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 de Goodpasture atípico como reto diagnóstico / Atypical Goodpasture syndrome as diagnostic challenge

Resumen La enfermedad de Goodpasture es una entidad clínica con una base fisiopatológica inmunológica como punto de partida, en la que la unión al dominio terminal NC1 afecta principalmente en la zona glomerular y pulmonar, con lo que su diagnóstico está orientado hacia estas insuficiencias orgánicas; su incidencia varía de 0.5 a 1 casos por millón de habitantes, por lo que se considera una enfermedad poco frecuente y de mortalidad elevada debido a las complicaciones derivadas del mecanismo inmunológico. La manifestación pulmonar de manera aislada representa un porcentaje aún menor; la hemoptisis es, incluso en 66% de los casos, la forma más frecuente de expresión clínica. El pronóstico de estos pacientes se asocia con la forma de manifestación de la enfermedad y la calidad en la atención recibida. El diagnóstico definitivo se realiza mediante toma de biopsia; sin embargo, la existencia de anticuerpos anti-MBG (antimembrana basal glomerular) mediante la prueba ELISA puede manejarse en el diagnóstico con buena sensibilidad.

Abstract Goodpasture's disease is a clinical entity with an immunological pathophysiological basis, where the union to terminal NC1 domain mainly affects glomerular and lungs; thus, its diagnosis is oriented to such organ failure; its incidence varies from 0.5 to 1 cases per million population, for this reason it is considered a rare disease with high mortality due to complications from immune mechanism. Isolated lung presentation accounts for an even fewer percentage; in two thirds of cases hemoptysis is the most common clinical expression of the disease. Prognosis of these patients is associated to the presentation of the disease and the quality of care provided during the same. The definitive diagnosis is made by biopsy; however, the presence of antiGBM antibodies (glomerular basement membrane) by ELISA can be handled in the diagnosis with good sensitivity.

HRCT diagnosis of Goodpasture syndrome / 实用放射学杂志

Objective To analyze the imaging features and follow-up changes of high-resolution CT(HRCT) in Goodpasture syndrome.Methods HRCT imaging features and follow-up findings of 15 cases Goodpasture syndrome confirmed by clinical were analyzed retrospectrively.The imaging features included extent,forms and follow-up changes.Results The lung lesion of Goodpasture syndrome involved two lobes(n=1), three lobes(n=2), four lobes(n=5) and five lobes (n=7).Upper lobe of the right lung was the most common involved region.Centered on the hilum of lung consolidations confused ground glass opacity (GGO) were showed in 7 cases, GGO distribution of pulmonary leaflets in 5 cases.On follow-up observation, lobar or segmental consolidation could change into GGO,GGO could disappear in short times.Conclusion Multiple lobar or segmental consolidations confused GGO without the lung bottom and periphery involvement is the imaging characteristics of Goodpasture syndrome patients with anemia and hemoptysis.HRCT is a helpful method for the diagnosis and following up of Goodpasture syndrome.

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.

A Case of Goodpasture's Syndrome in a Patient with Graves' Disease / 대한신장학회잡지

Goodpasture's syndrome (GS) is characterized by lung hemorrhage and glomerulonephritis and caused by autoimmune reaction between anti-glomerular basement membrane (GBM) autoantibodies and the alpha 3 (type IV) collagen chain. Some reports suggested that patients with anti-GBM autoantibody could be related with other autoimmune diseases including Graves' disease. We report a case of 14-year-old girl with Graves' disease treated with PTU for 4 years, who was admitted because of hemoptysis and dyspnea. Laboratory values included a serum creatinine value of 0.7 mg/dL, BUN 22 mg/dL, hemoglobin 3.9 g/mm3 and albumin 3.2 mg/dL. The thyroid function tests showed normal serum T3 and free T4, suppressed TSH, and elevated thyroglobulin Ab and TSH-R-Ab levels. Urinalysis showed 2+ for protein and many dysmorphic RBC/HPF. Both anti-GBM Ab and pANCA were positive serologically. In renal biopsy, the glomeruli showed mesangial proliferation and crescent formation with linear deposits of IgG along the GBM. This case is to represent the GS of patient with Graves' disease referring to medical documents.

A Case of Goodpasture's Syndrome with Normal Renal Function / 결핵및호흡기질환

Goodpasture's syndrome is a disease that is characterized by hemoptysis, anemia, and glomerulonephritis with renal failure. Goodpasture reported a case of a young man who expired as a result of a pulmonary hemorrhage and glomerulonephritis at the recovery phase after an influenza infection in 1919. In 1958, Stanton et al. described a combined case of these two diseases as Goodpasture's syndrome. Since then, antiglomerular basement membrane antibody(anti-GBM Ab) has been confirmed to play an important role in the mechanism of this syndrome, and it was reported that this syndrome was an autoimmune disease. The triad of alveolar hemorrhage, glomerulonephritis and circulating anti-GBM Ab forms the basis of a diagnosis of Goodpasture's syndrome. When patients are affected by disease, the relief of symptoms can be accomplished by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoabsorption. However, the patients usually die from a massive pulmonary hemorrhage when the diagnosis or treatment is delayed. The incidence of Goodpasture's syndrome is common in the western world, but it is extremely rare in Korea with only five cases being reported. In three of these cases, pulmonary hemorrhage and renal failure was the initial manifestation. Therefore, hemodialysis or plasmapheresis were absolutely essential treatments. We report a case of Goodpasture's syndrome in Korea with a normal renal function.

A Case of Goodpasture's Syndrome / 대한신장학회잡지

Goodpasture's syndrome is an autoimmune disease characterized by rapid progressive glomerulonephritis, alveolar hemorrhage and autoantibody to the NC1 domain of the alpha 3 chain of type IV collagen (anti-glomerular basement membrane antibody). It was common in Europe with peak incidence at third decades. However, Goodpasture's syndrome was reported only three cases in Korea. We reported a case of Goodpasture's syndrome in a 34 year old male. He admitted our hospital due to dyspnea and hemoptysis. We made diagnosis by detection of Anti-GBM Ab and biopsy of kidney. He was treated with hemodialysis, plasma exchanges and immunosuppressive agents. However, his renal function was not recovered. Currently, he is receiving regular hemodialysis.

A Case of Renal Transplantation in a Patient with Goodpasture's Syndrome / 대한이식학회지

Goodpasture's syndrome is a clinical complex of anti-GBM (glomerular basement membrane) nephritis and lung hemorrhage. Anti-GBM nephritis is an autoimmune disease in which autoantibodies directed against type IV collagen induce RPGN (rapid progressive glomerulonephropathy) and crescentic glomerulonephritis. 50 to 70% of patients have pulmonary hemorrhage. We have one case of a successful renal transplantation in a patient with Goodpasture's syndrome. A 51 year old male had arrived in the emergency room due to dyspnea. 20 days prior to admission he had suffered from fever and then progressively developed nausea and weakness. He underwent hemodialysis with the dual lumen catheter which was inserted in the right internal jugular vein. Goodpasture's syndrome was confirmed by the measurement of serum anti-GBM Ab titer, renal biopsy and clinical manifestations of pulmonary hemorrhage. Renal biopsy findings showed diffuse proliferative glomerulonephritis on light microscopy and linear ribbon-like deposition of IgG along the GBM. The patient was placed on CAPD on the 30th hospital day. After six months of CAPD, the patient received a renal transplant from a HLA-haploidendical brother. Which was done after negative seroconversion of circulating antibody of GBM. With a induction of IL-2 receptor blocker (Basiliximab), cyclosporine-A and prednisone were administered for their immunosuppressants. He had good health for 37 months with excellent graft function.

A case of Goodpasture syndrome with diffuse pulmonary hemorrhage / 대한내과학회지

Goodpasture syndrome is an autoimmune disease with a triad of acute renal failure due to rapidly progressive glomerulonephritis (RPGN), pulmonay hemorrhage and circulating anti-glomerular basement membrane antibody (anti-GBM Ab). It was commonly reported from Europe in male with a peak incidence in their 20's. If patients are affected with the disease, relief of symptoms can be expected by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoadsorption. However, if the diagnosis or treatment is delayed, the patients usually die from massive pulmonary hemorrhage. It has been revealed that the main target of anti-GBM Ab's is NC1 domain on the alpha3 chain of type IV collagen. Currently there are many studies underway using this information as a basis to identify the pathogenesis of Goodpasture syndrome and to develop new therapeutic approach. The patient was a 20-year-old male with a chief complaint of edema. Unlike patients in the two previous cases, reported in Korea who had massive hemorrhage, he showed diffuse pulmonary hemorrhage which improved in only one week by hemodialysis. Renal biopsy demonstrated crescents in over 90% of glomeruli and showed signs of acute renal failure due to RPGN, with 618 U/mL (normal range <19.9 U/mL) of anti-GBM Ab titer.