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adult hsp nephritis

Neil Gaskill, Bruce Guido, Cynthia Magro
Henoch-Schonlein purpura is an immunoglobulin A (IgA)-immune complex mediated leukocytoclastic vasculitis that classically manifests with palpable purpura, abdominal pain, arthritis, and hematuria or proteinuria. The condition is much more predominant in children (90% of cases) and commonly follows an upper respiratory infection. We present a case of recurrent Henoch-Schonlein purpura (HSP) complicated by nephritis in an adult female initially categorized as IgA nephropathy (IgAN). We review the pathophysiologic basis of HSP nephritis as the variant of HSP accompanied by renal involvement and its pathogenetic commonality with IgA nephropathy...
15, 2016: Dermatology Online Journal
Lampros Fotis, Paul V Tuttle, Kevin W Baszis, Peri H Pepmueller, Terry L Moore, Andrew J White
BACKGROUND: A small percentage of children with Henoch-Schönlein purpura (HSP) develop a chronic form of the disease that often requires prolonged corticosteroid therapy. Disease modifying anti-rheumatic agents (DMARDs) or biologics have been successfully used to treat those refractory cases. Azathioprine is a DMARD that has been reported to be effective in HSP nephritis and in adult cutaneous leukocytoclastic vasculitis, a condition with cutaneous histology similar to HSP. CASE PRESENTATION: A description of 6 cases with relapsing HSP without significant renal involvement, treated with azathioprine are reported...
June 23, 2016: Pediatric Rheumatology Online Journal
Beth Vogt
Although kidney disease is much less common in children than in adults, clinicians must remain alert for the renal conditions that occur in this population because prompt diagnosis and management are essential. Acute postinfectious glomerulonephritis occurs as an uncommon sequela of streptococcal and other infections. Management is focused on providing supportive measures, including management of fluid-electrolyte imbalance and hypertension, until the patient recovers. Immunoglobulin (Ig) A nephropathy is a primary glomerulonephritis related to abnormal IgA glycosylation...
May 2016: FP Essentials
Fei Han, Liang-liang Chen, Ping-ping Ren, Jing-yun Le, Pei-jing Choong, Hong-ju Wang, Ying Xu, Jiang-hua Chen
OBJECTIVE: The treatment of Henoch-Schönlein purpura (HSP) with moderate proteinuria remains controversial. We retrospectively analyzed the efficacy of immune suppressants, with a particular emphasis on mycophenolate mofetil (MMF). METHODS: Ninety-five HSP patients with moderate proteinuria (1.0-3.5 g/24 h) after at least three months of therapy with angiotensin-converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) were divided into three groups: an MMF group (n=33) that received MMF 1...
September 2015: Journal of Zhejiang University. Science. B
Kunal Chaudhary, Ji-Young Shin, Georges Saab, Alan M Luger
Henoch-Schonlein purpura (HSP) frequently occurs in children under 15 years of age but is quite rare in adults. Most children who develop HSP nephritis completely recover from the illness, whereas up to 40% of adults have persistent hematuria and 10% develop chronic renal failure. Plasma exchange alone has been shown to be beneficial in children who have HSP nephritis. Adults have been treated using immunosuppressive drugs such as cyclophosphamide but effects are not certain. Here, we present a case of a 59-year-old white male who developed HSP nephritis while undergoing treatment with steroids...
October 2008: NDT Plus
Saiko Kato-Okada, Hiromichi Suzuki, Tsutomu Inoue, Tomohiro Kikuta, Hirokazu Okada
INTRODUCTION: Recently, Henoch-Schönlein purpura (HSP) has been observed in elderly people, although it was believed to be uncommon in these subjects. The increased risks of developing end-stage renal disease (ESRD) in adults in comparison with children were highlighted by different studies; however, limited data are available on the treatment of HSP nephritis in adults. METHODS: Between 2002 and 2008, five elderly Japanese patients (>65 years old) (mean age, 68 years, ranging from 65 to 72) with severe forms of HSP nephritis were entered into a prospective study to evaluate prednisolone therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes...
2015: Japanese Clinical Medicine
Shan Lu, Dong Liu, Jing Xiao, Wenming Yuan, Xiaoyang Wang, Xiaoxue Zhang, Jin Zhang, Zhangsuo Liu, Zhanzheng Zhao
BACKGROUND: Henoch-Schönlein purpura (HSP) mainly affects children, but age is also thought to be an important prognostic factor. Kidney involvement is a major cause of mortality in HSP patients. The purpose of this study was to analyze the clinicopathological correlations between adults and children. METHODS: A total of 208 children and 75 adult patients with HSP nephritis (HSPN) were evaluated. All patients underwent a renal biopsy. RESULTS: Extra-renal symptoms (arthritis and abdominal pain) were more common in the pediatric patient group than in the adult group (P < 0...
May 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Song Mao, Songming Huang
To evaluate the association between angiotensinogen (AGT) gene polymorphism and the risk of Henoch-Schönlein purpura (HSP)/Henoch-Schönlein purpura nephritis (HSPN) we searched the eligible studies through Pub Med, Embase, Cochrane, and China National Knowledge Infrastructure (CNKI) databases according to predefined criteria. A random-effects model was used to calculate the combined odds ratios (ORs) and its corresponding 95% confidence interval (CI). Five studies were recruited for the analysis of the association between AGT M235T gene polymorphism and HSP/HSPN risk...
February 2015: Renal Failure
Jean-Claude Davin, Rosanna Coppo
Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, in whom prognosis is mostly dependent upon the severity of renal involvement. Nephritis is observed in about 30% of children with HSP. Renal damage eventually leads to chronic kidney disease in up to 20% of children with HSP nephritis in tertiary care centres, but in less than 5% of unselected patients with HSP, by 20 years after diagnosis. HSP nephritis and IgA nephropathy are related diseases resulting from glomerular deposition of aberrantly glycosylated IgA1...
October 2014: Nature Reviews. Nephrology
Yoon Kang, Jin-su Park, You-Jung Ha, Mi-il Kang, Hee-Jin Park, Sang-Won Lee, Soo-Kon Lee, Yong-Beom Park
We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55...
February 2014: Journal of Korean Medical Science
R López-Mejías, B Sevilla Pérez, F Genre, S Castañeda, N Ortego-Centeno, J Llorca, B Ubilla, R Ochoa, T Pina, A Marquez, L Sala-Icardo, J A Miranda-Filloy, J Rueda-Gotor, J Martín, R Blanco, M A González-Gay
Henoch-Schönlein purpura (HSP) is the most common type of primary small-sized blood vessel vasculitis in children and an uncommon condition in adults. Interleukin (IL)-6 is a proinflammatory cytokine whose effect is controlled by the IL-6 receptor (IL-6R). IL-6 transducer (IL-6ST/gp130) is the signal-transducing subunit of the IL-6R. Two hundred and eighty five Spanish HSP patients and 877 sex and ethnically matched controls were genotyped for the IL6R rs2228145 and IL6ST/gp130 rs2228044 functional polymorphisms...
December 2013: Tissue Antigens
Maxime D Bérubé, Normand Blais, Sylvain Lanthier
Henoch-Shönlein purpura (HSP) is a systemic small vessel vasculitis. Most patients present during childhood. The characteristic association of purpura, arthralgia, abdominal pain, and nephritis reflects the predominant distribution of vasculitis. Headaches and mild behavioral changes suggest CNS involvement in one-third of HSP patients. Salient central nervous system (CNS) manifestations are rarer and mostly reported in adults and patients with a severe disease course. Diagnosis of CNS vasculitis is rarely confirmed by histopathology and generally relies on "suggestive" imaging showing brain hemorrhages, infarcts and edema, predominantly located in the parieto-occipital regions...
2014: Handbook of Clinical Neurology
S Kimura, S Takeuchi, Y Soma, T Kawakami
BACKGROUND: The long-term prognosis of Henoch-Schönlein purpura (HSP) is determined by the severity of renal involvement, known as HSP nephritis, which varies considerably from patient to patient. There is now increasing evidence that dysregulated cytokine production plays a crucial role in human autoimmune and inflammatory processes. AIM: To explore the possible contributions of serum antistreptolysin O, C-reactive protein, IgA, interleukin (IL)-6, IL-8, tumour necrosis factor (TNF)-α, anticardiolipin antibody (aCL) and antiphosphatidylserine-prothrombin complex antibody (anti-PSPT) in the pathogenesis of HSP, and to evaluate correlations between those biological parameters and the clinical features...
October 2013: Clinical and Experimental Dermatology
Chang-Joo Lee, Kwang-Sun Suh, Kyung-Hee Kim, Yoon-Kyung Chang, Ki-Ryang Na, Kang-Wook Lee
BACKGROUND: The presence of tubuloreticular inclusions (TRIs) in endothelial cells (ECs) always evokes suspicion of an association with underlying viral infections or autoimmune diseases. However, other underlying diseases can be associated with TRI expression. Since identification of the underlying disease is of primary consideration for management of glomerulonephritis (GN), it is important to clarify the clinical significance of TRI expression. METHODS: The authors studied 104 renal biopsy cases having TRI...
December 2013: Ultrastructural Pathology
Hesham Mohey, Blandine Laurent, Christophe Mariat, Francois Berthoux
BACKGROUND: We established earlier the absolute renal risk (ARR) of dialysis/death (D/D) in primary IgA nephropathy (IgAN) which permitted accurate prospective prediction of final prognosis. This ARR was based on the potential presence at initial diagnosis of three major, independent, and equipotent risk factors such as hypertension, quantitative proteinuria≥1 g per day, and severe pathological lesions appreciated by our local classification scoring≥8 (range 0-20). We studied the validity of this ARR concept in secondary IgAN to predict future outcome and focused on Henoch-Schönlein purpura (HSP) nephritis...
2013: BMC Nephrology
Cícera Sebastiana da Silva Padovani
INTRODUCTION: The aim of this study was to evaluate the epidemiologic profile of patients and difficulties of patients referred by basic health units (UBS) or other hospitals, outpatient screening of the Division of Nephrology, Hospital São Paulo (UNIFESP) for evaluation and treatment kidney disease. METHODS: From February to September 2009, has been evaluated 341 patients referred from UBS in São Paulo and other parts of the Country. RESULTS: Of these patients, 26% (86/341) required for new tests to confirm the diagnosis doubtful for referrals, incomplete, or because of the waiting period for the care and exams, which ranged from one week to three years, and part of them did not bring any kind of examination for the evaluation, 12% (45/341) returned for follow-up at the unit location, 13% (46/341) were referred for treatment site closest to their residence, 47% (164/341) for our sub-specialty Clinics of Nephrology (HSP): 24% (82/341) uremia, 8% (27/341) with polycystic kidney disease, 7% (23/341) for hypertension, 4% (16/341) renal Lithiasis and 4% (16/341) nephritis...
October 2012: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Lei Zhang, Changsong Han, Chuanhui Sun, Hongxue Meng, Fei Ye, Shiping Na, Fulai Chen, Duo Zhang, Xiaoming Jin
Approximately 40% of patients with Henoch-Schonlein purpura (HSP) develop Henoch-Schonlein purpura nephritis (HSPN) after 4 to 6 weeks of subcutaneous hemorrhaging. Immunoglobulin-A nephropathy (IgAN) and HSPN have numerous similarities, which can cause difficulty in correctly diagnosing the disorder during a differential diagnosis. The pathogenesis of the 2 diseases is not clear. We enrolled 137 patients with HSPN, 107 patients with IgAN, and 28 healthy (control) patients in our study. The levels of alpha-smooth muscle actin (α-SMA), c-Met, and Gal-deficient IgA1 (Gd-IgA1) in the 3 patient groups were determined and compared...
January 2013: Translational Research: the Journal of Laboratory and Clinical Medicine
Pingping Ren, Fei Han, Liangliang Chen, Yu Xu, Ying Wang, Jianghua Chen
BACKGROUND: Henoch-Schönlein purpura (HSP) is a form of systemic vasculitis that can progress to Henoch-Schönlein purpura nephritis (HSPN), and the most effective treatment remains controversial. Our aim was to compare the effects of oral mycophenolate mofetil (MMF) with low-dose prednisone and the full-dose corticosteroids (CS; prednisone) for the induction therapy of HSPN with large proteinuria. METHODS: Fifty-three patients with biopsy-proved HSPN with large proteinuria (>2...
2012: American Journal of Nephrology
Alessandra Soriano, Giovanni Galati, Umberto Vespasiani-Gentilucci, Paolo Gallo, Antonio de Vincentis, Antonio Picardi, Antonella Afeltra
Henoch-Schönlein Purpura (HSP) is a small vessel-vasculitis that usually affects children and adolescents; its onset in adults is uncommon.We describe a case of HSP complicated with nephritis and extensive deep vein thrombosis in an 81-year-old Caucasian woman, successfully treated with oral corticosteroids. Even at the extremes of age, HSP should be considered in the differential diagnosis of leukocytoclastic vasculitis, with a particular attention to renal involvement, because of its potential morbidity and mortality in the elderly; in addition, ruling out an occult thrombotic event in course of HSP is mandatory, especially in the presence of additional thrombotic risk factors...
October 2012: Aging Clinical and Experimental Research
Outi Jauhola, Jaana Ronkainen, Olli Koskimies, Marja Ala-Houhala, Pekka Arikoski, Tuula Hölttä, Timo Jahnukainen, Jukka Rajantie, Timo Örmälä, Matti Nuutinen
BACKGROUND: Corticosteroids have been shown not to prevent the development of Henoch-Schönlein nephritis. However, long-term follow-up data are scarce. METHODS: The long-term outcome of patients in a randomized placebo-controlled prednisone study was evaluated 8 years later with a health questionnaire completed by 160/171 (94%) patients and by urine and blood pressure screening (138/171, 81%). RESULTS: Twelve patients had hematuria and/or proteinuria and seven had hypertension...
June 2012: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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