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.

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Friday, December 2, 2016 9:08 am