Predicting cardiopulmonary involvement in patients with systemic sclerosis: complementary value of nailfold videocapillaroscopy patterns and disease-specific autoantibodies

Im, Markusse; Meijs, J.; B, De Boer; Ja, Bakker; Hpc, Schippers; Aa, Schouffoer; Marsan, Nina Ajmone; Ljm, Kroft; Mk, Ninaber; Huizinga, T.; Jk, de Vries-Bouwstra

doi:10.1093/rheumatology/kew402

Cited by 45 publications

(65 citation statements)

References 27 publications

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“…In addition, the single NVC pattern and its score may be of great value to predict novel future SSc organ involvement, as recently demonstrated [9,19,38]. Previous studies have demonstrated the positive effects of therapeutic approaches on microvascular damage in SSc [1214].…”

Section: Discussionmentioning

confidence: 99%

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study

et al. 2019

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Objective The aim of this observational study was to investigate the evolution of scleroderma microangiopathy throughout different nailfold videocapillaroscopy (NVC) patterns (‘early’, ‘active’, ‘late’) as well as the prevalence of organ involvement in SSc patients during a 12-year follow-up. Methods Thirty-four SSc patients showing at baseline (first capillaroscopic analysis) the ‘early’ NVC pattern of microangiopathy were enrolled and followed for 12 years (s.d. 2). Complete NVC analysis and clinical and serological findings were collected. Patients were in a standard therapeutic care setting. Statistical analysis was carried out by non-parametric tests. Results After a 12-year follow-up, the ‘early’ NVC pattern changed from baseline in 76% of the patients. The NVC pattern was found to be ‘active’ in 9 patients (26%), ‘late’ in 13 (38%) and characterized by non-specific capillary abnormalities in 4 (12%). In the subgroup whose microangiopathy progressed from the ‘early’ to the ‘late’ NVC pattern, the median time of progression from the ‘early’ to the ‘active’ pattern was significantly shorter (11 months) when compared with patients who progressed from the ‘early’ to the ‘active’ NVC pattern (55 months) (P = 0.002). The median time of progression between NVC patterns was significantly shorter in SSc patients showing either a nucleolar ANA pattern or Scl70 autoantibodies (P = 0.048). Organ involvement was progressively greater in SSc patients with ‘early’, ‘active’ and ‘late’ NVC patterns, respectively. Conclusions This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting.

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Section: Discussionmentioning

confidence: 99%

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study

et al. 2019

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“…Capillaroscopic abnormalities were associated with a mean 15% reduction in forced vital capacity and DLCO compared to participants with normal capillaroscopy findings. Multiple other studies have also found an association between abnormal capillaroscopy and the presence or severity of ILD [27,34,46].…”

Section: Interstitial Lung Diseasementioning

confidence: 89%

“…Because of its relatively lower prevalence than other organ specific manifestations, it is more difficult to explore the role of NFC in predicting its presence or severity but some studies have already shown some intriguing results. In a recent Dutch cohort of over 200 patients, NFC changes were independently predictive of cardio-respiratory complications and, notably, all patients with pulmonary hypertension had abnormal NFC [46]. The "late" pattern and reduced capillary density have both been associated with the presence and severity of pulmonary hypertension in SSc [47,48].…”

Section: Pulmonary Hypertensionmentioning

confidence: 98%

“…Many patients with MCTD, which again shares considerable clinical manifestation overlap with SSc, will have NFC abnormalities although this has not been studied in isolation [33]. The recent study by Markusse et al found that the "early" pattern was associated with a positive anti-RNP antibody, a hint that, similar to NFC in the IIM, MCTD may have differing underlying microvascular pathophysiology with less avascular areas or capillary drop-out compared to patients with SSc [46].…”

Section: Mixed Connective Tissue Diseasementioning

confidence: 99%

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The Established and Evolving Role of Nailfold Capillaroscopy in Connective-Tissue Disease

Parker¹,

McGill²

2020

Connective Tissue Disease - Current State of the Art

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Nailfold capillaroscopy (NFC) is a low-cost, non-invasive, rapid, highly specific and reproducible investigation well established in the diagnosis of systemic sclerosis and related conditions. This chapter will detail the relevant underlying scientific principles that underpin the investigation, the methods for performing NFC, the range of abnormalities that can be present and the currently available classification criteria before moving on to discuss the various established and emerging applications as relevant to the connective tissue diseases. In addition to its role in the diagnosis of SSc, highlighted by its inclusion in the most recent ACR/EULAR consensus classification criteria, NFC has been shown to predict disease activity, many organ-specific complications such as digital ulcers, pulmonary hypertension and interstitial lung disease, and even mortality. It is emerging as a useful investigation in other CTDs characterised by microvasculopathy, such as in the idiopathic inflammatory myopathies and mixed connective tissue disease, as well as being studied as a serial investigation in patients to act as a potential biomarker and measure of treatment efficacy. NFC can contribute to the earlier identification of patients with CTDs with clinically important complications and if applied accurately, therefore, can help improve outcomes in these often challenging diseases.

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“…It is well known that the patterns of NVC scleroderma spectrum abnormalities are closely correlated to organ involvement in patients with SSc [22]. Interstitial lung disease and pulmonary artery hypertension [23] and skin ulcers [24] are common in cases with NVC scleroderma spectrum abnormalities. In particular, the loss of capillaries with extensive avascular areas is an important finding, and there are many reports that a late NVC pattern suggests the presence of organ involvement.…”

Section: Correlation Between Patterns Of Nvc Scleroderma Spectrum Abnmentioning

confidence: 99%

The role of nailfold videocapillaroscopy in patients with systemic sclerosis

Kubo

Smith

Cutolo

et al. 2018

Immunological Medicine

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Predicting cardiopulmonary involvement in patients with systemic sclerosis: complementary value of nailfold videocapillaroscopy patterns and disease-specific autoantibodies

Cited by 45 publications

References 27 publications

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study

The Established and Evolving Role of Nailfold Capillaroscopy in Connective-Tissue Disease

The role of nailfold videocapillaroscopy in patients with systemic sclerosis

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