3 meses de 5,7% e uma taxa de mortalidade estimada em 1 ano de 12%. Treze pacientes necessitaram de hemodiálise no momento do diagnóstico; 7 pacientes saíram da diálise, com tempo médio para recuperação renal de 4,86 semanas (IQR 1,57 - 23,85 semanas). A positividade para C-ANCA (p < 0,001) e o pareamento de anticorpos C-ANCA mais PR3 (p = 0,005) foram estatisticamente significantes em GPA versus PAM. A positividade de P-ANCA foi observada em PAM versus GPA (p = 0,02) e duplo positivo versus GPA (p = 0,002), com pareamento de anticorpos P-ANCA e MPO em PAM versus GPA (p = 0,044). Trinta e sete dos 41 casos foram encaminhados localmente, 16 casos foram de dentro de um raio de 15 milhas dos condados de Albany, Schenectady e Saratoga. Conclusões: A vasculite por ANCA está associada à doença renal terminal e aumento da mortalidade. Nosso estudo sugere a possibilidade de maior incidência regional de GN pauci-imune no norte do estado de Nova York. Novos estudos devem investigar as causas do acúmulo de casos em regiões específicas.

Concurrent Anti-glomerular Basement Membrane Nephritis and IgA Nephropathy

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

Granulomatosis con poliangitis en pediatría: a propósito de un caso con síndrome pulmón-riñón / Granulomatosis with polyangiitis in children: report of a case with kidney-lung syndrome

La granulomatosis con poliangitis como causa del síndrome pulmón-riñón es infrecuente en niños. Presentamos el caso de un paciente de 13 años con hemorragia alveolar, glomerulonefritis rápidamente progresiva, escleritis y anticuerpos anti-PR3 (proteinasa 3). Hacemos referencia a las características de esta vasculitis asociada a anticuerpos contra el citoplasma de los neutrófilos (ANCA) en los niños y a su enfoque terapéutico.

Granulomatosis with polyangiitis as a cause of kidney-lung syndrome is uncommon in children. We report the case of a 13 year old patient with alveolar hemorrhage, rapidly progressive glomerulonephritis, episcleritis and anti PR3. We refer to features of the ANCA-associated vasculitis in children and to its therapeutic approach.

Diffuse Alveolar Hemorrhage / 결핵및호흡기질환

Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

Clinical analysis of anti glomerular basement membrane disease with normal renal function / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate the clinical and pathologic characteristics of anti-glomerular basement membrane(GBM) disease with normal renal function.</p><p><b>METHODS</b>The clinical and pathologic data of 6 patients with anti-GBM disease and normal renal function in Peking Union Medical College Hospital were reviewed retrospectively. Furthermore, 29 patients with anti-GBM disease and impaired renal function in the same period in the same hospital were enrolled as the control group. Factors that may influence the prognosis were analyzed.</p><p><b>RESULTS</b>Six (17.1%) of all 35 patients maintained normal renal function for 12-133 months during follow-up. Five patients had microhematuria and proteinuria, one had pulmonary hemorrhage only, and three manifested as Goodpasture syndrome. Renal biopsies from 4 patients revealed linear deposition of IgG 2+-3+ along the glomerular capillary walls by immunofluorescence. As shown by normal light microscopy, mild mesangial proliferation and crescentic glomerulonephritis with a large amount of fibrinoid necrosis of glomerular capillary walls were observed in different patients; however, most pathological changes were mild. Five of these six patients were treated with immunosuppressive drugs and/or plasma exchange. Compared with the control group, the 6 patients with normal renal function had significantly higher hemoglobin[(77.97±20.62 vs.(99.67±19.80 g/L P=0.024], lower titers of anti-GBM antibody[(224.34 ± 145.79 vs.(80.23 ± 85.73 EU/ml P=0.027], and lower ratio of glomeruli with crescents[(0.58±0.29 vs.(0.17±0.27 ,P=0.005]. These 6 patients with normal renal function were followed up for 12-133 months, among whom 4 patients achieved complete remission and 2 had mild proteinuria and microhematuria.</p><p><b>CONCLUSION</b>Anti-GBM disease with normal renal function is not uncommon. Most patients have mild pathologic changes and good prognosis.</p>

Cellular components of crescents in four common types of crescentic glomerulonephritis / 中华病理学杂志

<p><b>OBJECTIVE</b>To examine the cellular components at different stages of the crescent formation in four most common types of human crescentic glomerulonephritis (CGN), including anti-GBM disease (GBM-CGN), crescentic IgA nephropathy (IgA-CGN), ANCA associated pauci-immune CGN (ANCA-CGN) and crescentic lupus glomerulonephritis (LN-CGN).</p><p><b>METHODS</b>Renal biopsy specimens of patients with GBM-CGN (n = 10), IgA-CGN (n = 12), ANCA-CGN (n = 12), and LN-CGN (n = 11) were selected. Immunohistochemistry was adopted to identify the cellular components using different cell markers including cytokeratin (PEC), CD68 (macrophage), nestin (podocyte), podocalyxin (podocyte), CD3 (lymphocyte), CD15 (neutrophil) and PCNA.</p><p><b>RESULTS</b>There were different subtypes of cell components identified during the formation of a cellular crescent in 4 different types of human CGN. Mainly of PEC 11.4 (0.0, 95.0)%, macrophage 8.0 (0.0, 35.0)% and podocyte 5.5 (0.0, 22.0)% and their constitutive percentages were different among various CGNs (P < 0.01). In all the CGNs studied, there were 50% of cells were negative to all the cell markers adopted for this expeiment. Podocalyxin positive cells 0.5 (0.0, 9.6)% were significantly less than nestin positive cells 5.5 (0.0, 22.0)% in all CGNs. PCNA positive cells were 44.7 (16.7, 83.3)% in the cellular crescent of all CGNs and co-localized with nestin (38/45 cases), CK (42/45 cases) or CD68 (24/45 cases).</p><p><b>CONCLUSIONS</b>PEC, macrophage and podocyte might play important roles in the formation of crescents. The staining disparity of nestin and podocalyxin indicates that podocyte dedifferentiation may occur during the crescent formation. PEC, podocytes and macrophages may participate in the formation of crescent in common CGNs through active cellular proliferation.</p>

Association of anti-glomerular basement membrane antibody disease with dermatomyositis and psoriasis: case report / Associação de doença do anticorpo anti-membrana basal glomerular com dermatomiosite e psoríase: relato de caso

CONTEXT: Anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.

CONTEXTO: A síndrome do anticorpo anti-membrana basal glomerular (anti-MBG) é caracterizada pela deposição de anticorpos anti-MBG em tecidos afetados, associada à glomerulonefrite e/ou ao envolvimento pulmonar. Essa síndrome já foi descrita em associação a outras doenças autoimunes, mas até onde conhecemos, não há relatos de sua associação com dermatomiosite e psoríase. RELATO DE CASO: Um homem de 51 anos com antecedentes de dermatomiosite e psoríase vulgar apresentou quadro de polineuropatia sensitivo-motora de mãos e pés, perda de 4 kg, adinamia e febre. À admissão estava em uso crônico de ciclosporina e de anti-hipertensivos há três meses devido a hipertensão arterial leve. Exames laboratoriais mostraram anemia e leucocitose, creatinina e ureia séricas elevadas e urina com proteinúria, hematúria, leucocitúria e cilindros granulosos. A proteinúria de 24 horas foi de 2,3 g. A biópsia renal revelou uma glomerulonefrite crescêntica necrotizante com depósitos lineares de imunoglobulina G (IgG) na MBG à imunofluorescência, sugestivos de anticorpos anti-MBG. O paciente foi então tratado inicialmente com metilprednisolona e com ciclofosfamida mensalmente na forma de pulsoterapia.

Concomitancia de anticuerpos antimembrana basal y ANCA en el síndrome riñón-pulmón / Concomitance of glomerular basement membrane antibodies and ANCA in pulmonary - renal syndrome

La enfermedad antimembrana basal glomerular (anti-MBG) es una condición que se manifiesta clínicamente como glomerulonefritis rápidamente progresiva y hemorragia alveolar, también llamada Síndrome Riñón- Pulmón. Se asocia a la presencia de autoanticuerpos dirigidos contra el colágeno tipo IV de la membrana basal glomerular. Las vasculitis sistémicas asociadas a ANCA también pueden manifestarse como Síndrome Riñón-Pulmón, cuadro clínico a veces indistinguible de la enfermedad anti-MBG. La concomitancia de ANCA y anticuerpos anti-MBG en el Síndrome Riñón-Pulmón es del orden de un 30 por ciento, según distintos reportes de la literatura. El perfil clínico, el pronóstico y el rol fisiopatológico de cada anticuerpo en este grupo de pacientes todavía son materia de investigación. El mecanismo patogénico inicial parece ser el daño mediado por ANCA, que puede inducir la aparición de anticuerpos anti-MBG, los que perpetúan el daño en el glomérulo.

Anti-glomerular basement membrane (anti-MBG) disease is a condition that is manifested clinically as rapidly progressive glomerulonephritis and alveolar hemorrhage, also known as Pulmonary-Renal Syndrome. It is associated with the presence of autoantibodies directed against type IV collagen of the glomerular basement membrane. Systemic vasculitis associated with ANCA may also manifest as Pulmonary-Renal Syndrome, sometimes clinically indistinguishable from the anti-MBG disease.The concomitance of ANCA and anti-MBG antibodies in the Pulmonary-Renal Syndrome is about 30 percent, according to various reports in literature. The clinical profile, prognosis and physiopathologic roles of each antibody in this group of patients is still under investigation. The pathogenic mechanism appears to be the initial damage mediated by ANCA, which may induce the appearance of anti-MBG, those who perpetuate the glomerulus damage.

A Case of Membranoproliferative Glomerulonephritis with False-Positive Reaction for Anti-GBM Antibody / 대한신장학회지

Anti-glomerular basement membrane (Anti-GBM) nephritis is an autoimmune disorder characterized by rapidly progressive crescentic glomerulonephritis (RPGN). The treatment of anti-GBM nephritis with plasmapheresis, steroids and immunosuppressant has improved outcomes. An early diagnosis is essential for the survival of patients and a recovery of renal function. The diagnosis of anti-GBM disease has been traditionally based on the demonstration of linear deposits of immunoglobulins along the glomerular basement membrane by immunofluorescence (IF) microscopy. However, a kidney biopsy cannot always be easily performed in such ill patients. Recent development of specific enzyme immunoassays for anti-GBM antibody in the serum has made possible a provisional diagnosis without a kidney biopsy. A 46-year-old male patient with hypertension and hepatitis B presented with generalized edema and general weakness. Laboratory findings were compatible with acute renal failure and nephrotic syndrome with positive serum anti-GBM antibodies. After plasmapheresis with steroid pulse therapy, renal biopsy was performed and diagnosed as membranoproliferative glomerulonephritis (MPGN) with granular deposit of Ig G and C3. Follow-up antibody titers were negative. This case demonstrates the possibility of false-positive anti-GBM antibody in the serum. Therefore, enzyme immunoassay for anti-GBM antibody should be used only as a screening or follow-up test in patients that have been confirmed positive by IF microscopy.

The Role of MCP 1 and IL 6 on the Progress of Crescentic Glomerulonephritis / 대한신장학회지

PURPOSE: Growing data on the relationship between cytokine expression and the progression of renal diseases make these cytokines potential targets for therapeutic interventions. Weexamined the helper T1-cell- and macrophage-associated cytokines in anti-glomerular basement membrane (GBM) antibody-induced nephritis in mice and their temporal relationships with renal tissue fibrosis. METHODS: Kidneys were harvested on days 1, 3, 7, 11, and 16 after glomerulonephritis was induced with anti-GBM antibody. The progression of renal fibrosis was serially monitored to quantitate the accumulation of cortical extracellular matrix, and various cytokines were measured simultaneously. RESULTS: A single injection of anti-GBM antibody successfully produced severe crescentic glomerulonephritis. Proteinuria increased abruptly and both mesangial matrix expansion and interstitial fibrosis progressed rapidly. Cortical fibronectin and type III collagen increased continuously, reaching a peak on day 7, and the deposition of type III collagen followed the same pattern, in parallel with that of urinary transforming growth factor 1 (TGF-1) expression. Serial cytokine measurements revealed a sustained increase in interleukin (IL) 6 and monocyte chemoattractant protein 1 (MCP1) from day 3, but neither IL12, IL18, nor interferon changed significantly. Real-time polymerase chain reaction confirmed these features at the transcription level. CONCLUSION: MCP1 and IL6 correlated with the progression of renal fibrosis, with no increase in Th1- inducing cytokines. This confirms MCP1 and IL6 as attractive therapeutic targets for renal fibrosis in crescentic glomerulonephritis.

A Case of Anti-Glomerular Basement Membrane Disease Improved by Early Plasmapheresis and Immunosuppression Therapy / 대한신장학회지

Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/or pulmonary hemorrhage. The presence of severe crescentic glomerular inflammation with linear deposition of immunoglobulin G along the glomerular basement membrane is pathognomonic. Because renal function is rapidly and often irretrievably destroyed, many patients require hemodialysis all through their lifetime. We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary hemorrhage. The patient was treated with pulse methylprednisolone and plasmapheresis followed by oral corticosteroid and cyclophosphamide. His renal function was successfully recovered with early diagnosis and aggressive treatment.

Enfermedades glomerulares durante la gestación. Serie de casos y revisión de la literatura / Glomerular disease and pregnancy

Objetivos: determinar las glomerulopatías más frecuentes y su curso clínico en mujeres gestantes. Hacer una revisión de la literatura del tema. Materiales y métodos: serie de casos retrospectivos de pacientes gestantes con enfermedad glomerular activa diagnosticada por biopsia, entre enero de 2000 y septiembre de 2007, atendidas en el Hospital San Vicente de Paúl de Medellín, Colombia. No hubo exclusiones. Se determinaron sus características clínicas, la evolución de la enfermedad glomerular y el resultado materno perinatal. Resultados: se hizo el diagnóstico en 11 pacientes. Cuatro casos de nefritis lúpica, dos de glomerulonefritis rápidamente progresiva (GNRP) por GN extracapilar y granulomatosis de Wegener, respectivamente; tres con síndrome nefrótico por glomeruloesclerosis focal y segmentaria (GEFyS), una con GN membrana proliferativa tipo I, y una con GN proliferativa mediada por complejos inmunes. En dos casos se presentó eclampsia. En tres casos hubo muerte materna: dos por eclampsia y una por granulomatosis de Wegener; y en tres casos muerte fetal: dos por eclampsia y una en materna con nefritis lúpica y síndrome antifosfolípido. Las glomerulopatías primarias no mostraron empeoramiento durante la gestación. Conclusiones: en casos de GN durante la gestación hay riesgo incrementado de complicaciones materno-fetales.

A Case of Goodpasture's Syndrome in a Foundry Worker / 대한산업의학회지

BACKGROUND: Smoking, upper respiratory tract infection, genetic factors and hydrocarbons are known as risk factors of Goodpasture's syndrome. We studied a patient with Goodpasture's syndrome who had worked for 27 years in a foundry company. Based on a study on the work-relatedness of the syndrome, we describe and discuss our study results. CASE: A 46-year-old man, who had worked as a foundry worker for 27 years and had a 12 1/2 packyear history of smoking cigarettes, was admitted into a hospital on 15th February 2006 with coughing, chest pain and dyspnea. On admission, he had hematuria, proteinuria, severe restrictive pulmonary function disorder and rapid elevation of blood urea nitrogen/creatinine. Immunological examination showed ANA (+), ANCA (-) and Anti-GBM Ab (+). Kidney biopsy showed pauci-immune crescentic glomerulonephritis. Mild bleeding was revealed through bronchoscopy and no vasculitis and granuloma were present on at lung biopsy. Finally, we diagnosed the worker's illness as Goodpasture's syndrome and carried out hemodialysis and plasmapheresis. In the workplace survey, the exposure level of respirable crystalline silica exceeded the TLV-TWA (0.0106 mg/m3), which was calibrated for overtime. CONCLUSION: Based on both the clinical test and industrial hygiene examination, we concluded that the Goodpasture's syndrome in this case was caused by long-term silica exposure.

A Case of Goodpasture's Syndrome in a Foundry Worker / 대한산업의학회지

BACKGROUND: Smoking, upper respiratory tract infection, genetic factors and hydrocarbons are known as risk factors of Goodpasture's syndrome. We studied a patient with Goodpasture's syndrome who had worked for 27 years in a foundry company. Based on a study on the work-relatedness of the syndrome, we describe and discuss our study results. CASE: A 46-year-old man, who had worked as a foundry worker for 27 years and had a 12 1/2 packyear history of smoking cigarettes, was admitted into a hospital on 15th February 2006 with coughing, chest pain and dyspnea. On admission, he had hematuria, proteinuria, severe restrictive pulmonary function disorder and rapid elevation of blood urea nitrogen/creatinine. Immunological examination showed ANA (+), ANCA (-) and Anti-GBM Ab (+). Kidney biopsy showed pauci-immune crescentic glomerulonephritis. Mild bleeding was revealed through bronchoscopy and no vasculitis and granuloma were present on at lung biopsy. Finally, we diagnosed the worker's illness as Goodpasture's syndrome and carried out hemodialysis and plasmapheresis. In the workplace survey, the exposure level of respirable crystalline silica exceeded the TLV-TWA (0.0106 mg/m3), which was calibrated for overtime. CONCLUSION: Based on both the clinical test and industrial hygiene examination, we concluded that the Goodpasture's syndrome in this case was caused by long-term silica exposure.

Differentiation of glomerular from non-glomerular hematuria by three different methods of microscopic examinations of erythrocytes in urine

Morphological examinations of urinary erythrocytes can be of diagnostic value in initial evaluation of hematuria. Dysmorphic urinary red blood cells are known to indicate a glomerular origin of bleeding. We examined the clinical usefulness of this test in a population complained of hematuria by use of three different light microscopy, phase contrast microscopy, and Wright staining and compared their sensitivity and specificity. The study included 169 patients with hematuria [89 glomerular and 80 non-glomerular]. The urine specimens were collected before invasive procedures such as biopsy and cystoscopy. In each urine sample, 100 urinary erythrocytes were examined. Statistical analysis was performed using Student's t test, correlation coefficient, and x. Reliability parameters including sensitivity, specificity and predictive values of negative and positive tests were also evaluated. Dysmorphic red cells were recorded as acanthocytes, doughnut-like cells, yeast like cells with more than one blebs and ghost forms. Isomorphic erythrocytes had uniform size and shape. Significant difference was found in the number of urinary dysmorphic red cells between the two groups of patients. Statistical analysis showed that by using percentage of glomerular type erythrocytes and setting the cut-off at 20-25%, the specificity for three procedures was almost the same [? 97.5%]. But sensitivity for light microscopy, phase contrast microscopy, and Wright staining was in different ranges as 70.7%, 89.8%, and 86.5% respectively. It was concluded that with some limitations, these simple, non-invasive techniques were useful in identifying the source of bleeding in the work up of hematuria by considering that sensitivity of the methods were in the order of phase contrast microscopy, Wright staining, and light microscopy