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Scleroderma renal crisis

Marcin Okrój, Martin Johansson, Tore Saxne, Anna M Blom, Roger Hesselstrand
BACKGROUND: The complement system has been implicated in pathogenesis of systemic sclerosis (SSc). The goal of the present study was to evaluate improved complement biomarkers in SSc. METHODS: The presence of C4d, reflecting activation of the classical/lectin pathways, C3bBbP corresponding to activation of the alternative pathway, and soluble terminal complement complexes (all complement pathways), was measured in plasma samples by enzyme-linked immunosorbent assay and correlated to clinical parameters...
November 18, 2016: Arthritis Research & Therapy
Bernadette M Lynch, Edward P Stern, Voon Ong, Mark Harber, Aine Burns, Christopher P Denton
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy...
September 2016: Clinical and Experimental Rheumatology
Thasia G Woodworth, Yossra A Suliman, Daniel E Furst, Philip Clements
Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients...
November 2016: Nature Reviews. Nephrology
Norihiro Nagamura, Seikon Kin
Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC...
August 2016: Nagoya Journal of Medical Science
Cody M Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, Jean M Elwing
Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia...
2016: Case Reports in Rheumatology
Arnaud Devresse, Selda Aydin, Moglie Le Quintrec, Nathalie Demoulin, Patrick Stordeur, Catherine Lambert, Sara Gastoldi, Yves Pirson, Michel Jadoul, Johann Morelle
BACKGROUND: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis characterized by abrupt onset of hypertension, thrombotic microangiopathy, and kidney injury. The mechanisms of the disease remain ill-defined, but a growing body of evidence suggests that activation of the complement system may be involved. METHODS: Here, we report the case of a patient presenting with severe SRC and strong evidence of complement activation, both in serum and in the kidney, in the absence of genetic defect of the complement system...
July 2016: Medicine (Baltimore)
Carles Tolosa-Vilella, Maria Lluisa Morera-Morales, Carmen Pilar Simeón-Aznar, Begoña Marí-Alfonso, Dolores Colunga-Arguelles, José Luis Callejas Rubio, Manuel Rubio-Rivas, Maika Freire-Dapena, Alfredo Guillén-Del Castillo, Nerea Iniesta-Arandia, Maria Jesús Castillo-Palma, Marivi Egurbide-Arberas, Luis Trapiellla-Martínez, José A Vargas-Hitos, José Antonio Todolí-Parra, Mónica Rodriguez-Carballeira, Adela Marin-Ballvé, Xavier Pla-Salas, Juan José Rios-Blanco, Vicent Fonollosa-Pla
OBJECTIVE: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. METHODS: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE)...
October 2016: Seminars in Arthritis and Rheumatism
Julie J Paik, Ram Hirpara, Jennifer A Heller, Laura K Hummers, Fredrick M Wigley, Ami A Shah
OBJECTIVE: To demonstrate potential thrombotic complications after radial arterial line placement in patients with scleroderma. METHODS: This is a retrospective case series of 4 patients with scleroderma who were hospitalized in the intensive care unit (ICU) requiring invasive hemodynamic monitoring and developed severe complications after radial arterial line placement. We reviewed their medical records to assess their laboratory findings and clinical presentations...
October 2016: Seminars in Arthritis and Rheumatism
Matthew Turk, Janet E Pope
OBJECTIVE: Systemic sclerosis (SSc) leads to a high mortality from internal organ involvement. Scleroderma renal crisis (SRC) usually occurs in the diffuse cutaneous SSc (dcSSc) subset early in the disease, often with acute severe hypertension and renal failure. Prevalence of SRC since its classification in the early 1970s was determined in publications to assess whether the prevalence of SRC has changed over time because the proportion with the dcSSc subset is smaller in contemporary cohorts...
July 2016: Journal of Rheumatology
Antonietta Gigante, Biagio Barbano, Guido Granata, Silvia Quarta, Antonio Amoroso, Felice Salsano, Rosario Cianci, Edoardo Rosato
OBJECTIVES: The most important renal complication of systemic sclerosis (SSc) is scleroderma renal crisis (SRC). Many patients demonstrate less severe renal complications, most likely associated with reduced renal blood flow and a consequent reduction in glomerular filtration rate (GFR). The mechanism of this slowly progressive form of chronic renal disease is unclear. The aim of this study was to evaluate GFR by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) and the 7-variable Modification of Diet and Renal Disease (MDRD) equations in SSc patients and to correlate estimated GFR (eGFR) with clinical variables of the disease...
June 2016: Clinical Nephrology
Cecilia Beatrice Chighizola, Francesca Pregnolato, Pier Luigi Meroni, Christopher Paul Denton, Voon H Ong
OBJECTIVES: Although in scleroderma renal crisis (SRC) outcome has improved to a great extent with the introduction of ACE inhibitors, there remains significant mortality and morbidity with frequent requirement for renal replacement therapy. Therefore, novel biomarkers to identify patients at high risk of poor outcome would be valuable. The aim of this study was to assess the role of the N terminal fragment of pro Brain Natriuretic Peptide (N-TproBNP) as predictor of outcome in SRC. METHODS: 20 subjects with confirmed SRC were retrospectively enrolled...
September 2016: Clinical and Experimental Rheumatology
Daisuke Takada, Junichi Hoshino, Koichi Kikuchi, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Akinari Sekine, Masayuki Yamanouchi, Keiichi Sumida, Koki Mise, Tatsuya Suwabe, Noriko Hayami, Naoki Sawa, Kenmei Takaichi, Nobukazu Hayasi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
A 69-year-old Japanese man was presented with hypertensive crisis. Renal histology revealed malignant nephrosclerosis, including an onion skin pattern with fibrinoid necrosis of the small arteries from arterioles up to interlobular arteries. Immunological investigation clarified positive anti-RNA polymerase (RNAP) III antibody, and limited cutaneous systemic sclerosis (Lc SSc) was diagnosed by skin biopsy as the underlying disease causing scleroderma renal crisis (SRC). Angiotensin covering enzyme (ACE) inhibitor therapy and calcium antagonist were effective for his renal condition...
March 4, 2016: Modern Rheumatology
Jiri Vymetal, Martina Skacelova, Andrea Smrzova, Anna Klicova, Marketa Schubertova, Pavel Horak, Josef Zadrazil
BACKGROUND AND AIM: Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides...
March 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
Cybele Ghossein, John Varga, Andrew Z Fenves
Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication...
January 2016: Current Rheumatology Reports
R P Goswami, S Mondal, K Basu, S Das, P Ghosh, A Ghosh
No abstract text is available yet for this article.
November 2015: Indian Journal of Nephrology
Christie P Thomas, Carla M Nester, Andrew C Phan, Manisha Sharma, Amanda L Steele, Petar S Lenert
A 46-year-old female with interstitial lung disease presented with proximal muscle weakness, worsening hypertension, microangiopathic hemolysis, thrombocytopenia and deteriorating renal function. She had no sclerodactyly, but had abnormal capillaroscopy. She tested positive for PM-Scl antibodies, and a renal biopsy showed an acute thrombotic microangiopathy consistent with scleroderma renal crisis (SRC). She failed to respond to corticosteroids, plasmapheresis and renin-angiotensin pathway inhibitors. She recovered quickly with the anti-C5 antibody, eculizumab...
December 2015: Clinical Kidney Journal
Anna Postolova, Jennifer K Chen, Lorinda Chung
Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients...
February 2016: Rheumatic Diseases Clinics of North America
C P Simeón-Aznar, V Fonollosa-Plá, Carles Tolosa-Vilella, G Espinosa-Garriga, M Campillo-Grau, M Ramos-Casals, F J García-Hernández, M J Castillo-Palma, J Sánchez-Román, J L Callejas-Rubio, N Ortego-Centeno, M V Egurbide-Arberas, L Trapiellla-Martínez, L Caminal-Montero, L Sáez-Comet, J Velilla-Marco, M T Camps-García, E de Ramón-Garrido, E M Esteban-Marcos, L Pallarés-Ferreres, N Navarrete-Navarrete, J A Vargas-Hitos, R Gómez de la Torre, G Salvador-Cervello, J J Rios-Blanco, M Vilardell-Tarrés
Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15...
October 2015: Medicine (Baltimore)
Mouhamadou Moustapha Cisse, Sidy Mohamed Seck, Daher Abdoul Karim Oumar, Khodia Fall, Ahmed Tall Lemrabott, Moussa Diallo, Maria Faye, Moustapha Faye, Abdou Niang, Boucar Diouf
Scleroderma renal crisis (SRC) is defined as the new onset of accelerated arterial hypertension and /or rapidly progressive oliguric renal failure during the course of systemic sclerosis. It is a rare but life-threatening complication. This formerly serious complication has got a considerable brighter outlook since the introduction of angiotensin converting enzyme inhibitors (ACE) however the mortality is still remaining high. We report two cases of SRC which to our knowledge are the firsts described in Dakar...
2015: Pan African Medical Journal
Marie Hudson
PURPOSE OF REVIEW: There are important knowledge gaps concerning scleroderma renal crisis (SRC), in large part because its rarity has hampered research. Many studies to date share limitations, in particular small samples, prevalent cases, and retrospective study designs. This review features some of the most recent studies that attempt to shed new insights into SRC while trying to overcome those limitations. RECENT FINDINGS: The most important recent progress in the understanding of the pathophysiology of SRC includes identification of novel genetic and serological biomarkers...
November 2015: Current Opinion in Rheumatology
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