Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis in children

OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.

Collared Pecary (tayassu tajacu) as a new model of renal ischemic injury induced by clamping the renal artery

PURPOSE: The use of the collared peccary as an experimental model for ischemic nephropathy. METHODS: A total of 12 collared peccary (Tayassu tajacu) was used and ischemic nephropathy was induced in six of these animals that constituted the experimental group (G1) while the other six formed the control group (G2). Ischemic nephropathy was induced surgically by partial occlusion of the left renal artery. The disease course was assessed by hematological tests, serum chemistry, urinalysis, ultrasound (US) and doppler ultrasound function of the renal artery before induction, and at five, 10, 15 and 20 days after surgery. Twenty days after the occlusion, unilateral nephrectomy and histopathological examination were performed to assess renal morphology. RESULTS: Statistical analysis by Fischer's test showed a significant difference (p<0.05) between the control group and the experimental group. The histopathological examination showed glomerular, tubular and interstitial lesions. In the experimental group, 83.3% (5 /6) showed moderate renal lesions and only 16.7% (1/6) were classified with no lesions. The ultrasound examination of the right kidney presented statistical difference between day 5 and day 10 post occlusion. CONCLUSION: The collared peccary as a good experimental model for ischemic renal disease, because it could be manipulated during the research time without death, with health conditions that permit any subsequent procedure for disease therapy. .

Nonrejection pathology of renal allograft biopsies: 10 years experience from a tertiary care center in north India.

Background: Renal dysfunction in allograft transplant is common and its assessment is done using Revised Banff '97 working classification, which is the accepted formulation for the evaluation of histological appearance of renal allograft biopsies. The nonrejection category under the Banff working classification of renal allograft pathology forms a large group resulting in allograft dysfunction. Aim: To evaluate the spectrum of histopathological changes seen in renal allograft dysfunction. Materials and Methods: A total of 119 renal biopsies were studied over 10 years presenting with renal allograft dysfunction from a tertiary center in North India. Results: Majority of the biopsies were in the nonrejection category (47.1%), which included few cases of acute tubular necrosis (25.2%), cyclosporine nephrotoxicity (16%), infections (10.9%), and thrombotic microangiopathy (3.4%). The second largest category in our study was acute/active cellular rejection group (31.9%), which displayed moderate to severe tubulitis, mononuclear cell infiltrate in the interstitium, and vasculitis. Antibody-mediated rejection cases were seen in 28.6% of the renal biopsies followed by chronic allograft nephropathy cases (12.6%) showing features of tubular atrophy and interstitial fibrosis. Borderline changes with features of mild tubulitis contributed to 7.6% of the biopsies. The smallest group comprised of only 4.2%, which were within normal histological limits. Conclusion: Timely accurate diagnosis of renal allograft dysfunction is essential for prompt, effective management of renal transplant patients. Thus, nonrejection pathology forms a significant cause of renal dysfunction in patients with renal allograft transplantation.

Efecto del misoprostol en la prevención de la nefrotoxicina inducida por indometacina / The effect of the misoprostol in the prevention of the nephrotoxicity induced by indometacin

El uso de desinflamatorio no esteroides (DINE) inhibe la síntesis de prostaglandinas vasodilatadoras renales alterando el flujo plásmatico renal y la tasa de filtración glomerular, pudiendo producir falla renal aguda en pacientes nefrópatas o con factores de riesgo asociados. El misoprostol es un análogo sintético de la prostaglandina E1, que utilizado en estudios experimentales y clínicos ha demostrado propiedades nefroprotectoras, evitando así el deterrioro funcional y los cambios hemodinámicos intrarrenales nefastos por el uso de DINE como la indometacina. Lo novedoso de este tratamiento, a difencia de los estudios clínicos similares hasta el momento, radica en que todos los pacientes incluidos, no presetaban nefropatía previa ni factores de riesgo que los hiciera proclives a desarrollar nefrotoxicidad por desinflamatorios. El proposito de este estudio es demostrar si el uso de misoprostol previene las alteraciones funcionales renales producidas en pacientes sin nefropatía y sin factores de riesgo para el desarrollo de nefrotoxicidad por el uso de indometacina a corto plazo (10días). Se estudiaron 35 pacientes (17 hombres y 18 mujeres) ingresados en el Servicio de Medicina Interna del Hospital Militar "Dr. Carlos Arvelo" por artropatías dolorosas inflamatorias; en un trabajo aleatorio, prospectivo, doble ciego, comparado con placebo. Los pacientes se distribuyeron al azar en dos grupos. El grupo 1 recibió indometacina, 50 mg V.O.TID, más misoprostol, 200 Mg V.O.TID, y el grupo 2, indometacina a la misma dosis y placebo. Los parámetros paraclínicos utilizados para evaluar la función renal u nefrotoxicidad aguda fueron: tasa de filtración glomerular (depuración de creatinina sensibilizada con cimetidina) y proteinuria en 24 horas, antes y después del tratamiento. Treinta pacientes concluyeron el estudio. En el grupo 1 se observó que la depuración de cratinina promedio al final del estudio aumentó con respecto a la inicial en un 13,1 por ciento (de 107,7 a 121,8 cc/min/1,73 m. cuadrado) (p<0,001), mientras que en el grupo 2 la misma disminuyó en un 14,5 por ciento (de 112,75 a 96,4 cc/min/1,73 m. cuadrado) (P=0,001). La proteinuria no varió en ningún grupo (p>0,05). Se concluye, que el misoprostol previno en pacientes sin nefropatía previa ni factores de riesgo asociados, la disfunción renal ocacionada por la indometacina, en el tratamiento a corto plazo de artropatías inflamatorias dolorosas

Renal involvement in leprosy.

Renal involvement was studied in 70 patients with leprosy by urine analysis, detailed biochemical investigations and renal histopathology. Of these 70 patients, 40 had lepromatous and 30 had non-lepromatous leprosy. Creatinine clearance was reduced in 20 patients. Renal biopsies were studied in 50 cases (25 lepromatous, 25 non-lepromatous); of which in 13 cases (10 lepromatous, 3 non-lepromatous) abnormal histopathological lesions were found by light microscopy. Amyloidosis was seen in only one lepromatous patient. No acid-fast bacilli and leproma like lesion were demonstrated in any case.

Experimental Nephritis Induced by Homologous Placental Tissue as Observed with the Light, Fluorescent and Electron Microscope

Toxemia of pregnancy is a common complication of gestation, usually occurring in late pregnancy. Whether toxemia represents an exaggeration of changes incident to pregnancy or depends upon some wholly new factor is a moot point. Indeed, the cause of the toxemia of pregnancy, despite decades of intensive research, remains the great enigma of obstetrics and constitutes one of an important unsolved problems in the field of human reproduction. Glomerulonephritis can be induced in various animal species by numerous serums and tissue extracts. Its production by duck immune serum was first described in the rabbit by Masugi(1934). By using a potent standardized nephrotoxic duck serum or its gamma globulin, nephritis has be reproduced in a regular manner by Seegal, et al., (1936). The experiments recorded here show the results of injecting rabbit antidog-placenta serum into both pregnant and non-pregnant dogs as described by Seegal at al., (1955). The course of the resulting nephritis is compared with that following the injection of rabbit antidog-kidney serum. The large size of the animal permitted frequent bleeding and the gestation period allowed for observation of nephritis during pregnancy. The findings support the conclusion that rabbit antidog-placenta serum injected in the dog produced an acute nephritis which usually progressed to a chronic state comparable to that which follows the injection of anti-kidney serum. Pregnancy has not been terminated by this antiserum. Beveans et al.(1955) describe the lesions produced in these pregnant and non-pregnant dogs following injection of either rabbit anti-placenta or rabbit anti-kidney serum. Acute and chronic phases of the nephritis have been studied over a period of 10 months. The intravenous injection of rabbit antidog-placenta or antidog-kidney serum produced immediate evidence of glomerulonephritis in dogs and rabbits. The glomerulonephritis so induced may terminate in death within 8 days, may progress to a chronic form or may heal. Recently, Irino et al.(1967) induced renal 1esions in rats by placental extracts. These changes were observed with the electron microscope and the ranal glomerular alterations in rats with a clinical syndrome resembling toxemia of pregnancy showed the characteristic changes consisting of swelling with decreased density of tile basement membrane, a dense granular deposition within tile along capillary basement membranes, and marked swelling and slight proliferation of glomerular epithelium. The glomerular lesions, designated endothelial glomerulitis are apparently a result of an antigen-antibody reaction and present further evidence that human toxemia of pregnancy has an immune mechanism as a basis for its production. Kim(1969) attempted to establish the pathologic changes induced by sensitizing the rat against homologous placental tissue and to compare them with the lesions of the kidney in human toxemia. He found that renal lesions were closely related to that of human toxemia of pregnancy. The present investigation is aimed to study the lesions in the glomerulus of the pregnant rat kidneys induced by repeated injection of homologous placental tissue as observed with the light, the fluorescent and the electron microscope and adds further evidence for the view that the syndrome, as induced experimentally, constitutes an analog of toxemia of pregnancy as it affects the human.s