Rheumatology Book

Environmental Injury

  • Raynaud's Phenomenon

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Vasculitis

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Raynaud's PhenomenonAka: Raynaud's Syndrome, Raynaud Disease, Raynaud Phenomenon, Raynaud Syndrome

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  1. Epidemiology
    1. Affects 3-4% of U.S. adult population
    2. Predominately affects women by 3 to 1 ratio
    3. Onset from Puberty to age 30 years
    4. Family History responsible in 20-30% of patients
  2. Pathophysiology
    1. Exaggerated response to cold temperatures
    2. Increased Alpha Adrenergic Receptor responsiveness
    3. May be primary idiopathic or secondary cause
  3. Secondary Causes of Raynaud's Phenomenon
    1. Connective tissue disease
      1. Scleroderma (95% have Raynaud's)
      2. Systemic Lupus Erythematosus
      3. Sjogren's Syndrome
      4. Dermatomyositis
    2. Trauma
      1. Occupational tool use (vibratory tool)
      2. Carpal Tunnel Syndrome
    3. Occlusive vascular disease
      1. Atherosclerosis
      2. Systemic Vasculitis
      3. Thromboembolism
    4. Medications
      1. See provocative factors below
    5. Hyperviscosity state (e.g. Polycythemia Vera)
    6. Paraproteinemia
    7. Cryoglobulinemia
  4. Provocative Factors
    1. Tobacco
    2. Caffeine
    3. Amphetamines
    4. Cocaine
    5. Pseudoephedrine
    6. Phenylpropanolamine
    7. Ephedrine
    8. Phenylephrine
    9. Ergotamines
    10. Unopposed Estrogen
    11. Nonselective Beta Blockers
    12. Clonidine
    13. Chemotherapeutic medications (e.g. Bleomycin)
  5. Symptoms
    1. Hypersensitivity to cold temperatures
    2. Color changes of digits during cold or stress exposure
      1. "White attacks" suggest severe ischemia
      2. Mottling with acrocyanosis more common and benign
    3. Sensation of numbness, clumsiness or "pins and needles"
    4. One finger may be more sensitive than the others
  6. Signs
    1. Pallor or cyanosis of fingers or toes
    2. Thumb is not involved
  7. Evaluation
    1. Distal pulses
    2. Bruit Auscultation
    3. Signs of ischemia
    4. Allen's Test
    5. Assess for Thoracic Outlet Syndrome
  8. Labs
    1. Complete Blood Count (CBC)
    2. Serum Chemistry Panel (Chem7)
    3. Urinalysis
    4. Consider Antinuclear Antibody (ANA) when indicated
  9. Radiology
    1. Consider Arterial Doppler ultrasound
  10. Diagnosis: Cold Challenge (not necessary)
    1. Immerse patient's hand in ice water
      1. Blanching occurs in seconds
      2. Next cyanosis occurs
    2. Rewarm hand in warm water
      1. Erythema and pain may occur on rewarming
  11. Complications
    1. Cutaneous Ulceration of involved digit
    2. Gangrene
  12. Management: First Line
    1. Conservative Measures
      1. Smoking Cessation
      2. Avoid precipitating medications (see above)
      3. Avoid cold or reduce cold exposure
        1. Dress warmly in loose-fitting layers for the cold
        2. Wear a warm hat
        3. Wear mittens instead of gloves, and wear stockings
        4. Use hand warmers (chemical heat packets)
        5. Use a space heater at work
        6. Preheat car during winter
    2. Calcium Channel Blockers (Dihydropyridines)
      1. Nifedipine (Procardia) 15 to 60 mg PO qd
      2. Amlodipine
    3. Other medications
      1. Nitroglycerin ointment
      2. Sodium Nitrate with Ascorbic acid gel
        1. Tucker (1999) Lancet 354:1670
  13. Management: Severe Ischemia (e.g. CREST Syndrome related)
    1. See first-line measures above
    2. Alpha-adrenergic blockers
    3. Angiotensin Receptor Blockers
    4. Pentoxifylline (Trental)
  14. Management: Finger Temperature Feedback
    1. One Protocol
      1. Patient places fingertip on temperature monitor
      2. Tone played louder when finger gets colder
      3. Patient tries to warm finger to decrease noise
      4. Reduces symptoms by 92%
    2. Second Protocol
      1. Patient immerses hands in warm water
      2. Rest of patient's body cold (e.g. outside)
      3. Repeat tid, every other day, for 3 weeks
      4. New conditioned cold response: Vasodilation
  15. Management: Severe or ischemic digital ulcers
    1. Intravenous Prostaglandins
      1. PGI2 Analog: Iloprost (not available in U.S.)
      2. PGI2: Epoprostenol
    2. Cervical sympathectomy
      1. Proximal sympathectomy
      2. Localized microsurgical digital sympathectomy
      3. Local chemical sympathectomy with Lidocaine
  16. References
    1. Oreizi-Esfahani (1996) Consultant, p. 905-12
    2. Wigley (1999) Consultant p. 540-54
    3. Comfort-Adee (1993) Am Fam Physician 47(4):823

Raynaud Disease (C0034734)

Definition (CSP)intermittent attacks of ischemia in the fingers, toes, ears, or nose, accompanied by pain, pallor, and prickling; phenomenon applies to secondary symptoms, disease when cause is unknown.
Definition (MSH)An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or STRESS.
ConceptsDisease or Syndrome (T047)
ICD9443.0, 443.0
MSHD011928
EnglishRAYNAUD DIS, Raynaud Disease, Raynaud's Disease, Raynaud's Syndrome, RAYNAUDS DIS, Raynauds Disease, RAYNAUDS SYNDROME
Spanishenfermedad de Raynaud, enfermedad de Raynaud - RETIRADO -, sindrome de Raynaud
Parent ConceptsPeripheral Vascular Diseases (C0085096), Other peripheral vascular disease (C0553983), Non-Neoplastic Vascular Disorder (C1335053), Peripheral Arterial Constriction (C1373207), Raynaud Disease (C0034734), Systemic arterial finding (C0577829), Arteriopathic disease (C0852949), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, DXP, ICD9CM, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Raynaud Phenomenon (C0034735)

Definition (AIR)WHAT: Raynaud's phenomenon. Raynaud's Phenomenon: the paroxysmal constriction of the small arteries and arterioles of the hands or feet, usually precipitated by cold or emotional upset, resulting in pallor and cyanosis of the fingers or toes following a characteristic pattern. WHY: Raynaud's phenomenon may occur in mixed connective tissue disease, systemic lupus erythematosus, progressive systemic sclerosis, poly- myositis/dermatomyositis, and rheumatoid arthritis associated with Sjogren's syndrome. HOW: In Raynaud's phenomenon there are three classic color changes of the fingers or toes. First, vasoconstriction results in a white blanching of the fingertips. Second, vasodilatation with sludging of vascular flow follows and results in blue, cyanotic digits. Finally, with recovery, there is increased blood flow with resulting erythema of the fingers. With observation of two of the three color changes, Raynaud's phenomenon is considered present. Local body cooling (by placing the hands in ice cold water) may demonstrate Raynaud's phenomenon, but some cases require general body cooling before the characteristic color phases occur. Permanent tissue damage can be induced by this testing, which therefore must be done only when absolutely necessary. If the digits show persistent cyanosis or there is evidence of pre-existing necrosis, performing this test is especially hazardous. One or more digits may be involved in Raynaud's phenomenon, and this involvement may be unilateral. REFS: 1) Spittell, JA: "Raynaud's phenomenon and allied vasospastic disorders". In Juergens, JL et al. (eds.): Peripheral Vascular Diseases, pp. 555-83. Philadelphia: W.B. Saunders Co., 1980. 2) Porter, JM; Snider, RL; Bardana, EJ; Rosch, J and Eidemiller, LR: The diagnosis and treatment of Raynaud's phenomenon. Surgery 77:11, 1975. DN19300-3.
Definition (NCI)Intermittent bilateral attacks of ischemia of the fingers or toes and sometimes of the ears or nose, marked by severe pallor, and often accompanied by paresthesia and pain.
Definition (NCI)A set of symptoms characteristic of peripheral vascular disease, namely caused by an inappropriate response of the peripheral arteries in reaction to environmental stimuli, usually to the cold. The term is used when an underlying disease (mostly connective tissue/autoimmune disorders such as lupus, scleroderma, rheumatoid arthritis, etc.) is primarily causative and a range of symptoms including the peripheral vascular spasm is secondary. The term does not refer to the primary Raynauds or Raynauds disease.
ConceptsDisease or Syndrome (T047)
ICD9443.0
MSHD011928
EnglishParoxysmal digital cyanosis, Raynaud Phenomenon, Raynaud's phenomenon, Raynauds Phenomenon, Raynauds Syndrome, Secondary Raynauds
Spanishcianosis digital paroxistica, fenomeno de Raynaud
Parent ConceptsPhysical Examination (C0031809), Joint or joint related complaint (C0221585), Wrist(s) and/or Hand(s) (C0221592), Syndrome (C0039082), Peripheral Vascular Diseases (C0085096), Cyanosis (C0010520), Named sign of hand (C0575816)
SourcesAIR, COSTAR, CSP, DXP, MSH, MTH, MTHICD9, NCI, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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