Raynaud's phenomenon is a common clinical symptom caused by an exaggerated vascular response to triggers like cold. Out of the two types of Raynaud's phenomenon, namely primary and secondary, the latter needs more attention. Proper clinical history, laboratory diagnosis and imaging can help clinicians in looking for the underlying causes.

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What is Raynaud's phenomenon?

Raynaud's phenomenon is an exaggerated vascular response of the arterial circulation triggered by cold ambient temperature and emotional stress.

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Primary Raynaud’s phenomenon

Characteristic features:

1. Its prevalence is very common.
2. The onset of the condition is at a young age (<20 years). 
3. The severity of the condition can be mild to moderate.
4. There is a symmetric manifestation of symptoms seen.
5. The condition is not associated with any complications like digital ulcer or gangrene.
6. There is no negative antinuclear antibody observed.
7. No nail fold capillaroscopy changes can be seen in this type.

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Secondary Raynaud's phenomenon

It is defined as recurrent vasospastic events secondary to various aetiologies. It can be the first manifestation of underlying connective tissue disorder.

Common causes of secondary Raynaud’s phenomenon are:

1. Autoimmune disorders such as systemic sclerosis (most common), systemic lupus erythematosus, mixed connective tissue disease, Sjogren’s syndrome and idiopathic inflammatory myositis.
2. Occupational trauma can also be a cause.
3. Anti-migraine drugs like Ergotamine and its derivative, amphetamines, beta-blockers (non-specific), chemotherapeutic agents like bleomycin and cisplatin.
4. Haematological conditions like cryoglobulinemia, paraproteinemia, POEMS syndrome, myeloproliferative disorders etc. and obstructive vascular diseases like thoracic outlet syndrome, and atherosclerotic disorders can also lead to the condition.

Characteristic features of secondary Raynaud's phenomenon:

1. It is a less common condition.
2. The onset of the condition is at an older age (>30 years).
3. There is an asymmetric symptom manifestation seen.
4. Painful attacks are observed where the severity and intensity increases with attacks.
5. The condition is usually associated with complications like digital ulcer or gangrene, sclerodactyly.
6. Positive antinuclear antibodies and other disease-specific autoantibodies are observed.
7. Significant nail fold capillaroscopy changes are present.

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Approach to patients with Raynaud’s phenomenon

The clinical history of the patient should include the following:

• Age, gender, symmetry of presentation, frequency of episodes, the severity of the episode, and complications
• Any history that may be suggestive of connective tissue disorder - e.g skin thickening, joint pain, skin rash, painless oral or palatal ulcers, oral or ocular sicca symptoms, persistent prolonged dry cough, significant weight loss etc
• Drug history
• Associated comorbidities like hypothyroidism, and haematological malignancies

Laboratory analysis should include the following:

1. If suspecting connective tissue disorder: Complete blood count, peripheral smear, blood chemistry for renal and liver function, urinalysis, antinuclear antibody, rheumatoid factor, disease-specific antibodies, serum complements C3 & C4.
2. If suspecting haematological malignancy: Peripheral smear, serum protein electrophoresis, serum cryoglobulins, serum-free light chain assay etc.

Radiological imaging- If suspecting obstructive pathology, consider doing an ultrasound colour doppler study of upper limb vessels or if required CT or MR angiogram.

Nail fold capillaroscopy- This is to look for changes in capillary structures like giant or dilated capillaries, micro or macro haemorrhage, dropouts, avascular areas, neoangiogenesis.

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Management of Raynaud’s phenomenon

1. Goals
   1. To improve the quality of life.
   2. To prevent secondary complications.
   3. To follow and observe for the development of features of connective tissue disorder like systemic sclerosis for early diagnosis and treatment.
2. Non-pharmacological measures- It is a very essential component of effective management which includes:
   1. Patient education: To explain causes, read alarming symptoms and also treatment strategy.
   2. Cold protection:
      1. The most important way of prevention is to maintain a warm ambient temperature and avoid rapid transitioning of warm to cold temperature. To use loose and multilayered clothes to maintain warm core body temperature.
      2. Usage of woollen gloves and avoiding cold weather.
3. Lifestyle modification
   1. Smoking and drugs that can precipitate Raynaud’s condition should be avoided. 
   2. It is important to reduce and avoid emotional stress.
4. Pharmacological measures- Usage of oral or systemic vasodilator

Drugs used for managing the condition include:

• Dihydropyridine calcium channel blocker- First line, e.g. amlodipine, nifedipine etc.
• Phosphodiesterase 5 inhibitors- Sildenafil, tadalafil
• Oral and intravenous prostaglandin analogue (prostacyclin) such as alprostadil, Iloprost, epoprostenol etc.
• Endothelin receptor antagonist- Bosentan and ambrisentan

Other treatment options include:

• Oral or topical nitroglycerine
• Nitric oxide enhancer- Cilostazol, serotonin uptake blocker like fluoxetin
• In refractory cases- Botulinum injection or sympathectomy can be tried

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When to start pharmacological treatment?

Although primary Raynaud’s condition can be managed majorly with non-pharmacological measures, those patients whose quality of life is impacted or have moderate to severe intensity warrant a pharmacological measure. Pharmacological treatment can be considered when:

1. Patients are at risk of developing complications.
2. Patients have already developed complications like chronic digital ulcers or gangrene.
3. Patients present with severe Raynaud’s and associated complications.

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Disclaimer- The views and opinions expressed in this article are those of the author's and do not necessarily reflect the official policy or position of M3 India.

The author is a practising Rheumatologist from Bangalore.