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RAYNAUDS PHENOMENON

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									      RAYNAUD’S
     PHENOMENON
        Christina Page, M.D.
Olive View-UCLA Internal Medicine
               Epidemiology
Approximately 5-10% of the US population have
Raynaud’s Phenomenon
Affects mostly women and usually appears
between the ages of 15-20 (almost always before
the age of 40)
More prevalent in colder climate
In over 80% of patients with Raynaud’s
Phenomenon who are seen by an internist, the
condition is simply an exaggeration of the
physiologic response to cold temperatures
            Definition of
        Raynaud’s Phenomenon
Maurice Raynaud (1862): people exposed to cold
temperatures have transient digital ischemia
secondary to exaggerated response of CNS

Term is used to represent these episodic events
that represent vasoconstriction of the digital
arteries, precapillary arterioles and cutaneous
arteriovenous shunts
      What happens with
      Raynaud’s Phenomenon?
The ischemic phase of the attack is evidenced by
  demarcated pale or cyanotic skin limited to the
  digits.
 Starts in one or several digits after exposure to
  the cold or a stressful situation and then spreads
  symmetrically to all fingers of both hands
 Ends with rapid reflow of blood into the digits,
  shown as erythematous skin (reactive
  hyperemia)
 Mechanism of
color changes in
   Raynaud’s
Raynaud’s Phenomenon
         Diagnosis of
      Raynaud’s Phenomenon
Patient must have history of sensitivity to
the cold and have episodic pallor or
cyanosis of the distal portions of the digits
(or both) after exposure to the cold
 Photographs of the hands can be obtained
  during an attack and be used to confirm the
  history
 Discarded provocative test: immersion of the
  patient’s hand in ice water (cruel, and inhuman
  punishment)
     Active Raynaud’s Phenomenon
Panel A shows sharply demarcated pallor resulting from the
  closure of digital arteries
Panel B shows digital cyanosis of the fingertips
        Primary versus Secondary
        Raynaud’s Phenomenon
Clinical criteria are used to distinguish patients
with uncomplicated or primary Raynaud’s
Phenomenon from those with Raynaud’s
Phenomenon secondary to another illness.
   In Primary Raynaud’s Phenomenon, about one quarter
    of patients have a family history of Raynaud’s
    Phenomenon in a first-degree relative and usually the
    symptoms are generally milder than in Secondary
    Raynaud’s Phenomenon
   Criteria for the Diagnosis of
 Primary Raynaud’s Phenomenon
Vasospastic attacks precipitated by cold or
emotional stress
Symmetric attacks involving both hands
Absence of tissue necrosis, ulceration or gangrene
No history or physical findings suggestive of a
secondary cause
Normal ESR
Negative serologic findings,
   Particularly Negative ANA
Normal nail-fold capillaries
      Secondary Raynaud’s
      Phenomenon Criteria
Age at onset of more than 30 years
Episodes are intense, painful, asymmetric or
associated with ischemic skin lesions
Clinical features suggestive of a connective-
tissue disease
Specific autoantibodies (i.e., centromere,
SCL-70, Smith, dsDNA)
Evidence of microvascular disease on
microscopy of nail-fold capillaries
          Nail-Fold Capillaries in a Patient with
          CREST Syndrome or Limited Scleroderma

The capillaries are dilated and enlarged, and some areas are
  devoid of normal capillaries, a typical characteristic of
                        scleroderma.
Nailfold capillaroscopy
      Supplies
Nailfold capillaroscopy
       Technique
         Nailfold capillary
          abnormalities
Normal                             SSc




Adult DM            Childhood DM
SSc-specific antibodies


                                  Readily available
                                  •Anti-centromere
                                  •Anti-SCL 70
                                  •Anti-PM/Scl

                                  Not so available
                                  •Anti-Th/To
                                  •Anti-RNA
                                   polymerase III
                                  •Anti-fibrillarin
       Anti-centromere antibody
        Evaluating a Patient for
        Raynaud’s Phenomenon
Ask the following screening questions:
   1. Are your fingers unusually sensitive to cold?
   2. Do your fingers change color when they are exposed
    to cold temperatures?
   3. Do they turn white, blue, or both?


The diagnosis of Raynaud’s phenomenon is
excluded if the response to questions 2 and 3 is
negative.
The diagnosis is confirmed by positive response to
all three questions.
          Evaluating a Patient for
          Raynaud’s Phenomenon
If all three questions are positive, you next need to
exclude potential causative or aggravating factors.
   Drugs and toxins
        Chemo agents, interferon, estrogen, nicotine, narcotics,
         sympathomimetic agents, cyclosporine, cocaine, ergotamines,
         clonidine
   Environmental agents and injury
        Frostbite, repetitive occupational stress, hypothenar hammer
         syndrome
   Neuropathy
        Carpal tunnel syndrome
     Evaluating a Patient for
     Raynaud’s Phenomenon
Patients with:
   1. Normal findings on review of history
         and physical exam
   2. No digital lesions or gangrene
   3. Normal nail-fold capillaries
can be considered to have
    Primary Raynaud’s Phenomenon
           Evaluating a Patient for
           Raynaud’s Phenomenon
Patient’s with a history of any of the following:
  1. Single digit or asymmetric attacks
  2. Absent pulses
  3. Asymmetry of blood pressure
  4. Evidence of critical ischemia
Obtain: Angiography, Arterial Doppler Ultrasonography or
  Digital plethysmography

Abnormal findings indicate the presence of conditions
  causing large-artery disease such as Vasculitis,
  Atherosclerosis, Thromboangiitis obliterans, Embolic
  Disease or Thoracic Outlet Syndrome
           Evaluating a Patient for
           Raynaud’s Phenomenon
Patient’s with symptoms or signs suggestive of
  systemic disease
(Myalgias, arthralgias, fever, weakness, weight loss, rash,
  arthritis or symptoms of heart or lung disease)
Obtain:
CBC, Chemistry panel, Urinalysis, ANA, ESR,
 Rheumatoid Factor, C3 and C4 Complement
 levels and tests for disease specific auto-antibodies
           Evaluating a Patient for
           Raynaud’s Phenomenon
Patient with negative initial laboratory results, should
  undergo the following:
         1. Thyroid-function tests
         2. Serum protein electrophoresis
         3. Test for cryoglobulins
If results are negative,
    patient has PRIMARY RAYNAUD’S PHENOMENON
If results are positive, patient has other systemic disease,
    Hypothyroidism, Cancer, Cryoglobulinemia,
    Cryofibrinogenemia, Cold agglutinin syndrome
         Evaluating a Patient for
         Raynaud’s Phenomenon
Patient’s with positive initial laboratory results have
  rheumatic disease:
      Scleroderma
      Systemic lupus erythematosus
      Mixed connective-tissue disease
      Dermatomyositis/Polymyositis
      Sjogren’s Syndrome
      Undifferentiated connective-tissue disease
Non-pharmacological Treatments
for Raynauld’s Phenomenon
Quit Smoking
   Nicotine causes skin temperature to drop, which may
    lead to an attack

Control Stress
   Emotional upsets may trigger an attack

Keep Warm
   Avoid cold temperatures
   Dress warm: gloves, headgear, layers
   Air conditioning can trigger attacks
       Pharmacologic Treatment of
         Raynaud’s Phenomenon
  Calcium-Channel Blockers
(Nifedipine, Amlodipine, Felodipine)

      Placebo-controlled trials indicate benefit
           50-66% reduction in frequency of attacks

      SE: tachycardia, edema, flushing, HA, dizziness,
       constipation, orthostatic hypotension
    Pharmacologic Treatment of
      Raynaud’s Phenomenon

Effective Calcium-Channel Blockers †
   Nifedipine XL 30-120 mg QD (Procardia, Adalat-CC)
   Amlodipine 5-20 mg QD (Norvasc)
   Felodipine 2.5-10 mg QD (Plendil)
   Isradapine 2.5-5.0 mg QD (DynaCirc)
   Diltiazem 30-120 mg TID (Cardizem, Dilacor) *
   Diltiazem XR 120-300 mg QD (Cardizem SR or CD) *

* Diltiazem is the least effective of the bunch
† Not all CCB effective: verapamil is not effective
  Other Pharmacologic Options
for Treatment of Raynaud’s Disease

Prazosin
  Crossover trial found it to more effective than placebo

Losartan
  Reduced severity of attacks in patients with
   scleroderma
Fluoxetine
  Controlled study showed improved symptoms

Nitroglycerin
  Ointment effective--apply proximal 2/3 of finger

  Oral long-acting; patches--not well studied
                    References
1.   Wigley,F. Raynaud’s Phenomenon. NEJM 2002;347(23):1001-1008
2.   Thompson SE, et al. Calcium-channel blockers for Raynaud’s
     phenomenon in systemic sclerosis. Arthritis Rheum 2001,44:1841-7.
3.   Dziadzio M, et al. Losartan therapy for Raynaud’s phenomenon and
     scleroderma: clinical and biochemical findings in a fifteen week,
     randomized, parallel-group, controlled trial. Arthritis Rheum 1999,
     42:2646:55.
4.   Coleiro B, et al. Treatment of Raynaud’s phenomenon with the
     selective serotonin reuptake inhibitor fluoxetine. Rheumatology
     (Oxford) 2001,40:1038-43.
5.   Pope J, et al. Prazosin for Raynaud’s phenomenon in progressive
     systemic sclerosis. Cochrane Database Syst Rev 2000;2:CD000956.

								
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