Table of Contents
- Definition of Raynaud’s Disease
- Classification of Raynaud’s Disease
- Pathophysiology of Raynaud’s Disease
- Differential Diagnosis of Raynaud’s Disease
- Clinical Features of Raynaud’s Disease
- Diagnosis and Investigations of Raynaud’s Disease
- Treatment of Raynaud’s Disease
- Prognosis of Raynaud’s Disease
- Review Questions
For the very first time in 1862, the disorder was illustrated by Raynaud. At first look the disorder appears quite noticeable, owing to the point that the patient notifies the change in the color of their skin particularly in their limbs.
Primarily, the skin is cold and pallor owing to spasm in arterioles which may result in the coldness and cyanosis of limbs as the distension of veins occurs followed by the bluish skin color. Lastly, it turns warm and red as a result of arterioles’ response to vasodilatation. It took quite a few years of study to complete comprehension of the pathology and physiology of Raynaud that continues to be explained. The causes of Raynaud’s phenomena and the mechanisms have been explained under three groups by Henrick in 2005 namely:
- Neural abnormalities
Definition of Raynaud’s Disease
Raynaud’s disease is a disorder that causes pain at the margins in the cold environment. Reduction in the size of blood vessels, most usually within the margins occurs resulting in the fingers to change their color to blue from white and, lastly, as the stream of blood proceeds, they are turned red.
Raynaud’s phenomena is an ailment subsequently causing a specific sequence of color changes of the extremities when in contact to fluctuations in weather either hot or cold or may be through emotional changes. A great number of people with Raynaud’s phenomenon have extreme sensitivity to cold temperature.
Classification of Raynaud’s Disease
Raynaud’s disease is classified to:
- Primary Raynaud’s
- Secondary Raynaud’s
Raynaud appearing all alone is termed to as “Raynaud’s disease” or more appropriately as primary Raynaud’s phenomenon and when it appears accompanied by other disorders, it is referred to as secondary Raynaud’s phenomenon.
Raynaud’s is customarily a sign or symptom of different ailments which comprises of atherosclerosis, scleroderma, lupus and rheumatoid arthritis. It can also be a result of taking assured medicines, having frostbite, smoking or using vibrating power tools for numerous years thus sometimes named as secondary Raynaud’s.
The most usual generation of symptoms is disclosure to cold atmosphere. During cold atmosphere, it’s pretty typical for our body to constrict the minor vessels of blood to the skin and to expose the blood vessels to the exclusive parts of our body to keep the body warm and maintain our temperature through homeostasis. On the other hand with Raynaud’s, the body confines the flow of blood more to the skin than to other parts where it is needed. Additional triggers comprise of stress related to emotions and effects that have an impact on the flow of blood including some medicines, caffeine and smoking.
This is more frequent than the Raynaud’s syndrome and more likely to occur in both entitles female and male with an almost equal ratio. It seems tributary to the below-mentioned conditions.
It appears as if women have more adverse effects specifically with age ranging from 20–30 years. It has an enlightened advancement, the disorder is proportionally causing an effect upon digits from both our hands. Over a period of time, recurrent and sustained spasms are becoming more common.
The pathogenesis of Raynaud’s syndrome is characterized by an increased reaction of the arterioles to stimuli that is vasospastic exclusively in situations of emotional stress or cold. The sickness can happen both in our lower and upper limbs with chin, nose, and ears. In occasional situations, low oxygen supply can result in the occurrence of necrosis.
Pathophysiology of Raynaud’s Disease
People suffering from Raynaud’s phenomenon experience changes in the color of their skin. Changes in the color of skin happen for the reason that an anomalous spasm in the blood vessels may result in a reduced supply of blood to the localized tissues of the body.
Originally, the finger or toes involved can turn whitish in color owing to a decreased amount of blood supply. Then these extremities will turn into bluish color due to persistent deficiency of oxygen. As a final point, the blood vessels open themselves again, resulting in localized “flushing” occurrence, which in turn causes the extremities to reappear as red in color. Hence, this three-phase color system (whitish to blue to red), occurs more frequently upon the contact to cold atmosphere, is a significant characteristic of Raynaud’s phenomena.
Particular parts of a body are most often vulnerable to cold injury. A perfect mark of distinction is present among the unaffected and ischemic parts of the body.
The impacts are changeable, and they should be distinct from non-changeable origins of low blood supply including thrombosis or vasculitis. Barely, the necrosis of the tissues appears from a distal end of the particularly affected vessel, it most commonly appears at the periphery or margins of the particularly affected vasculature. It most normally has an adverse effect on the hand’s extremities, however, ears, toes, and nose can also be affected by it. Sometimes, even the involvement of tongue occurs.
The primary Raynaud’s phenomenon is correlated to purposeful modifications alone. In comparison, the secondary Raynaud’s phenomenon furthermore reveals fundamental micro-vascular anomalies.
A deficit of mediators of vasodilation, which comprises of the oxide of nitric, has been connected to the cause of Raynaud’s phenomenon. Moreover, effective vasoconstrictors generally present in our endothelium and known as endothelin-1 has been seen in to be flowing in elevated levels in people suffering from the secondary Raynauds’ phenomenon.
Discharge of the vasoconstrictor endothelin-1 is elicited by stimuli of vasoactivation which comprises of converting growth factor-beta (TGF-beta), vasopressin and angiotensin. Contradictory, consequences concerning the levels of endothelin-1 in people suffering from primary Raynaud’s phenomenon is renowned.
Angiotensin has both pro-fibrotic and vasoconstrictive impacts. The research by Kawaguchi et al disclosed that increased levels of angiotensin II in people suffering from Raynaud has been seen along with embedded cutaneous systemic sclerosis
In people suffering from systemic sclerosis, fundamental anomalies allied with vasculature fibrotic proliferation resulting into decreased flow of blood to the peripheral digits is usually seen which is a characteristic differentiation from the primary Raynaud’s disease.
Scientists discovered that originally, both the people having this ailment and those that are hale and hearty reacted to sound stimuli along with vasoconstriction in the extremities and vasodilation in muscles of the forearm.
Compromised vasodilation can also be part of Raynaud’s phenomenon. An essential neuropeptide, which is a intoxicating vasodilator, released by nerves supplying the vessels of blood commonly named as calcitonin which is, in fact, a gene-related peptide. A lessened amount of calcitonin gene–related peptide-secreting neurons has been seen in biopsy of skin samples of people suffering from systemic sclerosis and primary Raynaud.
Elevated vasoconstriction in Raynaud’s phenomenon can lead to increased activity of α2C-adrenoreceptors; vasoconstriction of the blood vessels has been seen to be cold-induced by these adrenoreceptors. There are two types of research about such phenomena by Furspan et al, exhibiting the fact that there is an increased contractile reaction to α2-adrenergic agonists along with cooling in people having the primary Raynaud’s phenomenon which is to be interrelated to improve the activity of protein tyrosine kinase.
Raynaud phenomenon is found to be linked with the below mentioned intravascular abnormalities:
- In systemic sclerosis and primary Raynaud, enhanced activation and accumulation of platelet have been revealed.
- An improved fabrication of thromboxane A 2 platelet that in actual is an extremely prevailing vasoconstrictor has been seen in people with Raynaud phenomenon
- Patients with systemic sclerosis have been seen to have weakened fibrinolytic scheme, perhaps subsidizing to obstruction in vessels.
- Oxidized stress occurring through oversensitive oxygen species seems to be involved as one of the causes of Raynaud’s phenomenon.
Differential Diagnosis of Raynaud’s Disease
- Sjogren’s syndrome
- Dermatomyositis and polymyositis
- Lupus erythematosus
- Rheumatoid arthritis
- Primary pulmonary hypertension
- Hepatitis C
- Hepatitis B
- Infections of mycoplasma
- Type 1 cryoglobulinemia
- Waldenstrom macroglobulinaemia
- Paraneoplastic syndromes
- Lung adenocarcinoma
Endocrinological and metabolic diseases
- Paroxysmal nocturnal hemoglobinuria
Diseases related to occupation
- Exposure to lead
- Exposure to arsenic
- Polyvinyl chloride exposure
Drug’s adverse reaction
- Oral contraceptives
- Ergot alkaloids
Clinical Features of Raynaud’s Disease
Throughout an occurrence of the Raynaud’s, the body confines the flow of blood to the feet and hands which cause them to feel numb and cold thus turning them white or pallor. As the blood flow comes back and the digits of toes and fingers become hot and warm, they began to turn red along with pounding and pain. In occasional situations, Raynaud’s can also affect the ears or nose.
It may appear most frequently only very little time. Nevertheless, in some situations, it may appear long-term ranging from one hour or more.
Diagnosis and Investigations of Raynaud’s Disease
Diagnostic criteria for the primary Raynaud’s phenomenon is below mentioned as:
- Occurrences activated by disclosure to stress or cold
- Lack of necrosis
- Symmetrical bilateral involvement
- Lack of a noticeable essential pathogenesis
- Normal findings of laboratory for inflammation
- Normal findings of capillaroscopy
- Lack of antinuclear factors
Since the nature of the Raynaud’s, analysis and diagnosis can be moderately met by inquiring the patient a sequence of queries. These inquiries are expected to contain an explanation of the signs and symptoms and some overall facts about habits, diet, and hobbies.
The subsequent contest is to discriminate among primary and secondary kinds of the ailment. For this, the physician can take a sample of patient’s skin from the base of a fingernail to inspect it underneath a compound microscope. This technique is termed as capillaroscopy. The physician will be observing the inflamed or abnormal capillaries which could be a signal of the secondary illness.
The test of blood might be essential in order to see and examine the antinuclear antibodies and to assess the sedimentation rate of RBC’s. They both can expose problems related to autoimmune system or connective tissues disorders.
Treatment of Raynaud’s Disease
General measures to be taken
- Patients should avoid trauma to the affected area.
- Protect the patient’s body from the cold atmosphere.
- Urgent treatment of any injury to the limbs.
- Moisturizing lotions and creams should be used.
- Stopping smoking is important.
- Emotional stress should be avoided.
- Eating of fish, that is highly enriched in omega 3 fatty acids, is recommended.
Medications and drugs
- Angiotensin II antagonist
- Vasodilators such as Calcium channel blockers
- Inhibitors of angiotensin altering enzyme, such as ACEIs and ARBS
Sympathectomy is accomplished when the illness obstructs the activity of daily livings of the patient, or failure of the drugs changes in the tropics. Thoracic and cervical sympathectomy give a short-term aid; on the other hand, lumbar sympathectomy is highly preferred as it gives an intense wide-ranging relief for a much longer duration.
Prognosis of Raynaud’s Disease
The prospects for patients suffering from primary Raynaud’s phenomenon is ordinarily pretty good, with little morbidity and no rate of mortality. In very occasional situations, yet, low oxygen supply of the affected part of a body can be an outcome in necrosis.
The prognosis for patients suffering from secondary Raynaud’s phenomenon is closely correlated to the underlying cause and disease. The prognosis for the digits involving these patients is interrelated to the extremity of the low oxygen supply and the efficiency of movements to reestablish the flow of blood.
The correct answers can be found below the references.
1. A 20 yrs old woman complains about extreme sensitivity to the cold atmosphere with pallor color of skin for few minutes. Capillaroscopy is showing inflamed vessels. What could be the possible diagnosis?
- Myasthenia Gravis
- Raynaud’s Disease
2. A smoker suffering from rheumatoid arthritis complains of discoloration of the periphery of the digits. What could be the most likely diagnosis?
- Raynaud’s disease
- Raynaud’s phenomena
- Sjogren’s syndrome
3. Which of the following are the diagnostic criteria for primary Raynaud’s?
- Lack of necrosis
- Renal Failure