This may be the case in the CREST syndrome, of which Raynauds is a part.Patients with secondary Raynauds can also have symptoms related to their underlying diseases. Raynauds phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.When Raynauds phenomenon is limited to one hand or one foot, it is referred to as unilateral Raynauds. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses. Mechanism
Its pathophysiology includes hyperactivation of the sympathetic nervous system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Diagnosis
Distinguishing Raynauds disease (primary Raynauds) from Raynauds phenomenon (secondary Raynauds) is important. Looking for signs of arthritis or vasculitis, as well as a number of laboratory tests, may separate them. Nail fold capillary examination or "capillaroscopy" is one of the most sensitive methods to diagnose RS with connective tissue disorders, i.e. distinguish a secondary from a primary form objectively.If suspected to be secondary to systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography.A careful medical history will seek to identify or exclude possible secondary causes. Digital artery pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive).
Primary Raynauds is thought to be at least partly hereditary, although specific genes have not yet been identified.Smoking increases frequency and intensity of attacks, and a hormonal component exists. Caffeine, estrogen, and nonselective beta-blockers are often listed as aggravating factors, but evidence that they should be avoided is not solid. Secondary
Raynauds phenomenon, or secondary Raynauds, occurs secondary to a wide variety of other conditions. Secondary Raynauds has a number of associations:
Connective tissue disorders:
Scleroderma
Systemic lupus erythematosus
Rheumatoid arthritis
Sjögrens syndrome
Dermatomyositis
Polymyositis
Mixed connective tissue disease
Cold agglutinin disease
Ehlers-Danlos syndrome
Eating disorders:
Anorexia nervosa
Obstructive disorders:
Atherosclerosis
Buergers disease
Takayasus arteritis
Subclavian aneurysms
Thoracic outlet syndrome
Drugs:
Beta-blockers
Cytotoxic drugs – particularly chemotherapeutics and most especially bleomycin
Cyclosporin
Bromocriptine
Ergotamine
Sulfasalazine
Anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen
Stimulant medications, such as those used to treat ADHD (amphetamine and methylphenidate)
OTC pseudoephedrine medications (Chlor-Trimeton, Sudafed, others)
Occupation:
Jobs involving vibration, particularly drilling and prolonged use of a string trimmer (weed whacker), experience vibration white finger
Exposure to vinyl chloride, mercury
Exposure to the cold (e.g., by working as a frozen food packer)
Others:
Physical trauma to the extremities
Lyme disease
Hypothyroidism
Cryoglobulinemia
Cancer
Chronic fatigue syndrome
Reflex sympathetic dystrophy
Carpal tunnel syndrome
Magnesium deficiency
Multiple sclerosis
Erythromelalgia (clinically presenting as the opposite of Raynauds, with hot and warm extremities, often co-exists in patients with Raynauds)
Chilblains (also clinically presenting as the opposite of Raynauds, with hot and itchy extremities, however it affects smaller areas than erythromelalgia, for instance the tip of a toe rather than the whole foot)Raynauds can precede these other diseases by many years, making it the first presenting symptom.