Monitoring the microcirculation in the diagnosis and follow‐up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations

Abstract

Systemic sclerosis (SSc) is a connective tissue disease with multi-factorial etiology and autoimmune pathogenesis. 1,2 SSc is characterized by structural and functional alterations of the microcirculation, with serious clinical implications such as Raynaud's Phenomenon (RP), digital ulcers (DU) and pulmonary arterial hypertension (PAH), 1,3-6 making morphological and functional assessment of the peripheral microvasculature a must for diagnosis, prognosis, and treatment response in SSc patients. 7-10 Nailfold videocapillaroscopy (NVC) is a safe, noninvasive, and validated method capable of detecting morphological microvascular abnormalities and differentiating between secondary RP due to SSc, primary RP and healthy subjects. 3,9,11,12 Differently from laser techniques, thermography, and other technologies, which can assess and quantify microvascular function at different skin sites, NVC allows for a morphological qualitative and semi-quantitative study of microcirculation. 3,5,9,13 The combination of these imaging techniques provides an accurate evaluation of microvascular damage in SSc and it is able to monitor response to treatment in the SSc microvasculature. 9,13-15 Therefore, it is advisable that rheumatology clinics avail themselves of a combination of morpho-structural and functional techniques, in order to create "a set" of safe and noninvasive tools for the early diagnosis and reliable quantification of SSc therapy response and disease progression. This review aims at providing updated information on the new acquisition standards for NVC and the functional techniques to

Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates

“…Full rheumatological assessments: fulfillment of the EULAR/ACR 2013 classification criteria [26], specifying when this was obtained through a very early diagnosis of systemic sclerosis (VEDOSS) features only [27]; modified Rodnan skin score (mRSS) and type of skin involvement (limited or diffuse) according to LeRoy classification [28]; nailfold video capillaroscopy (NVC) pattern [29]; presence of interstitial lung disease (ILD) on high resolution computed tomography (HRCT) and its functional assessment with pulmonary function tests, including forced vital capacity (FVC) and diffusion lung capacity of carbon oxide (DLco); history of digital ulcers (DUs) or current DUs; gastro-intestinal involvement; history of renal crisis; ongoing vasodilating; and/or ongoing and past immunosuppressive medications; All participants provided written informed consent. ALPC has full access to all the data in the study and takes responsibility for its integrity and data analysis.…”

What Role Does Trabecular Bone Score Play in Chronic Inflammatory Rheumatic Diseases?

“…SSc is a connective tissue disease characterized by early microvascular impairment, skin, and internal organ fibrosis (19)(20)(21)(22)(23)(24)(25)(26). Several studies have also recently demonstrated an increased risk of OP in SSc patients, correlated with multiple factors, i.e., low vitamin D levels (1,5,13,15,27).…”

“…Ruaro et al reported that SSc patients with a "Late" nailfold capillaroscopy pattern had lower TBS values than patients with an "Active" or "Early" pattern ("Late" vs. "Active" and "Early" pattern, p < 0.001) (5,13); whilst no statistically significant difference in BMD values was observed when comparing the three different capillaroscopy patterns (5,13). The negative correlation between the reduced bone microarchitecture, evaluated by TBS, and the progression of microvascular damage studied by nailfold videocapillaroscopy (NVC), suggested that the microvascular damage in SSc patients is also correlated to bone impairment and other systemic complications (5,13,22,26). Furthermore, these studies confirmed that SSc patients have a higher OP and osteopenia risk associated with the BMD obtained by DXA (1,2,5,13,15).…”

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

“…Systemic sclerosis (SSc), a heterogeneous disease, is characterized by immune dysfunction, often leading to organ damage due to inflammation, endothelial dysfunction and fibrosis [ 1 , 2 , 3 , 4 ]. SSc involves microcirculation structural and functional alterations [ 5 , 6 , 7 , 8 , 9 ]. The main cause of death in SSc patients is not only collagen overproduction but also the effects collagen overproduction has on the pulmonary system.…”