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Raynaud’s Syndrome – Classification, Diagnosis And Treatment

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Raynaud’s Syndrome

In 1862 Raynaud described the condition for the first time. This disease looks pretty spectacular at first sight, due to the fact that the patient notices the skin color change of the affected limbs. Initially, the skin is pale and cold due to arteriolar spasm, then the limbs become cyanotic and cold because vein dilation is added and the skin turns into blue. Finally it becomes red and hot due to reactive vasodilatation of the arterioles.

Raynaud’s syndrome is divided into:

  • Primary disease, which bears the same name and whose cause is unknown.
  • Raynaud’s phenomenon, which is more common than Raynaud’s disease and is secondary to a local or a systemic disease.

Raynaud’s Syndrome

It seems that the most affected are women, especially in the second and third decade of life. It has a progressive evolution, the disease is symmetrically affecting fingers from both hands. Over time the spasms are becoming more frequent and prolonged. The diagnosis of Raynaud’s syndrome is established when the phenomenon persists for more than three years, without a cause which can be highlighted.

The cause of Raynaud’s syndrome is represented by an excessive response of the arterioles to vasospastic stimuli, especially in conditions of  cold or emotional stress. The disease can occur both in the upper and lower limbs, nose, ears and chin. In rare cases ischemia can lead to necrosis.

Raynaud’s Phenomenon

It is more common than Raynaud’s syndrome and has a equal frequency to both women and men. It appears secondary to the following conditions:

Autoimmune Diseases:

  1. Scleroderma;
  2. Lupus erythematosus;
  3. Dermatomyositis and polymyositis;
  4. Rheumatoid arthritis;
  5. Sjogren’s syndrome;
  6. Vasculitis;
  7. Primary pulmonary hypertension.

Infectious Diseases:

  1. Hepatitis B or C;
  2. Mycoplasma infections.

Neoplastic disease:

  1. Lymphoma;
  2. Leukemia;
  3. Myeloma;
  4. Waldenstrom macroglobulinaemia;
  5. Polycythemia;
  6. Type 1 cryoglobulinemia;
  7. Lung adenocarcinoma;
  8. Other paraneoplastic syndromes.

Metabolic And Endocrine Diseases:

  1. Acromegaly;
  2. Myxedema;
  3. Diabetes mellitus;
  4. Pheochromocytoma.

Raynaud's SyndromeHematological Diseases:

  1. Paroxysmal nocturnal hemoglobinuria;
  2. Polycythemia;
  3. Cryofibrinogenaemia.

Occupational Diseases:

  1. Vibration;
  2. Polyvinyl chloride exposure;
  3. Frostbites;
  4. Exposure to arsenic
  5. Exposure to lead.

Adverse reaction to drugs:

  1. Oral contraceptives;
  2. Ergot alkaloids;
  3. Bromocriptine;
  4. Beta adrenergic;
  5. Cyclosporine;
  6. Alpha-interferon.

Raynaud’s Syndrome Diagnosis:

The clinical examination will insist on the following:

  • If you have numbness, pain or swelling in the affected area;
  • If the affected area has at least two colors: white, due to ischemia caused by vasospasm, blue due to cyanosis or red due to hyperemia. Skin color can changed in this order, although it is not mandatory;
  • How many fingers are affected during a crisis, because in the early attacks of Raynaud’s phenomenon can be affected only 1 or 2 fingers. As the disease progresses all fingers will be affected. The thumb is rarely affected;
  • If crises occur in conditions of cold or emotional stress;
  • If tactile sensitivity is reduced;
  • If in the affected area is a loss of function;
  • If the patient suffer from other disorders that could explain the association with Raynaud’s phenomenon;
  • If symptoms are unilateral, then Raynaud’s phenomenon is suspected, while in case of bilateral and symmetrical symptoms, Raynaud’s syndrome is suspected;
  • The number of daily crises, in serious damage may appear multiple attacks per day, with a longer duration (from several minutes to hours);
  • What profession has the patient.
Raynaud's Disease

Raynaud’s Disease

Raynaud’s Syndrome Treatment:

Treatment consists of:

  • General measures:
  1. Protection from the cold;
  2. To avoid trauma to the affected area;
  3. To use moisturizing creams and lotions;
  4. Stop smoking;
  5. Avoid emotional stress;
  6. Consumption of fish, rich in omega 3 fatty acids;
  7. To treat promptly any lesion to the affected limb.
  • Medications:
  1. Calcium channel blockers, to achieve vasodilation;
  2. Inhibitors of angiotensin converting enzyme, may decrease the severity and frequency of the attacks;
  3. Angiotensin II antagonist.
  • Surgical:
  1. Sympathectomy is performed when the disease interferes with daily activity of the patient, or trophic changes occur or medication treatment failed
  2. Cervical and thoracic sympathectomy provide a temporary relief, but lumbar sympathectomy  is preferred as it is providing a more complete relief with a longer duration.