Progression of Organ Involvement in Systemic Sclerosis Patients with Persistent “Late” Nailfold Capillaroscopic Pattern of Microangiopathy: A Prospective Study

Pizzorni, Carmen; Sulli, Alberto; Paolino, Sabrina; Ruaro, Barbara; Smith, Vanessa; Trombetta, Amelia Chiara; Cutolo, Maurizio

doi:10.3899/jrheum.170485

Cited by 13 publications

(13 citation statements)

References 8 publications

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“…However, these techniques lack the support of validation studies as to their application in clinical practice. Nevertheless, microvascular structure evaluation by NVC combined with functional investigation by laser techniques or TI, not only helps in the distinction between primary and SRP, but is also able to evaluate therapy response and disease progression (Filaci et al, 2001; Caramaschi et al, 2009; Guiducci et al, 2012; Cutolo et al, 2013, 2014, 2016; Smith et al, 2013, 2016b; Trombetta et al, 2016; Ruaro et al, 2017a, 2018a, Pizzorni et al, 2017a; Soulaidopoulos et al, 2017; Markusse et al, 2017).…”

Section: Discussionmentioning

confidence: 99%

Innovations in the Assessment of Primary and Secondary Raynaud’s Phenomenon

et al. 2019

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Objectives: Raynaud’s phenomenon (RP) is characterized by intense vasospasm of the digital arteries that causes characteristic color changes in fingers. There are two main types of RP: Primary RP (PRP) and Secondary RP (SRP). PRP is a benign condition. Whilst SRP is associated with several connective tissue diseases (CTD), in particular systemic sclerosis (SSc). The objectives of this report were: to present a short review on morphological (nailfold videocapillaroscopy, NVC) and functional techniques (laser tools and thermography) that allow for a correct diagnosis and treatment of RP and to investigate blood perfusion (BP) by laser speckle contrast analysis (LASCA) in different skin areas of hands and face in PRP, SRP to SSc, and healthy subjects (CNT). Methods: 31 PRP patients (LeRoy criteria), 70 SRP to SSc (ACR/EULAR criteria) and 68 CNT were enrolled. BP was assessed by LASCA at the level different areas of hands and face. NVC was performed to distinguish between PRP and SRP, and to detect the proper pattern of nailfold microangiopathy in SSc patients. Results: Both PRP and SRP showed a statistically significant lower BP than CNT at the level of fingertips ( p < 0.0001), periungual ( p < 0.0001), palmar aspect of 3rd finger ( p < 0.0001), and palm areas ( p < 0.0001). Moreover, BP was significantly lower in PRP than in SRP to SSc with the “Early” pattern of microangiopathy in the same areas as above ( p < 0.04). Conclusion: By considering a small cohort of patients, BP of hands was found lower in PRP than in SSc patients with the “Early” NVC pattern of microangiopathy.

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Section: Discussionmentioning

confidence: 99%

Innovations in the Assessment of Primary and Secondary Raynaud’s Phenomenon

et al. 2019

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“…Furthermore, some authors have shown cross-sectional and predictive associations between progressive capillaroscopic changes and the impairment of internal organ function, that is, lung disease, skin fibrosis and digital ulcers. [33][34][35][36][37][38][39][40][41][42][43][44] It has now become clear that SSc patients with a 'Late' NVC pattern have an increased risk of a complex disease and moderate to severe skin or visceral involvement, compared to patients with an 'Early' and 'Active' NVC pattern 33,[37][38][39][40][41] ( Tables 1 and 2).…”

Section: Correlations Between Scleroderma Patterns and Internal Organmentioning

confidence: 99%

Advances in nailfold capillaroscopic analysis in systemic sclerosis

Ruaro

Sulli

Smith

et al. 2018

Journal of Scleroderma and Related Disorders

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Systemic sclerosis is an autoimmune connective tissue disease characterized by early and persistent microvascular impairment which leads to functional and organic manifestations, with progressive fibrosis of the skin and internal organs. Morphological and functional assessment of the peripheral microvasculature is a must, not only for diagnosis but also for the prognosis and therapeutical follow-up of systemic sclerosis patients, as reported in recent studies. Nailfold videocapillaroscopy is the validated technique for the study of scleroderma microangiopathy as it is able to detect peripheral microvascular morphology and both classify and score the capillary abnormalities into different microangiopathy patterns ('Early', 'Active' and 'Late'). Indeed, the possibility to early diagnose and follow the microvascular changes and the safety of the technique have made nailfold videocapillaroscopy a mandatory tool for patient evaluation and included its assessment in the new systemic sclerosis classification criteria. Important links between nailfold videocapillaroscopy patterns and systemic sclerosis clinical manifestations have been described.

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“…Possible specific disease conditions that may increase the risk of malnutrition in SSc patients include microstomia, difficult mouth opening, sicca syndrome, reduced gastric motility and digital ulcers or sclerodactyly that limit feeding due to the altered hand ability in the advanced stages of the disease [ 11 , 30 , 31 ].…”

Section: Discussionmentioning

confidence: 99%

Nutritional Status and Bone Microarchitecture in a Cohort of Systemic Sclerosis Patients

et al. 2020

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Systemic sclerosis (SSc) is a connective tissue disease characterized by initial microvascular damage, immune system activation and progressive fibrosis with insufficiency of internal organs. Gastrointestinal (GI) involvement is characterized by atrophy of the smooth muscle and small bowel hypomotility, mainly resulting from an autonomic nerve dysfunction. These modifications significantly affect gut transit and nutrient absorption, thus leading to malnutrition deficit induced by malabsorption. Nutritional deficit induced by malabsorption might also lead to bone alterations. This study aims to evaluate the relationship between malnutrition and bone status. Thirty-six postmenopausal female patients fulfilling the ACR 2013 criteria for SSc underwent dual-energy X-ray absorptiometry scan (DXA) to detect quantitative lumbar spine bone mineral density (BMD) and trabecular bone score (TBS) analysis to detect bone quality. Data from DXA also allow to assess body composition and provide several quantitative parameters, including free fat mass index (FFMI) that identifies the patient with malnutrition (values <15 kg/m2 in women and 17 kg/m2 in men), according to the ESPEN criteria. Body mass index (BMI) was calculated for all SSc patients and every patient completed a diary reporting GI symptoms. Two groups of SSc patients with or without diagnosed malnutrition according to FFMI parameter were identified. Malnourished SSc patients showed significantly lower weight (p = 0.01) and BMI (p = 0.001), as well as lower serum levels of hemoglobin (p = 0.009), albumin (p = 0.002), PTH (p = 0.02) and 25OH-vitamin D (p = 0.008). DXA analysis showed significantly lower lumbar L1-L4 T-score (p = 0.009) and BMD values (p = 0.029) in malnourished SSc patients. Consistently, TBS values were significantly lower in malnourished patients (p = 0.008) and correlated with BMD (at any site) and serum albumin levels (p = 0.02). In addition, FFMI positively correlated with bone parameters as well as with symptoms of intestinal impairment in malnourished SSc patients. Finally, GI symptoms significantly correlated with BMD but not with TBS. This pilot study shows that in malnourished SSc patients (2015 ESPEN criteria: FFMI<15 kg/m2), an altered bone status significantly correlates with GI involvement, in terms of symptoms being mainly due to intestinal involvement together with the presence of selected serum biomarkers of malnutrition.

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Progression of Organ Involvement in Systemic Sclerosis Patients with Persistent “Late” Nailfold Capillaroscopic Pattern of Microangiopathy: A Prospective Study

Cited by 13 publications

References 8 publications

Innovations in the Assessment of Primary and Secondary Raynaud’s Phenomenon

Innovations in the Assessment of Primary and Secondary Raynaud’s Phenomenon

Advances in nailfold capillaroscopic analysis in systemic sclerosis

Nutritional Status and Bone Microarchitecture in a Cohort of Systemic Sclerosis Patients

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