Systemic sclerosis sine scleroderma presenting as pulmonary intersticial fibrosis

Sanchis, David Vañó; G, Garcia; Yglesias, P. J. Marchena

doi:10.1007/s10067-005-0026-z

Cited by 7 publications

(3 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Interestingly, anti-PM/Scl-associated ILD in the absence of overt CTD manifestations and similar to conditions reported in patients with anti-ARS antibodies 27,28 has not been reported previously. This condition has likely been underrecognized, because the anti-PM/Scl autoantibodies are often not measured in the absence of clinical myositis or SSc manifestations 29 . In addition, our series was character- www.jrheum.org Downloaded on May 28, 2024 from ized by the low prevalence of definite CTD, highlighting the need for careful assessment of patients presenting with ILD in search of manifestations of formes frustes of CTD, notably amyopathic DM (DM rash without myositis) and SSc sine scleroderma characterized by visceral organ involvement (lungs, kidneys, gastrointestinal tract) with abnormal nailfold capillaries and ANA 30 .…”

Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease Associated with Anti-PM/Scl or Anti-Aminoacyl-tRNA Synthetase Autoantibodies: A Similar Condition?

Lega

Cottin²,

Fabien³

et al. 2010

J Rheumatol

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease Associated with Anti-PM/Scl or Anti-Aminoacyl-tRNA Synthetase Autoantibodies: A Similar Condition?

Lega

Cottin²,

Fabien³

et al. 2010

J Rheumatol

View full text Add to dashboard Cite

show abstract

“…Patients with underlying CTD may be relatively free of musculoskeletal symptoms, especially early in their disease course, and ILD may be the initial manifestation which leads to their eventual diagnosis. 11,19 Indeed, evolution of apparent idiopathic ILD into a welldefined CTD is well described in the literature. [8][9][10][11][12][13] Our data did not show a significant difference relative to the EHS, which we postulate is due to the lower prevalence of RA in our CTD cohort (12.0%) compared with the initial discovery study (41.3%).…”

Section: Discussionmentioning

confidence: 99%

“…However, the current study indicates that even with a UIP pattern on CT, other imaging findings should be considered in achieving an accurate diagnosis, as UIP may be a manifestation of other causes of ILD, which might not be manifest initially. Patients with underlying CTD may be relatively free of musculoskeletal symptoms, especially early in their disease course, and ILD may be the initial manifestation which leads to their eventual diagnosis 11,19 . Indeed, evolution of apparent idiopathic ILD into a well-defined CTD is well described in the literature 8–13 …”

Section: Discussionmentioning

confidence: 99%

Computed Tomography Findings Suggestive of Connective Tissue Disease in the Setting of Usual Interstitial Pneumonia

Chung

Montner

Thirkateh

et al. 2021

J Comput Assist Tomogr

View full text Add to dashboard Cite

Purpose: A usual interstitial pneumonia (UIP) pattern is common in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD). The purpose of the study was to validate imaging findings differentiating CTD-ILD from IPF in UIP.Methods: Patients with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern on computed tomography and/or pathology were included in this study. Prevalence of 3 computed tomography findings shown to be associated with CTD-ILD (the straight edge sign [SES], the exuberant honeycombing sign, and the anterior upper lobe sign [AULS]) were tabulated in CTD-ILD and IPF subjects. The ability of each of these signs to discriminate between CTD-ILD and IPF was evaluated. Survival analysis was also performed using log-rank analysis. Results:The study cohort included 50 CTD-ILD and 100 IPF subjects with UIP. The SES and the AULS were more common in CTD-ILD than IPF (prevalence, 36.0% and 34.9% in CTD-ILD vs 8.3% and 17.2% in IPF, respectively [P = 0.0105 − <0.001]). The highest specificity (95.7%) of CTD-ILD diagnosis was seen with bilateral SES. Moreover, the SES was associated with improved survival (P = 0.0383), which appeared to be largely because of improvement in survival in IPF subjects. The presence of AULS was associated with pulmonary functional abnormalities. Conclusions:A radiographic UIP pattern with evidence of SES or the AULS should raise suspicion for CTD-ILD rather than IPF. Patients with IPF and SES have an attenuated disease course and might represent a different phenotype than those without the SES.

show abstract

The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications

Toya

Tzelepis

2009

Rheumatol Int

View full text Add to dashboard Cite

Scleroderma sine scleroderma (ssSSc) is an occult form of systemic sclerosis that may cause diagnostic difficulties due to the absence of skin involvement. Delays in the diagnosis of ssSSc means lost opportunites to address and treat the often lethal involvement of internal organs such as the lungs and heart. In this systemic review we collected all published cases of ssSSc using EMBASE, MEDLINE, PubMed, and Web of Science from 1950 to present. Our purpose was to describe the range and frequency of the clinical manifestations of ssSSc. A total of 108 published cases of ssSSc were analyzed. Lung involvement was present in 66% of cases. Peripheral vascular system involvement was present in all patients whereas gastrointestinal manifestations were present in 82% of the cases. Overall the clinical presentation is subtle and heightened clinical awareness is required to facilitate prompt recognition and treatment.

show abstract

Systemic sclerosis sine scleroderma presenting as pulmonary intersticial fibrosis

Cited by 7 publications

References 5 publications

Interstitial Lung Disease Associated with Anti-PM/Scl or Anti-Aminoacyl-tRNA Synthetase Autoantibodies: A Similar Condition?

Interstitial Lung Disease Associated with Anti-PM/Scl or Anti-Aminoacyl-tRNA Synthetase Autoantibodies: A Similar Condition?

Computed Tomography Findings Suggestive of Connective Tissue Disease in the Setting of Usual Interstitial Pneumonia

The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications

Contact Info

Product

Resources

About