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Raynaud’s Phenomenon and Disease

- See: vascular malformations of the upper extremity

- Discussion:
    - first described by Maurice Raynaud in 1862;
    - involves vasoconstriction of the cutaneous arteriovenous shunts, digital arteries, and precapillary arterioles;
    - pallor of the digits with or w/o cyanosis on exposure to cold;
    - Raynaud's disease (primary form):
           - definition: when the phenomenon occurs w/o another demonstrable or associated disease;
                 - absence of organic disease (causing the symptoms)
           - more common than Raynaud's phenomenon;
           - mainly occurs in young women (median age of onset is 14 years);
           - bilateral and symmetric involvement
           - absence of clinical occlussion of peripheral arteries
           - gangrene or trophic changes are absent or are limited to distal digital skin;
           - normal nail-fold capillaries;
           - negative test for antinuclear antibody;
           - normal erythrocyte sedimentation rate.
    - Raynaud's phenomenon (secondary form):
           - definition: when the phenomenon occurs with another inciting disorder;
           - most commonly seen in females in their late 20s to late 30s;
           - some of these patients will demonstrate others signs of vascular pathology such as migraines or pulmonary hypertension;
           - patients may note that symptoms are triggered by a cold room or by strong emotions or anxiety;
           - lab data demonstrates auto-antibodies;
                  - antinuclear antibodies
           - contributing disorders:
                  - scleroderma patients with scleroderma often first present with Raynaud's phenomenon;
                         - typically associated with anticentromere antibody
                         - look for ANA titer > 1000
                         - ref: Images in Clinical Medicine. Cold Hands Associated with Scleroderma
                  - other inflammatory arthritic conditions: RA SLE, dermatomyositis;
                  - neurologic disorders
                  - arterior occlusive disorders
                  - blood dyscrasias

- Exam:
    - usually all digits will be affected during vascular episodes;
    - triple color changes:
           - digital pallor, episodic cyanosis, hyperemia;
           - pallor results from arterial insufficiency, where as cyanosis results from deoxygenation of blood which remains in the finger;
           - reactive hyperemia occurs after the vascular episode is over;
           - ref: Images in Clinical Medicine. Primary Raynaud's Phenomenon
    - character and quality of right and left radial pulses (irregular heart beat and look for extinguished pulse with hyperabduction and E. 
           rotation of shoulder);
    - assess patency of radial and ulnar digital arteries with Allen's test, and then assess patency of the arteries to the digits using
           a digital Allen's test;

- Lab data:
    - laboratory findings will typically be negative in primary Raynaud's disease where as in Raynaud's phenomena, positive findings
           are not uncommon;
    - sed rate:
           - an increased sed rate may point to a collagen vascular disease;
    - thyroid panel;
           - these patients will often have a subtle hypothyroidism;
    - cryoglobulins:
           - many patients with significant amounts of cryoglobulins are asymptomatic; other patients develop purpura, Raynaud's
                   phenomena, cyanosis, and tissue necrosis when exposed to cold;
           - pts w/ mixed cryoglobulinemia frequently have vasculitis, glomerulonephritis, lymphoproliferative disorders, or chronic
                   infection, (esp w/ hepatitis B virus);
    - protein electrophoresis;
    - measurement of C3 and C4 compliment levels;
    - arterial ultrasound or arteriography in selected cases;

- Radiographs:
    - Images in Clinical Medicine: Band Acro-osteolysis

- Management:
    - patients with the primary form of the disease are more often successfully treated than patients w/ the secondary form;
    - patients with scleroderma will be more resistant to treatment than patients w/o scleroderma;
    - adress environmental conditions:
          - protection from the cold;
          - stop smoking;
          - vibrating machinery and repetitive conditions (carpal tunnel syndrome);
    - harmful medications:
          - estrogen, cocaine, clonidine, sympathomimetic agents, serotonin-receptor agonists;
    - beneficial medications:
          - ASA;
          - alpha blocking agents (dibenzyline), and myovascular relaxants (nicotinic acid, cyclospasmol, and NTG oitment)
          - catecholamine and or serotonin depletors (reserpine)
          - thyroxine;
          - calcium channel blockers:
                 - nifedipine (either short or long acting) may be more effective in preventing attacks than the other types of calcium
                         channel blockers;
    - surgical:
          - digital and or cervical sympathectomy;
          - sympathectomy at the level of the hand should include the radial and ulnar arteries as well as the superficial palmar arch;
          - cold stress testing may help indicate which patients will have a favorable response to sympathethectomy;
          - references: Adventitial stripping of the radial and ulnar arteries in Raynaud's disease


Treating Raynaud's phenomenon and its underlying causes. Wigley FM. J Musculoskel Med. 1998:53.

The microcirculatory effects of peripheral sympathectomy

Clinical practice. Raynaud's Phenomenon.

The Effect of Botulinum Neurotoxin-A on Blood Flow in Rats: A Potential Mechanism for Treatment of Raynaud Phenomenon

Bilateral Digital Ischemia

Images in clinical medicine. Cold hands associated with scleroderma.

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