Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Tzelepis, George E.; Toya, Sophie P.; Moutsopoulos, H. M.

doi:10.1183/09031936.00060107

Cited by 104 publications

(65 citation statements)

References 102 publications

(147 reference statements)

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“…Most often, interstitial pneumonia arises within the context of an established CTD, but it is not uncommon for the interstitial pneumonia to be the first, and possibly the sole, manifestation of an otherwise occult CTD [7][8][9][10]. Identifying underlying CTD in patients presenting with what is initially considered to be an IIP can be challenging [9,[11][12][13][14][15][16], as boundaries between IIPs and CTD-ILDs are not clearly defined.…”

Section: Introductionmentioning

confidence: 99%

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

et al. 2015

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Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort. @ERSpublications ERS/ATS task force provides nomenclature and classification criteria for patients with IIP and autoimmune features

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Section: Introductionmentioning

confidence: 99%

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

et al. 2015

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“…Since the spectrum of ILD associated with CTD is broad, careful evaluation for autoantibodies or other serologic tests in conjunction with clinical features of autoimmune disease is crucial [19,20]. Because of the variable incidence and outcome of ILD in CTD, biomarkers including autoantibodies are critical for diagnosis, prognosis, patient subtyping, and predicting response to treatment.…”

Section: Laboratory Tests and Biomarkersmentioning

confidence: 99%

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

Ishii¹

2017

Contemporary Topics of Pneumonia

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Interstitial pneumonia (IP) refers to involvement of the lung parenchyma by varying degrees of inflammation and fibrosis, in contrast to airspace disease typically seen in bacterial pneumonia. IP lies in the center of a heterogenous group of diffuse interstitial lung diseases (ILDs), either idiopathic or linked to underlying disorders. One of the major categories of disorders frequently associated with IP is a connective tissue disease (CTD), in which autoimmunemediated tissue injury leads to multiple organ impairment. Today, IP represents the most critical pulmonary complication in CTD, resulting in significant morbidity and mortality.Despite growing understanding of the pathology of IPs, as well as the accumulating knowledge from both basic and clinical studies of CTDs, the pathogenesis of CTD-associated IP remains unclear. This chapter will provide an overview of the general understanding of ILD and illustrate the current state of knowledge on IP associated with CTD, in order to fully comprehend the entirety of its complex pictures. Moreover, we will propose a new insight into the immune pathogenesis of CTD-IP by presenting evidence which robustly indicates that T cells trigger initial development of IP in polymyositis/dermatomyositis, suggesting potential approaches for controlling such particular T cells in therapeutic interventions for IP.

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“…Polymyositis/dermatomyositis patients may present with typical skin lesions (violaceous scaling papules -Gottron's papules and facial heliotrope rash), mechanic's hands, and muscle disease. 12,13 Patients with connective tissue disease-associated ILD often have a better response to immunosuppressive therapy and a better prognosis compared to patients with other ILD forms such as IPF. Furthermore, screening for connective tissue disease may lead to early treatment of other extrapulmonary systemic complications such as renal disease in scleroderma patients.…”

Section: Connective Tissue Diseasementioning

confidence: 99%

Diagnostic assessment of patients with interstitial lung disease

Gulati

2011

Primary Care Respiratory Journal

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The diagnosis of interstitial lung disease (ILD) is frequently delayed because clinical clues are neglected and respiratory symptoms are ascribed to more common pulmonary diagnoses such as chronic obstructive pulmonary disease (COPD) in the primary care setting. While ILD cases ultimately require referral to a pulmonologist, general practitioners can play a crucial role in recognising the need for, and initiating, a diagnostic evaluation. An initial assessment hinges upon a structured history and physical examination with careful attention paid to occupational, environmental and drug exposures as well as a history of symptoms suggesting connective tissue disease. Ultimately a surgical lung biopsy may be indicated, but high resolution computed tomography (HRCT) chest scans are essential to the diagnostic work-up since each ILD form is characterised by a specific pattern of abnormalities.

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Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Cited by 104 publications

References 102 publications

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

Diagnostic assessment of patients with interstitial lung disease

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