Interstitial Lung Disease in Scleroderma

Schoenfeld, Sara R.; Castelino, Flavia V.

doi:10.1016/j.rdc.2014.12.005

Cited by 80 publications

(64 citation statements)

References 53 publications

(63 reference statements)

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“…SSc is a chronic autoimmune CTD of unknown aetiology, associated with considerable morbidity and increased mortality [55]. Reported percentages of patients with SSc who develop ILD range 35-90%; SSc-ILD often manifests as NSIP and is a major cause of morbidity and mortality [56][57][58][59]. Although SSc can occur at any age, patients are usually 30-60 years of age, and women are more likely to develop SSc than men [55,60,61].…”

Section: Ssc-associated Ildmentioning

confidence: 99%

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

et al. 2018

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@ERSpublications ILDs with a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors. Further epidemiological studies are warranted to help identify these patients. http://ow.ly/SY6m30mWytM Cite this article as: Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27: 180077 [https://doi.ABSTRACT The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. Disease behaviour is well documented in idiopathic pulmonary fibrosis but idiosyncratic in other ILDs that may present a progressive fibrosing phenotype. Possible reasons may include the heterogeneous nature of the aetiology, the complexity of diagnosis (and subsequent documentation of cases) and the methods employed to retrospectively analyse patient databases. This review presents a broad overview of the epidemiological data available for ILDs that may present a progressive-fibrosing phenotype, collectively and stratified according to clinical classification. We also note where further data are needed in comparison to the well-studied IPF indication.

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Section: Ssc-associated Ildmentioning

confidence: 99%

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

et al. 2018

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“…29,49 The most prevalent pattern of SSc-ILD is non-specific interstitial pneumonia (NSIP), seen in up to 78% of cases, followed by usual interstitial pneumonia (UIP) in 25–40% of cases. 53,54 …”

Section: Systemic Sclerosismentioning

confidence: 99%

“…SSc-ILD is treated with low dose CS (up to 15mg oral prednisone daily) usually in conjunction with other immunosuppressants, 54 particularly in patients with NSIP and significant ground-glass opacification.…”

Section: Systemic Sclerosismentioning

confidence: 99%

Corticosteroids in Myositis and Scleroderma

Postolova

Chen

Chung

2016

Rheumatic Disease Clinics of North America

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Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering.

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“…A patient with diffuse cutaneous SS without any type of diffuse fibrotic ILD is uncommonly encountered [137] . Fibrotic ILD is also evident in a considerable proportion of patients with limited cutaneous SS [138] .…”

Section: Interstitial Lung Diseasementioning

confidence: 99%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

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Interstitial Lung Disease in Scleroderma

Cited by 80 publications

References 53 publications

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

Corticosteroids in Myositis and Scleroderma

Investigation of Lung Involvement in Connective Tissue Disorders

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