Raynaud's Phenomenon with Focus on Systemic Sclerosis

Abstract

Raynaud's phenomenon is a painful vascular condition in which abnormal vasoconstriction of the digital arteries causes blanching of the skin. The treatment approach can vary depending on the underlying cause of disease. Raynaud's phenomenon can present as a primary symptom, in which there is no evidence of underlying disease, or secondary to a range of medical conditions or therapies. Systemic sclerosis is one of the most frequent causes of secondary Raynaud's phenomenon; its appearance may occur long before other signs and symptoms. Timely, accurate identification of secondary Raynaud's phenomenon may accelerate a final diagnosis and positively alter prognosis. Capillaroscopy is fundamental in the diagnosis and differentiation of primary and secondary Raynaud's phenomenon. It is helpful in the very early stages of systemic sclerosis, along with its role in disease monitoring. An extensive range of pharmacotherapies with various routes of administration are available for Raynaud's phenomenon but a standardized therapeutic plan is still lacking. This review provides insight into recent advances in the understanding of Raynaud's phenomenon pathophysiology, diagnostic methods, and treatment approaches.

Keywords: Raynaud’s phenomenon; alprostadyl; capillaroscopy; iloprost; microcirculation; sulodexide; systemic sclerosis; vasculopathy.

Figure 1

Secondary causes of Raynaud’s phenomenon. Blue dashed line—connective tissue diseases; ASyS—antisynthetase syndrome.

Figure 2

Nailfold videocapillaroscopic (×200) patterns of microangiopathy: (a)—normal; capillaroscopic characteristics: density—normal, 8 capillaries in 1 linear mm; dimension: within normal limits; morphology: normal shapes of capillaries; haemorrhages: absent (b)—early; capillaroscopic characteristics: density—7 capillaries in 1 linear mm; dimension: presence of giants (homogeneous enlargement of all three limbs of the capillary with the diameter ≥50 µm); morphology: hairpin shaped capillaries; haemorrhages: absent (c)—active; capillaroscopic characteristics: density—lowered, 4 capillaries in 1 linear mm; dimension: presence of giants (homogeneous enlargement of all three limbs of the capillary with the diameter ≥50 µm); morphology: presence of abnormally shaped capillary; haemorrhages: present (d)—late capillaroscopic characteristics: density—lowered, 2 capillaries in 1 linear mm; dimension: not measured because of presence of abnormal shape; morphology: presence of abnormally shaped capillary; haemorrhages: absent.