Prospective capillaroscopy-based study on transition from primary to secondary Raynaud’s phenomenon: preliminary results

Abstract

summaryThe objective of this prospective study was to investigate the transition from primary (PRP) to secondary (SRP) Raynaud's phenomenon (RP), in a large cohort of patients affected by isolated RP. A total of 2065 patients with RP were investigated by clinical interview, laboratory examinations, and nailfold videocapillaroscopy (NVC). Patients with negative NVC at first visit were yearly followed to monitor either the appearance of specific morphological alterations at NVC, or clinical manifestations of an underlying disease. Capillary abnormalities at NVC were scored, as well as the qualitative patterns of microangiopathy (Early, Active and Late). NVC was found negative at first visit in 1500 subjects; among them, 412 patients were evaluable and they were followed for a mean time of 5±4 years (range 2-13 years). Sixty-eight patients (16%) achieved a diagnosis of SRP during follow-up, showing normal or not specific capillary alterations at NVC 4% of patients (the diagnosis was undifferentiated connective tissue diseases), Early scleroderma-pattern 57%, Active sclerodermapattern 7%, Late scleroderma-pattern 12%, and scleroderma-like pattern 18% of patients. The time of transition from normal/not specific capillary alterations to Early scleroderma-pattern was 4.4±3.8 years. Enlarged capillaries (diameter between 20 and 50 microns) and mild reduction of capillary density were found the more frequent markers at first NVC visit in patients who progressed to a scleroderma pattern (P=0.01). This study demonstrates in a large cohort, that almost 16% of patients initially diagnosed as affected by RP with negative NVC may transit to SRP during a mean follow-up of 4.4 years. PRP patients showing major notspecific alterations of nailfold capillaries at first NVC should be strictly monitored at least once a year since at higher risk of transition to SRP.

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

“…8,11,12,16,17 In healthy and primary RP subjects, NVC evaluation is characterized by good skin transparency, morphology of the capillary to "U" or "hairpin shape," morphological/structural homogeneity, evidencing 10-12 capillaries per linear millimeter, one capillary for each dermal papilla, diameters of capillary branches of <20 μm and the lack of morphological atypia. 8,11,12,16,17 Although it is quite common to observe normal nailfold capillaries in primary RP, capillaries with efferent branch enlargement or tortuosity may also be present. 8,9 Therefore, primary RP patients should have a careful capillaroscopic analysis every 6-12 months, in order detect the first signs of any transition to the secondary form of RP, in the most reliable manner.…”

“…8,9 Therefore, primary RP patients should have a careful capillaroscopic analysis every 6-12 months, in order detect the first signs of any transition to the secondary form of RP, in the most reliable manner. 7,8,12 Nailfold videocapillaroscopy images in secondary RP are characterized by morphological signs that represent the microvascular damage. These include giant capillaries, microhemorrhages, capillary loss, the presence of avascular areas, and angiogenesis.…”

“…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 …”

Infrared thermography and capillaroscopy in the diagnosis of Raynaud's phenomenon

“…By means of the cold pressor test (CPT) in combination with an analysis of temperature via infrared thermography (IRT), we can imitate the most common trigger factors of the disease and evaluate the acral perfusion functionality 10 . Assessment of morphological changes of acral perfusion is possible with the use of capillaroscope [11][12][13] .…”

Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis

“…The other laboratory tests, which included renal and liver function tests, rheumatoid factor and anti-citrullinated protein antibodies, were unremarkable. Nailfold videocapillaroscopy (NVC) was performed to evaluate Raynaud's phenomenon (1)(2)(3)(4). The NVC examination demonstrated a sclerodermapattern, characterized by an early pattern of microangiopathy, with a few giant capillaries, a few capillary haemorrhages and a relatively well-preserved capillary distribution, without evident capillary loss ( Figure 1A) (5-7).…”