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Scleroderma encephalopathy

David Regnault, Louis d'Alteroche, Charlotte Nicolas, Fanny Dujardin, Jean Ayoub, Jean Marc Perarnau
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available. PATIENTS AND METHODS: Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. RESULTS: Twenty-five patients with a wide variety of histological lesions (sinusoidal dilatations, granulomatosis, regenerative nodular hyperplasia, obliterative portal venopathy, or subnormal liver) and a wide variety of associated diseases (thrombophilia, sarcoidosis, common variable immunodeficiency, scleroderma, Castleman's disease, early primitive biliary cirrhosis, congenital liver fibrosis, chemotherapy, purinethol intake, and congenital varices) were included...
May 2018: European Journal of Gastroenterology & Hepatology
Sabrina Hoa, Edward P Stern, Christopher P Denton, Marie Hudson
OBJECTIVE: The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC. METHODS: We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Papers were eligible for inclusion if they were full-length articles in English whose main topic was SRC or scleroderma renal disease...
April 2017: Autoimmunity Reviews
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
Dorota Jasinska, Jerzy Boczon
PURPOSE OF REVIEW: We aim to illustrate the potential viability of MCTD as an underlying aetiology of Melkersson-Rosenthal syndrome. The case is probably the first description available in the literature of the Melkersson-Rosenthal as an early manifestation of mixed connective tissue disease. RECENT FINDINGS: The Melkersson-Rosenthal syndrome consists of a triad of recurrent lip and/or face swelling, fissured tongue, and intermittent facial palsy. Mixed connective tissue disease is a multisystemic disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by a high titer of antibodies to ribonucleoprotein...
December 23, 2015: European Journal of Medical Research
Emily Keeler, Gloria Fioravanti, Bensson Samuel, Santo Longo
SCLERODERMA: renal crisis (SRC), a somewhat rare but serious complication of systemic scleroderma, is one of only a few known rheumatologic emergencies; it presents in as many as 10% of patients with scleroderma. Before the use of angiotensin converting enzyme (ACE) inhibitors to treat SRC, the mortality rate for SRC was extremely high-as much as 90% after 1 year. However, the mortality rate has significantly improved with the early and aggressive use of ACE inhibitors. SRC typically includes acute renal failure and accelerated hypertension...
2015: Laboratory Medicine
Davor Lessel, Bidisha Saha, Fuki Hisama, Bahar Kaymakamzade, Gulay Nurlu, Yasemin Gursoy-Özdemir, Holger Thiele, Peter Nürnberg, George M Martin, Christian Kubisch, Junko Oshima
We describe a 28-year-old Turkish man with consanguineous parents who presented with an aged appearance with prematurely gray hair and scleroderma-like skin, spastic paraplegia, and apparent disability. The proband and each of his parents were heterozygous for a mutation in WRN, which could not explain his symptoms. Exome sequencing of the proband's blood DNA showed a homozygous c.626-1G > C mutation in intron 5 of the SAMHD1 gene, which encodes a triphosphohydrolase involved in the regulation of intracellular dNTP pools and which is mutated in Aicardi-Goutieres syndrome...
October 2014: American Journal of Medical Genetics. Part A
Luc Mouthon, Guillaume Bussone, Alice Berezné, Laure-Hélène Noël, Loïc Guillevin
Scleroderma renal crisis (SRC) is characterized by malignant hypertension and oligo-anuric acute renal failure. It occurs in 5% of patients with systemic sclerosis (SSc), particularly in patients with diffuse disease during the first years. SRC is more common in patients receiving corticosteroids, the risk increasing with increasing dose. The disease is sometimes triggered by use of nephrotoxic drugs and/or intravascular volume depletion. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features...
June 2014: Journal of Rheumatology
Xi-Lian Zhang, Rong Ma, Yan-Ming Xie, Meng Li, Ping Rong
OBJECTIVE: Analyze the characteristic features of Shenmai injections as used in pediatric clinics, to improve the specifications for use in children in order to decrease risks. METHOD: We preformed statistical analyses on data from the CNKI and VIP databases relevant to the clinical application of Shenmai injections in children. Pediatric indications, usage and dosage, length of treatment, solubility of the drugs in mediums and adverse reactions were summarized...
September 2013: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
Mohamed Allam, Mohamed Ghozzi
Scleromyxedema (SM) is a sclerotic variant of lichen or papular mucinosis in which lichenoid papules and scleroderma-like features are both present. It is a rare deposition disorder characterized by generalized papular and sclerodermoid eruptions, mucin deposition, increased fibroblast proliferation, fibrosis, and monoclonal gammopathy (also known as paraproteinemia) mainly of the immunoglobulin G-lambda type in the absence of thyroid disease. It usually affects middle-aged adults and shows no gender or racial predilection...
May 2013: Case Reports in Dermatology
Ayako Matsui, Hidekazu Ikeuchi, Aika Shimizu, Shunsuke Takahashi, Keiichiro Mishima, Noriyuki Sakurai, Toru Sakairi, Yoriaki Kaneko, Akito Maeshima, Keiju Hiromura, Yoshihisa Nojima
No abstract text is available yet for this article.
July 10, 2012: Nihon Naika Gakkai Zasshi. the Journal of the Japanese Society of Internal Medicine
Anmol Dhillon, Celso Velazquez, Chokkalingam Siva
Rheumatologic diseases have varied clinical presentations, and posterior reversible encephalopathy syndrome (PRES) can be one of their presentations. The exact etiology of PRES is unknown, but endothelial dysfunction and immunosuppressive medications seem to be the likely cause in rheumatologic diseases. Clinical features include headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. The diagnosis of PRES can be difficult because several neuropsychiatric illnesses are generally prevalent in rheumatologic diseases; however, a high index of suspicion among physicians along with neuroimaging can help in the accurate diagnosis...
December 2012: Rheumatology International
Loïc Guillevin, Alice Bérezné, Raphaèle Seror, Luis Teixeira, Jacques Pourrat, Alfred Mahr, Eric Hachulla, Christian Agard, Jean Cabane, Philippe Vanhille, Jean-Robert Harle, Isabelle Deleveaux, Luc Mouthon
OBJECTIVE: Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence. METHODS: Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls. RESULTS: Among the 91 SRC patients, 71 (78...
March 2012: Rheumatology
Guillaume Bussone, Laure-Hélène Noël, Luc Mouthon
Scleroderma renal crisis is characterized by malignant hypertension and oligo-anuric acute renal failure. Scleroderma renal crisis occurs in 2 to 5% of patients with systemic sclerosis, particularly those with diffuse cutaneous systemic sclerosis in the first years of disease evolution. High-dose corticosteroid therapy (> 15 mg/d) is associated with an increased risk of scleroderma renal crisis. Patients present with prominent left heart failure and hypertensive encephalopathy. Renal failure can be associated with moderate proteinuria, without hematuria...
June 2011: Néphrologie & Thérapeutique
Luc Mouthon, Alice Bérezné, Guillaume Bussone, Laure-Hélène Noël, Peter M Villiger, Loïc Guillevin
Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension and oligo/anuric acute renal failure. SRC occurs in 5% of patients with SSc, particularly in the first years of disease evolution and in the diffuse form. The occurrence of SRC is more common in patients treated with glucocorticoids, the risk increasing with increasing dose. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features...
April 2011: Clinical Reviews in Allergy & Immunology
C P Denton, G Lapadula, L Mouthon, U Müller-Ladner
Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia...
June 2009: Rheumatology
Donato Rigante, Domenica Battaglia, Ilaria Contaldo, Ilaria La Torraca, Laura Avallone, Stefania Gaspari, Giulia Bersani, Achille Stabile
We report a 7-year-old boy who unexpectedly developed a multi-drug resistant epilepsy with negative neuroimaging results, followed by the insidious appearance of linear localized scleroderma involving the right leg. When the boy was 16 and severely affected by epileptic encephalopathy, we have evaluated this case for the first time: his localized scleroderma had reached the right buttock and positive anti-nuclear antibody was the only positive laboratory test. Methotrexate administered for 12 months was ineffective in improving both the organization of his electroencephalographic pattern and seizure control, though seemed to stabilize the progression of linear scleroderma...
July 2008: Rheumatology International
Teru Kumagi, E Jenny Heathcote
Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus...
January 23, 2008: Orphanet Journal of Rare Diseases
Pam R Rajendran, Jerry A Molitor
The use of an angiotensin II type-1 receptor blocker for scleroderma renal crisis is controversial. We describe a 46-year-old woman presenting with a seizure secondary to hypertensive encephalopathy as the initial manifestation of scleroderma renal crisis. She had complete resolution of end organ damage with use of an angiotensin II type-1 receptor blocker. There may be a role for angiotensin II type-1 receptor blockers in the setting of scleroderma renal crisis with central nervous system involvement.
August 2005: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
B Shergill, C H Orteu, S R McBride, M H A Rustin
Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations have been described. In addition to these noncutaneous manifestations, about 15% of patients have central neurological symptoms such as psychosis, convulsions and encephalopathy. Successful therapy is difficult but high-dose intravenous immunoglobulin (IVIg) has been reported to be a successful treatment...
September 2005: British Journal of Dermatology
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