Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase

Solomon, Joshua J.; Swigris, Jeffrey J.; Brown, Kevin K.

doi:10.1590/s1806-37132011000100015

Cited by 226 publications

(97 citation statements)

References 78 publications

(120 reference statements)

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“…These criteria proposed that all patients with anti-synthetase syndrome must have evidence for a tRNA synthetase autoantibody, in addition to one or more of the following clinical features: mechanic’s hands, Raynaud’s phenomenon, myositis, ILD, arthritis, and/or unexplained fever (table 1). In 2011, Solomon et al proposed alternative, stricter criteria, requiring two major or one major and two minor criteria, in addition to the presence of an aminoacyl-tRNA synthetase autoantibody (10). Historically, patients with these antibodies were diagnosed with an IIM or were unrecognized given their atypical clinical features.…”

Section: Diagnosismentioning

confidence: 99%

The Diagnosis and Treatment of Antisynthetase Syndrome

Witt

Curran

Strek

2016

Clinical Pulmonary Medicine

168

159

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Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud’s phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results. Patients with anti-synthetase syndrome often require multi-modality immunosuppressive therapy in order to control the muscle and/or pulmonary manifestations of their disease. The long-term care of these patients mandates careful attention to the adverse effects and complications of chronic immunosuppressive therapy, as well as disease-related sequelae that can include progressive interstitial lung disease necessitating lung transplantation, pulmonary hypertension, malignancy and decreased survival. It is hoped that greater awareness of the clinical features of this syndrome will allow for earlier diagnosis and appropriate treatment to improve outcomes in patients with anti-synthetase syndrome.

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Section: Diagnosismentioning

confidence: 99%

The Diagnosis and Treatment of Antisynthetase Syndrome

Witt

Curran

Strek

2016

Clinical Pulmonary Medicine

168

159

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“…42 While Connors and colleagues suggested that the presence of a circulating tRNA synthetase antibody in the setting of ILD is sufficient to diagnose the anti-synthetase syndrome 43 , Solomon and colleagues proposed that inflammatory arthritis, Raynaud’s or mechanics hands (at least two of three) be present in such patients without overt polymyositis/dermatomyositis to meet criteria for anti-synthetase syndrome. 44 Mejia and colleagues recently demonstrated that all patients with ILD and a circulating tRNA synthetase antibody who failed to meet criteria for dermatomyositis subsequently met IPAF criteria and that survival was similar between the two groups. 45 Over one third of patients included in the IPAF cohort characterized by Chartrand and colleagues had a positive tRNA synthetase antibody, which given the proclivity of this group to respond to immunosuppression 46–48 , may explain the low mortality in this group compared to other IPAF cohorts.…”

Section: Unanswered Questionsmentioning

confidence: 99%

Interstitial Pneumonia With Autoimmune Features: Overview of Proposed Criteria and Recent Cohort Characterization

Lee

Oldham

2017

Clinical Pulmonary Medicine

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The accurate diagnosis of interstitial lung disease (ILD) is essential for optimal prognostication and management. While connective tissue disease (CTD) is among the most common causes of ILD, some patients have features suggestive of autoimmunity without meeting criteria for a specific CTD. To help define and study this disease entity more uniformly, a 2015 research statement proposed consensus-based criteria and coined the term “interstitial pneumonia with autoimmune features” (IPAF). In this review, we summarize and compare previously proposed criteria to characterize these patients, provide an overview of the IPAF criteria and highlight recent investigations aimed at characterizing IPAF cohorts. We then call attention to questions that have arisen with the application of the IPAF criteria and discuss future areas of study.

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“…Ос-новным критерием включения было наличие тяжелого поражения легких, резистентного к стандартной тера-пии, или острое течение в дебюте болезни, что является неблагоприятным прогностическим признаком и харак-терно для антисинтетазного синдрома [60]. 17 пациентов были резистентны к проводимой ранее стандартной ци-тотоксической терапии.…”

Section: материал и методыunclassified

Assessment of flares in lupus patients enrolled in a phase II/III study of rituximab (EXPLORER)

Merrill

Buyon

Furie

et al. 2011

Lupus

124

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The EXPLORER study was designed to assess the response to rituximab versus placebo in patients with moderate to severe extrarenal systemic lupus erythematosus (SLE) receiving background immunosuppression. The definition of response required reduced clinical activity without subsequent flares over 52 weeks, and the study did not meet its efficacy endpoint. The current exploratory analysis assessed flare rates in patients who achieved initial low disease activity response (British Isles Lupus Assessment Group [BILAG] C or better in all organs) during the study. Exploratory reanalysis of data from the EXPLORER trial was conducted, considering alternative definitions for flare. No difference was found between rituximab and placebo in preventing or delaying moderate to severe flares. However, when severe (BILAG A) flares alone were examined, rituximab reduced the risk of a subsequent first A flare (hazard ratio ¼ 0.61; p ¼ 0.052) and lowered mean AE SD annualized A flare rates (0.86 AE 1.47 vs. 1.41 AE 2.14; p ¼ 0.038). Eighty-four (49.7%) rituximab-treated patients achieved low disease activity without subsequent A flares versus 31 (35.2%) placebo-treated patients (p ¼ 0.027). Prednisone rescue for A flares was similar in rituximab-(24%) and placebo-treated (14%) patients (p ¼ 0.204). This post hoc analysis evaluates the hypothesis that assessment of BILAG A flares may distinguish potential treatment effects with greater sensitivity than assessment of BILAG B flares. Lupus (2011) 20, 709-716.

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Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase

Cited by 226 publications

References 78 publications

The Diagnosis and Treatment of Antisynthetase Syndrome

The Diagnosis and Treatment of Antisynthetase Syndrome

Interstitial Pneumonia With Autoimmune Features: Overview of Proposed Criteria and Recent Cohort Characterization

Assessment of flares in lupus patients enrolled in a phase II/III study of rituximab (EXPLORER)

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