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Arthritis Res Ther ; 22(1): 5, 2020 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-31915059


OBJECTIVE: To describe successful therapeutic strategies in statin-induced anti-HMGCR myopathy. METHODS: Retrospective data from a cohort of 55 patients with statin-induced anti-HMGCR myopathy, sequentially stratified by the presence of proximal weakness, early remission, and corticosteroid and IVIG use at treatment induction, were analyzed for optimal successful induction and maintenance of remission strategies. RESULTS: A total of 14 patients achieved remission with a corticosteroid-free induction strategy (25%). In 41 patients treated with corticosteroids, only 4 patients (10%) failed an initial triple steroid/IVIG/steroid-sparing immunosuppressant (SSI) induction strategy. Delay in treatment initiation was independently associated with lower odds of successful maintenance with immunosuppressant monotherapy (OR 0.92, 95% CI 0.85 to 0.97, P = 0.015). While 22 patients (40%) presented with normal strength, only 9 had normal strength at initiation of treatment. CONCLUSION: While corticosteroid-free treatment of anti-HMGCR myopathy is now a safe option in selected cases, initial triple steroid/IVIG/SSI was very efficacious in induction. Delays in treatment initiation and, as a corollary, delays in achieving remission decrease the odds of achieving successful maintenance with an SSI alone. Avoiding such delays, most notably in patients with normal strength, may reset the natural history of anti-HMGCR myopathy from a refractory entity to a treatable disease.

Artigo em Inglês | MEDLINE | ID: mdl-31535689


OBJECTIVE: Interstitial lung disease (ILD) is a leading cause of mortality in SSc. Little is known about the benefits of immunosuppressive drugs in mild ILD. Our aim was to determine whether use of CYC or MMF was associated with an improved ILD course in patients with normal or mildly impaired lung function. METHODS: A retrospective cohort of SSc subjects with ILD, disease duration below seven years and no exposure to CYC or MMF prior to the baseline visit was constructed from the Canadian Scleroderma Research Group registry. Subjects were categorized as having mild ILD if baseline forced vital capacity (FVC % predicted) was >85%. The primary exposure was any use of CYC or MMF at the baseline visit. FVC at one year was compared between exposed and unexposed subjects, using multivariate linear regression. RESULTS: Out of 294 eligible SSc-ILD subjects, 116 met criteria for mild ILD. In this subgroup, mean (s.d.) disease duration was 3.7 (2.0) years. Thirteen (11.2%) subjects were exposed to CYC or MMF at baseline. The one-year FVC was higher in exposed subjects compared with unexposed subjects, by a difference of 8.49% (95% CI: 0.01-16.98%). None of the exposed subjects experienced clinically meaningful progression over two years, whereas 24.6% of unexposed subjects did. CONCLUSION: In this real-world setting, CYC/MMF exposure at baseline was associated with higher FVC values and a lower risk of progression among subjects with mild ILD. These data suggest a window of opportunity to preserve lung function in SSc-ILD.

Arthritis Rheumatol ; 71(6): 964-971, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30614663


OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.

Eur Respir Rev ; 27(148)2018 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-29769294


Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.

Doenças Pulmonares Intersticiais/diagnóstico , Pulmão/fisiopatologia , Testes de Função Respiratória , Escleroderma Sistêmico/complicações , Biópsia , Progressão da Doença , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Valor Preditivo dos Testes , Prognóstico , Capacidade de Difusão Pulmonar , Reprodutibilidade dos Testes , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Fatores de Tempo , Tomografia Computadorizada por Raios X , Capacidade Pulmonar Total , Capacidade Vital
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
Semin Arthritis Rheum ; 46(4): 488-508, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27908534


OBJECTIVE: The aim of this review was to summarize key findings from the literature concerning the therapeutic role of intravenous immunoglobulins (IVIg) in idiopathic inflammatory myopathies (IIM), dissecting the evidence according to disease subtype and treatment indication, and to review the evidence relating to the mechanism of action of IVIg in IIM to ascertain rationale for continued research. METHODS: Medline (Ovid) and Pubmed databases were searched from inception to July 2016 using relevant keywords. Original and review articles were retrieved for full-text review. Bibliographies of selected articles were also hand-searched for additional references. Data were summarized qualitatively and in tabular form. RESULTS: The efficacy of IVIg in IIM is supported by 3 randomized controlled trials, involving dermatomyositis and polymyositis subjects, in refractory, relapsed, or steroid-dependent disease, as well as part of first-line therapy in elderly dermatomyositis subjects. Other indications for IVIg are supported by uncontrolled evidence only. Limitations of studies include open, uncontrolled or retrospective study designs, small and selected samples, short-term follow-up and ad hoc outcome measures. Despite the limited evidence, there is strong biological plausibility for the role of IVIg in IIM. CONCLUSION: Robust, controlled evidence to support the use of IVIg using validated outcome measures is urgently required to guide therapeutic decision-making and maximize outcomes in IIM.

Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Miosite/tratamento farmacológico , Humanos , Resultado do Tratamento