When the weather turns cold, the most exposed parts of the body can easily suffer. In particular, the hands can become chapped and very pale. However, there is a specific condition that is triggered by the cold but has symptoms and often much more complex causes: Raynaud’s phenomenon.”During a Raynaud’s episode, there is a vasospasm of the arterioles: these small vessels constrict, drastically reducing the blood supply to the affected area”, explains Alessandro Bellisario, cardiac surgeon and lecturer in vascular surgery at UniCamillus University. “This leads to temporary ischaemia, which manifests itself in the characteristic colour changes and pain”.
Two types of the disease, two clinical scenarios
There are two main types of Raynaud’s syndrome: primary Raynaud’s syndrome, which is less severe, and secondary Raynaud’s syndrome, which may be a sign of an underlying disease. “The primary type often affects young people and is usually benign”, continues Bellisario. “The secondary type is associated with connective tissue diseases, such as scleroderma, systemic lupus erythematosus (SLE), rheumatoid arthritis or polymyositis, and is more common in people over the age of 40”.
Raynaud’s is not uncommon: it affects about 4% of the population, with a clear prevalence in women compared to men (5:1 ratio). “There are hormonal factors that contribute to this difference. Other risk factors include living in cold climates, familiarity with the condition, exposure to chemicals such as mercury and vinyl chloride, and the use of certain medications such as beta blockers”, says Bellisario.
Raynaud’s or just chilblains?
In winter, however, the hands can become pale and develop discomfort, pain, itching and small ulcers: this is known as chilblains. How can we correctly differentiate them? In fact, they are very different in terms of symptoms and occurrence, although some effects may seem similar at first glance. “Raynaud’s phenomenon is characterised by three phases. The first is the ischaemic phase, in which the arteries close and the hands turn white due to the lack of arterial flow”, explains Massimo Gravante, dermatologist and lecturer in dermatology at UniCamillus University.
This first phase is then followed by a development that further distinguishes it from chilblains. “The second phase is the cyanotic phase: the blood cannot get out and blocks the venules, turning the skin blue. Finally, the third phase is the erythema phase, when the vasoconstriction resolves and the ‘tap’ reopens (usually after 15-20 minutes), and intense redness appears due to the return of blood flow”.
Chilblains, on the other hand, do not follow this dynamic pattern and are the result of chronic vascular damage caused by the cold, without the typical phenomenon of opening and closing the ‘thermal tap’.
Diagnosing Raynaud’s syndrome
Raynaud’s phenomenon can be worrying, especially when it occurs for the first time, as it may be a sign of a more serious underlying condition, such as an autoimmune disease. Early diagnosis is therefore essential to guide the patient to the right treatment. “When a patient presents with Raynaud’s for the first time, after taking a detailed clinical history and providing some general advice, we carry out a number of key diagnostic tests, such as serum anti-nuclear antibodies (ANA) and capillaroscopy—a test to observe the state of the capillaries”, explains Roberta Priori, rheumatologist and lecturer in rheumatology at UniCamillus.
The aim of these initial examinations is to understand whether Raynaud’s phenomenon is isolated or associated with a rheumatological disease. “If the initial examination reveals changes typical of a connective tissue disease, or if the ANAs are positive, we proceed with more specific diagnostic tests, such as ANA typing, which includes looking for anti-ENA antibodies. For example, the presence of anti-SCL70, even in the absence of other clinical manifestations, can be an important indicator in predicting the development of systemic sclerosis”.
Capillaroscopy is also crucial for detecting early signs of damage to the blood vessels. “The detection of an early scleroderma pattern on capillaroscopy is a sign that could indicate disease progression, so it is important to monitor the situation closely”, Priori suggests. Early diagnosis is becoming increasingly important, with the aim of avoiding permanent damage and intervening early to prevent future complications.
Capillaroscopy is an essential tool for monitoring Raynaud’s and a possible underlying cause, but it is not the only useful test. “There are other very valuable techniques such as skin plicometry, oesophageal manometry with pH measurement, lung capacity assessment, CO2 diffusion capacity and Doppler renal ultrasound”, Bellisario continues.
It seems clear that attention to detail and early detection of early signs can make a big difference in the management of Raynaud’s phenomenon, especially when it is associated with autoimmune disease. Early diagnosis not only improves the patient’s quality of life, but also allows for a targeted treatment plan to counteract the progression of the disease.
Treatment options and extreme cases
The treatment of Raynaud’s phenomenon varies according to its nature, but some general indications are fundamental for each type of patient. “In Raynaud’s phenomenon, vasodilator medications such as calcium channel blockers are generally used”, says Roberta Priori. “If Raynaud’s is associated with systemic sclerosis, other medicines such as phosphodiesterase inhibitors or the endothelin receptor antagonist (bosentan) can be used in the presence of new or active ulcers on the fingers”. However, prevention should never be neglected, which can also be achieved through a correct lifestyle and good habits that should not be forgotten. “The general advice is: do not smoke, do not take medicines that can worsen the condition such as beta blockers, avoid exposure to low temperatures and protect your hands from the cold”.
In rare cases, when medication and precautions are not enough, surgery can be used. “Endoscopic thoracic sympathectomy (ETS) is an option for patients with severe Raynaud’s. This technique aims to reduce the hyperactivation of the sympathetic system responsible for vasospasm, but it is only used in exceptional cases”, explains Bellisario.
Although many of the symptoms of Raynaud’s phenomenon are neurological, it is not a neurological disorder. “There is no neurological disorder associated with Raynaud’s phenomenon, on the contrary: a dysregulation of the autonomic nervous system (for various reasons, such as stress or anxiety) can facilitate Raynaud’s phenomenon”, explains Luigi Maria Grimaldi, lecturer in neurology at UniCamillus and head of the neurological surgery unit at the Fondazione Giglio in Cefalù. “Peripheral neuropathies can affect the vascular response. Raynaud’s phenomenon is therefore a vascular disease in which there are neurological symptoms such as altered sensitivity and pain perception, but it is not a neurological disorder per se”.
So, despite the difficulties that Raynaud’s can cause, early diagnosis and a multidisciplinary approach now offer increasingly effective tools to improve patients’ quality of life. Recognising Raynaud’s phenomenon as a warning sign makes it possible to take action at an early stage, thus preventing serious complications and promoting the personalised management of the patient.