Histopathology of Lung Disease in the Connective Tissue Diseases

Vivero, Marina; Padera, Robert F.

doi:10.1016/j.rdc.2014.12.002

Cited by 41 publications

(60 citation statements)

References 71 publications

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“…However, this is not part of our clinical experience in very wellselected pSs patients, especially the development of fibrotic ILDs, raising thoughts about the coexistence of a second CTD although not yet fulfilling undisputed diagnostic criteria [29] .…”

Section: Other Interstitial Pneumoniasmentioning

confidence: 98%

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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Section: Other Interstitial Pneumoniasmentioning

confidence: 98%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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“…The radiological and corresponding histological patterns defining each entity of CTD-associated IP are summarized in Table 2 [11]. Although there is substantial histological overlap among the pulmonary manifestations of different CTDs and with other etiologies, certain histologic patterns may favor one CTD over another, and occasionally distinctive histologic clues may be present [35,36]. It is possible in many cases to confirm CTD-ILD and guide patient management using histologic features.…”

Section: Pulmonary Histopathology In Connective Tissue Diseasementioning

confidence: 99%

“…Fibroblast foci (regions of new fibrosis) are found at the interface between central and peripheral regions [35]. While primary UIP pattern is found in 13-56% of RA-ILD cases, some studies have reported that the most frequent histologic pattern of ILD among RA patients is NSIP, followed closely by UIP, accounting for 30-67% and 13-57% of RA-ILD, respectively [36]. Studies of PM/DM-ILD identified an UIP pattern in 5-33% of cases.…”

Section: Usual Interstitial Pneumonia (Uip)mentioning

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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“…The limited SSc form is dominated by vascular manifestations with milder skin and internal organ fibrosis, generally accompanied by a low progression rate. In contrast, diffuse SSc patients is a rapidly progressive fibrosis of the internal organs and skin, leading to a high mortality risk [20,21].…”

Section: Overview Of Sscmentioning

confidence: 99%