Objectives: Early microvascular damage and dysfunction are clinically mirrored in Raynaud’s phenomenon (RP). Currently, nailfold capillaroscopy (NC) is applied to differentiate between primary RP (PRP) and secondary RP (PRP), associated with connective tissue disease. However, abnormal morphology can also be caused due to age-related changes and cardiovascular disease. Thermography (TG) is a non-invasive technique which enables quantification of cutaneous vascular function. An approach using both NC and TG could improve the differentiation between PRP and SRP. Methods: Thirty RP patients (PRP, n = 21; SRP, n = 9) underwent nailfold capillaroscopy and thermography. Morphologic features were scored and patients were categorized according to the guidelines of EULAR Study group on Microcirculation in Rheumatic Diseases. TG of the hand was performed before, directly and ten minutes after a cold challenge test. Baseline images and rewarming curves were analyzed. Results: Capillary abnormalities with NC were found in all SRP patients (9/9) and in 48% (10/21) of PRP patients. Out of 10 PRP patients with altered capillary morphology, 9 (90%) had a cardiovascular disease. For all patients mean temperature was significantly higher 10 minutes after cold induction than before (p < 0,01). The gradient of the rewarming curve was significantly lower in patients with SRP compared to PRP patients (p = 0.015). Conclusions: Nailfold capillaroscopy and thermography can reliably be used to measure microvascular damage and dysfunction. Additional thermography can assist in differentiating between PRP and SRP, especially in elderly patients or in presence of a cardiovascular disease. Keywords: Raynaud’s phenomenon; Nailfold capillaroscopy; Thermography
Raynaud's phenomenon (RP) is defined as episodic attacks of artery and arteriole vasoconstriction. To differentiate between the benign RP (pRP) and the form associated with connective tissue diseases (sRP) the capillary morphology can be studied using nailfold capillaroscopy (nCS). However, abnormal morphology can also be caused due to age-related changes and has been described in patients with diabetes and cardiovascular disease. In addition, this technique cannot provide functional information. Thermal imaging (thermography; TG) is a non-invasive technique which enables quantification of cutaneous blood vessel function. In veterinary medicine, thermal imaging is applied for various clinical settings. A combined approach using both nCS and TG could improve the differentiation between pRP and sRP.
Objectives
The aim of this pilot study was to determine which technique (TG versus nCS) allows the best discrimination amongst older patients with pRP and sRP.
Methods
Thirty patients with RP (pRP, n=21; sRP, n=9) underwent nCS (Olympus SZ51) and TG (Flirr B 620). Nailfold morphologic features were measured and scored on capillary density, giant capillaries, ramification and hemorrages. The patients were divided into three categories: normal, slightly abnormal (slightly enlarged capillaries) and severely abnormal (destruction of capillary structure and hemorrages). TG of the hand was performed before, directly after and 10 min after a cold challenge test with cold manchets of 3°C. Rewarming and reperfusion were monitored and baseline images and rewarming curves were analysed.
Results
Capillary abnormalities with nCS were found in all patients with sRP (9/9) and in 52% (11/21) of patients with pRP. Out of 11 pRP patients with altered capillary morphology, 7 (63%) had a cardiovascular disease. TG demonstrated a lower average temperature at baseline in the pRP group compared to the sRP group (d 1,68°C, p<0,01). In patients with pRP temperature decreased after cold induction (−2.34°C, p=0,01), whereas in sRP patient temperature stayed consistent (+0.07°C, p=0,46) (table 1). In both groups temperature increased ten minutes after cold induction (pRP +2.62°C, p<0,01; sRP +1.57°C, p<0,01). The gradient of the rewarming curve was significantly lower in patients with sRP compared to the pRP group (median 0.16 vs. 0.26 °C/min; p=0.015).
Conclusions
Nailfold capillaroscopy and thermography can reliably be used to measure microvascular damage and dysfunction. TG is better suitable to differentiate between older patients with pRP and sRP. Furthermore, in presence of cardiovascular disease, TG appears to be a more reliable technique than nCS for differentiating between patients with pRP and sRP.
Systemic sclerosis is an autoimmune disease characterized by fibrosis and small vessel vasculopathy, which affects various organ systems, such as the heart. Takotsubo cardiomyopathy is a transient cardiomyopathy in reaction to an emotional or physical trigger. There may be clinical and pathogenetic overlap between Takotsubo cardiomyopathy and primary systemic sclerosis heart disease, and some patients with systemic sclerosis have been diagnosed with recurrent Takotsubo cardiomyopathy. Our large systemic sclerosis clinical cohort was reviewed to identify cases diagnosed with Takotsubo cardiomyopathy. The clinical features, laboratory and imaging results were reviewed and evaluated to perform a comparison between cases. We identified five patients with systemic sclerosis, all female (age 68.6 ± 5.7 years), who were diagnosed with Takotsubo cardiomyopathy. Two of these patients had recurrent episodes: one case with a history of multiple episodes and the other with one recurrence. Typical features included repolarization abnormalities on the electrocardiogram and transient left ventricular dysfunction observed using echocardiography or cardiac magnetic resonance imaging. Our findings build upon previous reports and observations that systemic sclerosis may cause Takotsubo cardiomyopathy. To our knowledge, this is the largest case series of Takotsubo syndrome in patients with systemic sclerosis. This association may provide novel insights into the aetiopathogenesis of Takotsubo cardiomyopathy as part of primary systemic sclerosis heart involvement.
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