Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group

Sánchez‐Cano, Daniel; Ortego-Centeno, Norbérto; Callejas‐Rubio, José Luis; Plá, Vicent Fonollosa; Ríos-Fernández, Raquel; Tolosa-Vilella, Carles; Espinosa, Gerard; Colunga-Argüelles, Dolores; Egurbide-Arberas, María Victoria; Rubio‐Rivas, Manuel; Freire, Mayka; Ríos-Blanco, Juan José; Trapiella-Martínez, Luis; Rodríguez-Carballeira, Mónica; Marín-Ballvé, Adela; Pla-Salas, Xavier; Simeón-Aznar, Carmen Pilar

doi:10.1007/s00296-017-3916-x

Cited by 58 publications

(40 citation statements)

References 40 publications

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“…Studies in patients with progressive fibrosing ILDs have identified several factors that predict mortality, but these need to be interpreted carefully given the variation in the methodology used and the retrospective nature of most of the studies. Lower FVC is an established predictor of mortality in patients with progressive fibrosing ILDs, as evidenced by numerous studies spanning IPF [55][56][57], RA-ILD [4,49], SSc-ILD [58][59][60], chronic HP [61,62] and fibrotic iNSIP [53]. The same is true of DLco [55,56,60,[63][64][65], although this is harder to assess in multi-center studies due to a lack of standardization in its measurement.…”

Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

confidence: 99%

The natural history of progressive fibrosing interstitial lung diseases

2019

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A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.

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Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

confidence: 99%

The natural history of progressive fibrosing interstitial lung diseases

2019

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“…When considering patients with SARD as controls, it is important to rule out overlap syndromes 5. One example is the association of AIM with interstitial lung disease, which can occur in myositis and in other SARD and especially systemic sclerosis 6. The differences observed for anti-Jo-1 antibodies are surprising and concerning since those antibodies have been measured for many years,5 and proficiency testing programmes have shown mostly consistent results (eg, https://www.immqas.org.uk).…”

mentioning

confidence: 99%

Detection of myositis-specific antibodies: additional notes

Mähler

Fritzler

2018

Ann Rheum Dis

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“…El compromiso pulmonar en las ETC es un problema creciente. Mientras la mortalidad global por ETC parece venir disminuyendo 52,53 , datos actualizados de registros, cohortes y seguros muestran que el compromiso pulmonar en estos pacientes puede estar aumentado e incrementa la morbimortalidad de los enfermos [54][55][56] .…”

Section: Enfermedad Intersticial Asociada a Enfermedad Del Tejido Conunclassified

Enfermedades pulmonares intersticiales. Una perspectiva actual

et al. 2019

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Update on interstitial lung diseasesInterstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.

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Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group

Abstract: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

Cited by 58 publications

References 40 publications

The natural history of progressive fibrosing interstitial lung diseases

The natural history of progressive fibrosing interstitial lung diseases

Detection of myositis-specific antibodies: additional notes

Enfermedades pulmonares intersticiales. Una perspectiva actual

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