- 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 20's to late 30's;
- 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;
- 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;
- character and quality of right and left radial pulses (look for irregular heart beat and look for extinguished pulse with
hyperabduction and external rotation of the shoulder);
- carefully assess the patency of the
radial and
ulnar digital arteries with Allen's test, and then
assess the patency of the arteries to the digits using a digital Allens 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, particularly with hepatitis B virus;
- protein electrophoresis;
- measurement of C3 and C4 compliment levels;
- arterial ultrasound or arteriography in selected cases;
- 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.
FM Wigley MD.
J. of Musculoskeletal Medicine.
Sep 1998. p 53;
The microcirculatory effects of peripheral sympathectomy.
LA Koman et al.
J. Hand Surg. 1995. Vol 20-A. p 709-717.
Raynaud's Phenomenon. Fredrick M. Wigley, M.D. NEJM. Volume 347:1001-1008
September 26, 2002 No 13.
