IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship? / Vasculite por IgA (púrpura de Henoch-Schönlein), nefrite e psoríase em uma criança: existe relação?

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

The Oxford Classification predictors of chronic kidney disease in pediatric patients with IgA nephropathy / Preditores da doença renal crônica segundo a Classificação de Oxford em pacientes pediátricos com nefropatia por IgA

Abstract Objective: The Oxford Classification for Immunoglobulin A nephropathy (IgAN) identifies pathological variables that may predict the decline of renal function. This study aimed to evaluate the Oxford Classification variables as predictors of renal dysfunction in a cohort of Brazilian children and adolescents with IgAN. Methods: A total of 54 patients with IgAN biopsied from 1982 to 2010 were assessed. Biopsies were re-evaluated and classified according to the Oxford Classification. Multivariate analysis of laboratory and pathological data was performed. The primary outcomes were decline of baseline estimated glomerular filtration rate (eGFR) greater than or equal to 50%. Results: Mean follow-up was 7.6 ± 5.0 years. Mean renal survival was 13.5 ± 0.8 years and probability of decline ≥50% in baseline eGFR was 8% at five years of follow-up and 15% at ten years. Ten children (18.5%) had a decline of baseline eGFR ≥ 50% and five (9.3%) evolved to end-stage renal disease. Kaplan-Meier analysis showed that baseline proteinuria, proteinuria during follow-up, endocapillary proliferation, and tubular atrophy/interstitial fibrosis were associated with the primary outcome. Multivariate Cox analysis showed that only baseline proteinuria (HR, 1.73; 95% CI, 1.20-2.50, p = 0.003) and endocapillary hypercellularity (HR, 37.18; 95% CI, 3.85-358.94, p = 0.002) were independent predictors of renal dysfunction. No other pathological variable was associated with eGFR decline in the multivariate analysis. Conclusion: This is the first cohort study that evaluated the predictive role of the Oxford Classification in pediatric patients with IgAN from South America. Endocapillary proliferation was the unique pathological feature that independently predicted renal outcome.

Resumo Objetivo: A Classificação Oxford para a Nefropatia por Imunoglobulina A (IgAN) identificou variáveis patológicas de risco para disfunção renal. O presente estudo teve como objetivo avaliar as variáveis da Classificação de Oxford como preditores de disfunção renal em crianças brasileiras com IgAN. Métodos: Foram analisados 54 pacientes com diagnóstico de IgAN entre 1982-2010. As biópsias renais foram reavaliadas pela Classificação de Oxford. Foram feitas análises uni e multivariada das variáveis clínicas e patológicas. O desfecho primário foi queda da taxa de filtração glomerular (TFG) ≥ 50% da filtração basal. Resultados: O acompanhamento médio foi de 7,6 ± 5,0 anos. A sobrevida renal média foi de 13,5 ± 0,8 anos e a probabilidade de atingir o desfecho primário foi de 8% em cinco anos e 15% em 10 anos de seguimento. Dez crianças (18,5%) apresentaram queda na TFG basal ≥ 50% e cinco (9,3%) evoluíram para doença renal crônica terminal. A análise de Kaplan-Meier mostrou que a proteinúria basal e de seguimento, a proliferação endocapilar e a atrofia tubular/fibrose intersticial foram associadas com o desfecho primário. A análise multivariada de Cox mostrou que a proteinúria basal (HR = 1,73; IC95% 1,20-2,50, p = 0,003) e a proliferação endocapilar (HR = 37,18; IC95% 3,85-358,94, p = 0,002) foram preditores independentes de disfunção renal. Nenhuma outra variável patológica foi associada com declínio da TFG na análise multivariada. Conclusão: Este é o primeiro estudo brasileiro que avaliou a Classificação Oxford em crianças com IgAN. A proliferação endocapilar foi a única característica patológica capaz de predizer independentemente o declínio da função renal.

Púrpura de Schönlein-Henoch en adulto HIV positivo adicto a cocaína y ANCA-p positivo / Henoch-Schönlein purpura in a cocaine consumer man with HIV infection and ANCA-p positivity

La púrpura de Schönlein-Henoch (PSH) es una vasculitis de pequeños vasos con depósito de inmunocomplejos de IgA. La presentación en adultos es rara y grave. Los casos comunicados de PSH en pacientes infectados por HIV son escasos. Los anticuerpos anticitoplasma de neutrófilos (ANCA), detectados por inmunofluorescencia indirecta (IFI), se encuentran comúnmente en otras vasculitis sistémicas primarias pero rara vez en PSH y es aún más inusual el patrón perinuclear (ANCA-p). Por otra parte, la positividad de los ANCA puede ser detectada en una serie de condiciones patológicas distintas de las vasculitis de pequeños vasos en asociación a procesos infecciosos, incluyendo el HIV, o a consumo de cocaína, y en especial el patrón de ANCA-p vinculado a medicamentos o enfermedades autoinmunes e inflamatorias intestinales. Se presenta el caso de un varón de 35 años con hábitos tóxicos (cocaína, marihuana) que consultó por dolor abdominal, hematoquecia y lesiones purpúricas en extremidades inferiores, que agregó fiebre, artralgias y progresión de lesiones purpúricas asociadas a síndrome nefrítico y ANCA-p (+). Durante la internación se detectó infección por HIV. La biopsia renal evidenció una nefropatía a IgA que tuvo una respuesta favorable al tratamiento corticoideo y antiproteinúrico. La comunicación del caso obedece a la rareza de la presentación y a la complejidad para establecer el diagnóstico y la terapéutica. Aún resta dilucidar el rol de los ANCA en la etiopatogenia y el manejo de la PSH del adulto.

The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.

Co-existing proteinase 3-antineutrophil cytoplasmic antibody-associated vasculitis with immunoglobulin A nephropathy

No abstract available.

Nefropatia por IgA e polimiosite: uma rara associação / IgA nephropathy and polymyositis: a rare association

A polimiosite é uma miopatia inflamatória idiopática sistêmica que, além da manifestação muscular, pode eventualmente cursar com acometimento respiratório, do trato gastrintestinal e, raramente, renal. Neste último caso, há descrição de apenas dois casos de nefropatia por IgA em pacientes com miopatia, ambos em dermatomiosite. Em contrapartida, relatamos pela primeira vez esta rara associação em polimiosite.

Polymyositis is a systemic and idiopathic inflammatory myopathy that, besides muscle manifestation, may occur with respiratory involvement, gastrointestinal tract and rarely renal involvement. In this latter, there are only two cases of IgA nephropathy, but both in dermatomyositis. On the other hand, we reported, for the first time, a case of IgA nephropathy in polymyositis.

Corticotherapy response in primary IgA nephropathy / Resposta à corticoterapia na nefropatia da IgA primária

INTRODUCTION: Some beneficial effects from long-term use of corticosteroids have been reported in patients with IgA nephropathy. OBJECTIVE: This retrospective study aimed to evaluate the outcome of proteinuria and renal function according to a protocol based on a 6-month course of steroid treatment. METHOD: Twelve patients were treated with 1 g/day intravenous methylprednisolone for 3 consecutive days at the beginning of months 1, 3, and 5 plus 0.5 mg/kg oral prednisone on alternate days for 6 months (treated group). The control group included 9 untreated patients. RESULTS: Proteinuria (median and 25th and 75th percentiles) at baseline in the treated group was 1861 mg/24h (1518; 2417 mg/24h) and was 703 mg/24h (245; 983) and 684 mg/24h (266; 1023) at the 6th (p < 0.05 vs. baseline) and 12th months (p < 0.05 vs. baseline), respectively. In the control group the proteinuria was 1900 mg/24h (1620; 3197) at baseline and was 2290 mg/24h (1500; 2975) and 1600 mg/24h (1180; 2395) at the 6th and 12th months, respectively (not significant vs. baseline). When compared with the control group, the treated group showed lower proteinuria (p < 0.05) during the follow-up and a higher number of patients in remission (p < 0.05) at the 6th and 12th months. Renal function did not change during the follow-up and the adverse effects were mild in most of the patients. CONCLUSION: The 6-month course of steroid treatment was effective in reducing proteinuria during the 12 months of the follow-up, and was well-tolerated by most of the patients.

INTRODUÇÃO: Tem sido sugerido que tratamento mais prolongado com corticosteroides pode ser benéfico em pacientes com nefropatia da IgA primária. OBJETIVO: Neste estudo retrospectivo avaliamos os efeitos na proteinúria e na função renal após 12 meses do protocolo baseado no uso por 6 meses de corticosteroides. MÉTODO: Doze pacientes receberam pulsos de 1 g/dia de metilprednisolona intravenosa por 3 dias consecutivos no início dos meses 1, 3 e 5, seguidos por prednisona (0,5 mg/kg) por via oral em dias alternados após cada pulso durante 6 meses (grupo tratado). O grupo controle foi composto por nove pacientes não tratados. RESULTADOS: A proteinúria (mg/24h; mediana; 25º; 75º percentis) no período basal no grupo tratado foi de 1861 (1518; 2417) e de 703 (245; 983) e de 684 (266; 1023) nos 6º (p < 0,05 vs. basal) e 12º (p < 0,05 vs. basal) meses, respectivamente. No grupo controle, a proteinúria foi de 1900 (1620; 3197) no período basal e de 2290 (1500; 2975) e de 1600 (1180; 2395) nos 6º e 12º meses, respectivamente (não significantes vs. basal). Comparado com o grupo controle, o grupo tratado teve menor proteinúria (p < 0,05) e número maior de pacientes em remissão (p < 0,05) nos 6º e 12º meses. A função renal não teve alteração significante e eventos adversos foram de pequena intensidade na maioria dos pacientes. CONCLUSÃO: O protocolo terapêutico base-ado no uso por 6 meses de corticosteroides foi efetivo em reduzir a proteinúria durante os 12 meses de seguimento e foi bem tolerado pela maioria dos pacientes.

Renal Manifestations in 2007 Korean Patients with Behcet's Disease

PURPOSE: Behcet's disease (BD) theoretically affects all sizes and types of blood vessels and results in multi-organ involvement. However, renal BD has not been fully characterized, though the kidneys are histologically rich in blood vessels. MATERIALS AND METHODS: A total of 2007 patients who fulfilled the diagnostic criteria for BD were enrolled in this study. We reviewed the medical records and test results of the BD patients and used univariate and multivariate logistic regression analyses to determine the clinical significance of renal involvement in BD. RESULTS: Among the 2007 BD patients, we noted hematuria in 412 (20.5%) and proteinuria in 29 (1.4%). Univariate analysis showed that the BD patients with hematuria were predominantly female and older, had higher erythrocyte sedimentation rates (ESRs), and more frequently presented with genital ulcerations. BD patients with proteinuria had higher ESR levels compared to BD patients without proteinuria. In the multivariate analysis, age, sex, and ESR were found to be significantly associated with hematuria in BD patients, whereas only ESR was associated with proteinuria in BD patients. We also found that IgA nephropathy was the most common pathologic diagnosis in 12 renal BD patients who underwent renal biopsies. CONCLUSION: We suggest that routine urinalysis and serum renal function tests be performed for the early detection of renal BD, especially in older female BD patients with recurrent hematuria, high ESR levels, and frequent genital ulcers, as well as in BD patients with proteinuria and high ESR levels.

Renal outcomes and clinical course of nondiabetic renal diseases in patients with type 2 diabetes

BACKGROUND/AIMS: In several recent studies, renal biopsies in patients with type 2 diabetes and renal disease have revealed a heterogeneous group of disease entities. Our aim was to study the prognosis and clinical course of nondiabetic renal disease (NDRD) and to determine risk factors for NDRD in patients with type 2 diabetes. METHODS: Renal biopsy reports of 110 patients with type 2 diabetes who were seen at Kyung Hee University Medical Center and Kyung Hee University Hospital at Gangdong, Seoul, Korea between January 2000 and December 2011 were retrospectively analyzed. RESULTS: Of 110 patients with type 2 diabetes, 41 (37.3%) had diabetic nephropathy (DN), 59 (53.6%) had NDRD, and 10 (9.1%) had NDRD superimposed on DN. Immunoglobulin A nephropathy (43.5%) was the most common NDRD. Patients with NDRD had a shorter duration of diabetes, lower frequency of diabetic retinopathy, and better renal outcomes, which might have resulted from the use of aggressive disease-specific treatments such as steroids and immunosuppressants in patients with NDRD. CONCLUSIONS: Compared with DN, NDRD was associated with better renal outcomes in patients with type 2 diabetes, as evidenced by a higher cumulative renal survival rate and lower rate of end-stage renal disease (ESRD). Shorter duration of diabetes and absence of retinopathy were independent predictors of NDRD in patients with type 2 diabetes and renal involvement. Renal biopsy is recommended for patients with type 2 diabetes and risk factors for NDRD, to obtain an accurate diagnosis, prompt initiation of disease-specific treatment, and ultimately better renal outcomes with the avoidance of ESRD.

Beta-Thalassemia Minor Is Associated with IgA Nephropathy

No abstract available.

Desprendimiento de retina seroso con lupus fijo discoides y nefropatía por inmunoglobulina A / Serous retinal detachment in a patient with discoid fixed lupus and nephropathy by immunoglobulin A

El desprendimiento seroso de retina produce disminución de la agudeza visual debido al paso de fluido procedente de la coroides hacia el espacio subretiniano. Esta enfermedad tiene varias causas, entre las que se encuentran las idiopáticas, congénitas, posquirúrgicas, secundarias e uveítis infecciosas, autoinmune, vascular, hematológicas y neoplásicas.Objetivo: describir el caso inusual de un paciente joven con desprendimiento seroso de retina asociado a lupus fijo discoide y nefropatía por inmunoglobulina A con respuesta satisfactoria al uso de esteroides.Caso clínico: se presenta un paciente masculino de 18 años de edad que acudió a consulta de retina del centro oftalmológico del Hospital Universitario Manuel Ascunce Domenech, por disminución súbita de la agudeza visual en ambos ojos con diagnóstico inicial de coroidopatia serosa central, que posteriormente evolucionó hacia desprendimientos serosos bilaterales con afectación de todo el polo posterior. Por sus antecedentes se interconsultó con el especialista en dermatología y nefrología, donde se decidió iniciar tratamiento con esteroides a altas dosis, vitaminoterapia endovenosa, así como antiinflamatorios y midriáticos ciclopléjicos tópicos con respuesta excelente a la terapia

Serous retinal detachment causes visual acuity decrease due to the passage of fluid from the choroid into the subretinal space. This disease has several causes including idiopathic, congenital, postoperative, and secondary to infectious uveitis, autoimmune, vascular, hematological and neoplastic ones.Objective: to describe an unusual case of a young patient with serous retinal detachment associated with discoid fixed lupus and nephropathy by immunoglobulin A with satisfactory response to steroids use.Clinical case: a male patient of 18 years old was presented in the retina office of the Ophthalmological Center at the University Hospital Manuel Ascunce Domenech with sudden decrease of visual acuity in both eyes with an initial diagnosis of serous central choroidopathy, which later progressed into bilateral serous detachments with entire posterior pole involvement. By his antecedents, a specialty consultation in dermatology and nephrology was carried out, deciding to initiate treatment with high-dose steroids, intravenous vitamin therapy, and anti-inflammatory and topical cycloplegic mydriatic with excellent response to therapy

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

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

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

Anti-Proteinuric Effect of Sulodexide in Immunoglobulin A Nephropathy

PURPOSE: We conducted a multi-center randomized double-blind study to determine the effects of 6-month therapy with sulodexide on urinary protein excretion in patients with idiopathic Immunoglobulin A (IgA) nephropathy. MATERIALS AND METHODS: A total of seventy-seven patients participated in the study. They were randomly allocated to one of three groups: sulodexide 75 mg or 150 mg daily or the placebo for 6 months. The primary end point was the achievement, at 6 months, of at least 50% reduction in urine protein/creatinine ratio (UPCR) from the baseline value. RESULTS: At 6 months, the primary end point was achieved by 12.5% of the patients assigned to the placebo, 4.0% of the patients assigned to sulodexide 75 mg daily and 21.4% of those assigned to 150 mg (p=0.308). Treatment with sulodexide 150 mg daily for 6 months significantly reduced log UPCR from 6.38+/-0.77 at baseline to 5.98+/-0.94 at 6 months (p=0.045), while treatment with sulodexide 75 mg daily and placebo did not. CONCLUSION: A 6-month treatment with sulodexide did not achieve 50% reduction of urinary protein excretion in IgA nephropathy patients, but showed a tendency to increase the time-dependent anti-proteinuric effect. Therefore, long-term clinical trials on a larger scale are warranted to elucidate the hypothesis that sulodexide affords renal protection in IgA nephropathy patients.

Improvement in Erythropoieis-stimulating Agent-induced Pure Red-cell Aplasia by Introduction of Darbepoetin-alpha When the Anti-erythropoietin Antibody Titer Declines Spontaneously

Anti-erythropoietin antibodies usually cross-react with all kinds of recombinant erythropoietins; therefore, erythropoiesis-stimulating agent (ESA)-induced pure red-cell aplasia (PRCA) is not rescued by different ESAs. Here, we present a case of ESA-induced PRCA in a 36-yr-old woman with chronic kidney disease, whose anemic condition improved following reintroduction of darbepoetin-alpha. The patient developed progressive, severe anemia after the use of erythropoietin-alpha. As the anemia did not improve after the administration of either other erythropoietin-alpha products or erythropoietin-beta, all ESAs were discontinued. Oxymetholone therapy failed to improve the transfusion-dependent anemia and a rechallenge with ESAs continuously failed to obtain a sustained response. However, her anemia improved following reintroduction of darbepoetin-alpha at 3 yr after the initial diagnosis. Interestingly, anti-erythropoietin antibodies were still detectable, although their concentration was too low for titration. In conclusion, darbepoetin-alpha can improve ESA-induced PRCA when the anti-erythropoietin antibody titer declines and its neutralizing capacity is lost.

Clinicopathologic Characteristics of IgA Nephropathy with Steroid-responsive Nephrotic Syndrome

Nephrotic syndrome is an unusual manifestation of IgA Nephropathy (IgAN). Some cases respond to steroid treatment. Here we describe a case-series of IgAN patients with steroid-responsive nephrotic syndrome. Twelve patients with IgAN with steroidresponsive nephrotic syndrome were evaluated and followed up. All patients presented with generalized edema. Renal insufficiency was found in two patients. The renal biopsy of eight patients revealed wide foot process effacement in addition to the typical features of IgAN. They showed complete remission after steroid therapy. Seven relapses were reported in five patients; six of the relapsed cases responded to steroid therapy. Compared with steroid-non-responsive patients, the patients with steroid-responsive nephrotic syndrome had shorter symptom duration, more weight gain, more proteinuria, and lower histologic grade than did those that had steroid-non-responsive nephrotic syndrome at presentation. None of the responders progressed to end stage renal disease, whereas five (38%) non-responders required dialysis or renal transplantation. Patients with IgAN who have steroid-responsive nephrotic syndrome likely have both minimal change disease and IgAN. The clinical features of sudden onset of generalized edema, initial heavy proteinuria and initial severe hypoalbuminemia might help identify the subset of patients, especially in low grade IgAN.

Crohn's Disease in Association with IgA Nephropathy / 대한소화기학회지

Urological complications are not uncommon in Crohn's disease (CD). The most common manifestations are renal stones, enterovesical fistulas, and ureteral obstruction, but renal parenchymal disease has rarely been reported. IgA nephropathy, the most common form of primary glomerulonephritis, is usually isolated, but can be sometimes associated with chronic extrarenal disorders such as inflammatory bowel disease. We describe a case of 36 year-old man with CD associated with IgA nephropathy. He was diagnosed as CD 6 years ago and at that time, isolated proteinuria was observed. He presented recurrent proteinuria and elevation of creatinine level while he had been managed well with mesalamine and azathioprine. The renal biopsy was performed and IgA nephropathy (type IV) was diagnosed. Strict blood pressure control with angiotensin converting enzyme inhibitor and calcium channel blocker resulted in clinical improvement and normalization of serum creatinine level.

Crohn's Disease Associated with IgA Nephropathy / 대한소화기학회지

Crohn's disease is a condition of chronic inflammation potentially involving any location of the alimentary tract from mouth to anus. Numerous extraintestinal manifestations can also be present. Urologic complications of inflammatory bowel disease are seen in up to 25% of patients, but renal parenchymal disease has been rarely reported. IgA nephropathy is recognized worldwide as a most common form of primary glomerulonephritis. Clinical manifestations vary, ranging from microscopic hematuria to nephrotic syndrome. Recently, IgA nephropathy associated with systemic diseases has been reported. We describe a case of a 22 year-old man with Crohn's disease associated with IgA nephropathy. At the age of 8 years, microscopic hematuria appeared. After fourteen years, he presented with melena, mild fever, recurrent oral ulcer, microscopic hematuria and proteinuria. Colonoscopic examination revealed characteristic features of Crohn's disease such as multiple ulcers. Microscopic findings showed superficial ulceration with small noncaseating granulomas. Renal biopsy revealed IgA nephropathy. The patient was treated with oral prednisolone, olsalazine, and metronidazole followed by maintenance therapy with sulfasalazine and azathioprine resulting in clinical improvement of Crohn's disease and IgA nephropathy.

Renal Transplantation in a Patient with Idiopathic Thrombocytopenic Purpura

The combination of idiopathic thrombocytopenic purpura (ITP) and chronic renal failure (CRF) is uncommon. This report highlights a case of renal transplantation in a patient with ITP. A 35-year-old man with ITP was admitted with uremic symptoms. A renal transplant and splenectomy was simultaneously performed. A prophylactic pneumococcous vaccination was performed and intravenous immunoglobulin (1 g/kg) was administered before and after the operation. The patient's platelet count increased gradually after the splenectomy. During a two-year follow up period, the graft function was well maintained. Renal transplantation in a patient with ITP is recommended with a well-designed strategy to prevent potential complications.

Type 2 cryoglobulinemia treated with plasmapheresis associated with IgA nephropathy.

Cryoglobulinemia is caused by antibodies which precipitate in blood on exposure to cold, and redissolve on warming. The authors present a child with essential, Type 2 cryoglobulinaemia, successfully treated with plasmapheresis, who developed glomerulonephritis due to IgA nephropathy. Conservative treatment resulted in spontaneous improvement with no deterioration in renal function.