ABSTRACT
Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary. Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II. Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.
Resumo Introdução: Alguns casos de nefropatia membranosa (NM) apresentam glomeruloesclerose segmentar e focal (GESF) tipicamente associada a progressão da doença. Contudo, relatamos o caso de uma paciente que parece ter NM e GESF, ambas primárias. Apresentação do caso: Uma jovem branca de 17 anos de idade com edema de membros inferiores associado a episódios de urina espumosa e hipertensão apresentou-se com achados físicos e laboratoriais sugestivos de síndrome nefrótica. Foi realizada biópsia renal. GESF foi observada por microscopia de luz em alguns glomérulos que apresentavam lesões de ponta, enquanto em outros o achado era acompanhado por hipertrofia podocitária e descolamento de podócitos no espaço urinário, compatíveis com podocitopatia GESF. Além disso, as alças capilares estavam espessadas com irregularidades na membrana basal devido a "espículas" compatíveis com NM estágio II. Imunofluorescência revelou depósitos finamente granulares de IgG, IgG4 e PLA2R nas alças capilares. Microscopia eletrônica exibiu depósitos subepiteliais e apagamento de pedicelos. Tais achados morfológicos são compatíveis com GESF e NM estágio II. Conclusões: No presente caso, as características clínicas e morfológicas revelaram uma possível sobreposição de GESF primária e NM, uma vez que a glomeruloesclerose segmentar e focal não parece estar relacionada com a progressão da NM, mas com a podocitopatia GESF.
Subject(s)
Humans , Female , Adolescent , Glomerulosclerosis, Focal Segmental/complications , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulonephritis, Membranous/complications , Glomerulonephritis, Membranous/diagnosis , Nephrotic Syndrome/complications , Nephrotic Syndrome/diagnosis , Biopsy , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Glomerulosclerosis, Focal Segmental/pathology , Glomerulosclerosis, Focal Segmental/drug therapy , Glomerulonephritis, Membranous/pathology , Glomerulonephritis, Membranous/drug therapy , Treatment Outcome , Kidney/pathology , Nephrotic Syndrome/drug therapyABSTRACT
We report a 19 years old male presenting with knee pain, elevated liver enzymes and proteinuria. Further investigation found positive antinuclear and anti-smooth muscle antibodies and a liver biopsy revealed the presence of an autoimmune hepatitis. Treatment with corticosteroids and azathioprine was started, resulting in normalization of liver enzymes but proteinuria persisted and a kidney biopsy disclosed a focal segmental glomerulosclerosis. The use of lisinopril resulted in a significative reduction of proteinuria and, after 30 months of follow up, he continues with azathioprine, lisinopril and a low prednisone dose without evidence of liver or kidney disease activity.
Subject(s)
Humans , Male , Young Adult , Proteinuria/complications , Glomerulosclerosis, Focal Segmental/complications , Hepatitis, Autoimmune/complications , Proteinuria/diagnosis , Proteinuria/immunology , Proteinuria/drug therapy , Immunohistochemistry , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulosclerosis, Focal Segmental/immunology , Autoimmunity , Hepatitis, Autoimmune/diagnosis , Hepatitis, Autoimmune/immunology , Diagnosis, Differential , Kidney/pathology , Liver/pathologySubject(s)
Humans , Female , Child , Scleroderma, Systemic/complications , Glomerulosclerosis, Focal Segmental/etiology , Nephrotic Syndrome/etiology , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/drug therapy , Glomerulosclerosis, Focal Segmental/diagnosis , Methotrexate/administration & dosage , Immunosuppressive Agents/administration & dosage , Nails/blood supply , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/pathologyABSTRACT
Resumo Introdução: As glomerulopatias são as doenças renais mais frequentemente diagnosticáveis por biópsia. O levantamento epidemiológico das glomerulopatias permite identificar sua distribuição e principais etiologias e serve de subsídio para definição de estratégias de prevenção e tratamento. Objetivo: O presente estudo pretende identificar a frequência e a correlação clínico-patológica das glomerulopatias diagnosticadas por biópsia no HC-UFPR durante 5 anos. Métodos: Foram realizadas 131 biópsias no período de 1 de janeiro de 2008 a 31 de dezembro de 2012, submetidas à microscopia óptica e de imunofluorescência. Todas as lâminas de microscopia óptica foram revistas por um patologista. Dados clínicos e laboratoriais e resultados da microscopia de imunofluorescência foram obtidos por revisão dos prontuários. Resultados: Foram reanalisados 128 de 131 casos; 46,5% foram obtidos em homens. A idade média de realização da biópsia foi 43 anos para os homens e 38 anos para as mulheres. Em 99 casos identificou-se a indicação da biópsia; 49,5% apresentaram síndrome nefrótica; 17,17%, insuficiência renal aguda e 15,15% insuficiência renal crônica; 8,08%, síndrome nefrítica; 6,06%, proteinúria isolada e 4,04%, hematúria isolada. 61,21% tratavam-se de glomerulopatia secundária, 33,62% glomerulopatia primária e 5,17% não puderam ser classificados. Dentre as glomerulopatias secundárias, a mais frequente foi a nefrite lúpica (49,29%), e, dentre as primárias, glomeruloesclerose segmentar e focal (30,77%) e nefropatia membranosa (25,64%). Conclusão: O paciente com glomerulopatia neste serviço é adulto e portador de síndrome nefrótica. Ao contrário de outros relatos, observamos predomínio das glomerulopatias secundárias, refletindo possivelmente o perfil terciário de atendimento do HC-UFPR.
Resumo Introduction: The glomerulopathies are the most common biopsy-proven kidney diseases. The epidemiological investigation of glomerulopathies allows the identification of their distribution and main causes and enables the development of prevention and treatment strategies. Objective: This study aims to identify the frequency and clinical-pathological correlation of glomerular diseases diagnosed at the HC-UFPR over the period of 5 years. Methods: 131 biopsies were performed between January 1, 2008 and December 31, 2012 and were analysed by light and immunofluorescence microscopy. Histopathological slides were reviewed by a pathologist. Clinical and laboratory data and the immunofluorescence microscopy results were extracted from medical records. The findings were tabulated and analysed. Results: 128 of 131 cases were reanalysed. 46.5% were obtained from men. Patients' age averaged 43 years for men and 38 for women. In 99 cases, the indication of biopsy was identified; 49.5% cases presented nephrotic syndrome, 17.17%, acute renal failure and 15.15%, chronic renal failure; 8.08%, nephritic syndrome; 6.06%, isolated proteinuria and 4.04% isolated hematuria. In 61.21% an underlying disease related to the glomerulopathy could be identified; 33.62% corresponded to primary disease and in 5.17% of cases the nature of the glomerulopathy could not be determined. Among secondary glomerulopathies, the most frequent was Lupus Nephritis (49.29%), and among the primary, Focal Segmental Glomerulosclerosis (30.77%) and Membranous Nephropathy (25.64%). Conclusion: The average patient with glomerulopathy in this service is an adult with nephrotic syndrome. Unlike other reports, secondary glomerulopathies were predominant. These findings may reflect the tertiary characteristic of the assistance at HC-UFPR.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Kidney Diseases/diagnosis , Kidney Diseases/pathology , Biopsy , Lupus Nephritis/diagnosis , Brazil , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulonephritis, Membranous/diagnosis , Retrospective Studies , Tertiary Care Centers , Nephrotic Syndrome/diagnosisABSTRACT
BACKGROUND/AIMS: Recurrent focal segmental glomerulosclerosis (FSGS) following renal transplantation is relatively common. However, the risk factors and optimal pretransplant treatment preventing recurrence of FSGS remain controversial. METHODS: We retrospectively reviewed 27 adult renal transplant recipients with FSGS over a period of 10 years. We first compared possible risk factors for FSGS recurrence between the recurrence and nonrecurrence groups. Then we evaluated the effect of pretransplant plasmapheresis (PP; n = 4) and PP with rituximab (PP + RTX; n = 5) on recurrence of FSGS after transplantation compared to control patients that were not treated with these modalities. RESULTS: There were seven recurrences in 27 patients (25.9%), but there were no significant differences in possible risk factors for FSGS recurrence between the two groups. Recurrence rates between patients with pretransplant PP or PP + RTX and control patients were not significantly different (22.2% vs. 27.7%, p > 0.05). There was also no significant difference in recurrence between the pretransplant PP and PP + RTX groups (25% vs. 20%, p > 0.05). CONCLUSIONS: Pretransplant PP or PP + RTX do not significantly decrease the recurrence of FSGS in adult renal transplant candidates.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Glomerulosclerosis, Focal Segmental/diagnosis , Immunosuppressive Agents/administration & dosage , Kidney Transplantation/adverse effects , Plasmapheresis , Recurrence , Retrospective Studies , Risk Factors , Treatment OutcomeABSTRACT
The collapsing variant of focal segmental glomerulosclerosis (FSGS) is a renal injury that may be idiopathic or associated with various factors; it is characterized by glomerular collapse, which leads to steroid-resistant nephrotic syndrome (NS) and progressive chronic renal failure. FSGS has not been well studied in children, in which most of the cases are idiopathic. We report six cases of the collapsing variant of FSGS in HIV-negative children who were resistant to immunosuppressive treatment. Three of the children died.
La variedad colapsante de glomeruloesclerosis focal y segmentaria (GEFS) es una lesión renal que puede ser idiopática o estar asociada a diferentes factores; se caracteriza por colapso glomerular que lleva a un síndrome nefrótico corticorresistente y a falla renal crónica progresiva. Ha sido poco estudiada en niños y en ellos la mayoría de los casos son idiopáticos. Presentamos seis casos de esta variedad de GEFS en niños negativos para VIH, resistentes al tratamiento inmunosupresor; tres de ellos murieron.
Subject(s)
Child , Case Reports , Case Reports , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulosclerosis, Focal Segmental/pathology , Glomerulosclerosis, Focal Segmental/therapy , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulosclerosis, Focal Segmental/pathology , Glomerulosclerosis, Focal Segmental/therapyABSTRACT
A síndrome de Hadju-Cheney é uma doença genética caracterizada por dismorfismos craniofaciais e alterações ósseas responsáveis pelo fenótipo da doença. As alterações renais, como cistos renais corticais, refluxo vesico - ureteral e falência renal, são raramente relatadas, mas são incluídas como apresentações menos comuns. O diagnóstico genético ainda não está disponível e a patogênese é relacionada a mutações no gene NOTCH. Os autores relatam um caso de um homem de 26 anos; porém, com características fenotípicas de um paciente pediátrico. Ele se apresentou com síndrome nefrótica, hipertensão arterial, cistos renais corticais e insuficiência renal aguda requerendo hemodiálise. A biopsia renal evidenciou glomeruloesclerose focal e segmentar e o tratamento para esse paciente foi de suporte com terapia hemodialítica. O diagnóstico da síndrome de Hadju-Cheney foi dado durante investigação do quadro renal.
Hajdu-Cheney disease is characterized by craniofacial dimorphisms and skeletal changes. Renal disturbs; such as renal cortical cysts, vesico-ureteral reflux and renal failure are rarely related but it is included as a less common feature. The diagnosis is not yet available and the pathogenesis it is related with mutations in the NOTCH gene. The authors report a case of a 26-years-old boy; but with phenotypic characteristics of a pediatric patient. He presented nephrotic syndrome, hypertension, renal cortical cysts, nephrotic range proteinuria and acute renal failure requiring hemodialysis. The renal tissue showed global and segmental glomerulosclerosis and the treatment to this patient it was supporting with hemodialysis. The diagnosis of Hadju-Cheney disease was given during investigation of renal function.
Subject(s)
Adult , Humans , Male , Glomerulosclerosis, Focal Segmental/complications , Hajdu-Cheney Syndrome/complications , Renal Insufficiency/complications , Glomerulosclerosis, Focal Segmental/diagnosis , Hajdu-Cheney Syndrome/diagnosis , Renal Insufficiency/diagnosisABSTRACT
Some patients with clinical and/or laboratory diagnosis of systemic lupus erythematosus (SLE) present with nephritis which from the morphological point of view does not fit in one of the 6 classes described in the WHO classification of lupus nephritis. On the other hand, nonlupus nephritis in patients with confirmed SLE is rarely reported. This condition may not be so uncommon as it seems. The associated glomerular lesions most frequently described are amyloidosis and focal segmental glomerulosclerosis (FSGS). We report on a 46 year-old, caucasian woman, who fulfilled the American College of Rheumatology criteria for SLE diagnosis: arthritis, positive anti-DNA, ANA, anti-Sm antibodies, and cutaneous maculae. During the follow-up, she presented arthralgias, alopecia, vasculitis, lower extremities edema and decreased serum levels of C3 and C4. Proteinuria was initially nephrotic, but reached negative levels. The serum creatinine varied from 0.7 to 3.0 mg/dl. The patient was submitted to the first renal biopsy at admission and to the second one, 3 years later, with diagnosis of minimal change disease and FSGS, respectively. No deposits were demonstrated by immunofluorescence. In the present case, we believe that the patient had SLE and developed an idiopathic disease of the minimal change disease-FSGS spectrum.
Alguns pacientes com diagnóstico clínico e/ou laboratorial de lúpus eritematoso sistêmico (LES) apresentam nefrite que, do ponto de vista morfológico,não se enquadra em qualquer das 6 classes de nefrite lúpica descritas na classificação da OMS. Por outro lado, nefrites nãolúpicas em pacientes comLES confirmado são raramente relatadas. Essa condição pode não ser tão incomum como parece. As lesões glomerulares associadas maisfreqüentemente descritas são amiloidose e glomerulosclerose segmentar e focal (GESF). Nós relatamos o caso de uma mulher de 46 anos, branca, quepreenchia critérios da Associação Americana de Reumatologia para diagnóstico de LES: artrite, FAN, anti-DNA e anti-Sm positivos, presença de máculas cutâneas. Durante o seguimento, ela apresentou artralgias, alopécia, vasculite, edema de membros inferires e níveis baixos de C3 e C4 séricos. A proteinúria que inicialmente era nefrótica chegou a negativar. A creatinina sérica variou de 0,7 a 3,0 mg/dl. A paciente foi submetida à sua primeira biópsia renal quando da chegada ao serviço e à segunda, 3 anos mais tarde, com diagnósticos de doença de lesões mínimas e GESF, respectivamente; aimunofluorescência revelou-se negativa. No presente caso, acreditamos que a paciente tinha LES e desenvolveu uma doença idiopática do espectrodoença de lesões mínimas-GESF.
Subject(s)
Humans , Female , Middle Aged , Glomerulosclerosis, Focal Segmental/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathologyABSTRACT
La glomerulopatía colapsante (GC) es una forma agresiva de daño glomerular definida por los datos histológicos de colapso de los capilares glomerulares, lesión de las células epiteliales viscerales y daño tubulointersticial característico. Los pacientes con glomerulopatía colapsante presentan datos clínicos que consisten en síndrome nefrótico grave, generalmente con proteinuria mayor de 10 g/24 horas, y progresión rápida a insuficiencia renal terminal o a la muerte por complicaciones del síndrome nefrótico, a pesar de cualquier forma de tratamiento. La GC, afecta a personas de cualquier sexo con ligera predominancia en el sexo masculino y en personas jóvenes. Se puede presentar como recidiva o de novo en el injerto renal. En algunos países predomina en la raza negra como sucede con las enfermedades renales en general. La GC comparte algunos datos clínicos e histológicos con la esclerosis focal y segmentaria recidivante y la nefropatía asociada al VIH, por lo que ha habido cierta controversia cerca de que se trate de una variante de estas enfermedades. Existe, sin embargo, evidencia clinicopatológica suficiente para separarla como una entidad diferente, aunque es posible que la esclerosis focal y segmentaria recidivante, la nefropatía asociada al SIDA y la glomerulopatía colapsante compartan un mecanismo fisiopatogénico común
Subject(s)
Glomerulonephritis, Membranous/complications , AIDS-Associated Nephropathy/diagnosis , Nephrotic Syndrome/diagnosis , Glomerulosclerosis, Focal Segmental/diagnosis , Renal Insufficiency, Chronic/physiopathologyABSTRACT
La Hialinosis Focal y Segmentaria (HFS), idiopática o secundaria, es una afección frecuente. Su patogenia no es aún bien conocida, en los últimos años, al mecanismo de hiperfiltración e hipertensión glomerular se asocia el rol patogénico de los factores de crecimiento e hipertrofia glomerular. La presentación clínica más habitual es el Sindrome Nefrótico con pobre respuesta al tratamiento esteroideo y evolución en plazos variables a la insuficiencia renal extrema. Los factores predictivos de evolución a la pérdida de la función renal, son el rango nefrótico de la proteinuria, los niveles elevados de creatinina sérica inicial y la severidad de las lesiones túbulointersticiales. La mejor evolución de la función renal de los pacientes cuya proteinuria responde al tratamiento, justifica tratarlos
Subject(s)
Humans , Kidney Glomerulus/pathology , Glomerulosclerosis, Focal Segmental/drug therapy , Glomerulosclerosis, Focal Segmental/physiopathology , Nephrotic Syndrome/complications , Cyclosporine , Cyclosporine/therapeutic use , Glomerulosclerosis, Focal Segmental/diagnosis , Prednisone/therapeutic useABSTRACT
En un estudio colaborativo de 99 niños con síndrome nefrótico en un período de 30 meses, 8 (5 mujeres) eran menores de un año (mediana 7,5 meses) y constituyen el motivo de esta comunicación. Los niños cuyo síndrome nefrótico se expresa antes del cuarto mes de vida tienen mal pronóstico, pues morfológicamente sus lesiones se relacionan con esclerosis mesangial difusa o el tipo finlandés y fallecen precozmente de complicaciones infecciosas severas. Los pacientes menores de 1 año con glomeruloesclerosis focal y segmentaria o cambios mínimos se comportan como los de edades posteriores