Raynaud's syndrome I73.0

Seizure-like vasospasms associated with numbness (45% of patients), tingling paresthesias (20% of patients) and pain (60% of patients), mostly symmetrical, but also asymmetrical or isolated, functional, digital vasospasms, which can be primarily idiopathic or secondary and can be relieved by heat or medication.

The term "Raynaud's phenomenon" is to be equated with Raynaud's syndrome and does not require a separate assessment.

Primary Raynaud's syndrome (>50%) - conceptually equivalent to Raynaud's disease. Vasospasms of the fingers triggered by cold and emotional stress up to max. 30 min. duration.

Secondary Raynaud's syndrome (Raynaud's phenomenon): similar symptoms with symmetrical/asymmetrical affection mostly associated with diseases of the so-called collagenoses.

Paraneoplastic Raynaud's syndrome also belongs to this group. It is associated with lung carcinomas, carcinomas of the ovaries and uterus (Lai TS et al. 2020; Lokineni S et al. 2021).

The prevalence in the German population is about 8-10%; in Sweden about 20%, in Switzerland about 20-30%.

Vasospasms triggered by cold, emotional stress, local compression phenomena.

Especially the Digiti II-V of the hand are affected, less often also the toes. The thumbs are usually left out, as are the back of the hand and palms.

Mostly bilateral seizure symptoms with inital cyanosis followed by seizure-like white coloration which turns into an exuberant redness. In 1/3 of the patients, however, only a seizure-like cyanosis or only a white discoloration of the fingers occurs.

Permanent acral ischemia without seizure character is to be distinguished.

Embolism (duration of ischemia > 30 min.)

PAVK (DD and one of the causes of secondary Raynaud's syndrome)

Isolated acrocyanosis (painless, cyanotic discoloration of the fingers that does not occur in attacks)

Systemic sclerosis of the acroscleroderma type (besides vasospasms of the fingers flat digital swellings and inudurations, sclerotic nail folds, other signs of PSS with antibody detection/centromer-Ak; SCL70-Ak)

• Clarification and if necessary treatment of an underlying disease. Otherwise symptom- and phase-oriented prophylactic measures: protection against the effects of cold, wearing warm clothing, if necessary also pocket warmers or heated gloves.
• Smoking ban (vasoconstrictive effect of nicotine).
• No prescription of pharmaceuticals containing clonidine, ergotamine or epinephrine.
• Physiotherapy: robotic measures such as alternating warm hand baths with room temperature (not too cold!) and 37 °C. Alternative finger exercises, e.g. fango kneading or kneading of warmed millet (whole grains!) with a few drops of olive oil.
• Some patients can also increase the finger temperature by bio-feedback exercises or autogenic training.

Notice! Physical therapies such as alternating warm hand baths, fango kneading, kneading warm millet are helpful in Raynaud's symptoms!

Isosorbide dinitrate ointment formerly Isoket ointment, this is withdrawn from the market, therefore if necessary Rectogesic corresponding to chronic anal fissure, see there. or as an alternative preparation magistral formulation with diltiazem 2% as a cream (NRF 5.7) or gel (NRF 5.6). The vasodilator creams may also zzur lower blood pressure, headache, but less pronounced as with systemic vasomodulatory therapy. Use as a monotherapeutic or in addition to internal therapy.

In case of minor injuries, early disinfecting measures such as polyvidone-iodine (e.g. Braunovidon ointment).

Warm kerosene baths of the hands are felt to be pleasant (note: a simple and inexpensive paraffin-containing body oil is sufficient for this; bath time 5-8min.).

• Use of vasoactive agents.
  • Calcium antagonists (considered the gold standard): Therapy of 1st choice is nifedipine (e.g. Adalat 5) 10-20 mg each 3x /day p.o. as monotherapy or in combination with pentoxifylline (e.g. Trental) 600 mg/day p.o.; increase nifedipine dose up to 3-4 times/day 10 mg p.o. Cave! Orthostatic dysregulations with nifedipine! Long-term administration of pentoxifylline promotes skin bleeding!
  • Alternatively, diltiazem (e.g., Dilzem Tbl.) 60-120 mg/day p.o. or verapamil (e.g., Isoptin 80) 240-320 mg/day p.o.
• Other possible therapeutic regimens:
  • ACE inhibitors and angiotensin 1 receptor antagonists: Several studies with the ACE inhibitors captopril (25 mg/day) and enalapril (20 mg/day) showed sometimes good, sometimes conflicting results. Losartan (angiotensin 1 receptor antagonist) possessed a comparable effect to 40 mg nifedipine/day in a randomized trial.
  • Alpha 1-receptor blockers: prazosin (e.g., Minipress) initially creeping 1 mg p.o. at night, if necessary additionally in the morning, slowly increasing to maintenance dose of 4 mg/day.
  • Prostacyclins (evidence level A): e.g. iloprost (ilomedin) 0.5-2.0 ng/kg bw/min. Daily recommended infusion duration is 6-8 h. Therapy duration: 3-5 days. Therapy cycles are repeated after 3 months.
  • Prostavasin (data situation unclear): In case of manifest or threatening gangrene! Effective but costly procedure to improve acral blood flow. Dosage: Prostaglandin E1 (e.g., Prostavasin) 20 μg/hr i.v. over 3 hr.
  • Estrogens: If symptoms worsen during menses and menopause, peroral administration of estrogens (e.g., Trisequens) by gynecologists.
  • Endothelin antagonists ( bosentan): Hopeful approach, currently in multicenter trial. Dosage: Tracleer 2 times/day 125 mg p.o.
  • PDE-5 inhibitors: In severe cases of refractory acral ulceration, therapy with oral sildenafil (Viagra) at a dosage of 20-80 mg/day may be considered. In individual cases, iloprost can be combined with sildenafil.

Contributory causes of secondary Raynaud's syndrome:

• Connective tissue diseases
  • Progressive systemic scleroderma, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, dermatomyositis/polymyositis
• Arterial occlusive diseases
  • Arteriosclerosis, Endangiitis obliterans, Polyarteritis nodosa, Embolisms, Thromboses
• Shoulder girdle arm syndromes
  • Cervical rib syndrome, 1st rib syndrome, scalenus syndrome, costoclavicular syndrome, hyperabduction syndrome, pectoralis minor syndrome, malposition syndrome, narrow upper thoracic aperture syndrome, Klippel-Feil syndrome, combination forms
• Hematogenous diseases
  • Cold agglutinins, cold hemolysins, cryoglobulinemia, macroglobulinemia (Waldenström), paroxysmal hemoglobulinuria, hyperviscosity syndrome, thrombocytosis, polycythemia, thrombotic microangiopathy
• Neurological diseases
  • Neuritis, poliomyelitis, multiple sclerosis, syringomyelia, nucleus pulposus prolapse, spinal tumors, carpal tunnel syndrome, hemiplegia
• Intoxications
  • ergot alkaloids (ergotism), fungal toxins (folding tintling), vinyl chloride derivatives (vinyl chloride disease), trichloroethylene
• Chronic occupational trauma
  • Vibration syndromes when working with jackhammers, power saws, walking on crutches, etc.
• Trauma
  • Local vascular injuries, post-traumatic, cold damage
• Medicinal
  • Clonidine, sympathomimetics, ACE inhibitors, hormonal anticonceptives, beta receptor blockers, secale alkaloids (ergotism), bleomycin, vincristine, ciclosporin
• Other
  • surgery, Sudeck's atrophy, dialysis

From a naturopathic point of view, ofiizine rosemary oil is recommended, which is applied to the affected hands several times a day as an externum (by Schoen-Angerer T et al. 2018).

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