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Autoimmun Rev ; 16(4): 407-415, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28212921


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. Two reviewers independently screened eligible papers for final study selection. Data was extracted using a customized form. A web-based survey of members of the Scleroderma Clinical Trials Consortium was used to identify unpublished definitions of SRC. RESULTS: We identified 415 papers that met inclusion criteria. Forty original definitions of SRC were identified from 36 studies, 9 reviews and 2 editorials. There was significant heterogeneity in definitions. As a rule, though, in addition to new-onset hypertension and acute kidney injury, other common items used to define SRC included hypertensive encephalopathy and seizures, microangiopathic hemolytic anemia and characteristic changes on kidney biopsy. The web-based survey identified unpublished definitions of SRC that were largely consistent with the results of the published literature. CONCLUSION: SRC was defined in a minority of studies and criteria were heterogeneous. A consensus definition of SRC is urgently needed to standardize data collection on SRC and further our understanding of this serious problem.

Insuficiência Renal Crônica/etiologia , Escleroderma Sistêmico/complicações , Doenças Autoimunes , Humanos
Clin Exp Rheumatol ; 34 Suppl 100(5): 106-109, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27749244


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. Patients with SRC may recover renal function up to 3 years after the crisis, most often within 12 to 18 months.

Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/terapia , Hipertensão/diagnóstico , Hipertensão/terapia , Nefrologia/normas , Terapia de Substituição Renal/normas , Reumatologia/normas , Escleroderma Sistêmico/complicações , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/mortalidade , Inibidores da Enzima Conversora de Angiotensina/administração & dosagem , Antiarrítmicos/administração & dosagem , Anticonvulsivantes/administração & dosagem , Anti-Hipertensivos/administração & dosagem , Consenso , Procedimentos Clínicos/normas , Esquema de Medicação , Medicina Baseada em Evidências/normas , Humanos , Hipertensão/etiologia , Hipertensão/mortalidade , Valor Preditivo dos Testes , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/mortalidade , Escleroderma Sistêmico/terapia , Resultado do Tratamento , Reino Unido
Rheum Dis Clin North Am ; 41(3): 367-82, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26210124


Systemic sclerosis is a multisystem disorder with a high associated mortality. The hallmark abnormalities of the disease are in the immune system, vasculature, and connective tissue. Systemic sclerosis occurs in susceptible individuals and is stimulated by initiating events that are poorly understood at present. In order for the disease phenotype to appear there is dysfunction in the homoeostatic mechanisms of immune tolerance, endothelial physiology, and extracellular matrix turnover. The progression of disease is not sequential but requires simultaneous dysfunction in these normal regulatory mechanisms. Better understanding of the interplay of these factors is likely to contribute to improved treatment options.

Escleroderma Sistêmico/etiologia , Autoanticorpos/imunologia , Quimiocinas/fisiologia , Fator de Crescimento do Tecido Conjuntivo/fisiologia , Citocinas/fisiologia , Progressão da Doença , Sistema Endócrino/fisiologia , Feminino , Fibroblastos/fisiologia , Estudo de Associação Genômica Ampla , Antígenos HLA/imunologia , Humanos , Imunidade Celular/fisiologia , Interleucinas/fisiologia , Neoplasias/complicações , Doença de Raynaud/complicações , Escleroderma Sistêmico/genética , Escleroderma Sistêmico/imunologia , Vasoconstritores/farmacologia
BMC Nephrol ; 15: 133, 2014 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-25128003


BACKGROUND: A translational study in renal transplantation suggested YKL-40, a chitinase 3-like-1 gene product, plays an important role in acute kidney injury (AKI) and repair, but data are lacking about this protein in urine from native human kidneys. METHODS: This is an ancillary study to a single-center, prospective observational cohort of patients with clinically-defined AKI according to AKI Network serum creatinine criteria. We determined the association of YKL -40 ≥ 5 ng/ml, alone or combined with neutrophil gelatinase-associated lipocalin (NGAL), in urine collected on the first day of AKI with a clinically important composite outcome (progression to higher AKI stage and/or in-hospital death). RESULTS: YKL-40 was detectable in all 249 patients, but urinary concentrations were considerably lower than in previously measured deceased-donor kidney transplant recipients. Seventy-two patients (29%) progressed or died in-hospital, and YKL-40 ≥ 5 ng/ml had an adjusted odds ratio (95% confidence interval) for the outcome of 3.4 (1.5-7.7). The addition of YKL-40 to a clinical model for predicting the outcome resulted in a continuous net reclassification improvement of 29% (P = 0.04). In patients at high risk for the outcome based on NGAL concentrations in the upper quartile, YKL-40 further partitioned the cohort into moderate-risk and very high-risk groups. CONCLUSIONS: Urine YKL-40 is associated with AKI progression and/or death in hospitalized patients and improves clinically determined risk reclassification. Combining YKL-40 with other AKI biomarkers like NGAL may further delineate progression risk, though additional studies are needed to determine whether YKL-40 has general applicability and to define its association with longer-term outcomes in AKI.

Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/urina , Adipocinas/urina , Progressão da Doença , Hospitalização , Lectinas/urina , Lesão Renal Aguda/mortalidade , Idoso , Biomarcadores/urina , Proteína 1 Semelhante à Quitinase-3 , Estudos de Coortes , Feminino , Hospitalização/tendências , Humanos , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Estudos Prospectivos